Case Report Surgical Orthodontic Treatment of a Patient Affected by Type 1 Myotonic Dystrophy (Steinert Syndrome) Laura Cacucci, Beatrice Ricci, Maria Moretti, Giulio Gasparini, Sandro Pelo, and Cristina Grippaudo Universit` a Cattolica del Sacro Cuore, Largo A. Gemelli 8, 00168 Rome, Italy Correspondence should be addressed to Cristina Grippaudo; [email protected] Received 10 January 2017; Revised 18 April 2017; Accepted 7 May 2017; Published 31 May 2017 Academic Editor: H¨ usamettin Oktay Copyright © 2017 Laura Cacucci et al. is is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Myotonic dystrophy, or Steinert’s disease, is the most common form of muscular dystrophy that occurs in adults. is multisystemic form involves the skeletal muscles but affects also the eye, the endocrine system, the central nervous system, and the cardiac system. e weakness of the facial muscles causes a characteristic facial appearance frequently associated with malocclusions. Young people with myotonic dystrophy, who also have severe malocclusions, have bad oral functions such as chewing, breathing, and phonation. We present a case report of a 15-year-old boy with anterior open bite, upper and lower dental crowding, bilateral crossbite, and constriction of the upper jaw with a high and narrow palate. e patient’s need was to improve his quality of life. Because of the severity of skeletal malocclusion, it was necessary to schedule a combined orthodontic and surgical therapy in order to achieve the highest aesthetic and functional result. Although therapy caused an improvement in patient’s quality of life, the clinical management of the case was hard. e article shows a balance between costs and benefits of a therapy that challenges the nature of the main problem of the patient, and it is useful to identify the most appropriate course of treatment for similar cases. 1. Introduction Myotonic dystrophy, or Steinert’s disease, is the most com- mon form of muscular dystrophy that occurs in adults [1]. is multisystemic form involves the skeletal muscles but affects also the eye, the endocrine system, the central nervous system, and the cardiac system [2]. Myotonic dystrophy is an autosomal dominant disease affecting both sexes equally, and each child of an affected person has a 50% risk of being himself affected by the disease. e genetic alteration of the disease is an abnormal expansion of CTG triplet, located in the DMPK gene (Dystrophic Myotonic Protein Kinase) on chromosome 19 [3–7]. is is the most common form of dystrophy in the adult population, whose prevalence is calculated to be about 1–10 cases per 100.000 births [8]. Steinert’s disease is characterized primarily by the myotonic phenomenon, which is a continued constriction of a skeletal muscle aſter a voluntary or inducted constriction. e myotonia is related to an abnormal state of excitability in the muscle fiber membrane [9]. Another distinctive feature of the disease is the muscular strength deficit and the depletion of muscle mass trending slowly progressive. e strength deficit has characteristically a distal distribution (i.e., hands, forearms, feet, and legs, in particular the portion of the limb between the knee and the foot). Mimic muscles of the face are involved too, with atrophy of the temporal and masseter mus- cles, as well as eyelid ptosis. e use of ultrasound and elec- tromyography demonstrated the involvement of the masseter and temporal muscles [10, 11]. Instead, the involvement of the pterygoid muscles has never been proved. Malocclusion problems are related to masticatory muscles involvement [12– 16]. e trend of the loss of strength is gradual and involves in a second moment proximal districts too, but the progression varies greatly from person to person, following the expansion of CTG triplet. e weakness of the diaphragm and the alveolar hypoventilation, which cause chronic bronchitis and bronchiectasis, are common manifestations of the disease, as cardiac abnormalities; the latter are mostly due to a disorder of the cardiac conduction system, which causes bradycardia and increased PR interval. Patients with extreme bradycardia Hindawi Case Reports in Dentistry Volume 2017, Article ID 7957961, 9 pages https://doi.org/10.1155/2017/7957961
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Case ReportSurgical Orthodontic Treatment of a Patient Affected byType 1 Myotonic Dystrophy (Steinert Syndrome)
Laura Cacucci Beatrice Ricci Maria Moretti Giulio GaspariniSandro Pelo and Cristina Grippaudo
Universita Cattolica del Sacro Cuore Largo A Gemelli 8 00168 Rome Italy
Correspondence should be addressed to Cristina Grippaudo cristinagrippaudounicattit
Received 10 January 2017 Revised 18 April 2017 Accepted 7 May 2017 Published 31 May 2017
Academic Editor Husamettin Oktay
Copyright copy 2017 Laura Cacucci et al This is an open access article distributed under the Creative Commons Attribution Licensewhich permits unrestricted use distribution and reproduction in any medium provided the original work is properly cited
Myotonic dystrophy or Steinertrsquos disease is themost common form ofmuscular dystrophy that occurs in adultsThismultisystemicform involves the skeletal muscles but affects also the eye the endocrine system the central nervous system and the cardiac systemTheweakness of the facial muscles causes a characteristic facial appearance frequently associated withmalocclusions Young peoplewith myotonic dystrophy who also have severe malocclusions have bad oral functions such as chewing breathing and phonationWe present a case report of a 15-year-old boy with anterior open bite upper and lower dental crowding bilateral crossbite andconstriction of the upper jaw with a high and narrow palate The patientrsquos need was to improve his quality of life Because of theseverity of skeletal malocclusion it was necessary to schedule a combined orthodontic and surgical therapy in order to achieve thehighest aesthetic and functional result Although therapy caused an improvement in patientrsquos quality of life the clinicalmanagementof the case was hard The article shows a balance between costs and benefits of a therapy that challenges the nature of the mainproblem of the patient and it is useful to identify the most appropriate course of treatment for similar cases
1 Introduction
Myotonic dystrophy or Steinertrsquos disease is the most com-mon form of muscular dystrophy that occurs in adults [1]This multisystemic form involves the skeletal muscles butaffects also the eye the endocrine system the central nervoussystem and the cardiac system [2] Myotonic dystrophy isan autosomal dominant disease affecting both sexes equallyand each child of an affected person has a 50 risk of beinghimself affected by the disease The genetic alteration of thedisease is an abnormal expansion of CTG triplet locatedin the DMPK gene (Dystrophic Myotonic Protein Kinase)on chromosome 19 [3ndash7] This is the most common formof dystrophy in the adult population whose prevalence iscalculated to be about 1ndash10 cases per 100000 births [8]
Steinertrsquos disease is characterized primarily by themyotonic phenomenon which is a continued constriction ofa skeletal muscle after a voluntary or inducted constrictionThemyotonia is related to an abnormal state of excitability inthemuscle fibermembrane [9] Another distinctive feature of
the disease is the muscular strength deficit and the depletionof muscle mass trending slowly progressive The strengthdeficit has characteristically a distal distribution (ie handsforearms feet and legs in particular the portion of the limbbetween the knee and the foot) Mimicmuscles of the face areinvolved too with atrophy of the temporal andmassetermus-cles as well as eyelid ptosis The use of ultrasound and elec-tromyography demonstrated the involvement of themasseterand temporal muscles [10 11] Instead the involvement ofthe pterygoid muscles has never been proved Malocclusionproblems are related tomasticatorymuscles involvement [12ndash16]The trend of the loss of strength is gradual and involves ina second moment proximal districts too but the progressionvaries greatly from person to person following the expansionof CTG triplet The weakness of the diaphragm and thealveolar hypoventilation which cause chronic bronchitis andbronchiectasis are common manifestations of the disease ascardiac abnormalities the latter are mostly due to a disorderof the cardiac conduction system which causes bradycardiaand increased PR interval Patients with extreme bradycardia
HindawiCase Reports in DentistryVolume 2017 Article ID 7957961 9 pageshttpsdoiorg10115520177957961
2 Case Reports in Dentistry
or with a high degree of atrioventricular block may diesuddenly the insertion of a pacemaker is recommended forthese subjects The mitral valve prolapse and dysfunction ofthe left ventricle are less frequent The disease progressesslowly with gradual involvement of the proximal muscles ofthe limbs and trunk muscles
The age of onset and clinical manifestations are highlyvariable depending on the type of genetic alteration Markeddifferences are noticed in individuals and in the variousmembers of the same family
Some years ago the detection of a form clinically verysimilar to myotonic dystrophy but with proximal strengthdeficit and different genetic basis resulted in the introductionof DM1 symbol to indicate Steinertrsquos disease and DM2 toindicate this other form also known as PROMM (ProximalMyotonic Myopathy) Other subjects with similar clinicalcharacteristics did not show the alteration of the DM1 genenor that of DM2 DM1 or Steinertrsquos dystrophy is caused by thegene defect of myotonin protein kinase (DMPK) located onchromosome 19q133 The DM2 is rarer and it is secondaryto the defect of the Zinc Finger Protein Gene 9 (ZNF9)encoded by chromosome 3q21 There is still a third form(DM3) which however does not have a precise genetic andmolecular characterization
Both the forms of dystrophy are characterized by exces-sive repetition (ldquobabblingrdquo) of a sequence of nucleotides(triplets) that in normal subjects is repeated for a limitednumber of times In the subjects affected by the disease thesebase sequences (CTG for DM1 and CCTG for DM2) may berepeated from tens to thousands of times compromising thefunction of the gene In general the greater the expansion ofnucleotides is the more serious the clinical expression of thedisease is The expansion can vary in different tissues of thesame individual which explains the different manifestationsof the disease
In Steinertrsquos myotonic dystrophy the muscular involve-ment is especially evident in the distal districts (forearmhand leg and foot) and the mimic muscles of the facewith a reduction in the expression movements of the faceand drooping eyelids (ptosis) This disease affects the entireskeletal musculature with generalized weakness and easyfatigability In PROMM however the muscle involvementprevails in districts proximal limbs (shoulders arms pelvisand thigh) Both forms are characterized by myotonic phe-nomenon [17]
Severe congenital forms of DM1 exist childhood andadolescence related forms and adult forms which are themost common In one household different forms can coexisttoo without obvious symptoms (subclinical)
People with myotonic dystrophy affecting facial andmasticatory muscles have a characteristic facial appearancelong face gonial angle and mandibular divergence increasewhich are frequently associated with malocclusions In par-ticular these individuals have a constriction of the palateand an anterior open bite [15 16] Very often severe dentalmalocclusions create discomfort in oral functions and inparticular in chewing breathing and phonation [18 19]besides constituting a social disadvantage [20] In additionthe functional discomfort creates difficulty in swallowing and
Figure 1 Front and lateral photographs of the patient at the time ofthe first visit
promotes general dental problems related to dry mouth dueto the labial incompetence and mouth breathing with plaquebuildup and gingivitis and greater susceptibility to caries [21]
In the paper we present the case report of a patient withSteinertrsquos syndrome treated with orthodontic-surgical ther-apy Although therapy caused an improvement in patientrsquosquality of life the clinical management of the case was hardThe case report is aimed to show a balance between costs andbenefits of a therapy that challenges the nature of the mainproblem of the patient and it is useful to identify the mostappropriate course of treatment for similar cases
2 Case Presentation
21 Diagnosis The patient M M (male) affected by myo-tonic dystrophy type 1 came to our attention at the age of15 years and seven months because he was not satisfied withhis occlusion and his facial appearance Because of lip incom-petence the patient had difficulty in eating and swallow-ing and difficulty in pronouncing certain phonemes Stein-ertrsquos syndrome was diagnosed when the patient was 7 yearsold and he presented with macroglossia heart problemsmouth breathing and frequent pneumonia Genetic analysisshowed a class 2 expansion of about 660 CTG The familyhistory revealed that the father who died because of cardiaccomplications has been suffering from myotonic dystrophydiagnosed at age of 50 due to heart problems and difficulty ingrasping objects and deficit of strength in hands The grand-mother and the paternal aunt were suffering from myotonicdystrophy too while the sister was not affected Physicalexamination showed a patient with long narrow face mildbilateral ptosis lip incompetence and hypotonia of the peri-oral muscles The examination of the profile revealed a con-vex profile with increasing gonial angle (Figure 1) (informedconsent has been obtained by the patient for publication)
The intraoral examination revealed a significant anterioropen bite (OVBminus25mmOVJ 5mm) upper and lower dentalcrowding bilateral crossbite and constriction of the upperjaw with a high and narrow palate (Figure 2)
Case Reports in Dentistry 3
Figure 2 Intraoral photographs at the time of the first visit
Figure 3 Rx before treatment
The Illinois cephalometric analysis (Table 1) revealed asecond hyperdivergent skeletal class with mandibular retru-sion tendency of vertical growth and reduced inclination oflower and upper incisors (Figure 3)
22Therapy Because of the severity of skeletalmalocclusionthe patient received a combined orthodontic and surgicaltherapy in order to achieve the highest aesthetic and func-tional result Maxillary rapid palatal expander performedinitially two rapid expansion cyclesWhen the expansion wascompleted an orthodontic fixed multibrackets appliance wasapplied in upper and lower arch The presurgical therapyhad the purpose to improve the shape of both arches whichappeared ovoid and provide a correct expansion of theupper jaw in preparation for orthognathic surgery (Figure 4)Having completed the phase of orthodontic presurgical treat-ment the patient underwent maxillofacial surgery Arnett-Bergman soft tissues analysis [22 23] (Table 2) and surgical
visual treatment objects (VTO) were performed beforesurgery (Figure 5) A maxillary clockwise rotation of 5mmand BSSO of the mandible were planned A counterclockwiserotation of 18∘ of the occlusal plane was planned On VTO avalue of SNA of 77∘ SNB of 73∘ ANB of 5∘ IMPA of 72∘ andFMA of 48∘ were planned On the postsurgery cephalometricanalysis we had a value of SNA of 81∘ SNB of 76∘ ANB of5∘ IMPA of 73∘ and FMA of 49∘ Therefore the results weresimilar to those planned
Before surgery the patient underwent the extraction ofthe third lower molars in bone inclusion After the healingof mucosal wounds and after the finding of bone healing ofextraction sites using orthopanoramic Rx the patient had theorthognathic surgery intervention which consisted in max-illary clockwise rotation and sagittal mandibular osteotomyrepositioning in maximum intercuspidation of the dentalarches Photos (Figure 6 and Table 3) and Rx (Figure 7 andTable 4) show a facial and skeletal harmony improvement
4 Case Reports in Dentistry
Figure 4 Extraoral and intraoral photographs before surgery
Figure 5 Arnett-Bergman soft tissues analysis and VTO before surgery
Case Reports in Dentistry 5
Figure 6 Extraoral and intraoral photographs and Arnett-Bergman soft tissues analysis after surgery
The recovery after the operation was difficult because ofan extreme muscle weakness after surgery and this is thereason why the patient avoids the orthodontic checks for twomonths After surgery orthodontic treatment completed thealignment and coordination of the dental arches Intermaxil-lary elastics acted to optimize vertical occlusal relationshipsIn this phase the rapid maxillary expander has been keptin place to prevent the relapse of the maxillary constrictionBecause of an initial relapse of the open bite the expanderwasremoved and the patient began speech therapy to improvethe posture of the tongue Six months later there was arecurrence of the palatal constriction One year after surgery
Bollard screws were positioned to attach vertical elastics tofixed retainers in order to counter the open bite Two yearsafter surgery the fixed orthodontic appliance was removedand upper and lower Schwarz appliances of restraint weredelivered to the patient to keep the result in combinationwithfixed upper and lower retainers (Figure 8) The patient wassubjected to regular checks until after seven months afterthe removal of orthodontic appliances when it was decidedto hand over to the patient two thermoformed appliances(upper and lower) with box shaped elastics positioned onfrontal teeth because of the partial relapse of open bitedue to facial muscle deficits linked to the primary disease
6 Case Reports in Dentistry
Figure 7 Rx after surgery
Table 1 Pretreatment cephalometric analysis
Case NormFacial angle 782 883Convexity angle 171 5SNA 792 802SNB 717 77ANB 75 32Mandibular angle 576 224119910-axis 789 573Occlusal angle 376 9Interincisal angle 1214 129Li-occlusal plane 8 184Li-mandibular plane minus12 48UiSN 946 105UiA-Pg 87 5LiA-Pg 82 13MLSNL 631 NLSNL 12 Rotational type P1NOB Growth category 2
Today the patient periodically comes to visit tominimize therecurrence of the open bite and tomotivate the cooperation inwearing physical containment and to maintain oral hygiene
23 Discussion Myotonic dystrophy is a very complexmolecular pathology with multisystemic involvement [2ndash9]People with myotonic dystrophy type 1 frequently have acharacteristic facial appearance such as that observed in thepatient described in this paper [12 13 24]
Kiliaridis et al [15] and Mercier et al [16] claim thatcraniofacialmalformations in patientswithmyotonic atrophyare associated with malocclusions in patients who have aprecocious involvement of themusclesThe patient describedin the case shows a constriction of the upper jaw narrowface increasedmandibular angle and a vertical facial growthwhich are perhaps the result of increased neuromuscularfunction as reported by Staley et al [12]The patient presentsanterior open bite as frequently found in the pathology as
