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尾侧重复综合征系统评价——需要更好的多学科手术方法及随访

Caudal Duplication Syndrome Systematic Review-A Need for Better Multidisciplinary Surgical Approach and Follow-Up.

作者信息

Radu-Iulian Spătaru, Adelaida Avino, Dan-Alexandru Iozsa, Monica Ivanov, Dragos Serban, Florentina Tomescu Luminiţa, Cătălin Cirstoveanu

机构信息

Discipline of Pediatric Surgery, Faculty of Medicine, "Carol Davila" University of Medicine and Pharmacy, 020021 Bucharest, Romania.

Department of Pediatric Surgery, Emergency Clinic Hospital for Children "Maria S. Curie", 41451 Bucharest, Romania.

出版信息

Medicina (Kaunas). 2020 Nov 27;56(12):650. doi: 10.3390/medicina56120650.

Abstract

Caudal duplication syndrome is a rare association of anatomical anomalies describing duplication of the hindgut, spine, and uro-genital structures, leading to varied clinical presentations. The current literature focuses on case reports which describe the embryological etiology and anatomical spectrum of the condition giving little attention to the surgical preparation, the need for a well-structured follow-up program, or the transition into adult healthcare of these complex patients. No reviews have been published regarding this complex pathology. : A review of caudal duplication syndrome cases was done to assess the range of the clinical malformations, timing, and types of surgical interventions. Inconsistencies in multidisciplinary care, follow-up, and risk events were described. Hindgut duplication always involved the anorectal region. Anorectal malformations were evenly distributed as unilateral and bilateral. Colon duplication extended from the anal region to the transverse colon or ascending colon in most of the cases and less to terminal. In females, genital duplication was present in all cases. The follow-up period varied between 3 months and 12 years. In all adult females, the motive of presentation was related to pregnancy (complications after successful delivery, fertility evaluation) or late complications (fecalith obstruction of the end-to-side colon anastomosis, repeated UTIs with renal scarring). : Complex malformations affecting multiple caudal organs may have a strong impact in many aspects of the long-term quality of life; therefore, patients with caudal duplication syndrome need increased awareness and joined multidisciplinary treatment.

摘要

尾侧重复综合征是一种罕见的解剖学异常关联,描述了后肠、脊柱和泌尿生殖结构的重复,导致多种临床表现。当前文献主要集中在病例报告上,这些报告描述了该病症的胚胎学病因和解剖学范围,而很少关注手术准备、构建完善的随访计划的必要性,或这些复杂患者向成人医疗保健的过渡。尚未发表关于这种复杂病理学的综述。对尾侧重复综合征病例进行了回顾,以评估临床畸形的范围、手术干预的时机和类型。描述了多学科护理、随访和风险事件中的不一致之处。后肠重复总是累及肛门直肠区域。肛门直肠畸形以单侧和双侧均匀分布。在大多数病例中,结肠重复从肛门区域延伸至横结肠或升结肠,较少延伸至末端。在女性中,所有病例均存在生殖器重复。随访期为3个月至12年。在所有成年女性中,就诊动机与妊娠(成功分娩后的并发症、生育能力评估)或晚期并发症(端侧结肠吻合口粪石梗阻、反复发生的尿路感染伴肾瘢痕形成)有关。影响多个尾侧器官的复杂畸形可能会对长期生活质量的许多方面产生重大影响;因此,尾侧重复综合征患者需要提高认识并接受联合多学科治疗。

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本文引用的文献

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Cloacal Duplication: Single-center Experience in the Management of a Rare Anomaly.
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