Management of Hemorrhage During Cesarean Delivery in a Patient With Vascular Ehlers-Danlos Syndrome: A Case Report

Author:

Grant Orna1,Mulligan Karen2,Donnelly Jennifer234,Ní Áinle Fionnuala45,Mhuircheartaigh Róisín Ní1

Affiliation:

1. Anaesthesia

2. Gynaecology, Mater Misericordiae University Hospital, Dublin, Ireland

3. Department of Obstetrics and Gynaecology, Rotunda Hospital, Dublin, Ireland

4. University College Dublin School of Medicine, Dublin, Ireland

5. Department of Haematology, Mater Misericordiae University Hospital, Dublin, Ireland.

Abstract

Parturients with vascular Ehlers-Danlos syndrome are at particular risk of hemorrhage, and there is little evidence to guide prevention or management of hemorrhage in these patients. We present the case of a patient with vascular Ehlers-Danlos syndrome who had a cesarean delivery complicated by an intraoperative hemorrhage. Administration of desmopressin and tranexamic acid appeared to be of marked benefit in achieving hemostasis. To the best of our knowledge, this is the first report of the use of desmopressin during major obstetric hemorrhage in vascular Ehlers-Danlos syndrome.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

General Medicine

Reference14 articles.

1. Vascular type Ehlers-Danlos syndrome is associated with platelet dysfunction and low vitamin D serum concentration.;Busch;Orphanet J Rare Dis,2016

2. American College of Obstetricians and Gynecologists’ Presidential Task Force on Pregnancy and Heart Disease and Committee on Practice Bulletins—Obstetrics. ACOG Practice Bulletin No. 212: pregnancy and heart disease.;Obstet Gynecol,2019

3. Pregnancy-related deaths and complications in women with vascular Ehlers-Danlos syndrome.;Murray;Genet Med,2014

4. Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s).;Wiesmann;Orphanet J Rare Dis,2014

5. 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy: The Task Force for the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC).;Regitz-Zagrosek;Eur Heart J,2018

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