ABSTRACT
Congenital absence of portal vein and extrahepatic portocaval shunt, also referred to as an Abernethy type 1, is a rare malformation of the mesenteric vasculature. A 9-year-old girl presented with dyspnea on exertion and orthodeoxia. A diagnosis of an Abernethy malformation type 1b and hepatopulmonary syndrome (HPS) was made, and she underwent auxiliary partial orthotopic liver transplantation (APOLT). Symptoms and signs of HPS resolved 2 months after the operation. HPS in Abernethy syndrome is a manifestation of normal portal blood flow bypassing the liver and can be ameliorated by redirecting blood flow to a segment of liver with normal portal anatomy. APOLT is a feasible and successful surgical procedure for patients with Abernethy malformation and HPS.
Subject(s)
Hepatopulmonary Syndrome/surgery , Liver Circulation , Liver Transplantation , Portal Vein/abnormalities , Child , Dyspnea/etiology , Dyspnea/physiopathology , Dyspnea/surgery , Female , Hepatopulmonary Syndrome/complications , Hepatopulmonary Syndrome/pathology , Hepatopulmonary Syndrome/physiopathology , Humans , Magnetic Resonance Angiography , Phlebography , Portal Vein/pathology , Portal Vein/physiopathology , Regional Blood Flow , Treatment OutcomeABSTRACT
Primary spontaneous pneumothorax occasionally occurs in young healthy individuals. Most of the time, it is diagnosed before it progresses to acute respiratory failure. We present a healthy young girl who developed acute respiratory failure secondary to primary spontaneous pneumothorax. Prompt evaluation and management are essential because this entity can lead to fatal outcome if not diagnosed and treated immediately.
