title: Delayed diagnosis of alpha-1-antitrypsin deficiency following post-hepatectomy liver failure: A case report.
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.
authors.affiliation: Benjamin Norton, Peninsula College of Medicine and Dentistry, Tamar Science Park, Plymouth PL6 8BU, United Kingdom.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Norton
authors.affiliation: Benjamin Norton, Peninsula College of Medicine and Dentistry, Tamar Science Park, Plymouth PL6 8BU, United Kingdom.
authors.forename: Jemimah
authors.initials: J
authors.lastname: Denson
authors.affiliation: Benjamin Norton, Peninsula College of Medicine and Dentistry, Tamar Science Park, Plymouth PL6 8BU, United Kingdom.
authors.forename: Christopher
authors.initials: C
authors.lastname: Briggs
authors.affiliation: Benjamin Norton, Peninsula College of Medicine and Dentistry, Tamar Science Park, Plymouth PL6 8BU, United Kingdom.
authors.forename: Matthew
authors.initials: M
authors.lastname: Bowles
authors.affiliation: Benjamin Norton, Peninsula College of Medicine and Dentistry, Tamar Science Park, Plymouth PL6 8BU, United Kingdom.
authors.forename: David
authors.initials: D
authors.lastname: Stell
authors.affiliation: Benjamin Norton, Peninsula College of Medicine and Dentistry, Tamar Science Park, Plymouth PL6 8BU, United Kingdom.
authors.forename: Somaiah
authors.initials: S
authors.lastname: Aroori
mesh_terms: D000230:Adenocarcinoma / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000556:secondary / Q000601:surgery*; D001706:Biopsy; D017024:Chemotherapy, Adjuvant / Q000009:adverse effects; D015179:Colorectal Neoplasms / Q000473:pathology*; D057210:Delayed Diagnosis; D017809:Fatal Outcome; D006498:Hepatectomy / Q000009:adverse effects*; D006801:Humans; D017114:Liver Failure, Acute / Q000175:diagnosis / Q000209:etiology*; D008113:Liver Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000556:secondary / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications / Q000175:diagnosis*
keywords: Alpha-1-antitrypsin deficiency; Functional liver remnant; Hepatectomy; Liver resection; Post-hepatectomy liver failure