Kasabach–Merritt Phenomenon Associated with Congenital Hydrocephalus: A Case Report

Abstract Background: Kasabach–Merritt phenomenon (KMP) is a rare condition characterized by thrombocytopenia and consumptive coagulopathy in the setting of rapidly expanding vascular tumors. Association with congenital hydrocephalus has not been reported. Clinical Description: A one-and-a-half-month-old female infant presented with fever and progressively enlarging head size. Examination showed macrocephaly, bulging fontanelle, pallor and a large, irregular, firm, nontender lesion extending from the nape of the neck to the right scapular region with overlying reddish discoloration. Systemic examination was normal. Management and Outcome: Investigations showed thrombocytopenia, though the septic screen was otherwise negative. Calcium levels and cerebrospinal evaluation were normal. The prothrombin time (PT), activated partial thromboplastin time and international normalized ratio were normal, but fibrinogen was low, and D-dimer was raised. Brain imaging showed communicating hydrocephalus. Ultrasound and magnetic resonance angiography were consistent with Kaposiform hemangioendothelioma (KHE). A final diagnosis of KMP with KHE associated with hydrocephalus was made. The infant was successfully treated with platelet transfusions along with pulse steroids and vincristine and referred for neurosurgery. Conclusion: This case is perhaps the first describing an association of congenital hydrocephalus with KMP and KHE. Specific therapy for vascular tumor resulted in regression of the manifestations of KMP.