Catastrophic intraoperative bleeding due to congenital extrahepatic porto-systemic shunt anomaly: A surgical case report of two rare anomalies

摘要

Introduction Abernethy malformations are extremely rare congenital anomalous portosystemic shunts. We report the case of a patient with a rare variant Abernethy malformation between the superior mesenteric vein and left renal vein, associated with a massive jejunal diverticulum. Presentation of case A 37-year-old Caucasian female presented to our emergency department with severe abdominal pain and proceeded to laparotomy for a presumed small bowel obstruction. At laparotomy she was found to have a massive diverticulum at the duodeno-jejunal junction, which was intimately associated with a venous malformation and the anomalous portosystemic shunt. Whilst mobilising the diverticulum, the patient developed catastrophic haemorrhage from the malformation. The patient underwent a complicated post-operative course however was eventually stabilised. Discussion We discuss the anatomy and pathophysiology of anomalous portosystemic shunts and propose an embryological origin for our patients’ anomalies. Conclusion Abernethy malformations are rare however may be associated with other intra-abdominal pathology and extreme caution is required when operating on these patients.