Pregnancy and Fetal Outcomes in Patients With Ehlers-Danlos Syndrome: A Nationally Representative Analysis

Ehlers-Danlos syndrome (EDS) is a genetically and clinically heterogeneous group of hereditary connective tissue disorders that interfere with collagen synthesis and function, thus affecting the skin, ligaments, blood vessels, and organs.1 According to the 2017 EDS classification by the international EDS Consortium, 13 subtypes of EDS have been classified through clinical presentation and different genetic mutations affecting the coding of either collagen or collagen-modifying enzymes.2 The most common subtypes of EDS are classic EDS (type I and II), hypermobility EDS (type III), and vascular EDS (type IV).3 The combined prevalence of all subtypes is estimated from 1:5000 to 1:50.4,5 There is a broad spectrum of clinical manifestations in EDS, ranging from mild to lethal conditions. Classic EDS (cEDS) commonly manifests itself as mild conditions, such as skin hyperextensibility, atrophic scarring, and joint hypermobility. Mild cEDS conditions could be complicated by joint dislocations, but often resolve spontaneously or are self-manageable. Hypermobility EDS (hEDS) is diagnosed clinically with major criteria and the diagnosis is supported by minor criteria such as skin involvement, a Beighton score of ≥ 5/9, chronic joint pain and dislocations, and positive family history. Vascular-type EDS (vEDS) is associated with intracranial or aortic aneurysms with life-threatening consequences of rupture or dissection.6, 7, 8

Pregnancy outcomes in patients with EDS are not well established. Multiple papers have been published on obstetrical outcomes in pregnant people with EDS; however, the populations in these studies are relatively small, with a maximum of 386 patients with EDS involved in a single study.5 In addition, the results of these studies vary and lack consistency. Some studies revealed EDS is associated with a higher risk of obstetrical complications, and other studies of patients with EDS did not find increased risks of adverse outcomes such as preterm birth, and intrauterine growth restriction(IUGR).7, 8, 9, 10, 11 To determine whether patients with EDS experience higher rates of in-hospital delivery-related complications and poor fetal outcomes than non-EDS patients, we used the Health Care Utilization Project-Nationwide Inpatient Sample (HCUP-NIS) database, a large, nationally representative, and contemporary database.

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