Table 2 Arnett-Bergman soft tissues analysis before surgery
Case NormFacial angle 1493 165ndash173∘
Nasal projection 126 13ndash18mmNasolabial angle 988 94ndash110∘
Lower face height 99 57ndash74mmLower face 562 53ndash56
Upper lip length 149 F 18ndash22M 22ndash25mm
Maxillary sulcus 1268 127ndash147∘
Upper lip protrusion 84 3 plusmn 1mmInterlabial gap 363 1ndash5mm
Lower lip-chin length 484 F 43ndash50M 45ndash54
Mandibular sulcus 132 110ndash134∘
L lip protrusion 179 2 plusmn 1mmB-SnPg line 71 4 plusmn 1mmLower face-throat angle 134 96ndash110∘
Throat length 854 51ndash63mm
reported by Portelli et al [13] because of the prevalence ofthe force of gravity on the elevator muscles Kiliaridis andKatsaros [25] say that the increasing of the vertical growthis due to an alteration of the perioral muscles in combinationwith a minor involvement of suprahyoid muscles So a newsituation is established around the teeth which alter thetransversal relationships The tongue in a lower position isnot able to counterbalance the forces developed during thelowering of the lower jaw caused by the elongated facialmusculature This situation as Kiliaridis and Katsaros [25]say can also affect the vertical size decreasing thewidth of thepalate and causing a posterior crossbiteThe lowered positionof the mandible in combination with the reduction of theocclusal forces can allow a supereruption of the posteriorteeth with increased height of the palatal vault and thedevelopment of an anterior open bite
With regard to the surgical therapy as Manzon andPhilbert [24] say although the induction of general anesthesiais extremely safe in a healthy population severe reaction
Case Reports in Dentistry 7
Figure 8 Extraoral and intraoral photographs after orthodontic therapy
Table 3 Arnett-Bergman soft tissues analysis after surgery
Case NormFacial angle 1503 165ndash173∘
Nasal projection 18 13ndash18mmNasolabial angle 1309 94ndash110∘
Lower face height 90 57ndash74mmLower face 493 53ndash56
Upper lip length 209 F 18ndash22M 22ndash25mm
Maxillary sulcus 1687 127ndash147∘
Upper lip protrusion 66 3 plusmn 1mmInterlabial gap 0 1ndash5mm
Lower lip-chin length 661 F 43ndash50M 45ndash54
Mandibular sulcus 1737 110ndash134∘
L lip protrusion 78 2 plusmn 1mmB-SnPg line 49 4 plusmn 1mmLower face-throat angle 185 96ndash110∘
Throat length 71 51ndash63mm
to anesthetic and neuromuscular paralyzing agents in amyotonic patient can cause organ failure myocardial infarc-tion and respiratory failure Therefore given the complexityof the case and the disease a polispecialistic collaborationcomprisingmaxillofacial surgeons anesthetists-resuscitatorsneurologists pulmonologists and cardiologists is requiredin the management of postoperative moment in order toprevent pulmonary and cardiac complications In order toperform a correct postoperative monitoring the patientdescribed in the case report spent the first night after surgeryin a postoperative intensive care unit The danger of com-plications related to general anesthesia makes this precau-tion strongly advisable in all patients who have to undergosurgery with general anesthesia
Surgical movements of the skeletal bases must be pro-grammed in order to provide the greatest postoperativesurgical stability in the long term because there is a high riskof relapse because of the progressiveweakness of the temporalmuscle The weakness of the masticatory muscles can leadto recurrence of the anterior open bite closed by surgery[26 27] In our experience in favor of combined orthodontic-surgical therapy there is the improvement of oral functionsand the patientrsquos appearance However the high probability
8 Case Reports in Dentistry
Table 4 Postsurgery cephalometric analysis
Case NormFacial angle 80 883Convexity angle 103 5SNA 813 802SNB 759 77ANB 54 32Mandibular angle 493 224119910-axis 747 573Occlusal angle 214 9Interincisal angle 142 129Li-occlusal plane 108 184Li-mandibular plane minus171 48UiSN 918 105UiA-Pg 62 5LiA-Pg 36 13MLSNL 534 NLSNL 128 Rotational type P1NOB Growth category 2
of recurrence complications related to general anesthesia andthe difficulties of recovery after the intervention and the needof very long orthodontic therapy which favor the accumu-lation of bacterial plaque and increase the risk of caries[28] must be kept in mind In the case report described infact throughout the orthodontic treatment the condition oforal health deteriorated because of the buildup of plaqueresulting in gingivitis and multiple demineralization at thecollar level of the teeth Naturally to undertake this typeof very long and complex therapy patient cooperation isnecessary but we know that during long therapy it alwaysdecreases [29] These considerations lead us to assess thesituation of each patient individually It would be better towork on the prevention and early treatment of malocclusionof cooperative patients with muscular dystrophy in orderto counteract the effects of facial hypotonic muscles onocclusion and the appearance of the patient from the time ofinitial diagnosis of the disease [28] Even cases successfullytreated in developmental age over time may recur or worsenthe functional deficit therefore patients should be orthodon-tically monitored periodically
3 Conclusion
Myotonic dystrophy is a very complex multisystemic diseasein which the muscular involvement leads to a characteristicfacial phenotype The weakness of the facial muscles is thecause of malocclusions needing in adulthood orthodontic-surgical treatment The purpose of this study was to describethe case of a patient treated with surgery and orthodonticswhose satisfaction at the conclusion of therapy demonstratedthe importance of a malocclusionrsquos therapy to provide an
aesthetic improvement to the patient and a resolution of rela-tional and functional problemsThis work could be an incen-tive to evaluate patients with myotonic dystrophy in an earlyage to try to counter the worsening of malocclusions andavoiding surgery if possibleThepropose of an early interven-tion may seem like a challenge but it might be useful toidentify new care pathways that make better the daily livesof these young patients and facilitate their psychosomaticdevelopment during the delicate period of adolescence
Abbreviations
DM1 Myotonic dystrophy type 1DM2 Myotonic dystrophy type 2PROMM Proximal Myotonic MyopathyDMPK Myotonin protein kinaseDM3 Myotonic dystrophy type 3ZNF9 Zinc Finger Protein Gene 9OVB OverbiteOVJ OverjetRx Radiography
Conflicts of Interest
The authors declare that they have no conflicts of interest
References
[1] T Suominen L L Bachinski S Auvinen et al ldquoPopulation fre-quency of myotonic dystrophy higher than expected frequencyof myotonic dystrophy type 2 (DM2) mutation in FinlandrdquoEuropean Journal of Human Genetics vol 19 no 7 pp 776ndash7822011
[2] P S Haper Myotonic DystrophymdashThe Clinical Picture WBSaunders London UK 2001
[3] J D Brook M E McCurrach H G Harley et al ldquoMolecularbasis of myotonic dystrophy Expansion of a trinucleotide(CTG) repeat at the 31015840 end of a transcript encoding a proteinkinase family memberrdquo Cell vol 68 no 4 pp 799ndash808 1992
[4] J Buxton P Shelbourne J Davies et al ldquoDetection of anunstable fragment ofDNA specific to individuals withmyotonicdystrophyrdquo Nature vol 355 no 6360 pp 547-548 1992
[5] Y-H Fu A Pizzuti R G Fenwick Jr et al ldquoAn unstabletriplet repeat in a gene related tomyotonicmuscular dystrophyrdquoScience vol 255 no 5049 pp 1256ndash1258 1992
[6] H G Harley J D Brook S A Rundle et al ldquoExpansion ofan unstable DNA region and phenotypic variation in myotonicdystrophyrdquo Nature vol 355 no 6360 pp 545-546 1992
[7] M Mahadevan C Tsilfidis L Sabourin et al ldquoMyotonic dys-trophy mutation an unstable CTG repeat in the 31015840 untranslatedregion of the generdquo Science vol 255 no 5049 pp 1253ndash12551992
[8] J Mathieu P Allard L Potvin C Prevost and P Begin ldquoA 10-year study of mortality in a cohort of patients with myotonicdystrophyrdquo Neurology vol 52 no 8 pp 1658ndash1662 1999
[9] G Meola ldquoClinical aspects molecular pathomechanisms andmanagement of myotonic dystrophiesrdquo Acta Myologica vol 32no 3 pp 154ndash165 2013
[10] S KiliaridisM Engvall andMG Tzakis ldquoUltrasound imagingof themassetermuscle inmyotonic dystrophy patientsrdquo Journalof Oral Rehabilitation vol 22 no 8 pp 619ndash625 1995
Case Reports in Dentistry 9
[11] C Odman and S Kiliaridis ldquoMasticatory muscle activity inmyotonic dystrophy patientsrdquo Journal of Oral Rehabilitationvol 23 no 1 pp 5ndash10 1996
[12] R N Staley S E Bishara J W Hanson and A J Nowak ldquoCra-niofacial development in myotonic dystrophyrdquoTheCleft Palate-Craniofacial Journal vol 29 no 5 pp 456ndash462 1992
[13] M Portelli G Matarese A Militi R Nucera G Triolo and GCordasco ldquoMyotonic dystrophy and craniofacial morphologyclinical and instrumental studyrdquo European Journal of PaediatricDentistry vol 10 no 1 pp 19ndash22 2009
[14] E Gazit N Bornstein M Lieberman V Serfaty M Gross andA D Korczyn ldquoThe stomatognathic system in myotonic dys-trophyrdquo European Journal of Orthodontics vol 9 no 1 pp 160ndash164 1987
[15] S Kiliaridis C Mejersjo and B Thilander ldquoMuscle functionand craniofacial morphology a clinical study in patients withmyotonic dystrophyrdquo European Journal of Orthodontics vol 11no 2 pp 131ndash138 1989
[16] J Mercier F Bennani J Ferri and B Piot ldquoMaxillofacial mani-festations of Steinertrsquos myotonic dystrophy clinical and thera-peutic aspectsrdquo Revue de Stomatologie et de Chirurgie Maxillo-Faciale vol 96 pp 74ndash82 1995
[17] G Meola ldquoClinical aspects molecular pathomechanisms andmanagement of myotonic dystrophiesrdquo Acta Myologica vol 32no 3 pp 154ndash165 2013
[18] L Sjogreen M Engvall A-B Ekstrom A Lohmander S Kil-iaridis andM Tulinius ldquoOrofacial dysfunction in children andadolescents with myotonic dystrophyrdquoDevelopmental Medicineand Child Neurology vol 49 no 1 pp 18ndash22 2007
[19] B Ercolin F C Sassi L D Mangilli L I Z Mendonca S CO Limongi and C R F De Andrade ldquoOral motor movementsand swallowing in patients with myotonic dystrophy type 1rdquoDysphagia vol 28 no 3 pp 446ndash454 2013
[20] L Dimberg K Arnrup and L Bondemark ldquoThe impact ofmal-occlusion on the quality of life among children and adolescentsa systematic review of quantitative studiesrdquo European Journal ofOrthodontics vol 37 no 3 pp 238ndash247 2015
[21] M Engvall L Sjogreen H Kjellberg A Robertson S Sundelland S Kiliaridis ldquoOral health in children and adolescents withmyotonic dystrophyrdquo European Journal of Oral Sciences vol 115no 3 pp 192ndash197 2007
[22] G W Arnett and R T Bergman ldquoFacial keys to orthodonticdiagnosis and treatment planning Part Irdquo American Journal ofOrthodontics and Dentofacial Orthopedics vol 103 no 4 pp299ndash312 1993
[23] G W Arnett and R T Bergman ldquoFacial keys to orthodonticdiagnosis and treatment planning Part IIrdquo American Journalof Orthodontics and Dentofacial Orthopedics vol 103 no 5 pp395ndash411 1993
[24] S Manzon and R Philbert ldquoOrthognathic Surgery in a PatientWith Myotonic Dystrophy Review of Literature and Report ofa Caserdquo Journal of Oral andMaxillofacial Surgery vol 65 no 12pp 2575ndash2579 2007
[25] S Kiliaridis andC Katsaros ldquoThe effects ofmyotonic dystrophyand Duchenne muscular dystrophy on the orofacial musclesand dentofacial morphologyrdquo Acta Odontologica Scandinavicavol 56 no 6 pp 369ndash374 1998
[26] J Kaufman J M Friedman D Sadowsky and J Harris ldquoMyo-tonic dystrophy surgical and anesthetic considerations duringorthognathic surgeryrdquo Journal ofOral andMaxillofacial Surgeryvol 41 no 10 pp 667ndash671 1983
[27] E Zanoteli H K Yamashita H Suzuki A S B Oliveira and AA Gabbai ldquoTemporomandibular joint and masticatory muscleinvolvement in myotonic dystrophy a study by magnetic res-onance imagingrdquo Oral Surgery Oral Medicine Oral PathologyOral Radiology and Endodontics vol 94 no 2 pp 262ndash2712002
[28] Ministero della Salute ldquoLinee guida nazionali per la promo-zione della salute orale e la prevenzione delle patologie oraliin eta evolutivardquo 2013 httpwwwsalutegovitimgsC_17_pub-blicazioni_2073_allegatopdf
[29] K J Skidmore K J Brook W MThomson andW J HardingldquoFactors influencing treatment time in orthodontic patientsrdquoAmerican Journal of Orthodontics and Dentofacial Orthopedicsvol 129 no 2 pp 230ndash238 2006
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2 Case Reports in Dentistry
or with a high degree of atrioventricular block may diesuddenly the insertion of a pacemaker is recommended forthese subjects The mitral valve prolapse and dysfunction ofthe left ventricle are less frequent The disease progressesslowly with gradual involvement of the proximal muscles ofthe limbs and trunk muscles
The age of onset and clinical manifestations are highlyvariable depending on the type of genetic alteration Markeddifferences are noticed in individuals and in the variousmembers of the same family
Some years ago the detection of a form clinically verysimilar to myotonic dystrophy but with proximal strengthdeficit and different genetic basis resulted in the introductionof DM1 symbol to indicate Steinertrsquos disease and DM2 toindicate this other form also known as PROMM (ProximalMyotonic Myopathy) Other subjects with similar clinicalcharacteristics did not show the alteration of the DM1 genenor that of DM2 DM1 or Steinertrsquos dystrophy is caused by thegene defect of myotonin protein kinase (DMPK) located onchromosome 19q133 The DM2 is rarer and it is secondaryto the defect of the Zinc Finger Protein Gene 9 (ZNF9)encoded by chromosome 3q21 There is still a third form(DM3) which however does not have a precise genetic andmolecular characterization
Both the forms of dystrophy are characterized by exces-sive repetition (ldquobabblingrdquo) of a sequence of nucleotides(triplets) that in normal subjects is repeated for a limitednumber of times In the subjects affected by the disease thesebase sequences (CTG for DM1 and CCTG for DM2) may berepeated from tens to thousands of times compromising thefunction of the gene In general the greater the expansion ofnucleotides is the more serious the clinical expression of thedisease is The expansion can vary in different tissues of thesame individual which explains the different manifestationsof the disease
In Steinertrsquos myotonic dystrophy the muscular involve-ment is especially evident in the distal districts (forearmhand leg and foot) and the mimic muscles of the facewith a reduction in the expression movements of the faceand drooping eyelids (ptosis) This disease affects the entireskeletal musculature with generalized weakness and easyfatigability In PROMM however the muscle involvementprevails in districts proximal limbs (shoulders arms pelvisand thigh) Both forms are characterized by myotonic phe-nomenon [17]
Severe congenital forms of DM1 exist childhood andadolescence related forms and adult forms which are themost common In one household different forms can coexisttoo without obvious symptoms (subclinical)
People with myotonic dystrophy affecting facial andmasticatory muscles have a characteristic facial appearancelong face gonial angle and mandibular divergence increasewhich are frequently associated with malocclusions In par-ticular these individuals have a constriction of the palateand an anterior open bite [15 16] Very often severe dentalmalocclusions create discomfort in oral functions and inparticular in chewing breathing and phonation [18 19]besides constituting a social disadvantage [20] In additionthe functional discomfort creates difficulty in swallowing and
Figure 1 Front and lateral photographs of the patient at the time ofthe first visit
promotes general dental problems related to dry mouth dueto the labial incompetence and mouth breathing with plaquebuildup and gingivitis and greater susceptibility to caries [21]
In the paper we present the case report of a patient withSteinertrsquos syndrome treated with orthodontic-surgical ther-apy Although therapy caused an improvement in patientrsquosquality of life the clinical management of the case was hardThe case report is aimed to show a balance between costs andbenefits of a therapy that challenges the nature of the mainproblem of the patient and it is useful to identify the mostappropriate course of treatment for similar cases
2 Case Presentation
21 Diagnosis The patient M M (male) affected by myo-tonic dystrophy type 1 came to our attention at the age of15 years and seven months because he was not satisfied withhis occlusion and his facial appearance Because of lip incom-petence the patient had difficulty in eating and swallow-ing and difficulty in pronouncing certain phonemes Stein-ertrsquos syndrome was diagnosed when the patient was 7 yearsold and he presented with macroglossia heart problemsmouth breathing and frequent pneumonia Genetic analysisshowed a class 2 expansion of about 660 CTG The familyhistory revealed that the father who died because of cardiaccomplications has been suffering from myotonic dystrophydiagnosed at age of 50 due to heart problems and difficulty ingrasping objects and deficit of strength in hands The grand-mother and the paternal aunt were suffering from myotonicdystrophy too while the sister was not affected Physicalexamination showed a patient with long narrow face mildbilateral ptosis lip incompetence and hypotonia of the peri-oral muscles The examination of the profile revealed a con-vex profile with increasing gonial angle (Figure 1) (informedconsent has been obtained by the patient for publication)
The intraoral examination revealed a significant anterioropen bite (OVBminus25mmOVJ 5mm) upper and lower dentalcrowding bilateral crossbite and constriction of the upperjaw with a high and narrow palate (Figure 2)
Case Reports in Dentistry 3
Figure 2 Intraoral photographs at the time of the first visit
Figure 3 Rx before treatment
The Illinois cephalometric analysis (Table 1) revealed asecond hyperdivergent skeletal class with mandibular retru-sion tendency of vertical growth and reduced inclination oflower and upper incisors (Figure 3)
22Therapy Because of the severity of skeletalmalocclusionthe patient received a combined orthodontic and surgicaltherapy in order to achieve the highest aesthetic and func-tional result Maxillary rapid palatal expander performedinitially two rapid expansion cyclesWhen the expansion wascompleted an orthodontic fixed multibrackets appliance wasapplied in upper and lower arch The presurgical therapyhad the purpose to improve the shape of both arches whichappeared ovoid and provide a correct expansion of theupper jaw in preparation for orthognathic surgery (Figure 4)Having completed the phase of orthodontic presurgical treat-ment the patient underwent maxillofacial surgery Arnett-Bergman soft tissues analysis [22 23] (Table 2) and surgical
