Catastrophic intraoperative bleeding due to congenital extrahepatic porto-systemic shunt anomaly: A surgical case report of two rare anomalies. (2018)

• Record Type: Journal Article
• Authors: Mohtashami, Ali; Kiat, Andrew; Cross, Jane; Simon, Robert; Curtin, Austin
• Is Part Of: International journal of surgery case reports. Volume 44(2018)
• Page Start: 161
• Page End: 165
• Publication Date: 2018
• Subjects: CEPS anomalous extrahepatic porto-systemic shunt(s) -- CT computed tomography -- SMV superior mesenteric vein -- LRV left renal vein -- DJ duodeno-jejunal; Case report -- Abernethy malformation -- Porto-Systemic shunt -- Small bowel diverticulum; Surgery -- Periodicals; Surgical Procedures, Operative -- Periodicals; Surgery; Electronic journals; Periodicals; 617.005
• DOI: 10.1016/j.ijscr.2018.02.034
• Languages: English
• ISSNs: 2210-2612

Abstract

Highlights: A rare variation of Abernethy malformation with Associated small bowel anomaly. A case of emergency surgery in a patient with these anomalies. Pre-operative imaging and intra-operative findings are presented. The embryology of these anomalies pertaining to the case are discussed. Abstract: 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.