Hematuria and Rectal Bleeding in the Child with Klippel and Trenaunay Syndrome

Abstract

We have operated upon 588 patients with Klippel and Trenaunay syndrome. The underlying factor is a congenital malformation of the deep veins: agenesis, atresia or compression by fibrovascular bands of the popliteal, femoral or iliac veins. Of these 588 patients, 6 children between 15 months and 4 years of age had severe rectal bleeding and hematuria. One of these children died from massive bleeding of the rectum with septicemia. Another boy was saved by rectal resection and the last one by subtotal cystectomy. The important venogram shows an absence of the anterior venous pathway (superficial femoral vein) compensated by the abnormal development of 2 venous groups, the vein of the sciatic nerve and large veins along the external aspect of the inferior limb. These 2 venous groups penetrate into the pelvis by the sciatic and gluteal notches and terminate in the internal iliac vein which becomes enormous and has a very high flow. This overflow hinders drainage of the venous collateral from the rectum, the bladder and the vagina. The retro adductor vein, prolongated by the deep femoral vein, represents an anastomosis between the sciatic nerve vein and the common femoral vein. The surgeon must try to widen this pathway.

Involvement of Splenic Hemangioma and Rectal Varices in a Patient with Klippel - Trenaunay Syndrome

“…Gastrointestinal hemorrhage usually presents in the first decade of the patient's life and tends to be intermittent. 5 In the present case, the patient had suffered from recurrent hematochezia for several years and was diagnosed with rectal varix and iron deficiency anemia. In general, hematochezia may be caused by the posterior or compensatory venous pathways of the extremity, which drain into the internal vein.…”

“…2 The most common involvement in the gastrointestinal tract is diffuse cavernous hemangiomas of the distal colon and the rectum. 5,6 In one of the largest published studies of KTS patients, hematochezia was observed in six of the 588 patients. 3 Although rare, bleeding from jejunal hemangiomas and esophageal and gastric varices also have been reported.…”

Massive Hematuria in Adults with Klippel-Trenaunay Syndrome Associated with Vascular Malformation of the Bladder

“…Hematuria is a common symptom in patients with genitourinary and pelvic vascular malformation necessitating therapeutic intervention. Hematuria as a presenting symptom has been reported in children [1][2][3], but massive hematuria in adults associated with KTS is rare [7][8][9]. We report 2 cases of hematuria in adults with KTS.…”

“…Although the lower extremity is involved in the majority of cases [1], pelvic vascular involvement appears to be responsible for rectal bleeding and hematuria in children with this syndrome [3]. Both hemangioma and vascular malformations have been reported [1][2][3]. Hemangiomas are true benign tumors characterized by proliferating endothelial cells while congenital vascular malformations are defects of vascular morphogenesis which tend to be diffuse [4].…”

“…Congenital vascular malformations are usually located in the extremities [1]. However, the genitourinary tract may be involved [2,3].…”