visual treatment objects (VTO) were performed beforesurgery (Figure 5) A maxillary clockwise rotation of 5mmand BSSO of the mandible were planned A counterclockwiserotation of 18∘ of the occlusal plane was planned On VTO avalue of SNA of 77∘ SNB of 73∘ ANB of 5∘ IMPA of 72∘ andFMA of 48∘ were planned On the postsurgery cephalometricanalysis we had a value of SNA of 81∘ SNB of 76∘ ANB of5∘ IMPA of 73∘ and FMA of 49∘ Therefore the results weresimilar to those planned
Before surgery the patient underwent the extraction ofthe third lower molars in bone inclusion After the healingof mucosal wounds and after the finding of bone healing ofextraction sites using orthopanoramic Rx the patient had theorthognathic surgery intervention which consisted in max-illary clockwise rotation and sagittal mandibular osteotomyrepositioning in maximum intercuspidation of the dentalarches Photos (Figure 6 and Table 3) and Rx (Figure 7 andTable 4) show a facial and skeletal harmony improvement
4 Case Reports in Dentistry
Figure 4 Extraoral and intraoral photographs before surgery
Figure 5 Arnett-Bergman soft tissues analysis and VTO before surgery
Case Reports in Dentistry 5
Figure 6 Extraoral and intraoral photographs and Arnett-Bergman soft tissues analysis after surgery
The recovery after the operation was difficult because ofan extreme muscle weakness after surgery and this is thereason why the patient avoids the orthodontic checks for twomonths After surgery orthodontic treatment completed thealignment and coordination of the dental arches Intermaxil-lary elastics acted to optimize vertical occlusal relationshipsIn this phase the rapid maxillary expander has been keptin place to prevent the relapse of the maxillary constrictionBecause of an initial relapse of the open bite the expanderwasremoved and the patient began speech therapy to improvethe posture of the tongue Six months later there was arecurrence of the palatal constriction One year after surgery
Bollard screws were positioned to attach vertical elastics tofixed retainers in order to counter the open bite Two yearsafter surgery the fixed orthodontic appliance was removedand upper and lower Schwarz appliances of restraint weredelivered to the patient to keep the result in combinationwithfixed upper and lower retainers (Figure 8) The patient wassubjected to regular checks until after seven months afterthe removal of orthodontic appliances when it was decidedto hand over to the patient two thermoformed appliances(upper and lower) with box shaped elastics positioned onfrontal teeth because of the partial relapse of open bitedue to facial muscle deficits linked to the primary disease
6 Case Reports in Dentistry
Figure 7 Rx after surgery
Table 1 Pretreatment cephalometric analysis
Case NormFacial angle 782 883Convexity angle 171 5SNA 792 802SNB 717 77ANB 75 32Mandibular angle 576 224119910-axis 789 573Occlusal angle 376 9Interincisal angle 1214 129Li-occlusal plane 8 184Li-mandibular plane minus12 48UiSN 946 105UiA-Pg 87 5LiA-Pg 82 13MLSNL 631 NLSNL 12 Rotational type P1NOB Growth category 2
Today the patient periodically comes to visit tominimize therecurrence of the open bite and tomotivate the cooperation inwearing physical containment and to maintain oral hygiene
23 Discussion Myotonic dystrophy is a very complexmolecular pathology with multisystemic involvement [2ndash9]People with myotonic dystrophy type 1 frequently have acharacteristic facial appearance such as that observed in thepatient described in this paper [12 13 24]
Kiliaridis et al [15] and Mercier et al [16] claim thatcraniofacialmalformations in patientswithmyotonic atrophyare associated with malocclusions in patients who have aprecocious involvement of themusclesThe patient describedin the case shows a constriction of the upper jaw narrowface increasedmandibular angle and a vertical facial growthwhich are perhaps the result of increased neuromuscularfunction as reported by Staley et al [12]The patient presentsanterior open bite as frequently found in the pathology as
Table 2 Arnett-Bergman soft tissues analysis before surgery
Case NormFacial angle 1493 165ndash173∘
Nasal projection 126 13ndash18mmNasolabial angle 988 94ndash110∘
Lower face height 99 57ndash74mmLower face 562 53ndash56
Upper lip length 149 F 18ndash22M 22ndash25mm
Maxillary sulcus 1268 127ndash147∘
Upper lip protrusion 84 3 plusmn 1mmInterlabial gap 363 1ndash5mm
Lower lip-chin length 484 F 43ndash50M 45ndash54
Mandibular sulcus 132 110ndash134∘
L lip protrusion 179 2 plusmn 1mmB-SnPg line 71 4 plusmn 1mmLower face-throat angle 134 96ndash110∘
Throat length 854 51ndash63mm
reported by Portelli et al [13] because of the prevalence ofthe force of gravity on the elevator muscles Kiliaridis andKatsaros [25] say that the increasing of the vertical growthis due to an alteration of the perioral muscles in combinationwith a minor involvement of suprahyoid muscles So a newsituation is established around the teeth which alter thetransversal relationships The tongue in a lower position isnot able to counterbalance the forces developed during thelowering of the lower jaw caused by the elongated facialmusculature This situation as Kiliaridis and Katsaros [25]say can also affect the vertical size decreasing thewidth of thepalate and causing a posterior crossbiteThe lowered positionof the mandible in combination with the reduction of theocclusal forces can allow a supereruption of the posteriorteeth with increased height of the palatal vault and thedevelopment of an anterior open bite
With regard to the surgical therapy as Manzon andPhilbert [24] say although the induction of general anesthesiais extremely safe in a healthy population severe reaction
Case Reports in Dentistry 7
Figure 8 Extraoral and intraoral photographs after orthodontic therapy
Table 3 Arnett-Bergman soft tissues analysis after surgery
Case NormFacial angle 1503 165ndash173∘
Nasal projection 18 13ndash18mmNasolabial angle 1309 94ndash110∘
Lower face height 90 57ndash74mmLower face 493 53ndash56
Upper lip length 209 F 18ndash22M 22ndash25mm
Maxillary sulcus 1687 127ndash147∘
Upper lip protrusion 66 3 plusmn 1mmInterlabial gap 0 1ndash5mm
Lower lip-chin length 661 F 43ndash50M 45ndash54
Mandibular sulcus 1737 110ndash134∘
L lip protrusion 78 2 plusmn 1mmB-SnPg line 49 4 plusmn 1mmLower face-throat angle 185 96ndash110∘
Throat length 71 51ndash63mm
to anesthetic and neuromuscular paralyzing agents in amyotonic patient can cause organ failure myocardial infarc-tion and respiratory failure Therefore given the complexityof the case and the disease a polispecialistic collaborationcomprisingmaxillofacial surgeons anesthetists-resuscitatorsneurologists pulmonologists and cardiologists is requiredin the management of postoperative moment in order toprevent pulmonary and cardiac complications In order toperform a correct postoperative monitoring the patientdescribed in the case report spent the first night after surgeryin a postoperative intensive care unit The danger of com-plications related to general anesthesia makes this precau-tion strongly advisable in all patients who have to undergosurgery with general anesthesia
Surgical movements of the skeletal bases must be pro-grammed in order to provide the greatest postoperativesurgical stability in the long term because there is a high riskof relapse because of the progressiveweakness of the temporalmuscle The weakness of the masticatory muscles can leadto recurrence of the anterior open bite closed by surgery[26 27] In our experience in favor of combined orthodontic-surgical therapy there is the improvement of oral functionsand the patientrsquos appearance However the high probability
8 Case Reports in Dentistry
Table 4 Postsurgery cephalometric analysis
Case NormFacial angle 80 883Convexity angle 103 5SNA 813 802SNB 759 77ANB 54 32Mandibular angle 493 224119910-axis 747 573Occlusal angle 214 9Interincisal angle 142 129Li-occlusal plane 108 184Li-mandibular plane minus171 48UiSN 918 105UiA-Pg 62 5LiA-Pg 36 13MLSNL 534 NLSNL 128 Rotational type P1NOB Growth category 2
of recurrence complications related to general anesthesia andthe difficulties of recovery after the intervention and the needof very long orthodontic therapy which favor the accumu-lation of bacterial plaque and increase the risk of caries[28] must be kept in mind In the case report described infact throughout the orthodontic treatment the condition oforal health deteriorated because of the buildup of plaqueresulting in gingivitis and multiple demineralization at thecollar level of the teeth Naturally to undertake this typeof very long and complex therapy patient cooperation isnecessary but we know that during long therapy it alwaysdecreases [29] These considerations lead us to assess thesituation of each patient individually It would be better towork on the prevention and early treatment of malocclusionof cooperative patients with muscular dystrophy in orderto counteract the effects of facial hypotonic muscles onocclusion and the appearance of the patient from the time ofinitial diagnosis of the disease [28] Even cases successfullytreated in developmental age over time may recur or worsenthe functional deficit therefore patients should be orthodon-tically monitored periodically
3 Conclusion
Myotonic dystrophy is a very complex multisystemic diseasein which the muscular involvement leads to a characteristicfacial phenotype The weakness of the facial muscles is thecause of malocclusions needing in adulthood orthodontic-surgical treatment The purpose of this study was to describethe case of a patient treated with surgery and orthodonticswhose satisfaction at the conclusion of therapy demonstratedthe importance of a malocclusionrsquos therapy to provide an
aesthetic improvement to the patient and a resolution of rela-tional and functional problemsThis work could be an incen-tive to evaluate patients with myotonic dystrophy in an earlyage to try to counter the worsening of malocclusions andavoiding surgery if possibleThepropose of an early interven-tion may seem like a challenge but it might be useful toidentify new care pathways that make better the daily livesof these young patients and facilitate their psychosomaticdevelopment during the delicate period of adolescence
Abbreviations
DM1 Myotonic dystrophy type 1DM2 Myotonic dystrophy type 2PROMM Proximal Myotonic MyopathyDMPK Myotonin protein kinaseDM3 Myotonic dystrophy type 3ZNF9 Zinc Finger Protein Gene 9OVB OverbiteOVJ OverjetRx Radiography
Conflicts of Interest
The authors declare that they have no conflicts of interest
References
[1] T Suominen L L Bachinski S Auvinen et al ldquoPopulation fre-quency of myotonic dystrophy higher than expected frequencyof myotonic dystrophy type 2 (DM2) mutation in FinlandrdquoEuropean Journal of Human Genetics vol 19 no 7 pp 776ndash7822011
[2] P S Haper Myotonic DystrophymdashThe Clinical Picture WBSaunders London UK 2001
[3] J D Brook M E McCurrach H G Harley et al ldquoMolecularbasis of myotonic dystrophy Expansion of a trinucleotide(CTG) repeat at the 31015840 end of a transcript encoding a proteinkinase family memberrdquo Cell vol 68 no 4 pp 799ndash808 1992
[4] J Buxton P Shelbourne J Davies et al ldquoDetection of anunstable fragment ofDNA specific to individuals withmyotonicdystrophyrdquo Nature vol 355 no 6360 pp 547-548 1992
[5] Y-H Fu A Pizzuti R G Fenwick Jr et al ldquoAn unstabletriplet repeat in a gene related tomyotonicmuscular dystrophyrdquoScience vol 255 no 5049 pp 1256ndash1258 1992
[6] H G Harley J D Brook S A Rundle et al ldquoExpansion ofan unstable DNA region and phenotypic variation in myotonicdystrophyrdquo Nature vol 355 no 6360 pp 545-546 1992
[7] M Mahadevan C Tsilfidis L Sabourin et al ldquoMyotonic dys-trophy mutation an unstable CTG repeat in the 31015840 untranslatedregion of the generdquo Science vol 255 no 5049 pp 1253ndash12551992
[8] J Mathieu P Allard L Potvin C Prevost and P Begin ldquoA 10-year study of mortality in a cohort of patients with myotonicdystrophyrdquo Neurology vol 52 no 8 pp 1658ndash1662 1999
[9] G Meola ldquoClinical aspects molecular pathomechanisms andmanagement of myotonic dystrophiesrdquo Acta Myologica vol 32no 3 pp 154ndash165 2013
[10] S KiliaridisM Engvall andMG Tzakis ldquoUltrasound imagingof themassetermuscle inmyotonic dystrophy patientsrdquo Journalof Oral Rehabilitation vol 22 no 8 pp 619ndash625 1995
Case Reports in Dentistry 9
[11] C Odman and S Kiliaridis ldquoMasticatory muscle activity inmyotonic dystrophy patientsrdquo Journal of Oral Rehabilitationvol 23 no 1 pp 5ndash10 1996
[12] R N Staley S E Bishara J W Hanson and A J Nowak ldquoCra-niofacial development in myotonic dystrophyrdquoTheCleft Palate-Craniofacial Journal vol 29 no 5 pp 456ndash462 1992
[13] M Portelli G Matarese A Militi R Nucera G Triolo and GCordasco ldquoMyotonic dystrophy and craniofacial morphologyclinical and instrumental studyrdquo European Journal of PaediatricDentistry vol 10 no 1 pp 19ndash22 2009
[14] E Gazit N Bornstein M Lieberman V Serfaty M Gross andA D Korczyn ldquoThe stomatognathic system in myotonic dys-trophyrdquo European Journal of Orthodontics vol 9 no 1 pp 160ndash164 1987
[15] S Kiliaridis C Mejersjo and B Thilander ldquoMuscle functionand craniofacial morphology a clinical study in patients withmyotonic dystrophyrdquo European Journal of Orthodontics vol 11no 2 pp 131ndash138 1989
[16] J Mercier F Bennani J Ferri and B Piot ldquoMaxillofacial mani-festations of Steinertrsquos myotonic dystrophy clinical and thera-peutic aspectsrdquo Revue de Stomatologie et de Chirurgie Maxillo-Faciale vol 96 pp 74ndash82 1995
[17] G Meola ldquoClinical aspects molecular pathomechanisms andmanagement of myotonic dystrophiesrdquo Acta Myologica vol 32no 3 pp 154ndash165 2013
[18] L Sjogreen M Engvall A-B Ekstrom A Lohmander S Kil-iaridis andM Tulinius ldquoOrofacial dysfunction in children andadolescents with myotonic dystrophyrdquoDevelopmental Medicineand Child Neurology vol 49 no 1 pp 18ndash22 2007
[19] B Ercolin F C Sassi L D Mangilli L I Z Mendonca S CO Limongi and C R F De Andrade ldquoOral motor movementsand swallowing in patients with myotonic dystrophy type 1rdquoDysphagia vol 28 no 3 pp 446ndash454 2013
[20] L Dimberg K Arnrup and L Bondemark ldquoThe impact ofmal-occlusion on the quality of life among children and adolescentsa systematic review of quantitative studiesrdquo European Journal ofOrthodontics vol 37 no 3 pp 238ndash247 2015
[21] M Engvall L Sjogreen H Kjellberg A Robertson S Sundelland S Kiliaridis ldquoOral health in children and adolescents withmyotonic dystrophyrdquo European Journal of Oral Sciences vol 115no 3 pp 192ndash197 2007
[22] G W Arnett and R T Bergman ldquoFacial keys to orthodonticdiagnosis and treatment planning Part Irdquo American Journal ofOrthodontics and Dentofacial Orthopedics vol 103 no 4 pp299ndash312 1993
[23] G W Arnett and R T Bergman ldquoFacial keys to orthodonticdiagnosis and treatment planning Part IIrdquo American Journalof Orthodontics and Dentofacial Orthopedics vol 103 no 5 pp395ndash411 1993
[24] S Manzon and R Philbert ldquoOrthognathic Surgery in a PatientWith Myotonic Dystrophy Review of Literature and Report ofa Caserdquo Journal of Oral andMaxillofacial Surgery vol 65 no 12pp 2575ndash2579 2007
[25] S Kiliaridis andC Katsaros ldquoThe effects ofmyotonic dystrophyand Duchenne muscular dystrophy on the orofacial musclesand dentofacial morphologyrdquo Acta Odontologica Scandinavicavol 56 no 6 pp 369ndash374 1998
[26] J Kaufman J M Friedman D Sadowsky and J Harris ldquoMyo-tonic dystrophy surgical and anesthetic considerations duringorthognathic surgeryrdquo Journal ofOral andMaxillofacial Surgeryvol 41 no 10 pp 667ndash671 1983
[27] E Zanoteli H K Yamashita H Suzuki A S B Oliveira and AA Gabbai ldquoTemporomandibular joint and masticatory muscleinvolvement in myotonic dystrophy a study by magnetic res-onance imagingrdquo Oral Surgery Oral Medicine Oral PathologyOral Radiology and Endodontics vol 94 no 2 pp 262ndash2712002
[28] Ministero della Salute ldquoLinee guida nazionali per la promo-zione della salute orale e la prevenzione delle patologie oraliin eta evolutivardquo 2013 httpwwwsalutegovitimgsC_17_pub-blicazioni_2073_allegatopdf
[29] K J Skidmore K J Brook W MThomson andW J HardingldquoFactors influencing treatment time in orthodontic patientsrdquoAmerican Journal of Orthodontics and Dentofacial Orthopedicsvol 129 no 2 pp 230ndash238 2006
Submit your manuscripts athttpswwwhindawicom
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
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BioMed Research International
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Case Reports in Dentistry
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Oral ImplantsJournal of
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OrthopedicsAdvances in
Case Reports in Dentistry 3
Figure 2 Intraoral photographs at the time of the first visit
Figure 3 Rx before treatment
The Illinois cephalometric analysis (Table 1) revealed asecond hyperdivergent skeletal class with mandibular retru-sion tendency of vertical growth and reduced inclination oflower and upper incisors (Figure 3)
22Therapy Because of the severity of skeletalmalocclusionthe patient received a combined orthodontic and surgicaltherapy in order to achieve the highest aesthetic and func-tional result Maxillary rapid palatal expander performedinitially two rapid expansion cyclesWhen the expansion wascompleted an orthodontic fixed multibrackets appliance wasapplied in upper and lower arch The presurgical therapyhad the purpose to improve the shape of both arches whichappeared ovoid and provide a correct expansion of theupper jaw in preparation for orthognathic surgery (Figure 4)Having completed the phase of orthodontic presurgical treat-ment the patient underwent maxillofacial surgery Arnett-Bergman soft tissues analysis [22 23] (Table 2) and surgical
visual treatment objects (VTO) were performed beforesurgery (Figure 5) A maxillary clockwise rotation of 5mmand BSSO of the mandible were planned A counterclockwiserotation of 18∘ of the occlusal plane was planned On VTO avalue of SNA of 77∘ SNB of 73∘ ANB of 5∘ IMPA of 72∘ andFMA of 48∘ were planned On the postsurgery cephalometricanalysis we had a value of SNA of 81∘ SNB of 76∘ ANB of5∘ IMPA of 73∘ and FMA of 49∘ Therefore the results weresimilar to those planned
Before surgery the patient underwent the extraction ofthe third lower molars in bone inclusion After the healingof mucosal wounds and after the finding of bone healing ofextraction sites using orthopanoramic Rx the patient had theorthognathic surgery intervention which consisted in max-illary clockwise rotation and sagittal mandibular osteotomyrepositioning in maximum intercuspidation of the dentalarches Photos (Figure 6 and Table 3) and Rx (Figure 7 andTable 4) show a facial and skeletal harmony improvement
