Turkish Neurosurgery 3: 82-84, 1993 Iyigün: Klippel Trenaunay Syndrome Klippel Trenaunay Syndrome With Oeeipital Infaret ÖMER IYIGÜN. ZEKI SEKEReL CEMIL RAKUNT, F AHRETTIN ÇELIK Department of Neurosurgery (ö.I, 2.S, CR. F.ç.) Ondokuz Mayis University Samsun, Türkiye Abstract : Klippel - Trenaunay syndrome is a congenital angiodysplasia characterized by varicose veins, cutaeous haemangiomas and bony and soft tissue hypertrophy. This report describes a case of Klippel-Trenaunay Syndrome with dilated tor- INTRODUCTION The Klippel-Trenaunay Syndrome is a congenital angiodysplasia eharaeterized by a vaseular nevus,varieose veins and bony and soft tissue hyper- trophy as well as assoeiated anomalies such as pes equinovarus, syndaetyly, polydaetyly, eongenital disloeation of hips or shoulders, spina bifidia, seollosis or pelvie asymmetry. In 1900.Klippel and Trenaunay (5)deseribed this syndrome for the first time. Parkes Weber (IO)deseribed asimilar triad of findings. Arteriovenous fistulas. vaseular hyperplasia and bony hypertropy oeeur in both syndromes. In Klippel - Tre- naunay syndrome, the fistulas are sm all and numerous but in Parkes Weber sydrome theyare large and few and may lead to eireulatory distur- banees. All these symptoms may beeome apperent at any time from birth to adulthood (12).Osteohyper- trophy is frequently present at birth usually affeeting the limbs. The congenital haemangioIna varies in size and colour. frequently following a radieular distribu- tion although the variees are eongenital but may in- erease in size (1). The etiology of Klippel -Trenaunay syndrome is unknown. It does not appear to be hereditary nor is there sex preferenee (2) In this report we present and unusual ease of Klippel- Trenaunay Syndrom with oecipital infaret 82 tuous vessels in the retina and left occipital infarct Key words: Cerebral infarct.K1ippel-Trenaunay Sydrome, Retinal Involvement. CAS E REPORT A 55 -year-old female pres en ted of our neurosurgieal centre with a l5-day history of mild headaehe. There was an associated history of enlarg- ed left arm and leg and aecompanying erythematous lesion over the faee and neek. There was no history of eonvulsions. vomiting, loss of eonseiousness or foeal neurologieal defieit. Her family had notieed the erythematous lesions on her faee and left upper and lower limbs whieh inereased in size in proportion to her general growth. Her left hand and foot. however beeame disproportionately large. she has four sibl- ings none of who m is affeeted. Physial examination revealed that there was evidenee of portwine on her faee and over the left forearin and left leg (FigI)The left han d and foot were disproportionately large (Fig 2a-2b). The rest of the systemie examination did not reveal any obvious ab- normality. Total and differential blood eounts in- duding platelet eount were within normal limits. Femoral angiography was proposed but the patient refused. Oeular examination revealed a visual aeuity of 20/20 in both eyes. The eonjuetiva and iris did not reveal any angioma or other disorder.On fundus ex- amination of the right eye. the optie dise was hyperaemie and minimaly elevated in appearanee. The retinal veins were markedly dilated and tortuous
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ÖMER IYIGÜN. ZEKI SEKEReL CEMIL RAKUNT, FAHRETTIN ÇELIK
Department of Neurosurgery (ö.I, 2.S, CR. F.ç.) Ondokuz Mayis University Samsun, Türkiye
Abstract : Klippel - Trenaunay syndrome is a congenitalangiodysplasia characterized by varicose veins, cutaeoushaemangiomas and bony and soft tissue hypertrophy. This reportdescribes a case of Klippel-Trenaunay Syndrome with dilated tor-
INTRODUCTION
The Klippel-Trenaunay Syndrome is a congenitalangiodysplasia eharaeterized by a vaseularnevus,varieose veins and bony and soft tissue hypertrophy as well as assoeiated anomalies such as pesequinovarus, syndaetyly, polydaetyly, eongenitaldisloeation of hips or shoulders, spina bifidia,seollosis or pelvie asymmetry.
In 1900.Klippel and Trenaunay (5)deseribed thissyndrome for the first time. Parkes Weber(IO)deseribed asimilar triad of findings.Arteriovenous fistulas. vaseular hyperplasia and bonyhypertropy oeeur in both syndromes. In Klippel - Trenaunay syndrome, the fistulas are sm all andnumerous but in Parkes Weber sydrome theyarelarge and few and may lead to eireulatory disturbanees. All these symptoms may beeome apperentat any time from birth to adulthood (12).Osteohypertrophy is frequently present at birth usually affeetingthe limbs. The congenital haemangioIna varies in sizeand colour. frequently following a radieular distribution although the variees are eongenital but may inerease in size (1).
