New-Onset Scleral Icterus in a School-Aged Female

Abstract

A 9-year-old, previously healthy, African American female was admitted with 2 weeks of mild, diffuse abdominal pain and 2 days of pronounced scleral icterus. She initially presented to an outside institution where she was noted to have marked hepatomegaly. Further examination at our institution was notable for an illappearing child with a pulse of 144 beats per minute and respiratory rate of 48 breaths per minute. She had a nontender, but distended, abdomen with a firm liver edge palpable 5 cm below the right costal margin. She had nonpitting edema in both lower extremities and palmar erythema.Laboratory studies revealed a microcytic anemia (hemoglobin 9.1 g/dL [range 11.5-15.5g/dL], mean corpuscular volume 76 fL [range 77-95 fL]), elevated transaminases (AST 419 U/L [range 22-55 U/L), ALT 78 U/L [range 11-30 U/L]), and hyperbilirubinemia (total bilirubin 5.5 mg/dL [range 0.2-1.0 mg/dL]). Serum α-fetoprotein was markedly elevated at 100 000 ng/mL. Abdominal ultrasound demonstrated a large, right-sided hepatic mass. Contrast-enhanced computed tomography (CT) scan was notable for a nodular liver, a large mixed density mass measuring 15 cm × 12 cm × 18 cm within the right hepatic lobe ( Figure 1A), multiple smaller hepatic lesions, splenomegaly, and diffuse pulmonary nodules concerning for metastatic disease. Hospital CourseThe patient underwent open wedge liver biopsy, which revealed a well-differentiated hepatocellular carcinoma (HCC) with active micronodular cirrhosis ( Figure 1B) that was HBsAg positive ( Figure 1C). α-Fetoprotein immunostaining was positive in the tumor cells ( Figure 1D). Investigation for an underlying etiology revealed positive serum HBsAg without any detectable hepatitis B surface antibody (anti-HBs). Hepatitis B virus (HBV) DNA was detected in the blood at 100 000 IU/mL and she was hepatitis B envelope antigen (HBeAg) positive. Her HBV genotype was type A with a P120T surface antigen mutation. Testing was negative for HIV-1, hepatitis C virus, and inherited cholestatic diseases that could predispose her to HCC.Evaluation of her mother's prenatal records revealed that the mother was HBsAg positive during her pregnancy. She was unaware of her diagnosis and denied receiving any further testing or treatment. At the birth hospital, the patient was born full term via Caesarean section due to failure to progress, and was given hepatitis B vaccine and hepatitis B immune globulin (HBIg) on day 1 of life. She received 3 additional doses of the hepatitis B vaccine at 3, 5, and 18 months of age. There were no records of an evaluation for hepatitis B infection, including HBsAg and anti-HBs testing after she completed her vaccination series. She was never breastfed. She did not have any other identified risk, either via blood-borne or sexual exposure, for hepatitis B virus infection by history.The patient rapidly deteriorated postbiopsy progressing to multisystem organ failure. She developed respiratory failure requiring endotracheal intubation and had echocardiogram findings consistent with hepatopulmo...