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Year : 1990  |  Volume : 56  |  Issue : 1  |  Page : 52-53

Klippel trenaunay syndrome associated with unusual eye changes

Correspondence Address:
L Mohan

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A young female had Klippel-Trenaunay syndrome with involvement from the head the toe on the left ide. The radiological, ophthalmological and histopathological findings were consistent with Klippel-Trenaunay syndrome. The unique feature was negalocornea which has not been reported earlier.

Keywords: Klinnel-Trenaunay syndrome, Megalocornea

How to cite this article:
Mohan L, Mukhija R D, AroraSSK, Prasad V N, Jain R K, Grover S. Klippel trenaunay syndrome associated with unusual eye changes. Indian J Dermatol Venereol Leprol 1990;56:52-3

How to cite this URL:
Mohan L, Mukhija R D, AroraSSK, Prasad V N, Jain R K, Grover S. Klippel trenaunay syndrome associated with unusual eye changes. Indian J Dermatol Venereol Leprol [serial online] 1990 [cited 2020 May 26];56:52-3. Available from: http://www.ijdvl.com/text.asp?1990/56/1/52/3483

Klippel and Trenaunay described a congeni­tal vascular abnormality manifesting with, (1) flat telangiectatic vascular nevi of portwine stain type, (2) hypertrophy involving the bones and soft tissues of one or more limbs, and (3) dilated varicose superficial veins.[1] The affected tissues do not contain haemodynamically significant arterio-venous communication.[2] The vascular nevus is most often present at birth, but may appear during early childhood.[3],[4],[5],[6] Usually, the vascular nevi occur on the affected limb, but distant lesions may be observed.[3] It is commoner among boys.[3] In a review of 48 cases,[3] the nevus was seen in a limb in 75%, the ipsilateral side of the body in 17%, and the contralateral limb in 15% of the cases. Hemihypertrophy is seen invo­lving the thorax, the arm and the leg on the same side,[6] and there may be bilateral involvement of the two or four limbs.[6] Compensatory scoliosis may be observed because of difference in the leg lengths.[3],[7] Venous varicosities may be apparent during late childhood. It may be associated with venous malformations in the orbit.[2]

  Case Report Top

An 8-year-old girl came with a diffuse macular hemangioma on the left side involving the right side at certain places. The hemangioma was bilateral but asymmetrical on the back. A few lesions were present on the right leg and the thigh. On the face, it was descending obliquely involving the lips and the oral mucosa The left upper and lower extremities were enlar. ged in length and girth. Scoliosis was present, The dilated veins and venous varicosities were present on the suprapubic region, upper part of the thigh and the abdomen.

Measurement from the . anterior superior iliac spine to the medial malleolus was 61 cm on the left side and 58.5 cm on the right side Girth was 14.5 cm on the left side and 13.8 cm on the right side just above the medial malleolus, 20.3 cm on the left side and 19.8 cm on the right side just below the knee joint, 32.5 cm on the left side and 31.5 cm on the right side on the upper part of the thigh, and 11.5 cm on the left side and 10.5 cm on. the right side on the lower part of the forearm.

Histopathologically, the capillaries were dilated in the papillary dermis with an increase in the number of venules in the deep dermis and subcutis.

The left eye showed hemangioma on both the lids. There was megalocornea on the left side, the diameter being 15 mm. Pupil was semidilated and reacted sluggishly to light on the left side. Fundoscopy showed a normal optic disc but tortuous retinal veins on the left side.

On X-rays of the upper extremity, the humerus, the radius and the ulna were 0.8 cm 0.5 cm and 0.2 cm longer on the left side. The length and width of the metacarpals were also more on the left side (3.0/2.9 cm and 0.7/0.6 cm respectively). On X-rays of the lower extremity, the femur, the tibia and the fibula were longer on the left side by 1.5 cm, 1.0 cm and 0.7 cm respectively. The metatarsals were 0.1 to 0.2 cm longer on the left side, Venography of the left leg showed dilated and tortuous deep veins with thrombosis but the left popliteal vein was not visualised.

  Comments Top

Several features observed in our patient are well known, but unilateral megalocornea has not been reported earlier.

  References Top

1.Klippel M and Trenaunay P : Du Noevus Variqueux Osteohypertrophiques, Arch Genetic Med (Paris), 1900; 185 : 641-672.  Back to cited text no. 1    
2.Lindenauer SM : The Klippel-Trenaunay syndrome : varicosity hypertrophy and heman­gioma with no arteriovenous fistula, Ann Surgery, 1965; 162 :303-314.  Back to cited text no. 2    
3.Baskerville PA, Ackroyd JS, Thomas ML et al: The Klippel-Trenaunay syndrome : clinical, radiological and haemodynamic features and management, Brit J Surgery, 1985; 72 : 232-236.  Back to cited text no. 3    
4.Kinmonth JB, Young AE, Edwards JMM et al Mixed vascular deformities of the lower limbs in particular reference to lymphography and surgical treatment, Brit J Surgery, 1976; 63 : 899­906.  Back to cited text no. 4    
5.Lindenauer SM : Congenital arteriovenous fistula and the Klippel Trenaunay syndrome, Ann Surgery, 1971; 174 : 248-263.  Back to cited text no. 5    
6.Atherton DJ and Rook A : Klippel Trenaunay syndrome, in : Textbook of Dermatology, Fourth ed, Editors, Rook A, Wilkinson DS, Ebling FJG et al : Oxford University Press, Bombay, 1987; p 214-216.  Back to cited text no. 6    
7.Letts RM : Orthopedic treatment of hemangio­matous hypertrophy of the lower extremity, J Bone Joint Surgery, 1977; 59 : 777-783.  Back to cited text no. 7    


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