• • Diffuse Cavernous Hemangioma of the Colon in the Klippel-Trenaunay Syndrome 1 Gary G. Ghahremanl, M.D., Hushang Kangarloo, M.D., Frank Volberg, M.D., and Morton A. Meyers, M.D. Large, infiltrative cavernous hemangiomas of the distal colon were present in 3 children with the Kiippel-Trenaunay syndrome, 2 of whom are the subject of this report. The le- sion is manifested clinically as intermittent rectal bleeding starting in the first 5 years of life. The radiographic features include (a) varicose lesions in the wall of the rectosig- moid, ranging from a network of distended submucosal veins to nodular defects, (b) nar- rowing of the rectum by the surrounding extramural portion of the hemangioma, and (c) phleboliths within the lesion. Inferior mesenteric angiography and sigmoidoscopy are useful for preoperative confirmation. The occurrence of visceral hemangiomas in the Klippel-Trenaunay syndrome and its relevant diagnostic and therapeutic implications are discussed. INDEX TERMS: Abdomen, calcification. Angioma • Colon, calcification. Colon, neo- plasms. Klippel-Trenaunay Syndrome Radiology 118:673-678, March 1976 • • Pediatric Radiology V ASCULAR dysplasias are uncommon in the gastro- intestinal tract; when present, they generally occur as localized lesions of the colon or rectum (2, 3, 10, 17), where they tend to cause intermittent and some- times massive rectal hemorrhage. This is the usual pre- senting symptom of diffuse cavernous hemangioma of the colon (DCHC). The lesion is manifested clinically in early life, but the correct diagnosis often is not made until adulthood (1, 8, 13, 16). About 50 cases of DCHC have been reported thus far, and a characteristic pat- tern has emerged which should permit an early and ac- curate diagnosis in most instances. We wish to report 2 cases of large, infiltrative cavernous hemangioma in- volving the left side of the colon in children. Both pa- tients had the classic triad of cutaneous hemangiomas, bone and soft-tissue hypertrophy of the lower extremi- ties, and congenital varicose veins characteristic of the Klippel-Trenaunay syndrome (8, 9, 11, 12). The occur- rence of visceral hemangiomatosis in this rare syn- drome was originally mentioned in a clinical report of our second case (8). However, the striking radiological features of DCHC in patients with the Klippel-Trenaunay syndrome have not been described previously. The clini- cal significance of this association will be emphasized. CASE REPORTS CASE I: An 8-year-old black boy was admitted because of fever, swollen and painful lower extremities, and profuse rectal bleeding. Intermittent passage of fresh blood in the stools had first been noted three years earlier, and a diagnosis of proctitis associated with hem- orrhoids had been made elsewhere. Physical examination revealed that the patient was severely anemic, with edematous legs which were markedly tender on palpation. The circumference of the left calf was 2.5 cm larger than the right. There was a cord-like superfi- cial mass in the scrotum and a blue angiomatous tumor within the glans penis. On rectal examination, blood oozed from several irregu- lar, firm masses in the anorectal region. Laboratory data included a hemoglobin level of 5.4 g/100 ml, a white blood cell count of 10,600/mm 3 , microcytic hypochromic anemia, and elevated IgGand IgM immunoglobulins. Clotting factors and bleeding times were within normal limits. Radiographs of the legs showed several phleboliths in the soft tissues of the right foot with a moderate degree of hypertrophy and accelerated maturation of the bones of the left leg. Numerous faintly opaque phleboliths were also noted in the pelvis, conforming to the general contour of the rectum. Barium enema examination demon- strated widening of the presacral space, tubular narrowing of the rectum, and many small, nodular indentations in the rectosigmoid wall (Fig. 1). Proctosigmoidoscopy under general anesthesia revealed irregu- larly elevated, inflamed, and friable rectal mucosa. There were ex- tensive bluish angiomatous submucosal structures in the rectosig- moid, some of which appeared to be indurated. Biopsy of the lateral rectal wall resulted in brisk hemorrhage which was controlled by li- gation. These findings and microscopic examination of the biopsy material confirmed the diagnosis of diffuse cavernous hemangioma. Following repeated blood transfusions and medical management, the rectal bleeding ceased and anemia was corrected. The pedal edema and tenderness of the legs also disappeared. Venography of the lower extremities subsequently documented diffuse phlebecta- sia of the deep venous system, which intercommunicated via many tortuous vessels and had deformed valves (Fig. 2). The superficial veins were incompletely opacified, but the delayed radiographs sug- gested retention of contrast material within a hemangioma in the left popliteal fossa. Angiographic studies of the abdominal aorta, femo- ral arteries, and inferior vena cava were unremarkable. The patient was readmitted 1% years later because of recurrence of grossly bloody stools and development of a perianal abscess. Se- lective inferior mesenteric angiography demonstrated markedly de- layed retention of contrast material in the sinusoids of the hemangi- oma of the rectosigmoid and scrotum (Fig. 3). A definite source of hemorrhage was not recognized on this study. At surgery, several bleeding sites in the rectal wall were ligated. Due to diffuse exten- sion of the hemangioma into all layers of the rectosigmoid wall, rad- ical resection of these segments and permanent colostomy were 1 From the Departments of Radiology, Virginia Commonwealth University/Medical College of Virginia Hospitals. Richmond, Va. (G. G. G., H. K.) and New York Hospital and Memorial Hospital for Cancer and Allied Diseases/Cornell University Medical Center, New York, N. Y. (F. V., M. A. M.). Accepted for publication in September 1975. sjh 673