Clinical Aspects of Cleft Palate Repair Ahmed Atef, Msc, MRCS Specialist of plastic surgery Mataria Teaching Hospital
Jul 06, 2015
Clinical Aspects of Cleft Palate Repair
Ahmed Atef, Msc, MRCSSpecialist of plastic surgery
Mataria Teaching Hospital
Objective
• Incidence
• Surgical Anatomy
• Embryology
• Classification / Cleft Variants
• Clinical Effects
• Management
• Future
Cleft Palate / Cleft lip is the the most commoncraniofacial malformation
Second most common congenital defect
Isolated Cleft palate
• No racial variation
• 1:2000 live birth
• M:F = 1:2
• Left : Right : B/L = 6:3:1
Surgical Anatomy
The palate forms a dynamic boundary between the oral cavity and the nasal cavity. It is composed of the hard palate anteriorlyand the soft palate posteriorly.
Normal Palate
• Primary Palate
• Secondary Palate
Hard Palate
Soft Palate
Surgical Anatomy
Surgical Anatomy
The hard palate includes the palatal processes of the maxilla and the horizontal plate of the palatine bone with adherent mucoperiosteum (attached to bone by Sharpey’s fibres).
Surgical Anatomy
Three pairs of foramina mark the surface of the bony palate
• Incisive Foramen
• Greater Palatine Foramen
• Lesser Palatine Foramen
The soft palate is a dynamic structure that acts as a valve between the oropharynx and nasopharynx.
An intact and functioning soft palate is essential for normal speech and feeding.
Soft palate• Mucosa• Five paired muscles &
central aponeurosisTensor veli palatiniLevator veli palatiniPalatoglossusPalatopharyngrusUvualis
*Veli (Latin) means curtain
Surgical Anatomy
Tensor palatiOrigin: scaphoid fossa of the medial
pterygoid plate, the lateral part of the cartilaginous auditory tube then passes around the pterygoidhamulus as a tendon
Insertion: broad triangular tendon at the posterior aspect of the hard palate as part of the palatine aponeurosis
Action: tense the soft palate to form a platform that the other muscles may elevate or depress.
Surgical Anatomy
Levator palati
Origin: petrous bone and the medial part of the auditory tube
Insertion: middle third of upper surface of the soft palate at upper surface of the palatine aponeurosis as far as the midline
Surgical Anatomy
Levator palati
The paired muscles form
a ‘V’-shaped sling pulling
the soft palate upwards
and backwards to close
the nasopharynx.
Surgical Anatomy
Palatoglossus
Origin: Palatine aponeurosis
Insertion: Side of tongue
Action: Pulls root of tongue upward and backward, narrows transverse diameter of oropharynx
Surgical Anatomy
Palatopharyngeus
Origin: Palatine aponeurosis
Insertion: Posterior border of thyroid cartilage
Action: Elevates wall of pharynx, pulls palatopharyngeal folds medially
Surgical Anatomy
Musculus uvulae
Origin: Posterior border of hard palate
Insertion: Mucous membrane of uvula
Action: Elevates uvula
Surgical Anatomy
The soft palate is raised by the contraction of the levator palati.
At the same time, the upper fibers of the superior constrictor muscle pull the posterior pharyngeal wall forward.
The palatopharyngeus muscles contract to pull palatopharyngealarches medially, like side curtains.
Surgical Anatomy
By this means The intact palate can periodically, selectively, and completely isolate the nasopharynx from the oropharynx during Feeding & Speech
Surgical Anatomy
This harmony in muscular action is necessary for
Velopharyngeal Competence
Surgical Anatomy
Surgical Anatomy
Surgical Anatomy
Embryology
Development of the face begins in the fourth week in utero, when neural cells migrate and fuse with mesodermal elements to form the facial primordium.
It results from the fusion
– Two mandibularprocesses
– One frontonasal process
– Two maxillary processes
Embryology
The palate develops between the 5th and the 12th week
CRITICAL period of palatal development is between the 6th and the 9th week.
Soft palate development is completed at 12th week
Embryology
Primary palate : Median palatine process from the medial nasal prominences.
Secondary palate : Lateral palatine process from the maxillary prominence
Embryology
6th – 9th week: Initially, the palatine processes are oriented vertically on either side of the developing tongue.
The tongue is displaced inferiorly as the head grows and the neck straightens, the lateral palatine processes are elevated and grow medially to fuse with the septum
Embryology
Is Cleft a Deficiency?
Embryology
Interference with fusion results in Cleft
Three theories:
i) Failure of fusion of the lateral shelves
ii) Failure of mesodermal penetration of the shelves:
iii) Mechanical interference (the tongue) such as in Pierre Robbin Sequence
Embryology
Gato et al. 2002, expression of chondroitin sulfate proteoglycan is important in palatal shelf adhesion and is supposed to be regulated by TGF-b3
Gato A, Martinez ML, Tudela C, Alonso I, Moro JA, Formoso MA, Ferguson MWJ, Martinez-lvarez C (2002) TGF-b3-induced
chrondroitin sulphate proteoglycan mediates palatal shelf adhesion.
Bush et al. 2003; Herr et al. 2003, Expression of T box transcription factor Tbx22 is found in the inferior nasal septum and the palatal shelf before fusion.
