Giant Cell Hepatitis and Primary Peritonitis Complicating Neonatal Escherichia coli Sepsis and Meningitis

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Purpose: Giant cell hepatitis (GCH) and primary peritonitis complicating neonatal sepsis and meningitis is very unusual. We report a 14-day-old male infant who presented with fever, decreased oral intake and increased irritability. One week prior to arrival he had undergone circumcision. Physical examination was remarkable for ill-appearing lethargic infant with a temperature of 99.4 rectally, tachycardia, scleral and skin icterus, distended abdomen with hypoactive bowel sounds, enlarged left scrotal area with ecchymosis. Laboratory data revealed a white blood cell count 11.0 cmm, Platelet count 127,000 cmm, granulocytes 49%, bands 11%, lymphocytes 31%, monocytes 8%, Na 140 meq/L, K 3.9 meg/L, Cl 107 meq/L, bicarb 14 meq/L, BUN 17 mg/dL, creatinine 0.3 mg/dL, glucose 74 mg/dL, total bilirubin 18.7 mg/dL, direct bilirubin 0.5 mg/dL, albumin 2.5 g/dL, alkaline phosphatase 438 u/L, AST 95 u/L, ALT 97 u/L, anion gap 19, PT 16.1 sec, PTT 29.4 sec, and INR 1.56. Cerebrospinal fluid (CSF) revealed protein 145 mg/dL, glucose 0 mg/dL, WBC 1255 cmm. RBC 13 cmm, monocytes 19/100 cells, Gram stain 4+ gram-negative rods. Pt was started on cefotaxime and ampicillin. Blood and CSF culture grew out Escherichia coli. Within 12 hours of admission, the patient clinically deteriorated further with bilious vomiting, progressive abdominal distension, shock and respiratory failure. An exploratory laparotomy revealed peritonitis and miliary nodules on the liver. Peritoneal fluid culture grew E. coli. Liver biopsy revealed extensive syncytial giant cell transformation of hepatocytes representing GCH. Metabolic work-up for inborn errors of metabolism including galactosemia, tyrosinemia, and phenylketonuria were negative. Urine was negative for reducing substances and organic acids. Alpha-1-antitrypsin deficiency was excluded. The infant received three weeks of IV cefotaxime therapy and made a slow clinical recovery with resolution of the hepatitis. The differential diagnosis of GCH is broad and included infectious etiologies; congenital infection (i.e., TORCH organisms), hepatitis with hemolytic anemia, paramyxovirus, rubeola, and HIV infection. Ductal cholestasis resulting from biliary atresia or hypoplasia, or a choledochal cyst can also cause formation of giant cells, as can metabolic diseases such as neonatal hemochromatosis, alpha-one-antitrypsin deficiency, and galactosemia, and inborn errors of bile acid metabolism. This case serves a reminder that GCH can occur due to E. coli sepsis. Animal studies have suggested that giant cell transformation of the liver occurs through induction by the E. coli endotoxin supporting the theory that the giant cell transformation is the immature/neonatal liver's reaction insulting aggressive stimuli.