Journal «Angiology and Vascular Surgery» • 

2021 • VOLUME 27 • №2

Multiple ruptures of major vessels and womb in a parturient woman with Ehlers-Danlos syndrome

Chernyadiev S.A.1, Malgina G.B.2, Leshchinskaya A.Yu.1, Erofeev E.N.2, Bochegov V.S.3, Makarov R.A.2, Tarasova N.V.2, Krokhaleva Ya.M.4

1 Department of Surgical Diseases and Cardiovascular Surgery of the Therapeutic and Preventive Faculty, Ural State Medical University of the RF Ministry of Public Health,
2 Ural Scientific Research Institute of Mother and Child Care under the RF Ministry of Public Health,
3 Department of Vascular Surgery, City Clinical Hospital № 40,
4 Department of Paediatric Surgery, Sverdlovsk Regional Pathologoanatomical Bureau, Yekaterinburg, Russia

Presented herein is a clinical case report demonstrating uncontrolled development of fatal complications in a young parturient woman with vascular Ehlers–Danlos syndrome. The disease had not been known previously. In the third period of natural term delivery we revealed a complete linear vertical uterine rupture with a clinical picture of haemorrhagic shock. After hysterectomy we encountered sequential ruptures of the iliac veins, arteries, abdominal portion of the aorta and inferior vena cava. Attempts at restoring the integrity of vessels followed by ligation thereof, terminating in cutting of vascular walls, with the appearance of new sources of bleeding. The diagnosis was later on verified by the findings of autopsy and genetic typing.

KEY WORDS: vascular Ehlers-Danlos syndrome, pregnancy, uterine rupture, hysterectomy, rupture of major vessels.

p. 158

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