Intracranial Hemorrhage and Vitamin K Deficiency Associated with Biliary Atresia: Summary of 15 Cases and Review of the Literature

Abstract

Biliary atresia (BA) is a rare disease, characterized by progressive and obliterative cholangiopathy, and is one of the major causes of secondary vitamin K deficiency in infancy. We describe 15 infants (10 female, 5 male) with BA, presenting with intracranial hemorrhage (ICH), including 10 subdural hemorrhages, 4 subarachnoid hemorrhages, 2 intraventricular hemorrhages, and 1 intraparenchymal hemorrhage. The age at onset of ICH ranged from 26 to 79 (mean 54.2) days. Eight patients underwent successful surgical evacuation of ICH, following administration of vitamin K. All 15 patients underwent Kasai portoenterostomy for BA 8–30 days after onset. During a mean follow-up period of 86.8 (range 2–352) months, 4 patients died of liver failure despite lack of neurological sequelae. Two patients underwent living-related donor and 1 patient living-unrelated donor liver transplantation. Only 2 patients suffered neurological signs and symptoms, including mental retardation and epilepsy, whereas 3 were noted to have temporary hemiparesis which recovered completely during the follow-up period. The possibility of BA should be considered in the treatment of ICH due to vitamin K deficiency, since it is reported to be one of the major causes of secondary vitamin K deficiency. Urgent surgical intervention for ICH can be performed successfully following sufficient administration of vitamin K or fresh frozen human plasma. Moreover, early performance of Kasai portoenterostomy is possible even for patients who have undergone craniotomy.

Vitamin K deficiency in hyperemesis gravidarum as a potential cause of fetal intracranial hemorrhage and hydrocephalus

“…In neonates, it is well known that vitamin K deficiency is associated with intracranial hemorrhage (Akiyama et al, 2006;Hubbard and Tobias, 2006;Waseem, 2006). Thus, we emphasize that not only the neonate but also the fetus has a potential risk of intracranial hemorrhage caused by vitamin K deficiency.…”

Hemorragia intracraneal como forma de presentación de atresia biliar: presentación de dos casos

“…5,6 En una serie de 15 casos de niños con atresia de vías biliares que se presentaron como hemorragia intracraneana; 10 fueron subdurales, 4 subaracnoideas, 2 intraventriculares y una intraparenquimatosa. Ocho requirieron evacuación quirúrgica, mientras que el resto mejoró con administración de vitamina K. 7 Nuestra primera paciente se presentó con hemorragia subdural y requirió evacuación quirúrgica, mientras que, la segunda, lo hizo con hemorragia intraventricular y parenquimatosa temporal derecha que se corrigieron con administración de vitamina K. La evolución neurológica de ambas fue buena, sin secuelas en el seguimiento. La edad de presentación de nuestras pacientes fue 122 y 56 días de vida, respectivamente, y la primera paciente superó ampliamente en edad a los casos previamente publicados, que informan intervalos de edad para el episodio hemorrágico de entre 26 y 79 días de vida.…”

“…La edad de presentación de nuestras pacientes fue 122 y 56 días de vida, respectivamente, y la primera paciente superó ampliamente en edad a los casos previamente publicados, que informan intervalos de edad para el episodio hemorrágico de entre 26 y 79 días de vida. 7 En ambas niñas, la combinación de centellograma con HIDA y exploración quirúrgica confirmó el diagnóstico de atresia de vías biliares. El uso del centellograma con HIDA en el diagnósti-co diferencial es variable en distintos centros.…”

Subdural hemorrhage: A unique case involving secondary vitamin K deficiency bleeding due to biliary atresia

“…prolonged prothrombin time (PT), and rapid improvement of PT and/or cessation of bleeding after administration of vitamin K [10,11]. VKDB has been categorized into two forms: idiopathic and secondary.…”

“…VKDB has been categorized into two forms: idiopathic and secondary. EHBA is one of the major causes of secondary VKDB due to cholestasis-induced fat malabsorption [11]. Because of cholestasis, decreased micelle entry into the small intestine leads to decreased vitamin K absorption.…”