Case Report
Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Mar 21, 2016; 22(11): 3289-3295
Published online Mar 21, 2016. doi: 10.3748/wjg.v22.i11.3289
Delayed diagnosis of alpha-1-antitrypsin deficiency following post-hepatectomy liver failure: A case report
Benjamin Norton, Jemimah Denson, Christopher Briggs, Matthew Bowles, David Stell, Somaiah Aroori
Benjamin Norton, Peninsula College of Medicine and Dentistry, Tamar Science Park, Plymouth PL6 8BU, United Kingdom
Jemimah Denson, Department of Histopathology, Plymouth Hospitals NHS Trust, Plymouth PL6 8DH, United Kingdom
Christopher Briggs, Matthew Bowles, David Stell, Somaiah Aroori, Peninsula HPB Unit, Derriford Hospital, Plymouth PL6 8DH, United Kingdom
Author contributions: Norton B and Aroori S wrote the paper; Denson J, Briggs C, Bowles M, Stell D and Aroori S were the doctors involved in the case, and advised with regard to the research; Aroori S provided the CT images and Denson J provided the histology images; all authors have read and approved the final manuscript.
Institutional review board statement: This case report was exempt from the Institutional Review Board standards at Peninsula School of Medicine and Dentistry.
Informed consent statement: The patient’s relative involved in this study gave there verbal informed consent authorising use and disclosure of there protected health information.
Conflict-of-interest statement: All the authors have no conflicts of interests to declare.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Somaiah Aroori, MS, MD, FRCS, EBS in HPB Surgery, Consultant HPB Surgeon, Peninsula HPB unit, Derriford Hospital, PL6 8DH Plymouth, United Kingdom. s.aroori@nhs.net
Telephone: +44-7837388342
Received: May 24, 2015
Peer-review started: May 25, 2015
First decision: July 19, 2015
Revised: September 5, 2015
Accepted: November 30, 2015
Article in press: December 1, 2015
Published online: March 21, 2016
Processing time: 294 Days and 14.9 Hours
Abstract

Post-hepatectomy liver failure (PHLF) is a leading cause of morbidity and mortality following major liver resection. The development of PHLF is dependent on the volume of the remaining liver tissue and hepatocyte function. Without effective pre-operative assessment, patients with undiagnosed liver disease could be at increased risk of PHLF. We report a case of a 60-year-old male patient with PHLF secondary to undiagnosed alpha-1-antitrypsin deficiency (AATD) following major liver resection. He initially presented with acute large bowel obstruction secondary to a colorectal adenocarcinoma, which had metastasized to the liver. There was no significant past medical history apart from mild chronic obstructive pulmonary disease. After colonic surgery and liver directed neo-adjuvant chemotherapy, he underwent a laparoscopic partially extended right hepatectomy and radio-frequency ablation. Post-operatively he developed PHLF. The cause of PHLF remained unknown, prompting re-analysis of the histology, which showed evidence of AATD. He subsequently developed progressive liver dysfunction, portal hypertension, and eventually an extensive parastomal bleed, which led to his death; this was ultimately due to a combination of AATD and chemotherapy. This case highlights that formal testing for AATD in all patients with a known history of chronic obstructive pulmonary disease, heavy smoking, or strong family history could help prevent the development of PHLF in patients undergoing major liver resection.

Keywords: Post-hepatectomy liver failure; Alpha-1-antitrypsin deficiency; Hepatectomy; Functional liver remnant; Liver resection

Core tip: A report of a 60-year-old male who underwent an extended right hepatectomy for metastatic colorectal adenocarcinoma. He subsequently developed post-hepatectomy liver failure secondary to a delayed diagnosis of alpha-1-antitrypsin deficiency. Clinicians should be aware of, and test for, the possibility of undiagnosed alpha-1-antitrypsin deficiency in patients with known chronic obstructive pulmonary disease or a family history, undergoing major liver resection.