Turkish Neurosurgery 3: 82-84, 1993 Iyigün: Klippel Trenaunay Syndrome

Klippel Trenaunay Syndrome With Oeeipital Infaret

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

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tuous vessels in the retina and left occipital infarct

Key words: Cerebral infarct.K1ippel-Trenaunay Sydrome, RetinalInvolvement.

CAS E REPORT

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

Turkish Neurosurgery 3: 82-84, 1993 Iyigün: Klippel Trenaunay Syndrome

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.

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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

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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.

6. Lqamar 1. Farber G . O'Quinn S: Klippel- Trenaunay syn­drome.Arch Dermatol 1965, 91: 58·59.

7. Lindenauer SM The Klippel-Trenaunay syndrome. varicosityhypertrophy and hemangioma with no arteriovenous fistula.Ann Surg 1965.162:303-314.

8. Oyesiku NM.Gahm NH. Goldman RL:Cerebral arteriovenousfistula in the Klippel· Trenaunay Webwr sndrome. Dev Medand Child Neurol 1988.30:245-251.

9. Smith Jr JA,Dixon JA: Neodymium Yag laser irradition of blad­der hemangioma. Urol 24: 134-136.1984.

10. Weber FP: Angioma formation in connection with hypper­trophy of limbs and hemihpertrophy.Br J Dermol19231-233,1907

11. Weber FP: Hhemangiectatic hypertrophy of limbs congenitalphlebarteriectasis and so cal1ed congenital varicase veins. BritJ Child Dis 1918.15:13.

12. You CK. Rees J,Gillis DA. Steeves J: Klippel -Trenaunay syn­drome A review.Can J Surg 26: 399-403.1983.