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联体双胎中的宫外双胎输血综合征:患病率及临床意义

Extrauterine twin-twin transfusion syndrome in conjoined twins: prevalence and clinical implications.

作者信息

Edun Tasnim Rubina, Al Aujan Abdulrahman Abdulmohsen, Al Khalaf Hamad Abdullah, Azzubi Moutasem Abdulrahim, Al Namshan Mohammed Khabti, Al Mohrij Saad Abdulaziz, Al Rabeeah Abdullah Abdulaziz

机构信息

Department of Pediatrics, King Abdullah Specialized Children's Hospital, King Abdulaziz Medical City, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia.

King Abdullah International Medical Research Center, King Saud Bin Abdulaziz University for Health Sciences, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia.

出版信息

Pediatr Surg Int. 2025 Apr 28;41(1):126. doi: 10.1007/s00383-025-06026-6.

DOI:10.1007/s00383-025-06026-6
PMID:40295339
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12037641/
Abstract

PURPOSE

Conjoined twins present a rare and unique clinical phenomenon known as extrauterine twin-twin transfusion syndrome (EUTTTS), which is underrecognized and underreported in the literature. The present study aimed to conduct a comprehensive analysis of EUTTTS and highlight its various clinical manifestations to help physicians effectively identify and manage this condition.

METHODS

A retrospective review was conducted on 10 sets of conjoined twins managed by a single medical team over a 3-year period. The impact of cross-circulation, management approaches, and clinical outcomes were evaluated.

RESULTS

Seven of the sets (70%) exhibited features of EUTTTS. Four of these sets (57%) had nutritional imbalances, and three (43%) had medication crossover, which included fluid administration. Both craniopagus sets (29%) had hemodynamic instability. These clinical manifestations posed significant diagnostic challenges and management complexities. Furthermore, donor twins had a higher risk of morbidity and mortality as compared to recipients.

CONCLUSION

EUTTTS is a prevalent condition among conjoined twins, and it manifests as a spectrum of clinical presentations independent of the type of conjoined twins. Awareness of these presentations is essential for physicians managing conjoined twins to ensure timely diagnosis and optimal management strategies.

摘要

目的

联体双胎呈现一种罕见且独特的临床现象,即宫外双胎输血综合征(EUTTTS),该综合征在文献中未得到充分认识和报道。本研究旨在对EUTTTS进行全面分析,并突出其各种临床表现,以帮助医生有效识别和处理这种情况。

方法

对一个医疗团队在3年期间管理的10对联体双胎进行回顾性研究。评估交叉循环的影响、管理方法和临床结果。

结果

其中7对(70%)表现出EUTTTS的特征。其中4对(57%)存在营养失衡,3对(43%)存在药物交叉,包括液体输注。两对颅联体(29%)均有血流动力学不稳定。这些临床表现带来了重大的诊断挑战和管理复杂性。此外,与受血儿相比,供血儿的发病和死亡风险更高。

结论

EUTTTS在联体双胎中是一种普遍存在的情况,它表现为一系列独立于联体双胎类型的临床表现。对于管理联体双胎的医生来说,了解这些表现对于确保及时诊断和优化管理策略至关重要。

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Twin-twin transfusion syndrome complicated by single ventricle physiology: A case report.双胎输血综合征合并单心室生理学:病例报告。
Paediatr Anaesth. 2021 Nov;31(11):1255-1258. doi: 10.1111/pan.14276. Epub 2021 Aug 20.
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Successful separation of xypho-omphalopagus conjoined twins with extrauterine twin-twin transfusion syndrome: a case report.成功分离剑突-脐部联体双胎并伴有宫外双胎输血综合征:一例报告
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