Klippel-Trenaunay syndrome with gastrointestinal bleeding, splenic hemangiomas and left inferior vena cava

Abstract

Klippel-Trenaunay syndrome is a congenital vascular anomaly characterized by a triad of varicose veins, cutaneous capillary malformation, and hypertrophy of bone and (or) soft tissue. Gastrointestinal vascular malformations in Klippel-Trenaunay syndrome may present with gastrointestinal bleeding. The majority of patients with splenic hemangiomatosis and/or left inferior vena cava are asymptomatic. We herein report a case admitted to the gastroenterology clinic with life-threatening hematochezia and symptomatic iron deficiency anemia. Due to the asymptomatic mild intermittent hematochezia, splenic hemangiomas and left inferior vena cava, the patient did not seek any help for gastrointestinal bleeding until his admittance to our department for evaluation of massive gastrointestinal bleeding. He was referred to angiography because of his serious pathogenetic condition and inefficiency of medical therapy. The method showed that hemostasis was successfully achieved in the hemorrhage site by embolism of corresponding vessels. Further endoscopy revealed vascular malformations starting from the stomach to the descending colon. On the other hand, computed tomography revealed splenic hemangiomas and left inferior vena cava. To the best of our knowledge, this is the first Klippel-Trenaunay syndrome case presenting with gastrointestinal bleeding, splenic hemangiomas and left inferior vena cava. The literature on the evaluation and management of this case is reviewed.

Klippel-Trenaunay Syndrome of the Rectosigmoid Colon Presenting as Severe Anemia

“…The vascularity of the lesions makes surgical repair an arduous task. Selective angiography with embolization and clipping has been utilized emergently as a bridge to surgery and to reduce risk of intraoperative bleeding 8,9. Proximal embolization reduces the perfusion pressure to the malformation but does not eliminate it.…”

Klippel-Trenaunay Syndrome Causing Life-Threatening GI Bleeding: A Case Report and Review of the Literature

“…Esophageal varices secondary to prehepatic portal hypertension from cavernous transformation or hypoplasia of portal vein can be another significant source of GI bleeding [15–17]. Vascular malformations involving the whole GI tract are rare [5]. …”

“…These lesions should not be biopsied since it may lead to severe bleeding [11, 24]. The whole GI tract should be evaluated endoscopically for the exact localization and extension of the vascular malformations and for effective management of GI hemorrhage [5]. Therapeutic interventional measurements are used when patients do not tolerate endoscopy in case of active bleeding.…”

“…Vascular malformations have been reported to affect the gastrointestinal (GI) tract, liver, spleen, and heart and follow a progressive course [2, 5]. Left-sided inferior vena cava (IVC), secondary to persistence of the left and regression of the right supracardinal vein, has been reported [5, 6].…”

“…Left-sided inferior vena cava (IVC), secondary to persistence of the left and regression of the right supracardinal vein, has been reported [5, 6]. Vascular malformations can also affect genitourinary tract (kidney, bladder, penis, scrotum, vagina, and vulva) and manifest as intrapelvic or retroperitoneal vascular masses [7].…”