A rare case of spontaneous hyphema secondary to gestational alloimmune liver disease.

Hyphema is rarely seen in neonates. Although most cases are secondary to instrument-assisted delivery, neonatal hyphema can occur spontaneously or result from an underlying coagulopathy. We report the case of an infant who was born with unilateral hyphema and was subsequently found to have gestational alloimmune liver disease-a condition where maternal antibodies attack the infant's liver, leading to a hypocoagulable state.

Staged biliary reconstruction after liver transplantation: A novel surgical strategy for high acuity pediatric transplant recipients.

Introduction: Biliary complications after pediatric orthotopic liver transplantation remain causes of significant patient morbidity. Staged operative approach in complex hepatobiliary surgery has improved postoperative outcomes but has not been evaluated in pediatric orthotopic liver transplantation. We sought to analyze the outcomes of staged biliary reconstruction after orthotopic liver transplantation in high acuity patients.

Frailty in Children with Liver Disease: A Prospective Multicenter Study.

Objective: To assess frailty, a measure of physiologic declines in multiple organ systems, in children with chronic liver disease using a novel pediatric frailty tool.

Study Design: We performed a prospective cross-sectional multicenter study at 17 liver transplantation (LT) centers. 71 children (5-17 years of age), 36 with compensated chronic liver disease (CCLD) and 35 with end-stage liver disease (ESLD) and listed for LT, were assessed for frailty using validated pediatric tools to assess the 5 classic Fried Frailty Criteria-slowness, weakness, exhaustion, diminished physical activity, and shrinkage.

Type 2 Abernethy malformation presenting as a portal vein-coronary sinus fistula.

We report the case of a child with Abernethy malformation with an anomalous connection between the portal vein and the coronary sinus. After 30 months of close follow-up, the patient developed hepatoblastoma, a previously documented complication of the Abernethy malformation. This case reports a unique variant of Abernethy malformation and documents the first reported case of hepatoblastoma in a patient with type 2 Abernethy malformation.

HIDA, percutaneous transhepatic cholecysto-cholangiography and liver biopsy in infants with persistent jaundice: can a combination of PTCC and liver biopsy reduce unnecessary laparotomy?

Background: Historically, HIDA is the initial diagnostic test in the evaluation of biliary atresia (BA). Non-excreting HIDA scans can yield false-positive results leading to negative laparotomy.

Objective: Cholestatic infants must be evaluated promptly to exclude biliary atresia (BA) and other treatable hepatic conditions.

Inflammatory bowel disease in children 5 years of age and younger.

Objectives: Clinicians are becoming increasingly aware that inflammatory bowel disease (IBD) can affect all age groups, although it has not been well described in infants and young children. Our aim was to evaluate early onset IBD in patients 5 yr of age and younger.

Methods: Medical records of patients diagnosed with early onset IBD at The Children's Hospital of Philadelphia between 1977 and 2000 were reviewed.