Abernethy malformation (Type 1B) presenting in a 6-year-old boy with hematochezia and hematuria: a case report Abernethy malformation is a rare congenital vascular abnormality defined by the diversion of portal blood flow to the inferior vena cava or its tributaries. Clinical presentations include neonatal cholestasis, liver tumors, and encephalopathy but variables in timing and symptomatology. Herein, we present a 6-year-old boy was referred to our hospital with complaints of hematochezia, hematuria, fecal, and urinary incontinence. A diagnosis of type 1b malformation was made depending on magnetic resonance angiography and cardiac catheterization findings, which demonstrated that the superior mesenteric vein and splenic vein joined to form a common trunk measuring 38 mm diameter and then drained into the dilated inferior vena cava with the absence of portal vein abnormalities in the liver. With further investigations, we indicated the presence of many arteriovenous malformations and urogenital abnormalities. The patient was managed conservatively.
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http://dx.doi.org/10.1016/j.radcr.2022.06.045 | DOI Listing |
Introduction Fetal extrahepatic portosystemic Venous Shunt (FEPSVS) are vascular malformations that divert placental and bowel blood from the liver into the systemic circulation. When uncorrected, it can lead to severe pathologic consequences after birth. Objective To report our method of prenatal diagnosis, the developing insight regarding prenatal counseling, and postnatal treatment.
View Article and Find Full Text PDFArab J Gastroenterol
January 2025
Department of Radiology, West China Hospital, Sichuan University, Chengdu, PR China. Electronic address:
Congenital extrahepatic portosystemic shunt, also known as Abernethy malformation, is a rare anatomic vascular malformation. Patients with Abernethy malformation may present with abdominal pain, abnormal liver function tests, hepatopulmonary syndrome, pulmonary hypertension, and/or portosystemic encephalopathy. Accurate identification of the shunt and portal vein and effective management of complications is vital in these patients.
View Article and Find Full Text PDFExp Clin Transplant
October 2024
From the Department of General Surgery, Gazi University, Ankara, Türkiye.
Abernethy syndrome is a rare congenital vascular anomaly. In this condition, blood from the portal system is diverted to the caval system through a portocaval shunt, entirely or partially bypassing the liver. Prevalence figures in the literature range from 1 per 30000 population up to 1 per 50000 population.
View Article and Find Full Text PDFRadiol Case Rep
December 2024
Baylor University Medical Center, Department of Radiology, 3500 Gaston Avenue, Dallas, Texas, 75246, USA.
I review a case of a 30-year-old woman who presented with diarrhea, nausea and vomiting for several days. She was ultimately diagnosed with acute gastroenteritis but was incidentally found to have a congenital extrahepatic portosystemic shunt, also known as the Abernethy malformation. The Abernethy malformation, first described by Dr John Abernethy in the year 1793, is an exceptionally rare clinical entity.
View Article and Find Full Text PDFVasc Endovascular Surg
February 2025
Department of Vascular Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
Congenital extrahepatic portosystemic shunt (CEPS), also termed Abernethy malformation (AF) is a rare anomaly of the splanchnic venous system. Several approaches, including shunt closures through surgical or radiological interventions and liver transplantations, have been proposed, but clear comparisons among different treatment strategies are still unavailable. We report a case in which an unusual portosystemic shunt was present between the dilated inferior mesenteric vein (IMV) to the right ovarian vein.
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