Ehlers-Danlos syndrome during pregnancy: a case report and review of the literature. 1981

D J Taylor, and I Wilcox, and J K Russell

Since Ehlers-Danlos syndrome encompasses a wide spectrum of disease and only has an estimated incidence of approximately 1:150,000 (Beighton, 1968a), there is a paucity of data from large studies on which to base firm conclusions about the risks of pregnancy in association with the syndrome. Most reports refer to women with the gravis (type I) and ecchymotic (type IV) forms, unlike pregnancy in association with the mitis (type II), which is rarely reported despite the fact that it constitutes 35 per cent of all cases of Ehlers-Danlos syndrome (Beighton, 1970). We suspect this is because complications with the mitis form are rare. Women with the two severe forms of the syndrome, mentioned above, risk hemorrhage, severe trauma at delivery, and preterm labor, but whether this is the case in the mitis form is unproved. Our patient's course was uneventful, delivery by cesarean section being undertaken because of her hip fixity. Tyson (1972) suggested that delivery by cesarean section was indicated in all cases of Ehlers-Danlos syndrome, but this pertains to the severe forms of the syndrome. The question of vaginal delivery in the mitis form remains unanswered. We would hope that in future reports of pregnancy in association with Ehlers-Danlos syndrome that patients are classified according to type so that prognosis, particularly in the mitis form, can be more accurately assessed.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D007743 Labor, Obstetric The repetitive uterine contraction during childbirth which is associated with the progressive dilation of the uterine cervix (CERVIX UTERI). Successful labor results in the expulsion of the FETUS and PLACENTA. Obstetric labor can be spontaneous or induced (LABOR, INDUCED). Obstetric Labor
D008297 Male Males
D011247 Pregnancy The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH. Gestation,Pregnancies
D011248 Pregnancy Complications Conditions or pathological processes associated with pregnancy. They can occur during or after pregnancy, and range from minor discomforts to serious diseases that require medical interventions. They include diseases in pregnant females, and pregnancies in females with diseases. Adverse Birth Outcomes,Complications, Pregnancy,Adverse Birth Outcome,Birth Outcome, Adverse,Complication, Pregnancy,Outcome, Adverse Birth,Pregnancy Complication
D011295 Prenatal Care Care provided the pregnant woman in order to prevent complications, and decrease the incidence of maternal and prenatal mortality. Antenatal Care,Care, Antenatal,Care, Prenatal
D002585 Cesarean Section Extraction of the FETUS by means of abdominal HYSTEROTOMY. Abdominal Delivery,Delivery, Abdominal,C-Section (OB),Caesarean Section,Postcesarean Section,Abdominal Deliveries,C Section (OB),C-Sections (OB),Caesarean Sections,Cesarean Sections,Deliveries, Abdominal
D004535 Ehlers-Danlos Syndrome A heterogeneous group of autosomally inherited COLLAGEN DISEASES caused by defects in the synthesis or structure of FIBRILLAR COLLAGEN. There are numerous subtypes: classical, hypermobility, vascular, and others. Common clinical features include hyperextensible skin and joints, skin fragility and reduced wound healing capability. Cutis Elastica,Ehlers Danlos Disease,Ehlers-Danlos Disease,Danlos Disease, Ehlers,Disease, Ehlers Danlos,Disease, Ehlers-Danlos,Ehlers Danlos Syndrome,Syndrome, Ehlers-Danlos
D005260 Female Females
D006473 Postpartum Hemorrhage Excess blood loss from uterine bleeding associated with OBSTETRIC LABOR or CHILDBIRTH. It is defined as blood loss greater than 500 ml or of the amount that adversely affects the maternal physiology, such as BLOOD PRESSURE and HEMATOCRIT. Postpartum hemorrhage is divided into two categories, immediate (within first 24 hours after birth) or delayed (after 24 hours postpartum). Hemorrhage, Postpartum,Delayed Postpartum Hemorrhage,Immediate Postpartum Hemorrhage,Hemorrhage, Delayed Postpartum,Hemorrhage, Immediate Postpartum,Postpartum Hemorrhage, Delayed,Postpartum Hemorrhage, Immediate

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