A Practical Guide to the Management of the Fetus and Newborn With Hemophilia

Abstract

Newborns with hemophilia are at risk of intracranial hemorrhage, extracranial hemorrhage, and other bleeding complications. The safe delivery of a healthy newborn with hemophilia is a complex process that can begin even before conception, and continues throughout pregnancy, birth, and the newborn period. This process involves the expectant parents and a wide variety of health-care professionals: genetic counselors, obstetricians, neonatologists, pediatricians, radiologists, adult and pediatric hematologists, and nurses with expertise in hemophilia. Because of this multidisciplinary complexity, the relative rarity of births of newborns with hemophilia, and the lack of high-quality evidence to inform decisions, there is considerable variation in practice in this area. We present a comprehensive multidisciplinary approach, from preconception counseling to discharge planning after birth, and describe available options for management decisions. We highlight a number of areas of important uncertainty and controversy, including the preferred mode of delivery, the appropriate use and timing of neuroimaging tests, and the appropriate use of clotting factor concentrates in the newborn period. While the approach presented here will aid clinicians in planning and providing care, further research is required to optimize the care of newborns with hemophilia.

Keywords: bleeding; gynecology and obstetrics; hemophilia.

Figure 1.

Algorithm for prenatal counseling and diagnostic testing for carriers or possible carriers of hemophilia. *See Table 1 for testing options.

Figure 2.

Algorithm for choosing a mode of delivery. If there is no obstetric contraindication to a vaginal delivery, either a vaginal delivery or a caesarean section may be planned. The dashed pathway indicates that, during labor, a decision may be made to perform a caesarean section in order to avoid a difficult vaginal delivery or an operative vaginal delivery.

Figure 3.

Algorithm for the use of factor concentrates and neuroimaging for newborns with hemophilia. *If a newborn is born to a hemophilia carrier or possible carrier, but a diagnosis of hemophilia was not obtained antenatally, the newborn should be presumed to be affected and appropriate care provided until postnatal testing confirms that the newborn is not affected. The dashed pathways indicate that if, at any time and regardless of the events of birth, a newborn develops concerning symptoms or has bleeding that is observed either clinically or with imaging, the prompt administration of replacement factor is the priority, followed by appropriate investigation. CT indicates computed tomography; neuro, neurological; NICU, neonatal intensive care unit; MRI, magnetic resonance imaging; US, ultrasound.