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. Key Points Ehlers-Danlos syndrome diagnoses approximately doubled over the 5-year study period. Ehlers-Danlos was associated with a range of adverse obstetric outcomes. Ehlers-Danlos was associated with increased readmission risk.