Congenital Vascular Lesions of the Gastrointestinal Tract: Blue Rubber Bleb Nevus and Klippel-Trenaunay Syndromes

South Med J. 2001;94(4) 

In This Article

Case Report

A 26-year-old man was transferred to our institution for evaluation and management of recurrent rectal bleeding. Rectal bleeding had first occurred after birth, and at the age of 2 months he had a diverting colostomy for "congenital hemangioma" of the rectosigmoid. At the age of 7 months, the colostomy was revised because of recurrent bleeding. Since then, he had had infrequent episodes of rectal bleeding. Approximately 2 months before the present admission, he had noticed an increase in the frequency and severity of these episodes, requiring multiple blood transfusions. In addition, he had noticed intermittent painless hematuria. Physical examination disclosed a functioning diverting colostomy located in the left upper quadrant, with guaiac-negative stool. Anal inspection did not reveal any abnormalities, but on digital examination, bright red blood was noticed. A large left scrotal mass consistent with varicocele was palpated. No hemihypertrophy or varicosities were noted on examination of the extremities.

The hematocrit value was 25%, with a mean corpuscular volume of 79 fL. Flexible sigmoidoscopy showed extensive submucosal vascular lesions resembling varicosities that extended from the rectum to the proximal sigmoid colon. The mucosa was covered with fresh blood, but no definite bleeding site was identified. Computed tomography of the abdomen and pelvis showed low-density masses with multiple phleboliths involving the entire sigmoid and rectum, consistent with hemangiomas. Hemangiomas and varicosities were identified in the anterior abdominal wall, suprapubic area, urinary bladder, and scrotum. A large calcified cystic-appearing mass in the abdominal cavity measuring 8 x 6 x 5 cm was also identified (Fig 2).

Figure 2. Computed tomography shows large lymphangioma (broad arrow), thickened sigmoid colon due to intramural hemangiomatosis with calcifications (curved arrow), and urinary bladder wall hemangioma (thin arrow.)

Exploratory laparotomy was done, with abdominoperineal resection of the rectosigmoid, coloanal anastomosis, resection of the calcified abdominal mass, and partial cystectomy. Biopsy results of the bladder lesion and the rectosigmoid were consistent with cavernous hemangioma with transmural involvement; the calcified mass was consistent with a lymphangioma. The patient was discharged on postoperative day 7 in excellent condition.