Ehlers-Danlos syndrome as a cause of fatal obstetric complications


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Abstract

Background. The vascular type of Ehlers-Danlos syndrome is of the greatest importance in the practice of an obstetrician/gynecologist, since it is accompanied by various obstetric and general medical problems that are often life-threatening. The incidence is 1:5,000 to 1:250,000, depending on the form and severity of the disease; the mild forms of the disease are more commonly recorded; its subtle forms are not always diagnosed in childhood, but usually when specific complications appear. Case report. Clinical cases demonstrate difficulties in diagnosing and predicting maternal outcomes in patients with Ehlers-Danlos syndrome. Currently, there is no consensus on safe delivery in patients with Ehlers-Danlos syndrome, since any surgical intervention in this category of patients is fraught with bleeding associated with spontaneous ruptures of the defective vascular wall. Due to the difficulty of predicting fatal complications of Ehlers-Danlos syndrome in obstetric patients, any description of the clinical case is useful for obstetricians/ gynecologists, angiosurgeons, and anesthesiologists/resuscitators. Conclusion. Based on the results of the two cases described above, the authors believe that for a successful pregnancy outcome in these patients, one should pay attention to the major and minor phenotypic signs of Ehlers- Danlos syndrome and, if there is a little suspicion, recommend a molecular genetic study of type III collagen genes at the stage of pregnancy planning, as well as a consultation with a vascular surgeon and a geneticist. Planned abdominal delivery is recommended for patients with a confirmed diagnosis in a third-level perinatal center with the participation of a multidisciplinary team: highly skilled obstetricians/gynecologists, a team of vascular surgeons, and a team of anesthesiologists and transfusiologists.

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About the authors

Galina B. Malgina

Ural Research Ural Research Institute of Maternal and Infant Care, Ministry of Health of Russia

Email: galinamalgina@mail.ru
Dr. Med. Sci., Associate Professor, Director

Vitaliy F. Nesterov

Ural Research Ural Research Institute of Maternal and Infant Care, Ministry of Health of Russia

Email: dr.nesterov20l4@yandex.ru
PhD (in Medicine), Senior Researcher, Head of Obstetric Physiological Department

Eugeniy N. Erofeev

Ural Research Ural Research Institute of Maternal and Infant Care, Ministry of Health of Russia

Email: omm-erofeev@yandex.ru
PhD (in Medicine), Head of Clinic

Roman A. Makarov

Ural Research Ural Research Institute of Maternal and Infant Care, Ministry of Health of Russia

Email: r_makarov_ekb@mail.ru
PhD (in Medicine), Senior Researcher, Head of the Intensive Care and Intensive Care Unit

Alla Yu. Leshchinskaya

Ural State Medical University, Ministry of Health of Russia

Email: allales75@mail.ru
PhD (in Medicine), Associate Professor of the Department of Surgical Diseases and Cardiovascular Surgery of the Faculty of Medicine and Prevention

Yaroslava M. Krokhaleva

Sverdlovsk Regional Postmortem Bureau

Email: yakobeleva@ya.ru
doctor-pathologist, Head of the Department of Pediatric Pathology

Artem S. Kovalev

Ural Research Ural Research Institute of Maternal and Infant Care, Ministry of Health of Russia

Email: dr.kova94@yandex.ru
correspondence graduate student

Vladislav V. Kovalev

Ural State Medical University, Ministry of Health of Russia

Email: vvkovalev55@gmail.com
Dr. Med. Sci., Professor, Head of the Department of Obstetrics and Gynecology, Transfusiology, Faculty of Advanced Training and Professional Retraining of Specialists

Sergey R. Belomestnov

Ural Research Ural Research Institute of Maternal and Infant Care, Ministry of Health of Russia

Email: beldoctor@mail.ru
PhD (in Medicine), Associate Professor of the Department of Obstetrics and Gynecology, Transfusiology, Faculty of Advanced Training and Professional Retraining of Specialists

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