Delivery Outcomes and Postpartum Readmissions Associated with Ehlers-Danlos Syndrome

Objective Given that updated estimates of Ehlers-Danlos syndrome and risks for obstetric complications including postpartum readmission may be of public health significance, we sought to analyze associated obstetric trends and outcomes in a nationally representative population.Study Design The 2016 to 2020 Nationwide Readmissions Database was used for this retrospective cohort study. Delivery hospitalizations to women aged 15 to 54 with and without Ehlers-Danlos syndrome were identified. Temporal trends in Ehlers-Danlos syndrome diagnoses during delivery hospitalizations were analyzed using joinpoint regression to estimate the average annual percent change with 95% confidence intervals (CIs). To determine whether adverse obstetric outcomes during the delivery were associated with Ehlers-Danlos syndrome, unadjusted and adjusted logistic regression models were fit with unadjusted (odds ratio [OR]) and adjusted ORs with 95% CIs as measures of association. In addition to analyzing adverse delivery outcomes, risk for 60-day postpartum readmission was analyzed.Results An estimated 18,214,542 delivery hospitalizations were included of which 7,378 (4.1 per 10,000) had an associated diagnosis of Ehlers-Danlos syndrome. Ehlers-Danlos syndrome diagnosis increased from 2.7 to 5.2 per 10,000 delivery hospitalization from 2016 to 2020 (average annual percent change increase of 16.1%, 95% CI: 9.4%, 23.1%). Ehlers-Danlos syndrome was associated with increased odds of nontransfusion severe maternal morbidity (OR: 1.84, 95% CI: 1.38, 2.45), cervical insufficiency (OR: 2.14, 95% CI: 1.46, 3.13), postpartum hemorrhage (OR: 1.41, 95% CI: 1.17, 1.68), cesarean delivery (OR: 1.26, 95% CI: 1.17, 1.36), and preterm delivery (OR: 1.35, 95% CI: 1.16, 1.56). Estimates for transfusion, placental abruption, and placenta previa did not differ significantly. Risk for 60-day postpartum readmission was 3.0% among deliveries with Ehlers-Danlos (OR: 1.76, 95% CI: 1.37, 2.25).Conclusion Ehlers-Danlos syndrome diagnoses approximately doubled over the 5-year study period and was associated with a range of adverse obstetric outcomes and complications during delivery hospitalizations as well as risk for postpartum readmission.