2007 Volume 18 Issue 5 Pages 253-257
From 1994 to 2007, eight patients with Klippel-Trenaunay syndrome consulted our hospital. They consisted of 5 males and 3 females at the age of 17 to 52 years old. Among them three patients were associated with abnormalities of other organs.
Case 1: 24-year-old female .Extensive hypertrophy oft he left lower extremity has been present since birth. She has been done orthopedic surgery several times in her teens.S he consulted our hospital because of pyelonephritis.
Computed tomography demonstrated hypodensity round mass near the left kidney 12 cm in maximum diameter and mild splenomegaly with cystic lesions. Cavernous lymphangioma was strongly suspected.
Case 2: 52-year-old male. Extensive varicose veins and hypertrophy of the left lower extremity have been present since birth. He consulted our hospital because of a worsening motor disturbance. Choreoathetoic movements of the fingers and toes were noticeable. A (CAG)n repeat longer than the normal range was observed on chromosome 4p. He was diagnosed with Huntington’s disease.
Case 3: 40-year-old male. All of the extremities of this patient showed cutaneous port wine hemangiomas, superficial venous varicosities and hypertrophy. He had an intermittent anal bleeding since his adolescence. He was performed endoscopy and varices involving the rectum and sigmoid colon were detected.
Three patients (37.5%) out of eight patients with KTS in our series were associated with abnormalities of other organs. It is important to look for other possible congenital lesions in the diagnosis of KTS. No patient in our series except for two patients who were treated in other hospital needed operation. The patient must be urged to follow a protective policy usually by elastic support and high elevation whenever possible.
