Abstract
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
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Ehlers–Danlos syndrome diagnoses approximately doubled over the 5-year study period.
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Ehlers–Danlos was associated with a range of adverse obstetric outcomes.
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Ehlers–Danlos was associated with increased readmission risk.
Keywords
Ehlers–Danlos syndrome - maternal safety - maternal outcomes - severe maternal morbidity