The etiology of Klippel -Trenaunay syndrome isunknown. It does not appear to be hereditary noris there sex preferenee (2)
In this report we present and unusual ease ofKlippel- Trenaunay Syndrom with oecipital infaret
82
tuous vessels in the retina and left occipital infarct
A 55-year-old female pres en ted of ourneurosurgieal centre with a l5-day history of mildheadaehe. There was an associated history of enlarged left arm and leg and aecompanying erythematouslesion over the faee and neek. There was no historyof eonvulsions. vomiting, loss of eonseiousness orfoeal neurologieal defieit. Her family had notieed theerythematous lesions on her faee and left upper andlower limbs whieh inereased in size in proportion toher general growth. Her left hand and foot. howeverbeeame disproportionately large. she has four siblings none of who m is affeeted.
Physial examination revealed that there wasevidenee of portwine on her faee and over the leftforearin and left leg (FigI)The left han d and foot weredisproportionately large (Fig 2a-2b). The rest of thesystemie examination did not reveal any obvious abnormality. Total and differential blood eounts induding platelet eount were within normal limits.Femoral angiography was proposed but the patientrefused.
Oeular examination revealed a visual aeuity of20/20 in both eyes. The eonjuetiva and iris did notreveal any angioma or other disorder.On fundus examination of the right eye. the optie dise washyperaemie and minimaly elevated in appearanee.The retinal veins were markedly dilated and tortuous
medidne, The clinical sydrome named after these investigators. ineludes haemangiomas. hypertrofy ofthe soft tissue and bone with overgrowth of the extremity and variease veins, The vascular lesion inKlippel -Trenaunay syndrome is one of deep venous
Fig. 3 : Fluoresent angiography showed the presence of dilated.tortuous vessels in right eye but no leakage of dye.
Fig 2a - 2b: Minimal erythematous lesion and hypertrophy ofleft hand and leg of a 55-year-oid woman with klippelTrenaunay syndrome
DlSCUSSION
over the disc. No definitive choroidal angioma wasvisualized, Fluorescent angiography confirmed thepresence of dilated. tortuous vessels in the right eyebut no leakage of dye could be exhibited (Fig 3-4),
Cranial CT showed a hypodense. well circumscribed lesion with no eantrast enhancement at
the left occipital region (Fig5) which was thought tobe a low grade astrocytoma or infarct.
Occipital craniotomy was undertaken in junei990, Immediately beneath the dura the lesion wasseen over the occipital pole, The lesion was removed subtotally, The operative eaurse was uneventtuI.Histologic a diagnosis proved to be typical necroticmateriaI.
The Klippel - Trenaunay syndrome is a relatedcondition consisting of a triad of cutaneoushaemangioma extending over the limbs. varieasitiesof the affected limbs and soft tissue and bony hypertrophy (4.6).Ini900 Klippel and trenaunay (5)published an artiele entitled "Du nevaus variqueuxosteohypertrophique" in archives Generales de
Fig 1 : Massive erythematous lesions are seen on the face and nede
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Turkish Neurosurgery 3: 82-84, 1993
Fig.5: Cranial CT showed a hypodense well-drcumsaibed lesionat the left ocdpital region.
abnormality with insuffieieny (9),Parker Weber (io)
described asimilar triad in 1907, in a further reportin 1918(ll), he included arteriovenous fistulas as partof the syndrome.sinee then the names Klippel- Trenaunay and Klippel-Trenaunay Weber have been used interehangeably and indiseriminately:Klippel-Trenaunay syndrome for patients with noarteriovenous fistula and Klippel -Trenaunay - Webersydrome for those with a clinieally apparent fistulapopularized by lindenauer in 1965 (7)
Neurovaseular involvement in yhe KlippelTrenaunay Weber syndrome is very rare, Djindjianat al (3) described the oeeurenee of spinalarteriovenous malformations in five patients with thesyndrome.
In 1988, Oyesiku et al described a true eerebralarteriovenous fistula in the Klippel - TrenaunayWeber Syndrome.This is the first ease to be reported(8).
A higher incidenee of neurovaseular anomalies inthe Klippel-Trenaunay-Weber sydrome may beeameevident using eranial CT sean.
84
Iyigün: Klippel Trenaunay Syndrome
The oecipital infaret probably due to an undisclosed micro haemangioma of the oecipital brain tissue
if a ease of Klippel-Trenaunay Syndrome is encountered angiography of eerebraL.spinal and four extremities routine diagnostie tests of haemotologiedisorders must be performed.
Correspondence : Ömer Iyigün, M.D.Ondokuz Mayis ÜniversitesiTip FakültesiNörosirurji Anabilim DaliSamsun-TÜRKIYE
REFERENCES
1. Barek L, Ledor K: The Klippel-Trenaunay syndrome: A casereport and Review of the literature. Tte Mount Sinai Journalof Medicine 49:66-70, 1982.
2. Boron L, et al : Klippel- Trenaunaysches Syndrom.FortschrRoentgenstr 1975, 123:355.
3. Djindjian M, Djindjian R,Murth M, Rey A, Houdart R: Spinalcord arteriovenous malformations and the Klippel TrenauayWeber syndrome.Surg Neurol 8:229-237.1977.
4. Hockley NM, Bihrle R, Benneta RM, Curry JM: Congenitalgenitourinary hemangiomas in a patient with the KlippelTrenaunay syndrome :Management with the neodymium YAGlaser.J Urol 141 :940-941.1989.
5. Klippel M .Trenaunay P : Du naevus variquex osteohypertrophique.Arch Gen Med 3 : 641-672. 1900.