4 Case Reports in Dentistry
Figure 4 Extraoral and intraoral photographs before surgery
Figure 5 Arnett-Bergman soft tissues analysis and VTO before surgery
Case Reports in Dentistry 5
Figure 6 Extraoral and intraoral photographs and Arnett-Bergman soft tissues analysis after surgery
The recovery after the operation was difficult because ofan extreme muscle weakness after surgery and this is thereason why the patient avoids the orthodontic checks for twomonths After surgery orthodontic treatment completed thealignment and coordination of the dental arches Intermaxil-lary elastics acted to optimize vertical occlusal relationshipsIn this phase the rapid maxillary expander has been keptin place to prevent the relapse of the maxillary constrictionBecause of an initial relapse of the open bite the expanderwasremoved and the patient began speech therapy to improvethe posture of the tongue Six months later there was arecurrence of the palatal constriction One year after surgery
Bollard screws were positioned to attach vertical elastics tofixed retainers in order to counter the open bite Two yearsafter surgery the fixed orthodontic appliance was removedand upper and lower Schwarz appliances of restraint weredelivered to the patient to keep the result in combinationwithfixed upper and lower retainers (Figure 8) The patient wassubjected to regular checks until after seven months afterthe removal of orthodontic appliances when it was decidedto hand over to the patient two thermoformed appliances(upper and lower) with box shaped elastics positioned onfrontal teeth because of the partial relapse of open bitedue to facial muscle deficits linked to the primary disease
6 Case Reports in Dentistry
Figure 7 Rx after surgery
Table 1 Pretreatment cephalometric analysis
Case NormFacial angle 782 883Convexity angle 171 5SNA 792 802SNB 717 77ANB 75 32Mandibular angle 576 224119910-axis 789 573Occlusal angle 376 9Interincisal angle 1214 129Li-occlusal plane 8 184Li-mandibular plane minus12 48UiSN 946 105UiA-Pg 87 5LiA-Pg 82 13MLSNL 631 NLSNL 12 Rotational type P1NOB Growth category 2
Today the patient periodically comes to visit tominimize therecurrence of the open bite and tomotivate the cooperation inwearing physical containment and to maintain oral hygiene
23 Discussion Myotonic dystrophy is a very complexmolecular pathology with multisystemic involvement [2ndash9]People with myotonic dystrophy type 1 frequently have acharacteristic facial appearance such as that observed in thepatient described in this paper [12 13 24]
Kiliaridis et al [15] and Mercier et al [16] claim thatcraniofacialmalformations in patientswithmyotonic atrophyare associated with malocclusions in patients who have aprecocious involvement of themusclesThe patient describedin the case shows a constriction of the upper jaw narrowface increasedmandibular angle and a vertical facial growthwhich are perhaps the result of increased neuromuscularfunction as reported by Staley et al [12]The patient presentsanterior open bite as frequently found in the pathology as
Table 2 Arnett-Bergman soft tissues analysis before surgery
Case NormFacial angle 1493 165ndash173∘
Nasal projection 126 13ndash18mmNasolabial angle 988 94ndash110∘
Lower face height 99 57ndash74mmLower face 562 53ndash56
Upper lip length 149 F 18ndash22M 22ndash25mm
Maxillary sulcus 1268 127ndash147∘
Upper lip protrusion 84 3 plusmn 1mmInterlabial gap 363 1ndash5mm
Lower lip-chin length 484 F 43ndash50M 45ndash54
Mandibular sulcus 132 110ndash134∘
L lip protrusion 179 2 plusmn 1mmB-SnPg line 71 4 plusmn 1mmLower face-throat angle 134 96ndash110∘
Throat length 854 51ndash63mm
reported by Portelli et al [13] because of the prevalence ofthe force of gravity on the elevator muscles Kiliaridis andKatsaros [25] say that the increasing of the vertical growthis due to an alteration of the perioral muscles in combinationwith a minor involvement of suprahyoid muscles So a newsituation is established around the teeth which alter thetransversal relationships The tongue in a lower position isnot able to counterbalance the forces developed during thelowering of the lower jaw caused by the elongated facialmusculature This situation as Kiliaridis and Katsaros [25]say can also affect the vertical size decreasing thewidth of thepalate and causing a posterior crossbiteThe lowered positionof the mandible in combination with the reduction of theocclusal forces can allow a supereruption of the posteriorteeth with increased height of the palatal vault and thedevelopment of an anterior open bite
With regard to the surgical therapy as Manzon andPhilbert [24] say although the induction of general anesthesiais extremely safe in a healthy population severe reaction
Case Reports in Dentistry 7
Figure 8 Extraoral and intraoral photographs after orthodontic therapy
Table 3 Arnett-Bergman soft tissues analysis after surgery
Case NormFacial angle 1503 165ndash173∘
Nasal projection 18 13ndash18mmNasolabial angle 1309 94ndash110∘
Lower face height 90 57ndash74mmLower face 493 53ndash56
Upper lip length 209 F 18ndash22M 22ndash25mm
Maxillary sulcus 1687 127ndash147∘
Upper lip protrusion 66 3 plusmn 1mmInterlabial gap 0 1ndash5mm
Lower lip-chin length 661 F 43ndash50M 45ndash54
Mandibular sulcus 1737 110ndash134∘
L lip protrusion 78 2 plusmn 1mmB-SnPg line 49 4 plusmn 1mmLower face-throat angle 185 96ndash110∘
Throat length 71 51ndash63mm
to anesthetic and neuromuscular paralyzing agents in amyotonic patient can cause organ failure myocardial infarc-tion and respiratory failure Therefore given the complexityof the case and the disease a polispecialistic collaborationcomprisingmaxillofacial surgeons anesthetists-resuscitatorsneurologists pulmonologists and cardiologists is requiredin the management of postoperative moment in order toprevent pulmonary and cardiac complications In order toperform a correct postoperative monitoring the patientdescribed in the case report spent the first night after surgeryin a postoperative intensive care unit The danger of com-plications related to general anesthesia makes this precau-tion strongly advisable in all patients who have to undergosurgery with general anesthesia
Surgical movements of the skeletal bases must be pro-grammed in order to provide the greatest postoperativesurgical stability in the long term because there is a high riskof relapse because of the progressiveweakness of the temporalmuscle The weakness of the masticatory muscles can leadto recurrence of the anterior open bite closed by surgery[26 27] In our experience in favor of combined orthodontic-surgical therapy there is the improvement of oral functionsand the patientrsquos appearance However the high probability
8 Case Reports in Dentistry
Table 4 Postsurgery cephalometric analysis
Case NormFacial angle 80 883Convexity angle 103 5SNA 813 802SNB 759 77ANB 54 32Mandibular angle 493 224119910-axis 747 573Occlusal angle 214 9Interincisal angle 142 129Li-occlusal plane 108 184Li-mandibular plane minus171 48UiSN 918 105UiA-Pg 62 5LiA-Pg 36 13MLSNL 534 NLSNL 128 Rotational type P1NOB Growth category 2
of recurrence complications related to general anesthesia andthe difficulties of recovery after the intervention and the needof very long orthodontic therapy which favor the accumu-lation of bacterial plaque and increase the risk of caries[28] must be kept in mind In the case report described infact throughout the orthodontic treatment the condition oforal health deteriorated because of the buildup of plaqueresulting in gingivitis and multiple demineralization at thecollar level of the teeth Naturally to undertake this typeof very long and complex therapy patient cooperation isnecessary but we know that during long therapy it alwaysdecreases [29] These considerations lead us to assess thesituation of each patient individually It would be better towork on the prevention and early treatment of malocclusionof cooperative patients with muscular dystrophy in orderto counteract the effects of facial hypotonic muscles onocclusion and the appearance of the patient from the time ofinitial diagnosis of the disease [28] Even cases successfullytreated in developmental age over time may recur or worsenthe functional deficit therefore patients should be orthodon-tically monitored periodically
3 Conclusion
Myotonic dystrophy is a very complex multisystemic diseasein which the muscular involvement leads to a characteristicfacial phenotype The weakness of the facial muscles is thecause of malocclusions needing in adulthood orthodontic-surgical treatment The purpose of this study was to describethe case of a patient treated with surgery and orthodonticswhose satisfaction at the conclusion of therapy demonstratedthe importance of a malocclusionrsquos therapy to provide an
aesthetic improvement to the patient and a resolution of rela-tional and functional problemsThis work could be an incen-tive to evaluate patients with myotonic dystrophy in an earlyage to try to counter the worsening of malocclusions andavoiding surgery if possibleThepropose of an early interven-tion may seem like a challenge but it might be useful toidentify new care pathways that make better the daily livesof these young patients and facilitate their psychosomaticdevelopment during the delicate period of adolescence
Abbreviations
DM1 Myotonic dystrophy type 1DM2 Myotonic dystrophy type 2PROMM Proximal Myotonic MyopathyDMPK Myotonin protein kinaseDM3 Myotonic dystrophy type 3ZNF9 Zinc Finger Protein Gene 9OVB OverbiteOVJ OverjetRx Radiography
Conflicts of Interest
The authors declare that they have no conflicts of interest
References
[1] T Suominen L L Bachinski S Auvinen et al ldquoPopulation fre-quency of myotonic dystrophy higher than expected frequencyof myotonic dystrophy type 2 (DM2) mutation in FinlandrdquoEuropean Journal of Human Genetics vol 19 no 7 pp 776ndash7822011
[2] P S Haper Myotonic DystrophymdashThe Clinical Picture WBSaunders London UK 2001
[3] J D Brook M E McCurrach H G Harley et al ldquoMolecularbasis of myotonic dystrophy Expansion of a trinucleotide(CTG) repeat at the 31015840 end of a transcript encoding a proteinkinase family memberrdquo Cell vol 68 no 4 pp 799ndash808 1992
[4] J Buxton P Shelbourne J Davies et al ldquoDetection of anunstable fragment ofDNA specific to individuals withmyotonicdystrophyrdquo Nature vol 355 no 6360 pp 547-548 1992
[5] Y-H Fu A Pizzuti R G Fenwick Jr et al ldquoAn unstabletriplet repeat in a gene related tomyotonicmuscular dystrophyrdquoScience vol 255 no 5049 pp 1256ndash1258 1992
[6] H G Harley J D Brook S A Rundle et al ldquoExpansion ofan unstable DNA region and phenotypic variation in myotonicdystrophyrdquo Nature vol 355 no 6360 pp 545-546 1992
[7] M Mahadevan C Tsilfidis L Sabourin et al ldquoMyotonic dys-trophy mutation an unstable CTG repeat in the 31015840 untranslatedregion of the generdquo Science vol 255 no 5049 pp 1253ndash12551992
[8] J Mathieu P Allard L Potvin C Prevost and P Begin ldquoA 10-year study of mortality in a cohort of patients with myotonicdystrophyrdquo Neurology vol 52 no 8 pp 1658ndash1662 1999
[9] G Meola ldquoClinical aspects molecular pathomechanisms andmanagement of myotonic dystrophiesrdquo Acta Myologica vol 32no 3 pp 154ndash165 2013
[10] S KiliaridisM Engvall andMG Tzakis ldquoUltrasound imagingof themassetermuscle inmyotonic dystrophy patientsrdquo Journalof Oral Rehabilitation vol 22 no 8 pp 619ndash625 1995
Case Reports in Dentistry 9
[11] C Odman and S Kiliaridis ldquoMasticatory muscle activity inmyotonic dystrophy patientsrdquo Journal of Oral Rehabilitationvol 23 no 1 pp 5ndash10 1996
[12] R N Staley S E Bishara J W Hanson and A J Nowak ldquoCra-niofacial development in myotonic dystrophyrdquoTheCleft Palate-Craniofacial Journal vol 29 no 5 pp 456ndash462 1992
[13] M Portelli G Matarese A Militi R Nucera G Triolo and GCordasco ldquoMyotonic dystrophy and craniofacial morphologyclinical and instrumental studyrdquo European Journal of PaediatricDentistry vol 10 no 1 pp 19ndash22 2009
[14] E Gazit N Bornstein M Lieberman V Serfaty M Gross andA D Korczyn ldquoThe stomatognathic system in myotonic dys-trophyrdquo European Journal of Orthodontics vol 9 no 1 pp 160ndash164 1987
[15] S Kiliaridis C Mejersjo and B Thilander ldquoMuscle functionand craniofacial morphology a clinical study in patients withmyotonic dystrophyrdquo European Journal of Orthodontics vol 11no 2 pp 131ndash138 1989
[16] J Mercier F Bennani J Ferri and B Piot ldquoMaxillofacial mani-festations of Steinertrsquos myotonic dystrophy clinical and thera-peutic aspectsrdquo Revue de Stomatologie et de Chirurgie Maxillo-Faciale vol 96 pp 74ndash82 1995
[17] G Meola ldquoClinical aspects molecular pathomechanisms andmanagement of myotonic dystrophiesrdquo Acta Myologica vol 32no 3 pp 154ndash165 2013
[18] L Sjogreen M Engvall A-B Ekstrom A Lohmander S Kil-iaridis andM Tulinius ldquoOrofacial dysfunction in children andadolescents with myotonic dystrophyrdquoDevelopmental Medicineand Child Neurology vol 49 no 1 pp 18ndash22 2007
[19] B Ercolin F C Sassi L D Mangilli L I Z Mendonca S CO Limongi and C R F De Andrade ldquoOral motor movementsand swallowing in patients with myotonic dystrophy type 1rdquoDysphagia vol 28 no 3 pp 446ndash454 2013
[20] L Dimberg K Arnrup and L Bondemark ldquoThe impact ofmal-occlusion on the quality of life among children and adolescentsa systematic review of quantitative studiesrdquo European Journal ofOrthodontics vol 37 no 3 pp 238ndash247 2015
[21] M Engvall L Sjogreen H Kjellberg A Robertson S Sundelland S Kiliaridis ldquoOral health in children and adolescents withmyotonic dystrophyrdquo European Journal of Oral Sciences vol 115no 3 pp 192ndash197 2007
[22] G W Arnett and R T Bergman ldquoFacial keys to orthodonticdiagnosis and treatment planning Part Irdquo American Journal ofOrthodontics and Dentofacial Orthopedics vol 103 no 4 pp299ndash312 1993
[23] G W Arnett and R T Bergman ldquoFacial keys to orthodonticdiagnosis and treatment planning Part IIrdquo American Journalof Orthodontics and Dentofacial Orthopedics vol 103 no 5 pp395ndash411 1993
[24] S Manzon and R Philbert ldquoOrthognathic Surgery in a PatientWith Myotonic Dystrophy Review of Literature and Report ofa Caserdquo Journal of Oral andMaxillofacial Surgery vol 65 no 12pp 2575ndash2579 2007
[25] S Kiliaridis andC Katsaros ldquoThe effects ofmyotonic dystrophyand Duchenne muscular dystrophy on the orofacial musclesand dentofacial morphologyrdquo Acta Odontologica Scandinavicavol 56 no 6 pp 369ndash374 1998
[26] J Kaufman J M Friedman D Sadowsky and J Harris ldquoMyo-tonic dystrophy surgical and anesthetic considerations duringorthognathic surgeryrdquo Journal ofOral andMaxillofacial Surgeryvol 41 no 10 pp 667ndash671 1983
[27] E Zanoteli H K Yamashita H Suzuki A S B Oliveira and AA Gabbai ldquoTemporomandibular joint and masticatory muscleinvolvement in myotonic dystrophy a study by magnetic res-onance imagingrdquo Oral Surgery Oral Medicine Oral PathologyOral Radiology and Endodontics vol 94 no 2 pp 262ndash2712002
[28] Ministero della Salute ldquoLinee guida nazionali per la promo-zione della salute orale e la prevenzione delle patologie oraliin eta evolutivardquo 2013 httpwwwsalutegovitimgsC_17_pub-blicazioni_2073_allegatopdf
[29] K J Skidmore K J Brook W MThomson andW J HardingldquoFactors influencing treatment time in orthodontic patientsrdquoAmerican Journal of Orthodontics and Dentofacial Orthopedicsvol 129 no 2 pp 230ndash238 2006
Submit your manuscripts athttpswwwhindawicom
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral OncologyJournal of
DentistryInternational Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
International Journal of
Biomaterials
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
BioMed Research International
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Case Reports in Dentistry
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral ImplantsJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Anesthesiology Research and Practice
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Radiology Research and Practice
Environmental and Public Health
Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
The Scientific World JournalHindawi Publishing Corporation httpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Dental SurgeryJournal of
Drug DeliveryJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral DiseasesJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Computational and Mathematical Methods in Medicine
ScientificaHindawi Publishing Corporationhttpwwwhindawicom Volume 2014
PainResearch and TreatmentHindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Preventive MedicineAdvances in
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
EndocrinologyInternational Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
OrthopedicsAdvances in
4 Case Reports in Dentistry
Figure 4 Extraoral and intraoral photographs before surgery
Figure 5 Arnett-Bergman soft tissues analysis and VTO before surgery
Case Reports in Dentistry 5
Figure 6 Extraoral and intraoral photographs and Arnett-Bergman soft tissues analysis after surgery
The recovery after the operation was difficult because ofan extreme muscle weakness after surgery and this is thereason why the patient avoids the orthodontic checks for twomonths After surgery orthodontic treatment completed thealignment and coordination of the dental arches Intermaxil-lary elastics acted to optimize vertical occlusal relationshipsIn this phase the rapid maxillary expander has been keptin place to prevent the relapse of the maxillary constrictionBecause of an initial relapse of the open bite the expanderwasremoved and the patient began speech therapy to improvethe posture of the tongue Six months later there was arecurrence of the palatal constriction One year after surgery
Bollard screws were positioned to attach vertical elastics tofixed retainers in order to counter the open bite Two yearsafter surgery the fixed orthodontic appliance was removedand upper and lower Schwarz appliances of restraint weredelivered to the patient to keep the result in combinationwithfixed upper and lower retainers (Figure 8) The patient wassubjected to regular checks until after seven months afterthe removal of orthodontic appliances when it was decidedto hand over to the patient two thermoformed appliances(upper and lower) with box shaped elastics positioned onfrontal teeth because of the partial relapse of open bitedue to facial muscle deficits linked to the primary disease
6 Case Reports in Dentistry
Figure 7 Rx after surgery
Table 1 Pretreatment cephalometric analysis
Case NormFacial angle 782 883Convexity angle 171 5SNA 792 802SNB 717 77ANB 75 32Mandibular angle 576 224119910-axis 789 573Occlusal angle 376 9Interincisal angle 1214 129Li-occlusal plane 8 184Li-mandibular plane minus12 48UiSN 946 105UiA-Pg 87 5LiA-Pg 82 13MLSNL 631 NLSNL 12 Rotational type P1NOB Growth category 2
