PF330 MODE OF DELIVERY IN NEWBORNS WITH HEMOPHILIA: ASSOCIATION WITH FAMILY HISTORY/MATERNAL CARRIER STATUS, SEVERITY OF HEMOPHILIA AND INCIDENCE OF INTRACRANIAL/EXTRACRANIAL HEMORRHAGE

Abstract

Background:Intracranial hemorrhage (ICH) in neonates is one of the most severe clinical conditions often associated with neurological complications. The incidence of ICH in hemophilic neonates compared to healthy term infants is referred higher (2–4% versus 0.04%, respectively). Nevertheless, mode of delivery in cases of known history of hemophilia or maternal carriership still remains under debate.Aims:To correlate mode of delivery (vaginal delivery‐VD or cesarean section‐CS) of newborns with hemophilia having had family history of hemophilia/maternal carriership with the severity of the disease, birth order and incidence of intracranial/extracranial hemorrhage.Methods:Patients with severe, moderate and mild haemophilia A/B (FVIII/IX <1, 1–5, ≥5–25 u/dl, respectively) born between 1/1/2000 and 31/12/2017 were retrospectively included. Data presented with absolute and percentage distributions and the comparison of variables were made using Fisher's exact test. A cut‐off p‐value of 0.05 was considered statistically significant.Results:Of a total of 134 neonates, 115 (85.8%) had been diagnosed as hemophilia A and 19 (14.2%) as hemophilia B, whereas 56.7% had severe, 15.7% moderate and 27.6% mild hemophilia. Seventy four neonates (55.2%) were born by VD and 60 (44.8%) by CS, while 54.5% were delivered from nulliparous mothers. At delivery, family history of hemophilia/maternal status was known in 27.6% of cases. Higher percentage of children with severe/moderate hemophilia was born by VD in comparison to children with mild hemophilia (63% vs 35%, p < 0.01), whereas 67.6% of neonates with family history/maternal carriership were born by CS. Positive family history and/or maternal carriership at delivery were found in 17.5 % of neonates with severe/moderate hemophilia, in contrast to 54.1% of them with mild hemophilia (p < 0.01). Knowledge of carriership influenced significantly the decision for CS only in cases of non primigravidae mothers, in contrast to deliveries with unknown hemophilia history (88.2% vs 25%, p < 0.01). Among all children, 4 (3%) had been presented with cephalohematoma. One of them with hemophilia B had also cerebral hemorrhage (0.8%). All patients were born by VD from primiparae mothers (2 with assisted VD). ICH occurred in one of the two children born by instrumental vaginal delivery using vacuum extraction. Maternal carriership of hemophilia A was known only in one neonate with cephalohematoma, born by spontaneous vaginal delivery.Summary/Conclusion:In our study, the choice of cesarean section seems to be the preferable way for delivery in cases of potential hemophilia, if the mother, especially multipara, is carrier of mild hemophilia. On the contrary, in cases of severe hemophilia, vaginal delivery is more frequently performed, probably because there is no knowledge of hemophilia carriership at the time of delivery, because of the presence of a de novo mutation in the factor VIII gene, since in previously known carriership prenatal diagnosis had been performed. In any case, it has to be mentioned that neonates with severe hemophilia born by vaginal delivery, especially assisted with vacuum extraction or forceps, are at greater risk of extracranial or intracerebral hemorrhage.