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极低出生体重儿食管闭锁的预后和临床问题:病例系列。

Prognosis and clinical issues of esophageal atresia in extremely low birth weight neonates: a case series.

机构信息

Department of Pediatric Surgery, Hyogo Prefectural Kobe Children's Hospital, 650-0047 1-6-7, Minatojima Minamimachi, Chuo-Ku, Kobe, Japan.

Department of Neonatal Medicine, Hyogo Prefectural Kobe Children's Hospital, 650-0047 1-6-7, Minatojima Minamimachi, Chuo-Ku, Kobe, Japan.

出版信息

BMC Pediatr. 2023 Aug 16;23(1):401. doi: 10.1186/s12887-023-04237-1.

Abstract

BACKGROUND

Esophageal atresia (EA) in extremely low birth weight (ELBW) neonates is rare. This report aims to clarify EA's clinical courses and prognosis in ELBW neonates and the clinical issues of long-term survival cases.

METHODS

A retrospective analysis was conducted for 8 neonates diagnosed with esophageal atresia. Medical records of ELBW EA neonates treated at our institution were reviewed to assess patient demographics, clinical courses, and outcomes. Transferred patient data was obtained from their local physicians through questionnaires.

RESULTS

EA in ELBW neonates were included in 8 of EA infants (7%). Fatal respiratory and cardiovascular complications of trisomy 18 and complications related to immaturity such as liver failure and pulmonary hypertension were associated with poor prognosis. During primary operations, gastrostomy and esophageal banding were performed together in 50% of the cases, while gastrostomy was performed alone in 25%. The esophageal anastomosis was not performed during any primary operation. All causes of death, except for 1 case, were due to non-surgical causes. A long-term survival case of 17 years postoperatively was included.

CONCLUSION

Although ELBW EA has a poor prognosis, long-term survival is possible in some cases, so aggressive therapeutic intervention is considered essential. It is important to share information about the prognosis with parents and multidisciplinary specialists and to select an appropriate treatment strategy for each case.

摘要

背景

极低出生体重儿(ELBW)中食管闭锁(EA)较为罕见。本研究旨在阐明 ELBW 新生儿 EA 的临床过程和预后,以及长期存活病例的临床问题。

方法

对 8 例诊断为食管闭锁的新生儿进行回顾性分析。回顾性分析了在我院治疗的 ELBW 食管闭锁新生儿的病历,评估了患者的人口统计学、临床过程和结局。通过问卷调查获得了转院患者的当地医生的数据。

结果

EA 在 ELBW 新生儿中占 EA 患儿的 7%(8 例)。18 三体综合征致命的呼吸和心血管并发症以及与不成熟相关的并发症,如肝功能衰竭和肺动脉高压,与不良预后相关。在初次手术中,50%的病例同时进行了胃造口术和食管环扎术,25%的病例仅进行了胃造口术。在任何初次手术中均未进行食管吻合术。除 1 例外,所有死亡病例均由非手术原因引起。包括 1 例术后 17 年长期生存的病例。

结论

尽管 ELBW 伴 EA 预后不良,但部分病例可长期生存,因此积极的治疗干预被认为是必要的。与家长和多学科专家分享预后信息,并为每个病例选择合适的治疗策略非常重要。

相似文献

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1
Improving survival in patients with trisomy 18.提高 18 三体综合征患者的生存率。
Am J Med Genet A. 2022 Apr;188(4):1048-1055. doi: 10.1002/ajmg.a.62605. Epub 2021 Dec 8.
2
Trisomy 18 Trends over the Last 20 Years.18 三体综合征近 20 年的发病趋势
J Pediatr. 2021 Dec;239:206-211.e1. doi: 10.1016/j.jpeds.2021.07.062. Epub 2021 Aug 4.
3
Surveillance guidelines for children with trisomy 18.18 三体综合征患儿的监测指南。
Am J Med Genet A. 2021 Apr;185(4):1294-1303. doi: 10.1002/ajmg.a.62097. Epub 2021 Feb 2.
10
Surgical intervention for esophageal atresia in patients with trisomy 18.18三体综合征患者食管闭锁的外科干预
Am J Med Genet A. 2014 Feb;164A(2):324-30. doi: 10.1002/ajmg.a.36294. Epub 2013 Dec 5.

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