Intracranial Hemorrhage Associated With Vitamin K–deficiency Bleeding in Patients With Biliary Atresia

Abstract

Although vitamin K prophylaxis had been given during the neonatal period, ICH-associated VKD bleeding was still found in 7.95% of patients with BA. Persistent neurologic sequelae were found in 5 of 7 cases, with low-density area in the left hemisphere.

Biliary atresia and posterior fossa bleed: Chance or causality. A case report and review of the literature

“…Like our case, most patients with BA have no signs and symptoms during the 1st month of birth 3 . BA is reported to be associated with polysplenia, situs inversus, intestinal malrotation, cardiopulmonary dysplasia, and other anomalies, and rarely with intracranial bleeding 8,9 . Difficulty in diagnosis occurs due to the overlapping features between BA and the other cause of neonatal cholestasis.…”

“…Other causes of cerebellar hemorrhages include coagulopathy, biliary atresia, hemorrhagic transformation of ischemic stroke, arteriovenous malformation, dural venous fistula in tentorium, cerebral amyloid angiopathy, neoplasm, trauma, aneurysm, remote hemorrhages, etc. 5,8,13 In our case, due to the atypical location, rare association, and delayed diagnosis of biliary atresia, initially, the patient was even suspected of an intramural bleed harboring posterior fossa tumor, but follow-up after 1 month revealed complete resolution of the bleed, which proved by itself of it being simply blood rather than any tumor.…”

Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care

“…Frequent assessment of these fat‐soluble vitamins (Table ) is required, particularly in those awaiting LT. Standard vitamin supplements designed for children with fat malabsorption have inadequate levels of these vitamins for BA patients with elevated serum total bilirubin . Large doses of individual vitamins (Table ) are often required to prevent deficiency in BA patients with a serum direct bilirubin > 2 mg/dL, thus avoiding potentially fatal consequences such as intracranial hemorrhage . Bile acid–binding resins and iron should not be administered simultaneously with fat‐soluble vitamin supplements.…”

Prise en charge de la cholestase à début néonatal chez l’enfant : comment pouvons-nous faire mieux ?

“…Le déficit vitaminique a été retrouvé chez des patients qui avaient une hémorragie intracrânienne [34]. Elle survenait chez 7,95 % des AVB [35]. Selon certains auteurs, tout cas suspect d'AVB chez qui il est impossible d'investiguer rapidement doit être opéré [1].…”