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28周胎儿肢体-体壁复合体及文化信仰对母亲结局的影响:一例报告

Limb body wall complex in a 28-week fetus and impact of cultural beliefs on maternal outcomes: A case report.

作者信息

Ilyas Erum, Affan Muhammad, Dar Marrium S, Jobran Afnan Wm

机构信息

Department of Obstetrics and Gynecology, Sir Syed Medical Hospital and College, Karachi, Pakistan.

Department of Internal Medicine, Jinnah Sindh Medical University, Karachi, Pakistan.

出版信息

SAGE Open Med Case Rep. 2024 Aug 16;12:2050313X241272614. doi: 10.1177/2050313X241272614. eCollection 2024.

Abstract

The limb body wall complex is a rare congenital disorder that is marked by various anomalies such as thoracoschisis, gastroschisis, limb defects and exencephaly. These defects are not compatible with life and result in spontaneous abortion or stillbirth. It is important to diagnose the anomalies on time and counsel the patients extensively to make the right decision. In this case report, we present a woman in her early twenties pregnant with a fetus with multiple anomalies including ectopia cordis, gastroschisis, meningomyelocele, kyphoscoliosis and congenital talipes equinovarus diagnosed as limb body wall complex at 18 weeks of gestation. Despite extensive counselling by the consultant, the couple decided to continue the pregnancy due to cultural beliefs which ultimately affected the maternal health. The fetus was spontaneously delivered at 28 weeks when the mother came to the hospital with ruptured membranes and the fetus was pronounced dead at birth. The co-occurrence of all these anomalies poses a challenge in diagnosis and management. Extensive counselling is required for the patient to make the appropriate decision for better outcomes. Cultural beliefs can play a major role in the decision-making of the patients and their families which can have a significant effect on maternal outcomes.

摘要

肢体-体壁综合征是一种罕见的先天性疾病,其特征为多种异常,如胸裂、腹裂、肢体缺陷和无脑儿。这些缺陷无法维持生命,会导致自然流产或死产。及时诊断这些异常并为患者提供全面的咨询以做出正确决定非常重要。在本病例报告中,我们介绍了一名二十出头的女性,她怀有一个胎儿,该胎儿存在多种异常,包括心异位、腹裂、脊髓脊膜膨出、脊柱侧凸和先天性马蹄内翻足,在妊娠18周时被诊断为肢体-体壁综合征。尽管顾问进行了全面的咨询,但由于文化信仰,这对夫妇决定继续妊娠,这最终影响了母亲的健康。当母亲胎膜破裂前来医院时,胎儿在28周时自然分娩,胎儿出生时被宣布死亡。所有这些异常的同时出现给诊断和管理带来了挑战。需要为患者提供全面的咨询,以便做出适当的决定以获得更好的结果。文化信仰在患者及其家庭的决策中可能起主要作用,这可能对母亲的结局产生重大影响。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a7ce/11329893/6190154f96ac/10.1177_2050313X241272614-fig1.jpg

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