Head trauma in children with congenital coagulation disorders

Abstract

Bleeding is the most frequent cause of death in children with congenital coagulation disorders, and intracranial (IC) hemorrhage accounts for the majority of mortality in all age groups. Even minor head trauma may produce significant IC pathology. Immediate diagnosis and rapid medical management are mandatory if morbidity and mortality are to be minimized. Although computed tomographic (CT) scans provide accurate diagnostic information, reliable criteria for the use of this expensive technology in children with hemophilia and von Willebrand's disease have not been defined. In this study the clinical symptoms and the time of initial assessment and therapy of head-injured children with congenital coagulopathies were evaluated and correlated with CT findings. Between 1985 and 1992, 123 patients with hemophilia A, hemophilia B, or von Willebrand's's disease received follow-up at this institution. One hundred nine episodes of head injury were recorded in 43 patients, and 66 CT scans were obtained. The most frequent mechanism of injury was a simple fall at play (62%). Only 5 patients had an IC injury demonstrable with CT (4.5% of 110 episodes). Vomiting was reported in 4 of 5 patients with IC hemorrhage (ICH), and all 5 presented with an altered mental status (Glasgow coma scale [GCS] (x̄) = 10) and focal neurological deficit. These findings were infrequently observed (vomiting, 5 of 105; GCS (x̄) = 15; neurological deficits 0 of 105) in children who either did not undergo CT or whose CT scan results were normal. Conclusions are (1) the incidence of ICH in children with hemophilia A, hemophilia B, and von Willebrand's disease was low (4.5%); (2) symptoms commonly associated with significant head injury (ie, loss of consciousness, headache, and seizures) were reported infrequently; and (3) vomiting, a GCS ≤ 14, and focal neurological deficit were observed in all children with ICH. Therefore, the authors recommend that any child with congenital coagulopathy who sustains head trauma and presents with vomiting, an altered mental status (GCS ≤ 14), or focal neurological deficit should receive immediate factor replacement and have an emergency CT scan.