Outcome and Predisposing Factors for Intracranial Hemorrhage in Turkish Children with Hemophilia

Background/Objectives: Childhood hemophilia, a hereditary bleeding disorder predominantly affecting males, arises due to gene mutations encoding clotting factors VIII or IX. Intracranial hemorrhage represents a significant and life-threatening complication in pediatric patients with hemophilia. The incidence of intracranial hemorrhage in children with hemophilia, although relatively low, is notably higher compared to the general pediatric population. Methods: In this study, the objective is to examine patients with hemophilia who have experienced intracranial hemorrhage retrospectively. This study is a multicenter, retrospective analysis using data from three tertiary care centers in a provincial city in Turkey. Data were obtained from the participants' hospital records. The presence of inhibitors against FVIII in the participants and the prophylaxis used against them were included in the analysis. Trauma history was queried, with types of traumas examined, including traffic accidents, falls, and a traumatic vaginal delivery. The duration and causes of complaints among the participants were investigated. The causes of complaints were categorized as fever, hematoma, convulsions, loss of consciousness, and hemiparesis. The participants' Physical Examination Findings were classified as fever, hematoma, and loss of consciousness. The duration of hospital stays was evaluated. The hemorrhage location was classified into five groups: parenchymal, subdural, scalp, subarachnoid, and multiple hemorrhagic foci. The recurrence of bleeding, the need for transfusion, surgical intervention, and mortality were also examined. Results: A significant difference was identified between the participants' survival rates and age variables, as well as transfusion in <36 months. A total of 9 participants had spontaneous intracranial bleeding, 2 experienced cranial trauma as a result of traffic accidents, and 25 participants were exposed to head trauma due to falls. Of the remaining individuals, one suffered head trauma from a severe impact, and one had cranial trauma following a traumatic vaginal delivery. Fourteen participants required transfusion, and three underwent surgical intervention. Conclusions: According to the results of the statistical analyses, the variables Factor Level, Physical Examination Findings, Transfusion, Recurrent Bleeding, Inhibitor, and Prophylaxis were found to affect survival significantly. No significant relationship was determined between the other analyzed variables and survival. During our study, five of the participants examined died. Accordingly, the mortality rate identified in our study is 13.1%.