Retrospective evidence on outcomes and experiences of pregnancy and childbirth in epidermolysis bullosa in Australia and New Zealand

Abstract

BackgroundPregnancy in epidermolysis bullosa (EB) has not been comprehensively studied.ObjectiveWe aimed to develop a foundational database, which could provide peri-obstetric advice in EB.MethodsSurvey questionnaires were sent to obstetricians, unaffected mothers of EB babies, and mothers with EB. Results were analyzed using chi-square, Fisher exact, and t-tests.ResultsOut of 1346 obstetricians surveyed, 195 responded, and only 14 had encountered EB. All recommended normal vaginal delivery (NVD), except for one elective Caesarean section (CS). We received responses from 75 unaffected mothers who had delivered EB babies. They had significantly more complications in their EB pregnancies compared to their non-EB pregnancies. A further 44 women with various types of EB who had given birth responded. Most delivered via NVD and had no significant increase in complications in both their EB and non-EB pregnancies. In both groups, there were no significant differences in blistering at birth in babies delivered via NVD and CS.ConclusionIn conclusion, most patients with EB who are capable of giving birth do not have an increased risk for pregnancy-related complications and NVD appears to be safe. Awareness of this data amongst obstetricians and dermatologists should lead to improved quality of care for mothers and babies affected with EB.

Management of epidermolysis bullosa simplex in pregnancy: A case report

“…Malnutrition, severe anemia and chronic infection may be associated and should be addressed [10]. Genetic counseling should be offered to patients, given the inheritance pattern [8,9,14], as evident from our case.…”

“…Principles of care remain the same irrespective of type of EB. Pregnancy is relatively uncommon, due to the associated disability and concerns regarding affected children [8,9]. Nevertheless, successful pregnancy outcomes have been reported even in women with severe types of EB [3,[9], [10], [11], [12], [13]].…”

“…Nevertheless, successful pregnancy outcomes have been reported even in women with severe types of EB [3,[9], [10], [11], [12], [13]]. Women with EB are not at increased risk of pregnancy-related complications [8], and the skin itself does not seem to worsen during pregnancy [9]. Malnutrition, severe anemia and chronic infection may be associated and should be addressed [10].…”

“…Our patient desired a vaginal delivery; however, failed IOL, coupled with an abnormal fetal heart rate, necessitated performance of a CD. Normal vaginal delivery is considered safe and preferred to CD even if the mother is expected to deliver a baby with EB [8]. There is a theoretical risk of blistering of vaginal mucosa, possible sloughing of the cervical and perineal epithelium and damage to soft tissues of pelvic floor [9].…”

“…Prolonged labor and associated immobility may predispose to lesions in the lower back, buttocks and arms [10]. Episiotomy is acceptable to reduce perineal tears [8]. Besides obstetric indications, CD may be indicated with genital tract involvement to minimize perineal bullae [9].…”