BMC pregnancy and childbirth · 2025
Patient-reported outcomes during pregnancy and delivery in epidermolysis bullosa.
СтатьяНазвание и аннотация: Перевод в очереди
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Zott Anna, Diem Anja, Prodinger Christine, Ude-Schoder Katharina, Bauer Johann W, Welponer Tobias, Laimer Martin
Аннотация
BACKGROUND: Research on pregnancy in women with epidermolysis bullosa (EB) and its effect on maternal disease activity is limited. Lack of evidence poses significant challenges for patient management.
OBJECTIVE: This study aimed to retrospectively characterise the spectrum and course of patient-reported symptoms and complications during pregnancy referring to the EB House Austria patient cohort.
METHODS: Data on pregnancy outcomes, clinical symptoms, and EB disease activity before, during, and after pregnancy were collected using a structured questionnaire. To specify the impact of disease severity, the responders were classified into three groups based on the individual overall EB phenotype (mild, moderate, severe) prior to pregnancy (baseline). The pseudonymised data were analysed descriptively and complemented by a comprehensive literature review.
RESULTS: This study assessed 33 pregnancies in 19 women encompassing all major EB subtypes. Most reported parameters, including EB lesional burden, remained stable during pregnancy. A trend towards symptom exacerbation was reported by patients with moderate EB, while those with severe EB more often recalled some symptomatic improvement. Anaemia was documented in 30% of pregnancies, predominantly among moderate to severe EB patients. Vaginal delivery constituted the predominant mode of birth (67%). Caesarean section rates increased with EB severity (0% in mild, 38% in moderate, and 50% in severe EB groups). Among the caesarean deliveries, antenatal vulvovaginal lesions were recalled in 70% of cases. Severe perineal tears were the most common perinatal complication, occurring in 15% of pregnancies and primarily in the moderate and severe cohorts. Due to sample size limitations, no formal statistical hypothesis testing was conducted.
CONCLUSION: Referring to a limited cohort size, our findings suggest that pregnancy management in women with EB is feasible, though antenatal and perinatal complication rates rise with disease severity. Vaginal delivery appears to be a viable option and was the preferred method in most cases. In planned caesarean sections, neuraxial anaesthesia was not associated with adverse events. Early identification and treatment of anaemia in severe EB are critical, and proactive supportive strategies should be implemented to facilitate breastfeeding.
OBJECTIVE: This study aimed to retrospectively characterise the spectrum and course of patient-reported symptoms and complications during pregnancy referring to the EB House Austria patient cohort.
METHODS: Data on pregnancy outcomes, clinical symptoms, and EB disease activity before, during, and after pregnancy were collected using a structured questionnaire. To specify the impact of disease severity, the responders were classified into three groups based on the individual overall EB phenotype (mild, moderate, severe) prior to pregnancy (baseline). The pseudonymised data were analysed descriptively and complemented by a comprehensive literature review.
RESULTS: This study assessed 33 pregnancies in 19 women encompassing all major EB subtypes. Most reported parameters, including EB lesional burden, remained stable during pregnancy. A trend towards symptom exacerbation was reported by patients with moderate EB, while those with severe EB more often recalled some symptomatic improvement. Anaemia was documented in 30% of pregnancies, predominantly among moderate to severe EB patients. Vaginal delivery constituted the predominant mode of birth (67%). Caesarean section rates increased with EB severity (0% in mild, 38% in moderate, and 50% in severe EB groups). Among the caesarean deliveries, antenatal vulvovaginal lesions were recalled in 70% of cases. Severe perineal tears were the most common perinatal complication, occurring in 15% of pregnancies and primarily in the moderate and severe cohorts. Due to sample size limitations, no formal statistical hypothesis testing was conducted.
CONCLUSION: Referring to a limited cohort size, our findings suggest that pregnancy management in women with EB is feasible, though antenatal and perinatal complication rates rise with disease severity. Vaginal delivery appears to be a viable option and was the preferred method in most cases. In planned caesarean sections, neuraxial anaesthesia was not associated with adverse events. Early identification and treatment of anaemia in severe EB are critical, and proactive supportive strategies should be implemented to facilitate breastfeeding.