Bush JO, Lan Y, Maltby KM, Jiang R (2002) Isolation and developmental expression analysis of Tbx22, the mouse homolog of the human x-linked cleft palate gene. Dev Dyn 225: 322-326
Herr A, Meunier D, Mller I, Rump A, Fundele R, Ropers H-H, Nuber UA (2003) Expression of mouse Tbx22 supports its role in palatogenesis and glossogenesis. Dev Dyn 226:579–586
Embryology
Classification
Veau Classification 1931
Veau Class I: isolated soft palate cleft
Veau Class II: isolated hard and soft palate
Veau Class III: unilateral CLAP
Veau Class IV: bilateral CLAP
Classification
Striped Y by Kernahan 1971 Millard modification
Cleft Variant
Cleft Variant
Cleft Variant
Cleft Variant
syndrome Treacher-Collins
Syndromatic Cleft
Pierre Robin syndrome
Syndromatic Cleft
Syndromatic Cleft
Van der Woude’s syndrome
Clinical effects
Patients with cleft deformities experience a multitude of problems including
• Feeding problems
• Speech difficulties
• Otologic issues
• Midface growth impairment.
Clinical effects
Feeding
The infant is usually not able to suck efficiently due to inability to achieve negative pressure.
Nasal regurgitation.
Feeding regimen: includes the use of squeeze bottles and holding in a nearly sitting position during feeding
Clinical effects
Speech
Patients are unable to produce high intra-oral pressure.
Normal velopharyngeal closure is crucial for production of intelligible speech; any abnormalities in this mechanism can result in hypernasality, nasal emissions, imprecise production of consonants.
Hearing
Serous otitis media.
Abnormality of LVP which aids the TVP to dilate ET.
Nasal regurgitation.
Treatment with myringotomy tubes is required pre- and post-cleft repair.
Management requires a multidisciplinary approach spanning multiple specialties
• Plastic surgery
• Speech pathology
• Otolaryngology
• Genetics
• Pediatrics
• Orthodontics
• Audiology
Goal
Restoring the morphologic form & function
Production of a competent velopharyngealsphincter
Principles
• Closure of the defect
• Correction of the abnormally inserted muscles
• Reconstruction of the palatine sling
• Tension free repair
• 2 layer repair of the hard palate & 3 layer repair of the soft palate
Von Langenbeck 1861 pioneered the first bipediclemucoperiosteal flaps and relaxing incisions for palate closure surgery in one stage.
Langenbeck v, B. Uranoplasty by means of raising mucoperiosteal flaps. Arch klin chir. 1861;2:205
Veau 1931, The vomer flap and suturing of velar muscles aiming at lengthening the palate
Wardill and Kilner 1937, “pushback” theory V-Y retro positioning of the palate increases the length further.
By connecting the lateral incisions to the incisions made for the nasal turn in flaps.
Wardill WEM. The technique of operation for cleft palate. Br J Surg. 1937;25: 117-130
A different approach was described by Furlow 1986 with the double-opposing z-plasty without relaxing incisions
Furlow LT, Jr. Cleft palate repair by double opposing Z-plasty. Plastic and reconstructive surgery. 1986;78:724-738
The Bardach 1991 two-flap palatoplasty uses two large full-thickness hard palate flaps that are mobilized and closed anteriorly and medially without pushback
Bardach, J. and P. Nosal: Geometry of the two-flap palatoplasty. (2nd). St. Louis, Mosby-Year Book, 1991
2002 ., & Sommerlad et al2000 Rohrich et al.,
Closure of the palate can be performed in two stages. This involvesclosing the soft palate early, between 3 and 6 months
of age, and delaying the repair of the hard palate.Sommerlad BC, Mehendale FV, Birch MJ, Sell D, Hattee C, Harland K. Palate rerepairrevisited. Cleft Palate Craniofac J. 2002;39:295-307.
To limit the effect of the hard palate repair on maxillary growth. It is suggested that the subperiosteal scarring impairs midfacial growth.
Preoperative considerations
• Age: 9-12 month
• Associated anomalies
• Routine Lab. Investigations
• Booking a unit of packed RBCs after G/XM
• Otologic and audiologic assessment
Operative preparations
i) RAE tube
ii) Dingman
iii) Shoulder roll
iv) Head Donut
v) Local anesthetic with
1:200,000 epi
vi) Position: supine, neck
Extended, reverse trendlenberg
vii) Throat pack
Operative preparations
Steps
i) Inject 1 :200 000 epinepherine into the palate.ii) Don't inject in areas sutures will be placediii) Wait 7 minutes for the epinephrine to take effectiv) Make incision along the medial side of the cleft v) Make releasing incision to get to bone on both sidesvi) Use freer to elevate mucoperiosteal flapvii) Dissect nasal mucosavii) Strip LVP muscle off abnormal insertion & create palatine
slingviii) Three layer repair
Steps
Vomerian flap
Postoperative care
• Keep your eye on the airway
• AB
• Analgesic
• Feeding: fluids, soft diet, no bottles for 3w
• Arm restraints
ComplicationsEarly:
Haemorrhage
Airway obstruction
Dehiscence
Fistula
Late:
Bifid uvula
VPI
Maxillary hypoplasia
Dental malalignment
Tissue engineering advancements over the last decade has provided a plethora of materials that may be suitable for the healing of craniofacial defects like the cleft palate.
Future directions with regards to the use of stem cells especially ASCs in craniofacial repair are discussed, including possible scaffold for reconstruction of palatal defect
Quiz
Embryogenesis of primary & secondary palate?
Muscles of soft palate?
Velopharyngeal mech?
Clinical effects?
Preoperative preparations?
Principles of repair?
Postoperative care?