Growing Concerns: A 3-Year-Old Girl with Multiple Hepatic Masses and Gastrointestinal Bleeding

Excerpt

A 5-month-old female was observed to have massive hematemesis 48 h after surgical closure of a patent ductus arteriosus. Her prior medical history was significant for: intrauterine growth retardation, with a birth weight of 2 kg; delivery through a scheduled Cesarean section due to breech presentation; and a maternal history of antiphospholipid antibodies and thrombophilia with six prior miscarriages. An esophagogastroduodenoscopy (EGD) was performed emergently at the bedside; although the examination was limited due to respiratory instability, it was grossly normal except for a large portion of the stomach being obscured by a clot. Although she was initially stabilized, she had an acute drop in hemoglobin four days later. An abdominal ultrasound demonstrated cavernous transformation of the porta hepatis without visualization of portal vein, corroborated by magnetic resonance imaging (MRI), which additionally revealed esophageal and rectal varices and splenomegaly (Fig. 1a). Repeat EGD demonstrated multiple grade 4 varices in the distal esophagus (Fig. 2a). After receiving sclerotherapy to the largest varices, she had no further bleeding and was discharged home. Since an evaluation for hypercoagulability during admission was negative, it was presumed that at some point she may have developed a portal vein thrombus from maternal transfer of antiphospholipid antibodies with subsequent portal hypertension, though no thrombus was ever visualized by imaging.

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