This case report describes a 13-year 10-month-old girl who underwent a deceased-donor split LT for primary diagnosis of biliary atresia at the age of 12 months, who presented with a lower GI bleed. Ultrasound and CT revealed a venous vascular anomaly involving the cecum and ascending colon, with communication of the SMV and pelvic veins consistent with a CEPS. Associated varices were noted in the pelvis along the uterus and urinary bladder. These findings were confirmed by trans-hepatic porto-venography, which was diagnostic and therapeutic as a successful embolization of the CEPS was performed using micro-coils. There were no complications following the procedure and no further GI bleeding occurred, illustrating the efficacy of this treatment option for CEPS. We discuss the literature regarding the presenting complaint of GI bleeding post-LT, CEPS as a rare cause of GI bleeding and its association with PV, and the classification and treatment of CEPS.

Gastrointestinal stromal tumors (GISTs) represent the majority of primary nonepithelial neoplasms of the digestive tract, most frequently expressing the KIT protein detected by immunohistochemical staining for the CD117 antigen. Jejunal GISTs account for approximately 10 % of GISTs. Patients usually present with abdominal discomfort. Jejunal GISTs may cause symptoms secondary to obstruction or hemorrhage. Pressure necrosis and ulceration of the overlying mucosa may cause gastrointestinal bleeding, and patients who experience significant blood loss may suffer from malaise and fatigue. Literature has classified small-bowel GISTs on the basis of size, and various established guidelines have advised conservative management of small jejunal GISTs (<2 cm). We here report the clinical, macroscopic, and immunohistological features of a small jejunal GIST presenting with acute lower gastrointestinal hemorrhage in a 50-year-old postmenopausal woman necessitating an emergency laparotomy to control the bleed. The management of very small (<2 cm) small-bowel GISTs is controversial. While guidelines are primarily based on the risk of malignancy in GISTs, no guideline predicting the risk of complications in small-bowel GISTs exists. Hence, these tumors should be removed even if incidentally detected.