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Obstet Gynecol. 2009 Feb;113(2 Pt 2):515-8. doi: 10.1097/AOG.0b013e3181898cbf.

A maternal and perinatal mortality in pregnancy complicated by the kyphoscoliotic form of Ehlers-Danlos syndrome.

Author information

1
Division of Maternal Fetal Medicine, Department of Obstetrics and Gynecology, Allegheny General Hospital, Pittsburgh, Pennsylvania, USA. esakajb@yahoo.com

Abstract

BACKGROUND:

Ehlers-Danlos syndrome is a group of inherited connective tissue diseases demonstrating autosomal-dominant, autosomal-recessive, and X-linked inheritance patterns. The diagnosis can be established by clinical, biochemical, and genetic findings.

CASE:

Our nulliparous patient presented with an unspecified diagnosis of Ehlers-Danlos syndrome. Laboratory testing confirmed the kyphoscoliotic type. Based on clinical and phenotypic similarities with the vascular type of Ehlers-Danlos syndrome, termination was advised. Minor trauma in the third trimester led to delivery of a stillborn fetus, which was followed by disseminated intravascular coagulopathy and death of the mother. Maternal autopsy revealed that there had been a spontaneous rupture of the right iliac artery.

CONCLUSION:

Practitioners should be aggressive in recommending effective birth control in patients with the kyphoscoliotic form of Ehlers-Danlos syndrome. In cases of established pregnancy, patients should be made fully aware of their risks of death and severe complications.

PMID:
19155940
DOI:
10.1097/AOG.0b013e3181898cbf
[Indexed for MEDLINE]

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