Excerpt
Methods: This is a retrospective review of autopsies from 14 patients diagnosed with AFLF or ACLF and listed for liver transplantation who died at our institution over 10 years (2004-2013).
Results: 325 patients were listed for liver transplant; 35 (10.8%) died prior to transplant. Autopsies were performed on 14 (40%). Diagnoses included biliary atresia (4/14), neonatal hemochromatosis (2/14), viral-induced hepatic failure (2/14), drug-induced hepatic failure (1/14), diffuse infantile hepatic hemangiomatosis (1/14), and liver failure of unclear etiology (4/14). 100% of patients required mechanical ventilation, 85% (12/14) required vasopressors, and 57% (8/14) had acute renal failure requiring dialysis. 50% (7/14) of patients had bacteremia, while only 14% (2/14) patients exhibited positive bacterial cultures on autopsy alone. 100% of patients exhibited severe coagulopathy. Cardiac arrest occurred in 21% (3/14). 14% (2/14) met criteria for brain death; the remaining 64% (9/14) withdrew medical support. Post-mortem examination revealed alveolar hemorrhage in 79% (11/14). Diffuse alveolar damage was seen in 43% (6/14). 50% (7/14) exhibited cardiomegaly and 43% (6/14) had evidence of ventricular hypertrophy. Cerebral edema was found in 64% (9/14) and 43% (6/14) showed presence of metabolic gliosis or Alzheimer’s type 2 astrocytosis. Renal tubular necrosis was seen in 64% (9/14) and 71% (10/14) had evidence of hepatomegaly or hepatocyte necrosis. Only 29% (4/14) exhibited areas of micro-thrombi.
Conclusions: This is the first study describing autopsy findings in children who have died from liver failure. This highlights the diversity of histopathology findings indicating that these patients die from complex multisystem organ failure due to primary liver dysfunction.