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

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.