The incidence and outcome of intracranial haemorrhage in newborns with haemophilia: analysis of the Nationwide Inpatient Sample database

Abstract

The incidence of intracranial haemorrhage (ICH) in newborns with haemophilia is unknown. Retrospective studies, estimate the incidence to be around 3%. Because of this uncertainty, we analysed the largest inpatient database in the USA, the Nationwide Inpatient Sample (NIS), to better approximate the incidence of ICH in these patients. ICD-9 coding data were used to reference NIS entries of haemophilia (A, B or C) or von Willebrand's disease (VWD), with intraventricular (IVH), subarachnoid (SAH), subdural (SDH) and/or intraparenchymal (IPH) haemorrhage. Of 9.2 x 10(7) hospitalizations from 1988 to 2001, 11% or 1 x 10(7) were newborns. Of these, 0.00527%, or 580 were diagnosed with haemophilia or VWD. Twenty of 580, or 3.4%, experienced an ICH. The ICH rate in non-haemophilic newborns was 0.11% (P value: <0.0001). The rate of ICH among term haemophilic newborns without sepsis, respiratory distress syndrome (RDS) or congenital heart disease (CHD), delivered without vacuum assist was 1.9%. One death occurred on the day of birth in a term neonate with haemophilia C. The mean length of stay for ICH patients with haemophilia was 28 days (median 28, range: 6-143 days). The mean hospital charges for the group were 102,072 dollars (median 67,551 dollars, range: 9624-467,132 dollars). These data add credence to the estimates of ICH in haemophilic newborns and may guide treatment strategies around the time of their birth. Further, uncomplicated delivery of term, otherwise healthy haemophilic newborns may carry a lesser risk of ICH.

Clinical Characteristics and Prognostic Factors in Hemophiliacs with Intracranial Hemorrhage: A Single-Center, Retrospective Experience

“…The incidence of ICH has been found to range from 2.2 to 7.5 % in patients with hemophilia [5,6,[12][13][14]. In one study, the cumulative hazard of ICH for the entire cohort over the comprehensive follow-up period was 26.7 per 1000 patients, and the annual rate of ICH was 2.50 events per 1000 patients (95 % CI = 1.90-3.31) [11].…”

Management of Type 2B von Willebrand Disease during Pregnancy

“…Our patient proceeded to caesarean section as ventouse and forceps delivery are associated with a higher risk of intracranial and extracranial bleeding than spontaneous vaginal delivery and caesarean section in the general population and this increased risk is mirrored in neonates with haemophilia [7]. A large population study showed a higher rate of intracranial haemorrhage in neonates with haemophilia or von Willebrand disease compared to those without (3.4 vs. 0.11%) Following exclusion of ventouse delivery and other comorbidities including sepsis, acute respiratory distress syndrome, and congenital heart disease, the rate of intracranial haemorrhage decreased to 1.9% in the affected group [8]. …”

Intracranial Hemorrhage in a Child of Hemophilia: A Case Report

“…These findings strongly suggest that long-term follow-up of children with ICH, including neuropsychological assessment and intervention, is critical. [6] Because of ICH's elevated risks and high mortality rate, it is a true medical emergency and should be treated presumptively prior to any evaluation.…”