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孕期伴有巨大膀胱憩室的埃勒斯-当洛综合征——一例罕见且有趣的病例报告

Ehlers-Danlos syndrome with huge bladder diverticulum in pregnancy - A rare and interesting case report.

作者信息

Pradhan Neekita, Shilawant Jyothi, Akkamahadevi C H, Shivakumar K S, Sundaresh D C

机构信息

Department of Obstetrics and Gynaecology, Sri Sathya Sai Institute of Higher Medical Sciences, EPIP Area, Whitefield, Bengaluru 560066, India.

Department of Obstetrics and Gynaecology, Sri Sathya Sai Institute of Higher Medical Sciences, EPIP Area, Whitefield, Bengaluru 560066, India.

出版信息

Eur J Obstet Gynecol Reprod Biol. 2020 Jul;250:231-234. doi: 10.1016/j.ejogrb.2020.05.001. Epub 2020 May 15.

DOI:10.1016/j.ejogrb.2020.05.001
PMID:32480169
Abstract

Ehlers-Danlos syndrome (EDS) is one of the commonest inheritable connective tissue disorders (CTD) affecting one in 5000 people globally. The incidence of bladder diverticula (BD) is reported to be 1.7% seen more commonly in children without any bladder outlet obstruction. BD are associated with congenital syndromes, namely, EDS. We report a case of huge BD in pregnancy that became symptomatic as the pregnancy progressed to term with urinary retention, recurrent urinary infections and fetal malpresentation. The patient was taken for elective Lower segment cesarean section (LSCS) wherein intra-operative findings of soft abdominal wall, velvety and jelly-like rectus muscle made us suspect connective tissue disorder. A thorough retrospective evaluation of medical history, physical examination along with orthopedic evaluation led to the diagnosis of EDS. To the best of our knowledge, this is the first case report of EDS with huge BD in pregnancy.

摘要

埃勒斯-当洛综合征(EDS)是最常见的遗传性结缔组织疾病(CTD)之一,全球每5000人中就有1人受其影响。据报道,膀胱憩室(BD)的发病率为1.7%,在没有任何膀胱出口梗阻的儿童中更为常见。BD与先天性综合征有关,即EDS。我们报告一例妊娠合并巨大BD的病例,随着孕周增加至足月,出现了尿潴留、反复尿路感染和胎位异常等症状。患者接受了选择性下段剖宫产术(LSCS),术中发现腹壁柔软、腹直肌呈天鹅绒样和果冻样,这使我们怀疑存在结缔组织疾病。通过对病史、体格检查以及骨科评估进行全面的回顾性评估,最终诊断为EDS。据我们所知,这是首例妊娠合并巨大BD的EDS病例报告。

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Ehlers-Danlos syndrome with huge bladder diverticulum in pregnancy - A rare and interesting case report.孕期伴有巨大膀胱憩室的埃勒斯-当洛综合征——一例罕见且有趣的病例报告
Eur J Obstet Gynecol Reprod Biol. 2020 Jul;250:231-234. doi: 10.1016/j.ejogrb.2020.05.001. Epub 2020 May 15.
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