Purpose or Case Report: the aim of the study is to report the importance of the link between intracranial bleeding (ICB) and biliary atresia in neonates. Nowadays, the incidence of bleeding secondary to vitamin K deficiency has well-decreased, thanks to systematic vitamin K prophylaxis in neonates. However, ICB remains a severe complication of Vitamine K deficiency, with a high mortality rate and possible neurological disabilities.
Biliary atresia (BA) is a life-threatening condition in neonates, commonly diagnosed in the first 2 months of life, based on jaundice, cholestasis, pale-colored stools and dark urine. Occasionally, BA can be revealed later (25 to 65 days after birth), with severe symptoms such as intracranial, nasal or gastrointestinal bleedings.
Methods & Materials:
Results: A 2 month-old boy was admitted in the emergency department with anorexia, vomiting, lethargy, and impaired consciousness, without fever. He also was slightly jaundiced with normal stools. He was born full-term and breastfed with a vitamin K supplementation as recommended. The examination revealed a 3-centimeter hematoma in the lumbar area and a bulging fontanelle. Cerebral CT-scan and MRI were performed rapidly and showed subdural hemorrhages and a frontal right intraparenchymal hematoma. Laboratory tests reflected anemia, cholestasis and coagulation disorder with deficiency of vitamin K-dependent factors. Surgical evacuation of intracranial hemorrhage was performed after intravenous vitamin K. An abdominal ultrasound showed afterwards an atrophic gallbladder, compatible with the diagnosis of BA without other ultrasonography signs of BA. Patient's stools and urine became respectively pale-colored and dark but only after the onset of neurological bleeding symptoms
The child was then rapidly transferred to another pediatric hospital, in order to perform a Kasaï intervention. The surgery went well but was complicated a few weeks later by a cytomegalovirus infection and a cholangitis.
Conclusions: BA should be strongly considered in infants up to 2 month old, presenting with a sudden tendency to bleed, especially when vitamin K-dependent factors are decreased. The presentation of BA can be atypical and revealed primarily by neurological symptoms, without any modification of stools or urine. ICB can be severe and life-threatening, hence the necessity of knowing these atypical presentations of BA.