Today the patient periodically comes to visit tominimize therecurrence of the open bite and tomotivate the cooperation inwearing physical containment and to maintain oral hygiene
23 Discussion Myotonic dystrophy is a very complexmolecular pathology with multisystemic involvement [2ndash9]People with myotonic dystrophy type 1 frequently have acharacteristic facial appearance such as that observed in thepatient described in this paper [12 13 24]
Kiliaridis et al [15] and Mercier et al [16] claim thatcraniofacialmalformations in patientswithmyotonic atrophyare associated with malocclusions in patients who have aprecocious involvement of themusclesThe patient describedin the case shows a constriction of the upper jaw narrowface increasedmandibular angle and a vertical facial growthwhich are perhaps the result of increased neuromuscularfunction as reported by Staley et al [12]The patient presentsanterior open bite as frequently found in the pathology as
Table 2 Arnett-Bergman soft tissues analysis before surgery
Case NormFacial angle 1493 165ndash173∘
Nasal projection 126 13ndash18mmNasolabial angle 988 94ndash110∘
Lower face height 99 57ndash74mmLower face 562 53ndash56
Upper lip length 149 F 18ndash22M 22ndash25mm
Maxillary sulcus 1268 127ndash147∘
Upper lip protrusion 84 3 plusmn 1mmInterlabial gap 363 1ndash5mm
Lower lip-chin length 484 F 43ndash50M 45ndash54
Mandibular sulcus 132 110ndash134∘
L lip protrusion 179 2 plusmn 1mmB-SnPg line 71 4 plusmn 1mmLower face-throat angle 134 96ndash110∘
Throat length 854 51ndash63mm
reported by Portelli et al [13] because of the prevalence ofthe force of gravity on the elevator muscles Kiliaridis andKatsaros [25] say that the increasing of the vertical growthis due to an alteration of the perioral muscles in combinationwith a minor involvement of suprahyoid muscles So a newsituation is established around the teeth which alter thetransversal relationships The tongue in a lower position isnot able to counterbalance the forces developed during thelowering of the lower jaw caused by the elongated facialmusculature This situation as Kiliaridis and Katsaros [25]say can also affect the vertical size decreasing thewidth of thepalate and causing a posterior crossbiteThe lowered positionof the mandible in combination with the reduction of theocclusal forces can allow a supereruption of the posteriorteeth with increased height of the palatal vault and thedevelopment of an anterior open bite
With regard to the surgical therapy as Manzon andPhilbert [24] say although the induction of general anesthesiais extremely safe in a healthy population severe reaction
Case Reports in Dentistry 7
Figure 8 Extraoral and intraoral photographs after orthodontic therapy
Table 3 Arnett-Bergman soft tissues analysis after surgery
Case NormFacial angle 1503 165ndash173∘
Nasal projection 18 13ndash18mmNasolabial angle 1309 94ndash110∘
Lower face height 90 57ndash74mmLower face 493 53ndash56
Upper lip length 209 F 18ndash22M 22ndash25mm
Maxillary sulcus 1687 127ndash147∘
Upper lip protrusion 66 3 plusmn 1mmInterlabial gap 0 1ndash5mm
Lower lip-chin length 661 F 43ndash50M 45ndash54
Mandibular sulcus 1737 110ndash134∘
L lip protrusion 78 2 plusmn 1mmB-SnPg line 49 4 plusmn 1mmLower face-throat angle 185 96ndash110∘
Throat length 71 51ndash63mm
to anesthetic and neuromuscular paralyzing agents in amyotonic patient can cause organ failure myocardial infarc-tion and respiratory failure Therefore given the complexityof the case and the disease a polispecialistic collaborationcomprisingmaxillofacial surgeons anesthetists-resuscitatorsneurologists pulmonologists and cardiologists is requiredin the management of postoperative moment in order toprevent pulmonary and cardiac complications In order toperform a correct postoperative monitoring the patientdescribed in the case report spent the first night after surgeryin a postoperative intensive care unit The danger of com-plications related to general anesthesia makes this precau-tion strongly advisable in all patients who have to undergosurgery with general anesthesia
Surgical movements of the skeletal bases must be pro-grammed in order to provide the greatest postoperativesurgical stability in the long term because there is a high riskof relapse because of the progressiveweakness of the temporalmuscle The weakness of the masticatory muscles can leadto recurrence of the anterior open bite closed by surgery[26 27] In our experience in favor of combined orthodontic-surgical therapy there is the improvement of oral functionsand the patientrsquos appearance However the high probability
8 Case Reports in Dentistry
Table 4 Postsurgery cephalometric analysis
Case NormFacial angle 80 883Convexity angle 103 5SNA 813 802SNB 759 77ANB 54 32Mandibular angle 493 224119910-axis 747 573Occlusal angle 214 9Interincisal angle 142 129Li-occlusal plane 108 184Li-mandibular plane minus171 48UiSN 918 105UiA-Pg 62 5LiA-Pg 36 13MLSNL 534 NLSNL 128 Rotational type P1NOB Growth category 2
of recurrence complications related to general anesthesia andthe difficulties of recovery after the intervention and the needof very long orthodontic therapy which favor the accumu-lation of bacterial plaque and increase the risk of caries[28] must be kept in mind In the case report described infact throughout the orthodontic treatment the condition oforal health deteriorated because of the buildup of plaqueresulting in gingivitis and multiple demineralization at thecollar level of the teeth Naturally to undertake this typeof very long and complex therapy patient cooperation isnecessary but we know that during long therapy it alwaysdecreases [29] These considerations lead us to assess thesituation of each patient individually It would be better towork on the prevention and early treatment of malocclusionof cooperative patients with muscular dystrophy in orderto counteract the effects of facial hypotonic muscles onocclusion and the appearance of the patient from the time ofinitial diagnosis of the disease [28] Even cases successfullytreated in developmental age over time may recur or worsenthe functional deficit therefore patients should be orthodon-tically monitored periodically
3 Conclusion
Myotonic dystrophy is a very complex multisystemic diseasein which the muscular involvement leads to a characteristicfacial phenotype The weakness of the facial muscles is thecause of malocclusions needing in adulthood orthodontic-surgical treatment The purpose of this study was to describethe case of a patient treated with surgery and orthodonticswhose satisfaction at the conclusion of therapy demonstratedthe importance of a malocclusionrsquos therapy to provide an
aesthetic improvement to the patient and a resolution of rela-tional and functional problemsThis work could be an incen-tive to evaluate patients with myotonic dystrophy in an earlyage to try to counter the worsening of malocclusions andavoiding surgery if possibleThepropose of an early interven-tion may seem like a challenge but it might be useful toidentify new care pathways that make better the daily livesof these young patients and facilitate their psychosomaticdevelopment during the delicate period of adolescence
Abbreviations
DM1 Myotonic dystrophy type 1DM2 Myotonic dystrophy type 2PROMM Proximal Myotonic MyopathyDMPK Myotonin protein kinaseDM3 Myotonic dystrophy type 3ZNF9 Zinc Finger Protein Gene 9OVB OverbiteOVJ OverjetRx Radiography
Conflicts of Interest
The authors declare that they have no conflicts of interest
References
[1] T Suominen L L Bachinski S Auvinen et al ldquoPopulation fre-quency of myotonic dystrophy higher than expected frequencyof myotonic dystrophy type 2 (DM2) mutation in FinlandrdquoEuropean Journal of Human Genetics vol 19 no 7 pp 776ndash7822011
[2] P S Haper Myotonic DystrophymdashThe Clinical Picture WBSaunders London UK 2001
[3] J D Brook M E McCurrach H G Harley et al ldquoMolecularbasis of myotonic dystrophy Expansion of a trinucleotide(CTG) repeat at the 31015840 end of a transcript encoding a proteinkinase family memberrdquo Cell vol 68 no 4 pp 799ndash808 1992
[4] J Buxton P Shelbourne J Davies et al ldquoDetection of anunstable fragment ofDNA specific to individuals withmyotonicdystrophyrdquo Nature vol 355 no 6360 pp 547-548 1992
[5] Y-H Fu A Pizzuti R G Fenwick Jr et al ldquoAn unstabletriplet repeat in a gene related tomyotonicmuscular dystrophyrdquoScience vol 255 no 5049 pp 1256ndash1258 1992
[6] H G Harley J D Brook S A Rundle et al ldquoExpansion ofan unstable DNA region and phenotypic variation in myotonicdystrophyrdquo Nature vol 355 no 6360 pp 545-546 1992
[7] M Mahadevan C Tsilfidis L Sabourin et al ldquoMyotonic dys-trophy mutation an unstable CTG repeat in the 31015840 untranslatedregion of the generdquo Science vol 255 no 5049 pp 1253ndash12551992
[8] J Mathieu P Allard L Potvin C Prevost and P Begin ldquoA 10-year study of mortality in a cohort of patients with myotonicdystrophyrdquo Neurology vol 52 no 8 pp 1658ndash1662 1999
[9] G Meola ldquoClinical aspects molecular pathomechanisms andmanagement of myotonic dystrophiesrdquo Acta Myologica vol 32no 3 pp 154ndash165 2013
[10] S KiliaridisM Engvall andMG Tzakis ldquoUltrasound imagingof themassetermuscle inmyotonic dystrophy patientsrdquo Journalof Oral Rehabilitation vol 22 no 8 pp 619ndash625 1995
Case Reports in Dentistry 9
[11] C Odman and S Kiliaridis ldquoMasticatory muscle activity inmyotonic dystrophy patientsrdquo Journal of Oral Rehabilitationvol 23 no 1 pp 5ndash10 1996
[12] R N Staley S E Bishara J W Hanson and A J Nowak ldquoCra-niofacial development in myotonic dystrophyrdquoTheCleft Palate-Craniofacial Journal vol 29 no 5 pp 456ndash462 1992
[13] M Portelli G Matarese A Militi R Nucera G Triolo and GCordasco ldquoMyotonic dystrophy and craniofacial morphologyclinical and instrumental studyrdquo European Journal of PaediatricDentistry vol 10 no 1 pp 19ndash22 2009
[14] E Gazit N Bornstein M Lieberman V Serfaty M Gross andA D Korczyn ldquoThe stomatognathic system in myotonic dys-trophyrdquo European Journal of Orthodontics vol 9 no 1 pp 160ndash164 1987
[15] S Kiliaridis C Mejersjo and B Thilander ldquoMuscle functionand craniofacial morphology a clinical study in patients withmyotonic dystrophyrdquo European Journal of Orthodontics vol 11no 2 pp 131ndash138 1989
[16] J Mercier F Bennani J Ferri and B Piot ldquoMaxillofacial mani-festations of Steinertrsquos myotonic dystrophy clinical and thera-peutic aspectsrdquo Revue de Stomatologie et de Chirurgie Maxillo-Faciale vol 96 pp 74ndash82 1995
[17] G Meola ldquoClinical aspects molecular pathomechanisms andmanagement of myotonic dystrophiesrdquo Acta Myologica vol 32no 3 pp 154ndash165 2013
[18] L Sjogreen M Engvall A-B Ekstrom A Lohmander S Kil-iaridis andM Tulinius ldquoOrofacial dysfunction in children andadolescents with myotonic dystrophyrdquoDevelopmental Medicineand Child Neurology vol 49 no 1 pp 18ndash22 2007
[19] B Ercolin F C Sassi L D Mangilli L I Z Mendonca S CO Limongi and C R F De Andrade ldquoOral motor movementsand swallowing in patients with myotonic dystrophy type 1rdquoDysphagia vol 28 no 3 pp 446ndash454 2013
[20] L Dimberg K Arnrup and L Bondemark ldquoThe impact ofmal-occlusion on the quality of life among children and adolescentsa systematic review of quantitative studiesrdquo European Journal ofOrthodontics vol 37 no 3 pp 238ndash247 2015
[21] M Engvall L Sjogreen H Kjellberg A Robertson S Sundelland S Kiliaridis ldquoOral health in children and adolescents withmyotonic dystrophyrdquo European Journal of Oral Sciences vol 115no 3 pp 192ndash197 2007
[22] G W Arnett and R T Bergman ldquoFacial keys to orthodonticdiagnosis and treatment planning Part Irdquo American Journal ofOrthodontics and Dentofacial Orthopedics vol 103 no 4 pp299ndash312 1993
[23] G W Arnett and R T Bergman ldquoFacial keys to orthodonticdiagnosis and treatment planning Part IIrdquo American Journalof Orthodontics and Dentofacial Orthopedics vol 103 no 5 pp395ndash411 1993
[24] S Manzon and R Philbert ldquoOrthognathic Surgery in a PatientWith Myotonic Dystrophy Review of Literature and Report ofa Caserdquo Journal of Oral andMaxillofacial Surgery vol 65 no 12pp 2575ndash2579 2007
[25] S Kiliaridis andC Katsaros ldquoThe effects ofmyotonic dystrophyand Duchenne muscular dystrophy on the orofacial musclesand dentofacial morphologyrdquo Acta Odontologica Scandinavicavol 56 no 6 pp 369ndash374 1998
[26] J Kaufman J M Friedman D Sadowsky and J Harris ldquoMyo-tonic dystrophy surgical and anesthetic considerations duringorthognathic surgeryrdquo Journal ofOral andMaxillofacial Surgeryvol 41 no 10 pp 667ndash671 1983
[27] E Zanoteli H K Yamashita H Suzuki A S B Oliveira and AA Gabbai ldquoTemporomandibular joint and masticatory muscleinvolvement in myotonic dystrophy a study by magnetic res-onance imagingrdquo Oral Surgery Oral Medicine Oral PathologyOral Radiology and Endodontics vol 94 no 2 pp 262ndash2712002
[28] Ministero della Salute ldquoLinee guida nazionali per la promo-zione della salute orale e la prevenzione delle patologie oraliin eta evolutivardquo 2013 httpwwwsalutegovitimgsC_17_pub-blicazioni_2073_allegatopdf
[29] K J Skidmore K J Brook W MThomson andW J HardingldquoFactors influencing treatment time in orthodontic patientsrdquoAmerican Journal of Orthodontics and Dentofacial Orthopedicsvol 129 no 2 pp 230ndash238 2006
Submit your manuscripts athttpswwwhindawicom
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral OncologyJournal of
DentistryInternational Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
International Journal of
Biomaterials
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
BioMed Research International
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Case Reports in Dentistry
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral ImplantsJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Anesthesiology Research and Practice
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Radiology Research and Practice
Environmental and Public Health
Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
The Scientific World JournalHindawi Publishing Corporation httpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Dental SurgeryJournal of
Drug DeliveryJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral DiseasesJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Computational and Mathematical Methods in Medicine
ScientificaHindawi Publishing Corporationhttpwwwhindawicom Volume 2014
PainResearch and TreatmentHindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Preventive MedicineAdvances in
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
EndocrinologyInternational Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
OrthopedicsAdvances in
Case Reports in Dentistry 5
Figure 6 Extraoral and intraoral photographs and Arnett-Bergman soft tissues analysis after surgery
The recovery after the operation was difficult because ofan extreme muscle weakness after surgery and this is thereason why the patient avoids the orthodontic checks for twomonths After surgery orthodontic treatment completed thealignment and coordination of the dental arches Intermaxil-lary elastics acted to optimize vertical occlusal relationshipsIn this phase the rapid maxillary expander has been keptin place to prevent the relapse of the maxillary constrictionBecause of an initial relapse of the open bite the expanderwasremoved and the patient began speech therapy to improvethe posture of the tongue Six months later there was arecurrence of the palatal constriction One year after surgery
Bollard screws were positioned to attach vertical elastics tofixed retainers in order to counter the open bite Two yearsafter surgery the fixed orthodontic appliance was removedand upper and lower Schwarz appliances of restraint weredelivered to the patient to keep the result in combinationwithfixed upper and lower retainers (Figure 8) The patient wassubjected to regular checks until after seven months afterthe removal of orthodontic appliances when it was decidedto hand over to the patient two thermoformed appliances(upper and lower) with box shaped elastics positioned onfrontal teeth because of the partial relapse of open bitedue to facial muscle deficits linked to the primary disease
6 Case Reports in Dentistry
Figure 7 Rx after surgery
Table 1 Pretreatment cephalometric analysis
Case NormFacial angle 782 883Convexity angle 171 5SNA 792 802SNB 717 77ANB 75 32Mandibular angle 576 224119910-axis 789 573Occlusal angle 376 9Interincisal angle 1214 129Li-occlusal plane 8 184Li-mandibular plane minus12 48UiSN 946 105UiA-Pg 87 5LiA-Pg 82 13MLSNL 631 NLSNL 12 Rotational type P1NOB Growth category 2
Today the patient periodically comes to visit tominimize therecurrence of the open bite and tomotivate the cooperation inwearing physical containment and to maintain oral hygiene
23 Discussion Myotonic dystrophy is a very complexmolecular pathology with multisystemic involvement [2ndash9]People with myotonic dystrophy type 1 frequently have acharacteristic facial appearance such as that observed in thepatient described in this paper [12 13 24]
Kiliaridis et al [15] and Mercier et al [16] claim thatcraniofacialmalformations in patientswithmyotonic atrophyare associated with malocclusions in patients who have aprecocious involvement of themusclesThe patient describedin the case shows a constriction of the upper jaw narrowface increasedmandibular angle and a vertical facial growthwhich are perhaps the result of increased neuromuscularfunction as reported by Staley et al [12]The patient presentsanterior open bite as frequently found in the pathology as
Table 2 Arnett-Bergman soft tissues analysis before surgery
Case NormFacial angle 1493 165ndash173∘
Nasal projection 126 13ndash18mmNasolabial angle 988 94ndash110∘
Lower face height 99 57ndash74mmLower face 562 53ndash56
Upper lip length 149 F 18ndash22M 22ndash25mm
Maxillary sulcus 1268 127ndash147∘
Upper lip protrusion 84 3 plusmn 1mmInterlabial gap 363 1ndash5mm
Lower lip-chin length 484 F 43ndash50M 45ndash54
Mandibular sulcus 132 110ndash134∘
L lip protrusion 179 2 plusmn 1mmB-SnPg line 71 4 plusmn 1mmLower face-throat angle 134 96ndash110∘
Throat length 854 51ndash63mm
reported by Portelli et al [13] because of the prevalence ofthe force of gravity on the elevator muscles Kiliaridis andKatsaros [25] say that the increasing of the vertical growthis due to an alteration of the perioral muscles in combinationwith a minor involvement of suprahyoid muscles So a newsituation is established around the teeth which alter thetransversal relationships The tongue in a lower position isnot able to counterbalance the forces developed during thelowering of the lower jaw caused by the elongated facialmusculature This situation as Kiliaridis and Katsaros [25]say can also affect the vertical size decreasing thewidth of thepalate and causing a posterior crossbiteThe lowered positionof the mandible in combination with the reduction of theocclusal forces can allow a supereruption of the posteriorteeth with increased height of the palatal vault and thedevelopment of an anterior open bite
