Disorders of the neonatal liver and bile ducts.

INTRODUCTION Liver and biliary tract disorders in the neonate are relatively rare and often complex. AIMS To evaluate the incidence of neonatal liver and biliary tract disorders, main causes, clinical presentation, treatment and outcome. MATERIAL AND METHODS Clinical, imagiological, laboratory, pathological and autopsy data concerning all newborns with liver and biliary tract disorder admitted to the neonatal intensive care unit of five tertiary medical centers from the north of Portugal, between 1997 and 2006, were retrospectively analysed. RESULTS 77 neonates (incidence 0.5% - 77/14505 admissions); 44M/33F; gestational age 34 weeks (25-41); preterm 50 (65%); birthweight 1980 g (570-4130), < 1500 g 29 (38%). Several causes were identified and classified as infectious, metabolic, anatomic/structural, neoplastic, vascular, traumatic, immune, genetic and idiopathic. Clinical signs appeared between days 1 and 61 of life. Jaundice was the most frequent clinical sign (92%). Cholestasis occurred in 67 (87%) patients. Duration of hospital stay was 35 days (5-146); 18 patients (23%) were deceased. Autopsy study was diagnostic in 8 cases (10%). CONCLUSIONS Nosocomial and intrauterine infection were the most common causes of liver and biliary tract disease. Several other rare causes represented an important challenge in diagnosis and treatment, and some were fatal. Awareness of the spectrum of liver and bile duct disorders in the neonate and recognition of the key clinical features are essential to optimize outcome.