Abstract - Preterm premature rupture of membranes in a patient with the hypermobility type of the Ehlers-Danlos syndrome. A case report

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M De Vos, L Nuytinck, C Verellen, and A De Paepe

Preterm premature rupture of membranes in a patient with the hypermobility

type of the Ehlers-Danlos syndrome. A case report.

Fetal Diagn Ther, July 1, 1999; 14(4): 244-7.

OBJECTIVES:

This report wants to focus on the risk of severe prematurity in patients

with the hypermobility type of the Ehlers-Danlos syndrome (EDS), a heritable

disorder of connective tissue. Although various obstetrical complications

have been reported in patients with EDS, most reports specifically comment

on the severe complications in patients with the vascular type of EDS,

including uterine and arterial rupture. Pregnancy outcome in patients

presenting the hypermobility type of EDS is poorly documented.

CASE:

A 33-year-old nullipara was referred for preconceptual genetic counseling

with a history of easy bruising, generalized joint hypermobility and chronic

arthralgia and myalgia. The diagnosis of the hypermobility type of EDS was

confirmed on clinical examination. During her first pregnancy, she underwent

a prophylactic Mc cerclage at 14 weeks' gestation. Premature rupture

of membranes occurred at 23 weeks' gestation. A female infant was delivered

at 26 weeks and died 3 h after birth. Electron-microscopic examination

showed collagen fibre abnormalities in the fetus' skin, which were

compatible with the diagnosis of EDS.

CONCLUSIONS:

Patients with the hypermobility type of EDS can have an increased risk for

pregnancy complications, including prematurity due to cervical incompetence

and to premature rupture of membranes. We therefore demand the clinician's

alertness for possible signs of this underdiagnosed type of EDS and

recommend the collaboration between the obstetrician and the medical

geneticist in the obstetrical management of these patients.