With regard to the surgical therapy as Manzon andPhilbert [24] say although the induction of general anesthesiais extremely safe in a healthy population severe reaction
Case Reports in Dentistry 7
Figure 8 Extraoral and intraoral photographs after orthodontic therapy
Table 3 Arnett-Bergman soft tissues analysis after surgery
Case NormFacial angle 1503 165ndash173∘
Nasal projection 18 13ndash18mmNasolabial angle 1309 94ndash110∘
Lower face height 90 57ndash74mmLower face 493 53ndash56
Upper lip length 209 F 18ndash22M 22ndash25mm
Maxillary sulcus 1687 127ndash147∘
Upper lip protrusion 66 3 plusmn 1mmInterlabial gap 0 1ndash5mm
Lower lip-chin length 661 F 43ndash50M 45ndash54
Mandibular sulcus 1737 110ndash134∘
L lip protrusion 78 2 plusmn 1mmB-SnPg line 49 4 plusmn 1mmLower face-throat angle 185 96ndash110∘
Throat length 71 51ndash63mm
to anesthetic and neuromuscular paralyzing agents in amyotonic patient can cause organ failure myocardial infarc-tion and respiratory failure Therefore given the complexityof the case and the disease a polispecialistic collaborationcomprisingmaxillofacial surgeons anesthetists-resuscitatorsneurologists pulmonologists and cardiologists is requiredin the management of postoperative moment in order toprevent pulmonary and cardiac complications In order toperform a correct postoperative monitoring the patientdescribed in the case report spent the first night after surgeryin a postoperative intensive care unit The danger of com-plications related to general anesthesia makes this precau-tion strongly advisable in all patients who have to undergosurgery with general anesthesia
Surgical movements of the skeletal bases must be pro-grammed in order to provide the greatest postoperativesurgical stability in the long term because there is a high riskof relapse because of the progressiveweakness of the temporalmuscle The weakness of the masticatory muscles can leadto recurrence of the anterior open bite closed by surgery[26 27] In our experience in favor of combined orthodontic-surgical therapy there is the improvement of oral functionsand the patientrsquos appearance However the high probability
8 Case Reports in Dentistry
Table 4 Postsurgery cephalometric analysis
Case NormFacial angle 80 883Convexity angle 103 5SNA 813 802SNB 759 77ANB 54 32Mandibular angle 493 224119910-axis 747 573Occlusal angle 214 9Interincisal angle 142 129Li-occlusal plane 108 184Li-mandibular plane minus171 48UiSN 918 105UiA-Pg 62 5LiA-Pg 36 13MLSNL 534 NLSNL 128 Rotational type P1NOB Growth category 2
of recurrence complications related to general anesthesia andthe difficulties of recovery after the intervention and the needof very long orthodontic therapy which favor the accumu-lation of bacterial plaque and increase the risk of caries[28] must be kept in mind In the case report described infact throughout the orthodontic treatment the condition oforal health deteriorated because of the buildup of plaqueresulting in gingivitis and multiple demineralization at thecollar level of the teeth Naturally to undertake this typeof very long and complex therapy patient cooperation isnecessary but we know that during long therapy it alwaysdecreases [29] These considerations lead us to assess thesituation of each patient individually It would be better towork on the prevention and early treatment of malocclusionof cooperative patients with muscular dystrophy in orderto counteract the effects of facial hypotonic muscles onocclusion and the appearance of the patient from the time ofinitial diagnosis of the disease [28] Even cases successfullytreated in developmental age over time may recur or worsenthe functional deficit therefore patients should be orthodon-tically monitored periodically
3 Conclusion
Myotonic dystrophy is a very complex multisystemic diseasein which the muscular involvement leads to a characteristicfacial phenotype The weakness of the facial muscles is thecause of malocclusions needing in adulthood orthodontic-surgical treatment The purpose of this study was to describethe case of a patient treated with surgery and orthodonticswhose satisfaction at the conclusion of therapy demonstratedthe importance of a malocclusionrsquos therapy to provide an
aesthetic improvement to the patient and a resolution of rela-tional and functional problemsThis work could be an incen-tive to evaluate patients with myotonic dystrophy in an earlyage to try to counter the worsening of malocclusions andavoiding surgery if possibleThepropose of an early interven-tion may seem like a challenge but it might be useful toidentify new care pathways that make better the daily livesof these young patients and facilitate their psychosomaticdevelopment during the delicate period of adolescence
Abbreviations
DM1 Myotonic dystrophy type 1DM2 Myotonic dystrophy type 2PROMM Proximal Myotonic MyopathyDMPK Myotonin protein kinaseDM3 Myotonic dystrophy type 3ZNF9 Zinc Finger Protein Gene 9OVB OverbiteOVJ OverjetRx Radiography
Conflicts of Interest
The authors declare that they have no conflicts of interest
References
[1] T Suominen L L Bachinski S Auvinen et al ldquoPopulation fre-quency of myotonic dystrophy higher than expected frequencyof myotonic dystrophy type 2 (DM2) mutation in FinlandrdquoEuropean Journal of Human Genetics vol 19 no 7 pp 776ndash7822011
[2] P S Haper Myotonic DystrophymdashThe Clinical Picture WBSaunders London UK 2001
[3] J D Brook M E McCurrach H G Harley et al ldquoMolecularbasis of myotonic dystrophy Expansion of a trinucleotide(CTG) repeat at the 31015840 end of a transcript encoding a proteinkinase family memberrdquo Cell vol 68 no 4 pp 799ndash808 1992
[4] J Buxton P Shelbourne J Davies et al ldquoDetection of anunstable fragment ofDNA specific to individuals withmyotonicdystrophyrdquo Nature vol 355 no 6360 pp 547-548 1992
[5] Y-H Fu A Pizzuti R G Fenwick Jr et al ldquoAn unstabletriplet repeat in a gene related tomyotonicmuscular dystrophyrdquoScience vol 255 no 5049 pp 1256ndash1258 1992
[6] H G Harley J D Brook S A Rundle et al ldquoExpansion ofan unstable DNA region and phenotypic variation in myotonicdystrophyrdquo Nature vol 355 no 6360 pp 545-546 1992
[7] M Mahadevan C Tsilfidis L Sabourin et al ldquoMyotonic dys-trophy mutation an unstable CTG repeat in the 31015840 untranslatedregion of the generdquo Science vol 255 no 5049 pp 1253ndash12551992
[8] J Mathieu P Allard L Potvin C Prevost and P Begin ldquoA 10-year study of mortality in a cohort of patients with myotonicdystrophyrdquo Neurology vol 52 no 8 pp 1658ndash1662 1999
[9] G Meola ldquoClinical aspects molecular pathomechanisms andmanagement of myotonic dystrophiesrdquo Acta Myologica vol 32no 3 pp 154ndash165 2013
[10] S KiliaridisM Engvall andMG Tzakis ldquoUltrasound imagingof themassetermuscle inmyotonic dystrophy patientsrdquo Journalof Oral Rehabilitation vol 22 no 8 pp 619ndash625 1995
Case Reports in Dentistry 9
[11] C Odman and S Kiliaridis ldquoMasticatory muscle activity inmyotonic dystrophy patientsrdquo Journal of Oral Rehabilitationvol 23 no 1 pp 5ndash10 1996
[12] R N Staley S E Bishara J W Hanson and A J Nowak ldquoCra-niofacial development in myotonic dystrophyrdquoTheCleft Palate-Craniofacial Journal vol 29 no 5 pp 456ndash462 1992
[13] M Portelli G Matarese A Militi R Nucera G Triolo and GCordasco ldquoMyotonic dystrophy and craniofacial morphologyclinical and instrumental studyrdquo European Journal of PaediatricDentistry vol 10 no 1 pp 19ndash22 2009
[14] E Gazit N Bornstein M Lieberman V Serfaty M Gross andA D Korczyn ldquoThe stomatognathic system in myotonic dys-trophyrdquo European Journal of Orthodontics vol 9 no 1 pp 160ndash164 1987
[15] S Kiliaridis C Mejersjo and B Thilander ldquoMuscle functionand craniofacial morphology a clinical study in patients withmyotonic dystrophyrdquo European Journal of Orthodontics vol 11no 2 pp 131ndash138 1989
[16] J Mercier F Bennani J Ferri and B Piot ldquoMaxillofacial mani-festations of Steinertrsquos myotonic dystrophy clinical and thera-peutic aspectsrdquo Revue de Stomatologie et de Chirurgie Maxillo-Faciale vol 96 pp 74ndash82 1995
[17] G Meola ldquoClinical aspects molecular pathomechanisms andmanagement of myotonic dystrophiesrdquo Acta Myologica vol 32no 3 pp 154ndash165 2013
[18] L Sjogreen M Engvall A-B Ekstrom A Lohmander S Kil-iaridis andM Tulinius ldquoOrofacial dysfunction in children andadolescents with myotonic dystrophyrdquoDevelopmental Medicineand Child Neurology vol 49 no 1 pp 18ndash22 2007
[19] B Ercolin F C Sassi L D Mangilli L I Z Mendonca S CO Limongi and C R F De Andrade ldquoOral motor movementsand swallowing in patients with myotonic dystrophy type 1rdquoDysphagia vol 28 no 3 pp 446ndash454 2013
[20] L Dimberg K Arnrup and L Bondemark ldquoThe impact ofmal-occlusion on the quality of life among children and adolescentsa systematic review of quantitative studiesrdquo European Journal ofOrthodontics vol 37 no 3 pp 238ndash247 2015
[21] M Engvall L Sjogreen H Kjellberg A Robertson S Sundelland S Kiliaridis ldquoOral health in children and adolescents withmyotonic dystrophyrdquo European Journal of Oral Sciences vol 115no 3 pp 192ndash197 2007
[22] G W Arnett and R T Bergman ldquoFacial keys to orthodonticdiagnosis and treatment planning Part Irdquo American Journal ofOrthodontics and Dentofacial Orthopedics vol 103 no 4 pp299ndash312 1993
[23] G W Arnett and R T Bergman ldquoFacial keys to orthodonticdiagnosis and treatment planning Part IIrdquo American Journalof Orthodontics and Dentofacial Orthopedics vol 103 no 5 pp395ndash411 1993
[24] S Manzon and R Philbert ldquoOrthognathic Surgery in a PatientWith Myotonic Dystrophy Review of Literature and Report ofa Caserdquo Journal of Oral andMaxillofacial Surgery vol 65 no 12pp 2575ndash2579 2007
[25] S Kiliaridis andC Katsaros ldquoThe effects ofmyotonic dystrophyand Duchenne muscular dystrophy on the orofacial musclesand dentofacial morphologyrdquo Acta Odontologica Scandinavicavol 56 no 6 pp 369ndash374 1998
[26] J Kaufman J M Friedman D Sadowsky and J Harris ldquoMyo-tonic dystrophy surgical and anesthetic considerations duringorthognathic surgeryrdquo Journal ofOral andMaxillofacial Surgeryvol 41 no 10 pp 667ndash671 1983
[27] E Zanoteli H K Yamashita H Suzuki A S B Oliveira and AA Gabbai ldquoTemporomandibular joint and masticatory muscleinvolvement in myotonic dystrophy a study by magnetic res-onance imagingrdquo Oral Surgery Oral Medicine Oral PathologyOral Radiology and Endodontics vol 94 no 2 pp 262ndash2712002
[28] Ministero della Salute ldquoLinee guida nazionali per la promo-zione della salute orale e la prevenzione delle patologie oraliin eta evolutivardquo 2013 httpwwwsalutegovitimgsC_17_pub-blicazioni_2073_allegatopdf
[29] K J Skidmore K J Brook W MThomson andW J HardingldquoFactors influencing treatment time in orthodontic patientsrdquoAmerican Journal of Orthodontics and Dentofacial Orthopedicsvol 129 no 2 pp 230ndash238 2006
Submit your manuscripts athttpswwwhindawicom
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral OncologyJournal of
DentistryInternational Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
International Journal of
Biomaterials
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
BioMed Research International
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Case Reports in Dentistry
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral ImplantsJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Anesthesiology Research and Practice
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Radiology Research and Practice
Environmental and Public Health
Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
The Scientific World JournalHindawi Publishing Corporation httpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Dental SurgeryJournal of
Drug DeliveryJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral DiseasesJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Computational and Mathematical Methods in Medicine
ScientificaHindawi Publishing Corporationhttpwwwhindawicom Volume 2014
PainResearch and TreatmentHindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Preventive MedicineAdvances in
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
EndocrinologyInternational Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
OrthopedicsAdvances in
6 Case Reports in Dentistry
Figure 7 Rx after surgery
Table 1 Pretreatment cephalometric analysis
Case NormFacial angle 782 883Convexity angle 171 5SNA 792 802SNB 717 77ANB 75 32Mandibular angle 576 224119910-axis 789 573Occlusal angle 376 9Interincisal angle 1214 129Li-occlusal plane 8 184Li-mandibular plane minus12 48UiSN 946 105UiA-Pg 87 5LiA-Pg 82 13MLSNL 631 NLSNL 12 Rotational type P1NOB Growth category 2
Today the patient periodically comes to visit tominimize therecurrence of the open bite and tomotivate the cooperation inwearing physical containment and to maintain oral hygiene
23 Discussion Myotonic dystrophy is a very complexmolecular pathology with multisystemic involvement [2ndash9]People with myotonic dystrophy type 1 frequently have acharacteristic facial appearance such as that observed in thepatient described in this paper [12 13 24]
Kiliaridis et al [15] and Mercier et al [16] claim thatcraniofacialmalformations in patientswithmyotonic atrophyare associated with malocclusions in patients who have aprecocious involvement of themusclesThe patient describedin the case shows a constriction of the upper jaw narrowface increasedmandibular angle and a vertical facial growthwhich are perhaps the result of increased neuromuscularfunction as reported by Staley et al [12]The patient presentsanterior open bite as frequently found in the pathology as
Table 2 Arnett-Bergman soft tissues analysis before surgery
Case NormFacial angle 1493 165ndash173∘
Nasal projection 126 13ndash18mmNasolabial angle 988 94ndash110∘
Lower face height 99 57ndash74mmLower face 562 53ndash56
Upper lip length 149 F 18ndash22M 22ndash25mm
Maxillary sulcus 1268 127ndash147∘
Upper lip protrusion 84 3 plusmn 1mmInterlabial gap 363 1ndash5mm
Lower lip-chin length 484 F 43ndash50M 45ndash54
Mandibular sulcus 132 110ndash134∘
L lip protrusion 179 2 plusmn 1mmB-SnPg line 71 4 plusmn 1mmLower face-throat angle 134 96ndash110∘
Throat length 854 51ndash63mm
reported by Portelli et al [13] because of the prevalence ofthe force of gravity on the elevator muscles Kiliaridis andKatsaros [25] say that the increasing of the vertical growthis due to an alteration of the perioral muscles in combinationwith a minor involvement of suprahyoid muscles So a newsituation is established around the teeth which alter thetransversal relationships The tongue in a lower position isnot able to counterbalance the forces developed during thelowering of the lower jaw caused by the elongated facialmusculature This situation as Kiliaridis and Katsaros [25]say can also affect the vertical size decreasing thewidth of thepalate and causing a posterior crossbiteThe lowered positionof the mandible in combination with the reduction of theocclusal forces can allow a supereruption of the posteriorteeth with increased height of the palatal vault and thedevelopment of an anterior open bite
With regard to the surgical therapy as Manzon andPhilbert [24] say although the induction of general anesthesiais extremely safe in a healthy population severe reaction
Case Reports in Dentistry 7
Figure 8 Extraoral and intraoral photographs after orthodontic therapy
Table 3 Arnett-Bergman soft tissues analysis after surgery
Case NormFacial angle 1503 165ndash173∘
Nasal projection 18 13ndash18mmNasolabial angle 1309 94ndash110∘
Lower face height 90 57ndash74mmLower face 493 53ndash56
Upper lip length 209 F 18ndash22M 22ndash25mm
Maxillary sulcus 1687 127ndash147∘
Upper lip protrusion 66 3 plusmn 1mmInterlabial gap 0 1ndash5mm
Lower lip-chin length 661 F 43ndash50M 45ndash54
Mandibular sulcus 1737 110ndash134∘
L lip protrusion 78 2 plusmn 1mmB-SnPg line 49 4 plusmn 1mmLower face-throat angle 185 96ndash110∘
Throat length 71 51ndash63mm
to anesthetic and neuromuscular paralyzing agents in amyotonic patient can cause organ failure myocardial infarc-tion and respiratory failure Therefore given the complexityof the case and the disease a polispecialistic collaborationcomprisingmaxillofacial surgeons anesthetists-resuscitatorsneurologists pulmonologists and cardiologists is requiredin the management of postoperative moment in order toprevent pulmonary and cardiac complications In order toperform a correct postoperative monitoring the patientdescribed in the case report spent the first night after surgeryin a postoperative intensive care unit The danger of com-plications related to general anesthesia makes this precau-tion strongly advisable in all patients who have to undergosurgery with general anesthesia
Surgical movements of the skeletal bases must be pro-grammed in order to provide the greatest postoperativesurgical stability in the long term because there is a high riskof relapse because of the progressiveweakness of the temporalmuscle The weakness of the masticatory muscles can leadto recurrence of the anterior open bite closed by surgery[26 27] In our experience in favor of combined orthodontic-surgical therapy there is the improvement of oral functionsand the patientrsquos appearance However the high probability
8 Case Reports in Dentistry
Table 4 Postsurgery cephalometric analysis
Case NormFacial angle 80 883Convexity angle 103 5SNA 813 802SNB 759 77ANB 54 32Mandibular angle 493 224119910-axis 747 573Occlusal angle 214 9Interincisal angle 142 129Li-occlusal plane 108 184Li-mandibular plane minus171 48UiSN 918 105UiA-Pg 62 5LiA-Pg 36 13MLSNL 534 NLSNL 128 Rotational type P1NOB Growth category 2
of recurrence complications related to general anesthesia andthe difficulties of recovery after the intervention and the needof very long orthodontic therapy which favor the accumu-lation of bacterial plaque and increase the risk of caries[28] must be kept in mind In the case report described infact throughout the orthodontic treatment the condition oforal health deteriorated because of the buildup of plaqueresulting in gingivitis and multiple demineralization at thecollar level of the teeth Naturally to undertake this typeof very long and complex therapy patient cooperation isnecessary but we know that during long therapy it alwaysdecreases [29] These considerations lead us to assess thesituation of each patient individually It would be better towork on the prevention and early treatment of malocclusionof cooperative patients with muscular dystrophy in orderto counteract the effects of facial hypotonic muscles onocclusion and the appearance of the patient from the time ofinitial diagnosis of the disease [28] Even cases successfullytreated in developmental age over time may recur or worsenthe functional deficit therefore patients should be orthodon-tically monitored periodically
