Ehlers-Danlos Syndrome in Pregnancy: A Review

Kang, Jungwoo; Hanif, Moghees; Mirza, Eushaa; Jaleel, Shazia

doi:10.1016/j.ejogrb.2020.10.033

Cited by 17 publications

(16 citation statements)

References 43 publications

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“…Several authors recommend performing a prophylactic caesarean section because of the high risk of severe perineal lesions, uterine rupture and vascular ruptures during vaginal deliveries, despite a possible increase in the risk of hemorrhage and wound healing difficulties associated with performing a caesarean section [27] . The delivery route decision should be made based on the risk/benefit balance [23] , [24] , [25] , [26] , [27] . The risks of vaginal delivery being uterine rupture, tearing of the vaginal wall, perineal lesion difficult to suture and healing difficulty.…”

Section: Clinical Discussionmentioning

confidence: 99%

Spontaneous uterine rupture revealing vascular Ehlers-Danlos syndrome: An uncommon case report

Slaoui¹,

Mahtate²,

Lazhar³

et al. 2022

International Journal of Surgery Case Reports

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Section: Clinical Discussionmentioning

confidence: 99%

Spontaneous uterine rupture revealing vascular Ehlers-Danlos syndrome: An uncommon case report

Slaoui¹,

Mahtate²,

Lazhar³

et al. 2022

International Journal of Surgery Case Reports

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“…Pregnancy with vEDS is associated with serious complications such as vessel rupture, uterine rupture, and wound dehiscence. Total maternal mortality in pregnancy with vEDS is estimated to be 5%–6% [ 1 , 4 ]. In the present case, the patient's mother had died from cardiovascular rupture.…”

Section: Discussionmentioning

confidence: 99%

“…Ehlers-Danlos syndrome (EDS) is a rare inherited connective tissue disease that presents with systemic connective tissue fragility in the skin, joints, vasculature, and internal organs [ 1 ]. EDS is classified into 13 subtypes based on its genetic cause and symptoms [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

Twin pregnancy with untyped Ehlers-Danlos syndrome requiring prompt genetic testing: A case report

Ogawa

Mariya

Fujibe

et al. 2022

Case Reports in Women's Health

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Ehlers-Danlos syndrome is a rare genetic disorder that presents with a variety of pathologies depending on the disease type. Among them, vascular Ehlers-Danlos syndrome requires extremely careful management as there have been many reports of fatal perinatal complications such as uterine rupture. Although hypermobile Ehlers-Danlos syndrome is less likely to cause fatal complications, symptoms such as arthralgia, hip dislocation, and depression may be seen throughout pregnancy. We report here a case of twin pregnancy in which Ehlers-Danlos syndrome was first suspected at 19 weeks of gestation. Vascular Ehlers-Danlos syndrome could not be ruled out based on family medical history, making it difficult to determine the perinatal management strategy. Prompt genetic testing did however rule out the vascular type and the patient was diagnosed with hypermobile Ehlers-Danlos syndrome from the clinical symptoms, enabling us to manage the pregnancy safely until 34 weeks of gestation.

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“…The 2017 International Classification of the EDS recognized 13 subtypes of EDS, categorized by their clinical and molecular characteristics 3 . The life expectancy and risk of pregnancy and delivery in patients with EDS vary considerably, depending on the subtype 4 . Therefore, an accurate diagnosis is important for safe management of these patients during pregnancy and delivery.…”

Section: Introductionmentioning

confidence: 99%

The detailed obstetric course of the first Japanese patient with AEBP1‐related Ehlers–Danlos syndrome (classical‐like EDS, type 2)

Sanai

Nakamura

Koike

et al. 2023

J of Obstet and Gynaecol

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We reported a detailed obstetric course of a Japanese patient with Ehlers-Danlos syndrome (EDS) caused by biallelic pathogenic variants in the AEBP1 gene. She was diagnosed with classical EDS at 3 years of age. At 33 years, whole-exome sequencing revealed a homozygous nonsense variant (c.1894C > T:p.Arg632*) in AEBP1. This is the 10th case of AEBP1-related EDS (classical-like EDS type 2) and the first in Japan. She was managed as an inpatient at our hospital beginning at 20 weeks of gestation because of the possibility of high-risk pregnancy. She experienced painful urinary retention, migraines, and threatened premature labor. She delivered a healthy female via elective caesarean section at 32 weeks of gestation. She was treated in the intensive care unit for severe paralytic ileus, postoperatively. Conservative therapy resulted in favorable outcomes, and she was safely discharged on postdelivery day 22nd.

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Ehlers-Danlos Syndrome in Pregnancy: A Review

Cited by 17 publications

References 43 publications

Spontaneous uterine rupture revealing vascular Ehlers-Danlos syndrome: An uncommon case report

Spontaneous uterine rupture revealing vascular Ehlers-Danlos syndrome: An uncommon case report

Twin pregnancy with untyped Ehlers-Danlos syndrome requiring prompt genetic testing: A case report

The detailed obstetric course of the first Japanese patient with AEBP1‐related Ehlers–Danlos syndrome (classical‐like EDS, type 2)

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