title: Chronic diarrhea, ascites, and protein-losing enteropathy in an infant with hepatic venous outflow obstruction after liver transplantation.
abstract: An 18-month-old female status post-orthotopic liver transplant for biliary atresia presented nine months after transplant with severe diarrhea and intolerance of feeds. She was found to have a PLE as evidenced by a low serum albumin and a persistent elevation of fecal A1AT. Investigation eventually revealed that the cause of the PLE was a stricture at the anastomosis site between the hepatic vein and inferior cava, supported by resolution of the PLE after venoplasty of the stricture. The patient has subsequently required several repeat venoplasties for recurrence of her symptoms correlating with recurrence of the stricture. This is a very rare presentation of hepatic venous outflow obstruction. Moreover, normal duplex ultrasound imaging of liver vasculature and her unusual presentation led to a delay in her diagnosis highlighting the need for an increased index of suspicion.
authors.affiliation: Division of Pediatric Gastroenterology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
authors.forename: S K
authors.initials: SK
authors.lastname: Hourigan
authors.forename: R A
authors.initials: RA
authors.lastname: Anders
authors.forename: S E
authors.initials: SE
authors.lastname: Mitchell
authors.forename: K B
authors.initials: KB
authors.lastname: Schwarz
authors.forename: H
authors.initials: H
authors.lastname: Lau
authors.forename: W
authors.initials: W
authors.lastname: Karnsakul
mesh_terms: D000714:Anastomosis, Surgical; D001201:Ascites / Q000150:complications* / Q000209:etiology; D006502:Budd-Chiari Syndrome / Q000150:complications / Q000209:etiology*; D003251:Constriction, Pathologic; D003967:Diarrhea / Q000150:complications* / Q000209:etiology; D005260:Female; D006801:Humans; D007223:Infant; D008099:Liver / Q000000981:diagnostic imaging / Q000473:pathology; D017093:Liver Failure / Q000150:complications* / Q000628:therapy; D016031:Liver Transplantation / Q000009:adverse effects*; D011183:Postoperative Complications; D011504:Protein-Losing Enteropathies / Q000150:complications* / Q000209:etiology; D012008:Recurrence; D012709:Serum Albumin / Q000378:metabolism; D014463:Ultrasonography / Q000379:methods; D014656:Vascular Surgical Procedures / Q000379:methods
chemical_list: D012709:Serum Albumin