3 Conclusion
Myotonic dystrophy is a very complex multisystemic diseasein which the muscular involvement leads to a characteristicfacial phenotype The weakness of the facial muscles is thecause of malocclusions needing in adulthood orthodontic-surgical treatment The purpose of this study was to describethe case of a patient treated with surgery and orthodonticswhose satisfaction at the conclusion of therapy demonstratedthe importance of a malocclusionrsquos therapy to provide an
aesthetic improvement to the patient and a resolution of rela-tional and functional problemsThis work could be an incen-tive to evaluate patients with myotonic dystrophy in an earlyage to try to counter the worsening of malocclusions andavoiding surgery if possibleThepropose of an early interven-tion may seem like a challenge but it might be useful toidentify new care pathways that make better the daily livesof these young patients and facilitate their psychosomaticdevelopment during the delicate period of adolescence
Abbreviations
DM1 Myotonic dystrophy type 1DM2 Myotonic dystrophy type 2PROMM Proximal Myotonic MyopathyDMPK Myotonin protein kinaseDM3 Myotonic dystrophy type 3ZNF9 Zinc Finger Protein Gene 9OVB OverbiteOVJ OverjetRx Radiography
Conflicts of Interest
The authors declare that they have no conflicts of interest
References
[1] T Suominen L L Bachinski S Auvinen et al ldquoPopulation fre-quency of myotonic dystrophy higher than expected frequencyof myotonic dystrophy type 2 (DM2) mutation in FinlandrdquoEuropean Journal of Human Genetics vol 19 no 7 pp 776ndash7822011
[2] P S Haper Myotonic DystrophymdashThe Clinical Picture WBSaunders London UK 2001
[3] J D Brook M E McCurrach H G Harley et al ldquoMolecularbasis of myotonic dystrophy Expansion of a trinucleotide(CTG) repeat at the 31015840 end of a transcript encoding a proteinkinase family memberrdquo Cell vol 68 no 4 pp 799ndash808 1992
[4] J Buxton P Shelbourne J Davies et al ldquoDetection of anunstable fragment ofDNA specific to individuals withmyotonicdystrophyrdquo Nature vol 355 no 6360 pp 547-548 1992
[5] Y-H Fu A Pizzuti R G Fenwick Jr et al ldquoAn unstabletriplet repeat in a gene related tomyotonicmuscular dystrophyrdquoScience vol 255 no 5049 pp 1256ndash1258 1992
[6] H G Harley J D Brook S A Rundle et al ldquoExpansion ofan unstable DNA region and phenotypic variation in myotonicdystrophyrdquo Nature vol 355 no 6360 pp 545-546 1992
[7] M Mahadevan C Tsilfidis L Sabourin et al ldquoMyotonic dys-trophy mutation an unstable CTG repeat in the 31015840 untranslatedregion of the generdquo Science vol 255 no 5049 pp 1253ndash12551992
[8] J Mathieu P Allard L Potvin C Prevost and P Begin ldquoA 10-year study of mortality in a cohort of patients with myotonicdystrophyrdquo Neurology vol 52 no 8 pp 1658ndash1662 1999
[9] G Meola ldquoClinical aspects molecular pathomechanisms andmanagement of myotonic dystrophiesrdquo Acta Myologica vol 32no 3 pp 154ndash165 2013
[10] S KiliaridisM Engvall andMG Tzakis ldquoUltrasound imagingof themassetermuscle inmyotonic dystrophy patientsrdquo Journalof Oral Rehabilitation vol 22 no 8 pp 619ndash625 1995
Case Reports in Dentistry 9
[11] C Odman and S Kiliaridis ldquoMasticatory muscle activity inmyotonic dystrophy patientsrdquo Journal of Oral Rehabilitationvol 23 no 1 pp 5ndash10 1996
[12] R N Staley S E Bishara J W Hanson and A J Nowak ldquoCra-niofacial development in myotonic dystrophyrdquoTheCleft Palate-Craniofacial Journal vol 29 no 5 pp 456ndash462 1992
[13] M Portelli G Matarese A Militi R Nucera G Triolo and GCordasco ldquoMyotonic dystrophy and craniofacial morphologyclinical and instrumental studyrdquo European Journal of PaediatricDentistry vol 10 no 1 pp 19ndash22 2009
[14] E Gazit N Bornstein M Lieberman V Serfaty M Gross andA D Korczyn ldquoThe stomatognathic system in myotonic dys-trophyrdquo European Journal of Orthodontics vol 9 no 1 pp 160ndash164 1987
[15] S Kiliaridis C Mejersjo and B Thilander ldquoMuscle functionand craniofacial morphology a clinical study in patients withmyotonic dystrophyrdquo European Journal of Orthodontics vol 11no 2 pp 131ndash138 1989
[16] J Mercier F Bennani J Ferri and B Piot ldquoMaxillofacial mani-festations of Steinertrsquos myotonic dystrophy clinical and thera-peutic aspectsrdquo Revue de Stomatologie et de Chirurgie Maxillo-Faciale vol 96 pp 74ndash82 1995
[17] G Meola ldquoClinical aspects molecular pathomechanisms andmanagement of myotonic dystrophiesrdquo Acta Myologica vol 32no 3 pp 154ndash165 2013
[18] L Sjogreen M Engvall A-B Ekstrom A Lohmander S Kil-iaridis andM Tulinius ldquoOrofacial dysfunction in children andadolescents with myotonic dystrophyrdquoDevelopmental Medicineand Child Neurology vol 49 no 1 pp 18ndash22 2007
[19] B Ercolin F C Sassi L D Mangilli L I Z Mendonca S CO Limongi and C R F De Andrade ldquoOral motor movementsand swallowing in patients with myotonic dystrophy type 1rdquoDysphagia vol 28 no 3 pp 446ndash454 2013
[20] L Dimberg K Arnrup and L Bondemark ldquoThe impact ofmal-occlusion on the quality of life among children and adolescentsa systematic review of quantitative studiesrdquo European Journal ofOrthodontics vol 37 no 3 pp 238ndash247 2015
[21] M Engvall L Sjogreen H Kjellberg A Robertson S Sundelland S Kiliaridis ldquoOral health in children and adolescents withmyotonic dystrophyrdquo European Journal of Oral Sciences vol 115no 3 pp 192ndash197 2007
[22] G W Arnett and R T Bergman ldquoFacial keys to orthodonticdiagnosis and treatment planning Part Irdquo American Journal ofOrthodontics and Dentofacial Orthopedics vol 103 no 4 pp299ndash312 1993
[23] G W Arnett and R T Bergman ldquoFacial keys to orthodonticdiagnosis and treatment planning Part IIrdquo American Journalof Orthodontics and Dentofacial Orthopedics vol 103 no 5 pp395ndash411 1993
[24] S Manzon and R Philbert ldquoOrthognathic Surgery in a PatientWith Myotonic Dystrophy Review of Literature and Report ofa Caserdquo Journal of Oral andMaxillofacial Surgery vol 65 no 12pp 2575ndash2579 2007
[25] S Kiliaridis andC Katsaros ldquoThe effects ofmyotonic dystrophyand Duchenne muscular dystrophy on the orofacial musclesand dentofacial morphologyrdquo Acta Odontologica Scandinavicavol 56 no 6 pp 369ndash374 1998
[26] J Kaufman J M Friedman D Sadowsky and J Harris ldquoMyo-tonic dystrophy surgical and anesthetic considerations duringorthognathic surgeryrdquo Journal ofOral andMaxillofacial Surgeryvol 41 no 10 pp 667ndash671 1983
[27] E Zanoteli H K Yamashita H Suzuki A S B Oliveira and AA Gabbai ldquoTemporomandibular joint and masticatory muscleinvolvement in myotonic dystrophy a study by magnetic res-onance imagingrdquo Oral Surgery Oral Medicine Oral PathologyOral Radiology and Endodontics vol 94 no 2 pp 262ndash2712002
[28] Ministero della Salute ldquoLinee guida nazionali per la promo-zione della salute orale e la prevenzione delle patologie oraliin eta evolutivardquo 2013 httpwwwsalutegovitimgsC_17_pub-blicazioni_2073_allegatopdf
[29] K J Skidmore K J Brook W MThomson andW J HardingldquoFactors influencing treatment time in orthodontic patientsrdquoAmerican Journal of Orthodontics and Dentofacial Orthopedicsvol 129 no 2 pp 230ndash238 2006
Submit your manuscripts athttpswwwhindawicom
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral OncologyJournal of
DentistryInternational Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
International Journal of
Biomaterials
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
BioMed Research International
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Case Reports in Dentistry
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral ImplantsJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Anesthesiology Research and Practice
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Radiology Research and Practice
Environmental and Public Health
Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
The Scientific World JournalHindawi Publishing Corporation httpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Dental SurgeryJournal of
Drug DeliveryJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral DiseasesJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Computational and Mathematical Methods in Medicine
ScientificaHindawi Publishing Corporationhttpwwwhindawicom Volume 2014
PainResearch and TreatmentHindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Preventive MedicineAdvances in
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
EndocrinologyInternational Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
OrthopedicsAdvances in
Case Reports in Dentistry 7
Figure 8 Extraoral and intraoral photographs after orthodontic therapy
Table 3 Arnett-Bergman soft tissues analysis after surgery
Case NormFacial angle 1503 165ndash173∘
Nasal projection 18 13ndash18mmNasolabial angle 1309 94ndash110∘
Lower face height 90 57ndash74mmLower face 493 53ndash56
Upper lip length 209 F 18ndash22M 22ndash25mm
Maxillary sulcus 1687 127ndash147∘
Upper lip protrusion 66 3 plusmn 1mmInterlabial gap 0 1ndash5mm
Lower lip-chin length 661 F 43ndash50M 45ndash54
Mandibular sulcus 1737 110ndash134∘
L lip protrusion 78 2 plusmn 1mmB-SnPg line 49 4 plusmn 1mmLower face-throat angle 185 96ndash110∘
Throat length 71 51ndash63mm
to anesthetic and neuromuscular paralyzing agents in amyotonic patient can cause organ failure myocardial infarc-tion and respiratory failure Therefore given the complexityof the case and the disease a polispecialistic collaborationcomprisingmaxillofacial surgeons anesthetists-resuscitatorsneurologists pulmonologists and cardiologists is requiredin the management of postoperative moment in order toprevent pulmonary and cardiac complications In order toperform a correct postoperative monitoring the patientdescribed in the case report spent the first night after surgeryin a postoperative intensive care unit The danger of com-plications related to general anesthesia makes this precau-tion strongly advisable in all patients who have to undergosurgery with general anesthesia
Surgical movements of the skeletal bases must be pro-grammed in order to provide the greatest postoperativesurgical stability in the long term because there is a high riskof relapse because of the progressiveweakness of the temporalmuscle The weakness of the masticatory muscles can leadto recurrence of the anterior open bite closed by surgery[26 27] In our experience in favor of combined orthodontic-surgical therapy there is the improvement of oral functionsand the patientrsquos appearance However the high probability
8 Case Reports in Dentistry
Table 4 Postsurgery cephalometric analysis
Case NormFacial angle 80 883Convexity angle 103 5SNA 813 802SNB 759 77ANB 54 32Mandibular angle 493 224119910-axis 747 573Occlusal angle 214 9Interincisal angle 142 129Li-occlusal plane 108 184Li-mandibular plane minus171 48UiSN 918 105UiA-Pg 62 5LiA-Pg 36 13MLSNL 534 NLSNL 128 Rotational type P1NOB Growth category 2
of recurrence complications related to general anesthesia andthe difficulties of recovery after the intervention and the needof very long orthodontic therapy which favor the accumu-lation of bacterial plaque and increase the risk of caries[28] must be kept in mind In the case report described infact throughout the orthodontic treatment the condition oforal health deteriorated because of the buildup of plaqueresulting in gingivitis and multiple demineralization at thecollar level of the teeth Naturally to undertake this typeof very long and complex therapy patient cooperation isnecessary but we know that during long therapy it alwaysdecreases [29] These considerations lead us to assess thesituation of each patient individually It would be better towork on the prevention and early treatment of malocclusionof cooperative patients with muscular dystrophy in orderto counteract the effects of facial hypotonic muscles onocclusion and the appearance of the patient from the time ofinitial diagnosis of the disease [28] Even cases successfullytreated in developmental age over time may recur or worsenthe functional deficit therefore patients should be orthodon-tically monitored periodically
3 Conclusion
Myotonic dystrophy is a very complex multisystemic diseasein which the muscular involvement leads to a characteristicfacial phenotype The weakness of the facial muscles is thecause of malocclusions needing in adulthood orthodontic-surgical treatment The purpose of this study was to describethe case of a patient treated with surgery and orthodonticswhose satisfaction at the conclusion of therapy demonstratedthe importance of a malocclusionrsquos therapy to provide an
aesthetic improvement to the patient and a resolution of rela-tional and functional problemsThis work could be an incen-tive to evaluate patients with myotonic dystrophy in an earlyage to try to counter the worsening of malocclusions andavoiding surgery if possibleThepropose of an early interven-tion may seem like a challenge but it might be useful toidentify new care pathways that make better the daily livesof these young patients and facilitate their psychosomaticdevelopment during the delicate period of adolescence
Abbreviations
DM1 Myotonic dystrophy type 1DM2 Myotonic dystrophy type 2PROMM Proximal Myotonic MyopathyDMPK Myotonin protein kinaseDM3 Myotonic dystrophy type 3ZNF9 Zinc Finger Protein Gene 9OVB OverbiteOVJ OverjetRx Radiography
Conflicts of Interest
The authors declare that they have no conflicts of interest
References
[1] T Suominen L L Bachinski S Auvinen et al ldquoPopulation fre-quency of myotonic dystrophy higher than expected frequencyof myotonic dystrophy type 2 (DM2) mutation in FinlandrdquoEuropean Journal of Human Genetics vol 19 no 7 pp 776ndash7822011
[2] P S Haper Myotonic DystrophymdashThe Clinical Picture WBSaunders London UK 2001
[3] J D Brook M E McCurrach H G Harley et al ldquoMolecularbasis of myotonic dystrophy Expansion of a trinucleotide(CTG) repeat at the 31015840 end of a transcript encoding a proteinkinase family memberrdquo Cell vol 68 no 4 pp 799ndash808 1992
[4] J Buxton P Shelbourne J Davies et al ldquoDetection of anunstable fragment ofDNA specific to individuals withmyotonicdystrophyrdquo Nature vol 355 no 6360 pp 547-548 1992
[5] Y-H Fu A Pizzuti R G Fenwick Jr et al ldquoAn unstabletriplet repeat in a gene related tomyotonicmuscular dystrophyrdquoScience vol 255 no 5049 pp 1256ndash1258 1992
[6] H G Harley J D Brook S A Rundle et al ldquoExpansion ofan unstable DNA region and phenotypic variation in myotonicdystrophyrdquo Nature vol 355 no 6360 pp 545-546 1992
[7] M Mahadevan C Tsilfidis L Sabourin et al ldquoMyotonic dys-trophy mutation an unstable CTG repeat in the 31015840 untranslatedregion of the generdquo Science vol 255 no 5049 pp 1253ndash12551992
[8] J Mathieu P Allard L Potvin C Prevost and P Begin ldquoA 10-year study of mortality in a cohort of patients with myotonicdystrophyrdquo Neurology vol 52 no 8 pp 1658ndash1662 1999
[9] G Meola ldquoClinical aspects molecular pathomechanisms andmanagement of myotonic dystrophiesrdquo Acta Myologica vol 32no 3 pp 154ndash165 2013
[10] S KiliaridisM Engvall andMG Tzakis ldquoUltrasound imagingof themassetermuscle inmyotonic dystrophy patientsrdquo Journalof Oral Rehabilitation vol 22 no 8 pp 619ndash625 1995
Case Reports in Dentistry 9
[11] C Odman and S Kiliaridis ldquoMasticatory muscle activity inmyotonic dystrophy patientsrdquo Journal of Oral Rehabilitationvol 23 no 1 pp 5ndash10 1996
[12] R N Staley S E Bishara J W Hanson and A J Nowak ldquoCra-niofacial development in myotonic dystrophyrdquoTheCleft Palate-Craniofacial Journal vol 29 no 5 pp 456ndash462 1992
[13] M Portelli G Matarese A Militi R Nucera G Triolo and GCordasco ldquoMyotonic dystrophy and craniofacial morphologyclinical and instrumental studyrdquo European Journal of PaediatricDentistry vol 10 no 1 pp 19ndash22 2009
[14] E Gazit N Bornstein M Lieberman V Serfaty M Gross andA D Korczyn ldquoThe stomatognathic system in myotonic dys-trophyrdquo European Journal of Orthodontics vol 9 no 1 pp 160ndash164 1987
[15] S Kiliaridis C Mejersjo and B Thilander ldquoMuscle functionand craniofacial morphology a clinical study in patients withmyotonic dystrophyrdquo European Journal of Orthodontics vol 11no 2 pp 131ndash138 1989
[16] J Mercier F Bennani J Ferri and B Piot ldquoMaxillofacial mani-festations of Steinertrsquos myotonic dystrophy clinical and thera-peutic aspectsrdquo Revue de Stomatologie et de Chirurgie Maxillo-Faciale vol 96 pp 74ndash82 1995
[17] G Meola ldquoClinical aspects molecular pathomechanisms andmanagement of myotonic dystrophiesrdquo Acta Myologica vol 32no 3 pp 154ndash165 2013
[18] L Sjogreen M Engvall A-B Ekstrom A Lohmander S Kil-iaridis andM Tulinius ldquoOrofacial dysfunction in children andadolescents with myotonic dystrophyrdquoDevelopmental Medicineand Child Neurology vol 49 no 1 pp 18ndash22 2007
[19] B Ercolin F C Sassi L D Mangilli L I Z Mendonca S CO Limongi and C R F De Andrade ldquoOral motor movementsand swallowing in patients with myotonic dystrophy type 1rdquoDysphagia vol 28 no 3 pp 446ndash454 2013
[20] L Dimberg K Arnrup and L Bondemark ldquoThe impact ofmal-occlusion on the quality of life among children and adolescentsa systematic review of quantitative studiesrdquo European Journal ofOrthodontics vol 37 no 3 pp 238ndash247 2015
[21] M Engvall L Sjogreen H Kjellberg A Robertson S Sundelland S Kiliaridis ldquoOral health in children and adolescents withmyotonic dystrophyrdquo European Journal of Oral Sciences vol 115no 3 pp 192ndash197 2007
[22] G W Arnett and R T Bergman ldquoFacial keys to orthodonticdiagnosis and treatment planning Part Irdquo American Journal ofOrthodontics and Dentofacial Orthopedics vol 103 no 4 pp299ndash312 1993
[23] G W Arnett and R T Bergman ldquoFacial keys to orthodonticdiagnosis and treatment planning Part IIrdquo American Journalof Orthodontics and Dentofacial Orthopedics vol 103 no 5 pp395ndash411 1993
[24] S Manzon and R Philbert ldquoOrthognathic Surgery in a PatientWith Myotonic Dystrophy Review of Literature and Report ofa Caserdquo Journal of Oral andMaxillofacial Surgery vol 65 no 12pp 2575ndash2579 2007
[25] S Kiliaridis andC Katsaros ldquoThe effects ofmyotonic dystrophyand Duchenne muscular dystrophy on the orofacial musclesand dentofacial morphologyrdquo Acta Odontologica Scandinavicavol 56 no 6 pp 369ndash374 1998
[26] J Kaufman J M Friedman D Sadowsky and J Harris ldquoMyo-tonic dystrophy surgical and anesthetic considerations duringorthognathic surgeryrdquo Journal ofOral andMaxillofacial Surgeryvol 41 no 10 pp 667ndash671 1983
[27] E Zanoteli H K Yamashita H Suzuki A S B Oliveira and AA Gabbai ldquoTemporomandibular joint and masticatory muscleinvolvement in myotonic dystrophy a study by magnetic res-onance imagingrdquo Oral Surgery Oral Medicine Oral PathologyOral Radiology and Endodontics vol 94 no 2 pp 262ndash2712002
