Vitamin K deficiency bleeding with intracranial hemorrhage: focus on secondary form

Non-accidental trauma is the leading cause of intracranial hemorrhage (ICH) in infancy. In contrast, ICH as a part of vitamin K deficiency bleeding (VKDB) secondary to hepatobiliary disease is rare, but encountered even in the era of vitamin K (VK) prophylaxis. During 43 months, six cases with ICH were diagnosed as an initial presentation of VKDB. Clinical features and imaging findings of them were retrospectively reviewed. All cases were breastfed and received oral VK prophylaxis. Liver dysfunction was found in five. Brain CT showed hemorrhage in subdural and subarachnoid space in six, parenchyma in three, and ventricle in one. Abdominal ultrasound was positive in four with final diagnoses of biliary atresia in two, neonatal hepatitis in one, and milk allergy in one. Two cases with negative ultrasound were diagnosed as idiopathic VKDB. In conclusion, ICH with secondary VKDB is rare, but important in infancy in the era of VK prophylaxis.