Brar Bobby K, Bober Michael B, Gough Ethan, Hashmi S Shahrukh, Hecht Jacqueline T, Dujmusic Lorena, Little Mary E, Modaff Peggy, Pauli Richard M, Rodriguez-Buritica David F, Serna Maria E, Smid Cory, Legare Janet M, Hoover-Fong Julie E
Division of Maternal - Fetal Medicine, Department of Gynecology and Obstetrics, Johns Hopkins University, Baltimore, MD; Greenberg Center for Skeletal Dysplasias, Department of Genetic Medicine, Johns Hopkins University, Baltimore, MD.
Nemours/A.I. duPont Hospital for Children, Wilmington, DE.
Genet Med. 2023 Jul;25(7):100845. doi: 10.1016/j.gim.2023.100845. Epub 2023 Apr 12.
Pregnancies affected by maternal or fetal achondroplasia present unique challenges. The optimal route of delivery in fetuses with achondroplasia has not been established. Our objective was to determine whether the route of delivery affects postnatal achondroplasia-related surgical burden.
We conducted a secondary analysis of Achondroplasia Natural History Study (CLARITY), which is a multicenter natural history cohort study of patients with achondroplasia. Achondroplasia-related surgical morbidity, which we defined as the need for one or more postnatal achondroplasia-related surgeries, was assessed in relation to the route of delivery and whether the mother also had achondroplasia. Rate of each individual surgery type (otolaryngology, brain, foramen magnum, spine, and extremity) was also assessed in relation to the route of delivery.
Eight hundred fifty-seven patients with achondroplasia with known route of delivery and known maternal stature were included. Three hundred sixty (42%) patients were delivered vaginally, and 497 (58%) patients were delivered by a cesarean delivery. There was no difference in the odds of requiring any postnatal achondroplasia-related surgery in those with achondroplasia who were delivered vaginally compared with those delivered by cesarean birth (odds ratio 0.95, 95% CI = 0.68-1.34, P = .80). No difference was present in the odds of requiring any postnatal achondroplasia-related surgery when route of delivery was compared for fetuses born to 761 average stature mothers (odds ratio 1.05, 95% CI = 0.74-1.51, P = .78). There was also no difference in the odds of requiring each of the individual achondroplasia-related surgeries by route of delivery, including cervicomedullary decompression.
Our study suggests that it is reasonable for average stature patients carrying a fetus with achondroplasia to undergo a trial of labor in the absence of routine obstetric contraindications.
受母体或胎儿软骨发育不全影响的妊娠存在独特的挑战。软骨发育不全胎儿的最佳分娩途径尚未确定。我们的目标是确定分娩途径是否会影响出生后与软骨发育不全相关的手术负担。
我们对软骨发育不全自然史研究(CLARITY)进行了二次分析,这是一项针对软骨发育不全患者的多中心自然史队列研究。评估了与分娩途径以及母亲是否也患有软骨发育不全相关的软骨发育不全相关手术发病率,我们将其定义为需要进行一项或多项出生后与软骨发育不全相关的手术。还评估了每种单独手术类型(耳鼻喉科、脑部、枕骨大孔、脊柱和四肢)的发生率与分娩途径的关系。
纳入了857名已知分娩途径和母亲身高的软骨发育不全患者。360名(42%)患者经阴道分娩,497名(58%)患者剖宫产。与剖宫产分娩的软骨发育不全患者相比,经阴道分娩的患者出生后需要进行任何与软骨发育不全相关手术的几率没有差异(优势比0.95,95%置信区间=0.68-1.34,P=0.80)。对于761名平均身高母亲所生的胎儿,比较分娩途径时,出生后需要进行任何与软骨发育不全相关手术的几率也没有差异(优势比1.05,95%置信区间=0.74-1.51,P=0.78)。按分娩途径划分,包括颈髓减压在内的每种与软骨发育不全相关手术的几率也没有差异。
我们的研究表明,对于怀有软骨发育不全胎儿的平均身高患者,在没有常规产科禁忌证的情况下尝试经阴道分娩是合理的。