[28] Ministero della Salute ldquoLinee guida nazionali per la promo-zione della salute orale e la prevenzione delle patologie oraliin eta evolutivardquo 2013 httpwwwsalutegovitimgsC_17_pub-blicazioni_2073_allegatopdf
[29] K J Skidmore K J Brook W MThomson andW J HardingldquoFactors influencing treatment time in orthodontic patientsrdquoAmerican Journal of Orthodontics and Dentofacial Orthopedicsvol 129 no 2 pp 230ndash238 2006
Submit your manuscripts athttpswwwhindawicom
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral OncologyJournal of
DentistryInternational Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
International Journal of
Biomaterials
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
BioMed Research International
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Case Reports in Dentistry
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral ImplantsJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Anesthesiology Research and Practice
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Radiology Research and Practice
Environmental and Public Health
Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
The Scientific World JournalHindawi Publishing Corporation httpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Dental SurgeryJournal of
Drug DeliveryJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral DiseasesJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Computational and Mathematical Methods in Medicine
ScientificaHindawi Publishing Corporationhttpwwwhindawicom Volume 2014
PainResearch and TreatmentHindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Preventive MedicineAdvances in
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
EndocrinologyInternational Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
OrthopedicsAdvances in
8 Case Reports in Dentistry
Table 4 Postsurgery cephalometric analysis
Case NormFacial angle 80 883Convexity angle 103 5SNA 813 802SNB 759 77ANB 54 32Mandibular angle 493 224119910-axis 747 573Occlusal angle 214 9Interincisal angle 142 129Li-occlusal plane 108 184Li-mandibular plane minus171 48UiSN 918 105UiA-Pg 62 5LiA-Pg 36 13MLSNL 534 NLSNL 128 Rotational type P1NOB Growth category 2
of recurrence complications related to general anesthesia andthe difficulties of recovery after the intervention and the needof very long orthodontic therapy which favor the accumu-lation of bacterial plaque and increase the risk of caries[28] must be kept in mind In the case report described infact throughout the orthodontic treatment the condition oforal health deteriorated because of the buildup of plaqueresulting in gingivitis and multiple demineralization at thecollar level of the teeth Naturally to undertake this typeof very long and complex therapy patient cooperation isnecessary but we know that during long therapy it alwaysdecreases [29] These considerations lead us to assess thesituation of each patient individually It would be better towork on the prevention and early treatment of malocclusionof cooperative patients with muscular dystrophy in orderto counteract the effects of facial hypotonic muscles onocclusion and the appearance of the patient from the time ofinitial diagnosis of the disease [28] Even cases successfullytreated in developmental age over time may recur or worsenthe functional deficit therefore patients should be orthodon-tically monitored periodically
3 Conclusion
Myotonic dystrophy is a very complex multisystemic diseasein which the muscular involvement leads to a characteristicfacial phenotype The weakness of the facial muscles is thecause of malocclusions needing in adulthood orthodontic-surgical treatment The purpose of this study was to describethe case of a patient treated with surgery and orthodonticswhose satisfaction at the conclusion of therapy demonstratedthe importance of a malocclusionrsquos therapy to provide an
aesthetic improvement to the patient and a resolution of rela-tional and functional problemsThis work could be an incen-tive to evaluate patients with myotonic dystrophy in an earlyage to try to counter the worsening of malocclusions andavoiding surgery if possibleThepropose of an early interven-tion may seem like a challenge but it might be useful toidentify new care pathways that make better the daily livesof these young patients and facilitate their psychosomaticdevelopment during the delicate period of adolescence
Abbreviations
DM1 Myotonic dystrophy type 1DM2 Myotonic dystrophy type 2PROMM Proximal Myotonic MyopathyDMPK Myotonin protein kinaseDM3 Myotonic dystrophy type 3ZNF9 Zinc Finger Protein Gene 9OVB OverbiteOVJ OverjetRx Radiography
Conflicts of Interest
The authors declare that they have no conflicts of interest
References
[1] T Suominen L L Bachinski S Auvinen et al ldquoPopulation fre-quency of myotonic dystrophy higher than expected frequencyof myotonic dystrophy type 2 (DM2) mutation in FinlandrdquoEuropean Journal of Human Genetics vol 19 no 7 pp 776ndash7822011
[2] P S Haper Myotonic DystrophymdashThe Clinical Picture WBSaunders London UK 2001
[3] J D Brook M E McCurrach H G Harley et al ldquoMolecularbasis of myotonic dystrophy Expansion of a trinucleotide(CTG) repeat at the 31015840 end of a transcript encoding a proteinkinase family memberrdquo Cell vol 68 no 4 pp 799ndash808 1992
[4] J Buxton P Shelbourne J Davies et al ldquoDetection of anunstable fragment ofDNA specific to individuals withmyotonicdystrophyrdquo Nature vol 355 no 6360 pp 547-548 1992
[5] Y-H Fu A Pizzuti R G Fenwick Jr et al ldquoAn unstabletriplet repeat in a gene related tomyotonicmuscular dystrophyrdquoScience vol 255 no 5049 pp 1256ndash1258 1992
[6] H G Harley J D Brook S A Rundle et al ldquoExpansion ofan unstable DNA region and phenotypic variation in myotonicdystrophyrdquo Nature vol 355 no 6360 pp 545-546 1992
[7] M Mahadevan C Tsilfidis L Sabourin et al ldquoMyotonic dys-trophy mutation an unstable CTG repeat in the 31015840 untranslatedregion of the generdquo Science vol 255 no 5049 pp 1253ndash12551992
[8] J Mathieu P Allard L Potvin C Prevost and P Begin ldquoA 10-year study of mortality in a cohort of patients with myotonicdystrophyrdquo Neurology vol 52 no 8 pp 1658ndash1662 1999
[9] G Meola ldquoClinical aspects molecular pathomechanisms andmanagement of myotonic dystrophiesrdquo Acta Myologica vol 32no 3 pp 154ndash165 2013
[10] S KiliaridisM Engvall andMG Tzakis ldquoUltrasound imagingof themassetermuscle inmyotonic dystrophy patientsrdquo Journalof Oral Rehabilitation vol 22 no 8 pp 619ndash625 1995
Case Reports in Dentistry 9
[11] C Odman and S Kiliaridis ldquoMasticatory muscle activity inmyotonic dystrophy patientsrdquo Journal of Oral Rehabilitationvol 23 no 1 pp 5ndash10 1996
[12] R N Staley S E Bishara J W Hanson and A J Nowak ldquoCra-niofacial development in myotonic dystrophyrdquoTheCleft Palate-Craniofacial Journal vol 29 no 5 pp 456ndash462 1992
[13] M Portelli G Matarese A Militi R Nucera G Triolo and GCordasco ldquoMyotonic dystrophy and craniofacial morphologyclinical and instrumental studyrdquo European Journal of PaediatricDentistry vol 10 no 1 pp 19ndash22 2009
[14] E Gazit N Bornstein M Lieberman V Serfaty M Gross andA D Korczyn ldquoThe stomatognathic system in myotonic dys-trophyrdquo European Journal of Orthodontics vol 9 no 1 pp 160ndash164 1987
[15] S Kiliaridis C Mejersjo and B Thilander ldquoMuscle functionand craniofacial morphology a clinical study in patients withmyotonic dystrophyrdquo European Journal of Orthodontics vol 11no 2 pp 131ndash138 1989
[16] J Mercier F Bennani J Ferri and B Piot ldquoMaxillofacial mani-festations of Steinertrsquos myotonic dystrophy clinical and thera-peutic aspectsrdquo Revue de Stomatologie et de Chirurgie Maxillo-Faciale vol 96 pp 74ndash82 1995
[17] G Meola ldquoClinical aspects molecular pathomechanisms andmanagement of myotonic dystrophiesrdquo Acta Myologica vol 32no 3 pp 154ndash165 2013
[18] L Sjogreen M Engvall A-B Ekstrom A Lohmander S Kil-iaridis andM Tulinius ldquoOrofacial dysfunction in children andadolescents with myotonic dystrophyrdquoDevelopmental Medicineand Child Neurology vol 49 no 1 pp 18ndash22 2007
[19] B Ercolin F C Sassi L D Mangilli L I Z Mendonca S CO Limongi and C R F De Andrade ldquoOral motor movementsand swallowing in patients with myotonic dystrophy type 1rdquoDysphagia vol 28 no 3 pp 446ndash454 2013
[20] L Dimberg K Arnrup and L Bondemark ldquoThe impact ofmal-occlusion on the quality of life among children and adolescentsa systematic review of quantitative studiesrdquo European Journal ofOrthodontics vol 37 no 3 pp 238ndash247 2015
[21] M Engvall L Sjogreen H Kjellberg A Robertson S Sundelland S Kiliaridis ldquoOral health in children and adolescents withmyotonic dystrophyrdquo European Journal of Oral Sciences vol 115no 3 pp 192ndash197 2007
[22] G W Arnett and R T Bergman ldquoFacial keys to orthodonticdiagnosis and treatment planning Part Irdquo American Journal ofOrthodontics and Dentofacial Orthopedics vol 103 no 4 pp299ndash312 1993
[23] G W Arnett and R T Bergman ldquoFacial keys to orthodonticdiagnosis and treatment planning Part IIrdquo American Journalof Orthodontics and Dentofacial Orthopedics vol 103 no 5 pp395ndash411 1993
[24] S Manzon and R Philbert ldquoOrthognathic Surgery in a PatientWith Myotonic Dystrophy Review of Literature and Report ofa Caserdquo Journal of Oral andMaxillofacial Surgery vol 65 no 12pp 2575ndash2579 2007
[25] S Kiliaridis andC Katsaros ldquoThe effects ofmyotonic dystrophyand Duchenne muscular dystrophy on the orofacial musclesand dentofacial morphologyrdquo Acta Odontologica Scandinavicavol 56 no 6 pp 369ndash374 1998
[26] J Kaufman J M Friedman D Sadowsky and J Harris ldquoMyo-tonic dystrophy surgical and anesthetic considerations duringorthognathic surgeryrdquo Journal ofOral andMaxillofacial Surgeryvol 41 no 10 pp 667ndash671 1983
[27] E Zanoteli H K Yamashita H Suzuki A S B Oliveira and AA Gabbai ldquoTemporomandibular joint and masticatory muscleinvolvement in myotonic dystrophy a study by magnetic res-onance imagingrdquo Oral Surgery Oral Medicine Oral PathologyOral Radiology and Endodontics vol 94 no 2 pp 262ndash2712002
[28] Ministero della Salute ldquoLinee guida nazionali per la promo-zione della salute orale e la prevenzione delle patologie oraliin eta evolutivardquo 2013 httpwwwsalutegovitimgsC_17_pub-blicazioni_2073_allegatopdf
[29] K J Skidmore K J Brook W MThomson andW J HardingldquoFactors influencing treatment time in orthodontic patientsrdquoAmerican Journal of Orthodontics and Dentofacial Orthopedicsvol 129 no 2 pp 230ndash238 2006
Submit your manuscripts athttpswwwhindawicom
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral OncologyJournal of
DentistryInternational Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
International Journal of
Biomaterials
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
BioMed Research International
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Case Reports in Dentistry
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral ImplantsJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Anesthesiology Research and Practice
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Radiology Research and Practice
Environmental and Public Health
Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
The Scientific World JournalHindawi Publishing Corporation httpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Dental SurgeryJournal of
Drug DeliveryJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral DiseasesJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Computational and Mathematical Methods in Medicine
ScientificaHindawi Publishing Corporationhttpwwwhindawicom Volume 2014
PainResearch and TreatmentHindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Preventive MedicineAdvances in
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
EndocrinologyInternational Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
OrthopedicsAdvances in
Case Reports in Dentistry 9
[11] C Odman and S Kiliaridis ldquoMasticatory muscle activity inmyotonic dystrophy patientsrdquo Journal of Oral Rehabilitationvol 23 no 1 pp 5ndash10 1996
[12] R N Staley S E Bishara J W Hanson and A J Nowak ldquoCra-niofacial development in myotonic dystrophyrdquoTheCleft Palate-Craniofacial Journal vol 29 no 5 pp 456ndash462 1992
[13] M Portelli G Matarese A Militi R Nucera G Triolo and GCordasco ldquoMyotonic dystrophy and craniofacial morphologyclinical and instrumental studyrdquo European Journal of PaediatricDentistry vol 10 no 1 pp 19ndash22 2009
[14] E Gazit N Bornstein M Lieberman V Serfaty M Gross andA D Korczyn ldquoThe stomatognathic system in myotonic dys-trophyrdquo European Journal of Orthodontics vol 9 no 1 pp 160ndash164 1987
[15] S Kiliaridis C Mejersjo and B Thilander ldquoMuscle functionand craniofacial morphology a clinical study in patients withmyotonic dystrophyrdquo European Journal of Orthodontics vol 11no 2 pp 131ndash138 1989
[16] J Mercier F Bennani J Ferri and B Piot ldquoMaxillofacial mani-festations of Steinertrsquos myotonic dystrophy clinical and thera-peutic aspectsrdquo Revue de Stomatologie et de Chirurgie Maxillo-Faciale vol 96 pp 74ndash82 1995
[17] G Meola ldquoClinical aspects molecular pathomechanisms andmanagement of myotonic dystrophiesrdquo Acta Myologica vol 32no 3 pp 154ndash165 2013
[18] L Sjogreen M Engvall A-B Ekstrom A Lohmander S Kil-iaridis andM Tulinius ldquoOrofacial dysfunction in children andadolescents with myotonic dystrophyrdquoDevelopmental Medicineand Child Neurology vol 49 no 1 pp 18ndash22 2007
[19] B Ercolin F C Sassi L D Mangilli L I Z Mendonca S CO Limongi and C R F De Andrade ldquoOral motor movementsand swallowing in patients with myotonic dystrophy type 1rdquoDysphagia vol 28 no 3 pp 446ndash454 2013
[20] L Dimberg K Arnrup and L Bondemark ldquoThe impact ofmal-occlusion on the quality of life among children and adolescentsa systematic review of quantitative studiesrdquo European Journal ofOrthodontics vol 37 no 3 pp 238ndash247 2015
[21] M Engvall L Sjogreen H Kjellberg A Robertson S Sundelland S Kiliaridis ldquoOral health in children and adolescents withmyotonic dystrophyrdquo European Journal of Oral Sciences vol 115no 3 pp 192ndash197 2007
[22] G W Arnett and R T Bergman ldquoFacial keys to orthodonticdiagnosis and treatment planning Part Irdquo American Journal ofOrthodontics and Dentofacial Orthopedics vol 103 no 4 pp299ndash312 1993
[23] G W Arnett and R T Bergman ldquoFacial keys to orthodonticdiagnosis and treatment planning Part IIrdquo American Journalof Orthodontics and Dentofacial Orthopedics vol 103 no 5 pp395ndash411 1993
[24] S Manzon and R Philbert ldquoOrthognathic Surgery in a PatientWith Myotonic Dystrophy Review of Literature and Report ofa Caserdquo Journal of Oral andMaxillofacial Surgery vol 65 no 12pp 2575ndash2579 2007
[25] S Kiliaridis andC Katsaros ldquoThe effects ofmyotonic dystrophyand Duchenne muscular dystrophy on the orofacial musclesand dentofacial morphologyrdquo Acta Odontologica Scandinavicavol 56 no 6 pp 369ndash374 1998
[26] J Kaufman J M Friedman D Sadowsky and J Harris ldquoMyo-tonic dystrophy surgical and anesthetic considerations duringorthognathic surgeryrdquo Journal ofOral andMaxillofacial Surgeryvol 41 no 10 pp 667ndash671 1983
[27] E Zanoteli H K Yamashita H Suzuki A S B Oliveira and AA Gabbai ldquoTemporomandibular joint and masticatory muscleinvolvement in myotonic dystrophy a study by magnetic res-onance imagingrdquo Oral Surgery Oral Medicine Oral PathologyOral Radiology and Endodontics vol 94 no 2 pp 262ndash2712002
[28] Ministero della Salute ldquoLinee guida nazionali per la promo-zione della salute orale e la prevenzione delle patologie oraliin eta evolutivardquo 2013 httpwwwsalutegovitimgsC_17_pub-blicazioni_2073_allegatopdf
[29] K J Skidmore K J Brook W MThomson andW J HardingldquoFactors influencing treatment time in orthodontic patientsrdquoAmerican Journal of Orthodontics and Dentofacial Orthopedicsvol 129 no 2 pp 230ndash238 2006
Submit your manuscripts athttpswwwhindawicom
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral OncologyJournal of
DentistryInternational Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
International Journal of
Biomaterials
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
BioMed Research International
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Case Reports in Dentistry
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral ImplantsJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Anesthesiology Research and Practice
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Radiology Research and Practice
Environmental and Public Health
Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
The Scientific World JournalHindawi Publishing Corporation httpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Dental SurgeryJournal of
Drug DeliveryJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral DiseasesJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Computational and Mathematical Methods in Medicine
ScientificaHindawi Publishing Corporationhttpwwwhindawicom Volume 2014
PainResearch and TreatmentHindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Preventive MedicineAdvances in
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
EndocrinologyInternational Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
OrthopedicsAdvances in
Submit your manuscripts athttpswwwhindawicom
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral OncologyJournal of
DentistryInternational Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
International Journal of
Biomaterials
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
BioMed Research International
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Case Reports in Dentistry
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral ImplantsJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Anesthesiology Research and Practice
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Radiology Research and Practice
Environmental and Public Health
Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
The Scientific World JournalHindawi Publishing Corporation httpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Dental SurgeryJournal of
Drug DeliveryJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Oral DiseasesJournal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Computational and Mathematical Methods in Medicine
ScientificaHindawi Publishing Corporationhttpwwwhindawicom Volume 2014
PainResearch and TreatmentHindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Preventive MedicineAdvances in
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
EndocrinologyInternational Journal of
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014
OrthopedicsAdvances in
Related Documents
