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从头说起:单一机构中食管闭锁的类型、疾病严重程度分层及生存率

From the Ground Up: Esophageal Atresia Types, Disease Severity Stratification and Survival Rates at a Single Institution.

作者信息

Evanovich Devon Michael, Wang Jue Teresa, Zendejas Benjamin, Jennings Russell William, Bajic Dusica

机构信息

Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Boston, MA, United States.

Tufts School of Medicine, Tufts University, Boston, MA, United States.

出版信息

Front Surg. 2022 Mar 9;9:799052. doi: 10.3389/fsurg.2022.799052. eCollection 2022.

Abstract

Esophageal atresia (EA), although a rare congenital anomaly, represents one of the most common gastrointestinal birth defects. There is a gap in our knowledge regarding the impact of perioperative critical care in infants born with EA. This study addresses EA types, disease severity stratification, and mortality in a retrospective cohort at a single institution. Institutional Review Board approved our retrospective cross-sectional study of term-born ( = 53) and premature infants (28-37 weeks of gestation; = 31) that underwent primary surgical repair of EA at a single institution from 2009-2020. Demographic and clinical data were obtained from the electronic medical record, Powerchart (Cerner, London, UK). Patients were categorized by (i) sex, (ii) gestational age at birth, (iii) types of EA (in relation to respiratory tract anomalies), (iv) co-occurring congenital anomalies, (v) severity of disease (viz. American Society of Anesthesiologists (ASA) and Pediatric Risk Assessment (PRAm) scores), (vi) type of surgical repair for EA (primary anastomosis vs. Foker process), and (vii) survival rate classification using Spitz and Waterston scores. Data were presented as numerical sums and percentages. The frequency of anatomical types of EA in our cohort parallels that of the literature: 9.5% (8/84) type A, 9.5% (8/84) type B, 80% (67/84) type C, and 1% (1/84) type D. EA accounts for 88% (7/8) type A, 75% (6/8) type B, and 13% (9/67) type C in the cohort studied. Our novel results show a nearly equal distribution of sex per each EA type, and gestational age (term-born vs. premature) by anatomical EA type. PRAm scoring showed a wider range of disease severity (3-9) than ASA scores (III and IV). The survival rate in our EA cohort dramatically increased in comparison to the literature in previous decades. This retrospective analysis at a single institution shows incidence of EA per sex and gestational status for anatomical types (EA type A-D) and by surgical approach (primary anastomosis vs. Foker process for vs. EA, respectively). Despite its wider range, PRAm score was not more useful in predicting disease severity in comparison to ASA score. Increased survival rates over the last decade suggest a potential need to assess unique operative and perioperative risks in this unique population of patients. Presented findings also represent a foundation for future clinical studies of outcomes in infants born with EA.

摘要

食管闭锁(EA)虽是一种罕见的先天性畸形,但却是最常见的胃肠道出生缺陷之一。关于围手术期重症监护对EA患儿的影响,我们的认知存在差距。本研究在一家单一机构的回顾性队列研究中探讨了EA的类型、疾病严重程度分层及死亡率。机构审查委员会批准了我们对2009年至2020年在一家单一机构接受EA一期手术修复的足月儿(n = 53)和早产儿(孕28 - 37周;n = 31)进行的回顾性横断面研究。人口统计学和临床数据从电子病历Powerchart(英国伦敦的erner公司)中获取。患者按以下因素分类:(i)性别,(ii)出生时的孕周,(iii)EA的类型(与呼吸道畸形相关),(iv)合并的先天性畸形,(v)疾病严重程度(即美国麻醉医师协会(ASA)和儿科风险评估(PRAm)评分),(vi)EA的手术修复类型(一期吻合术与Foker术),以及(vii)使用Spitz和Waterston评分进行生存率分类。数据以数值总和及百分比形式呈现。我们队列中EA解剖类型的频率与文献报道相似:A型9.5%(8/84),B型9.5%(8/84),C型80%(67/84),D型1%(1/84)。在所研究的队列中,A型占88%(7/8),B型占75%(6/8),C型占13%(9/67)。我们的新结果显示,每种EA类型的性别分布以及按EA解剖类型划分的孕周(足月儿与早产儿)分布几乎相等。PRAm评分显示的疾病严重程度范围(3 - 9)比ASA评分(III级和IV级)更广。与过去几十年的文献相比,我们EA队列的生存率显著提高。在一家单一机构进行的这项回顾性分析显示了按解剖类型(EA A - D型)以及手术方式(分别针对I型和II型EA的一期吻合术与Foker术)划分的不同性别和孕周状态的EA发病率。尽管PRAm评分范围更广,但与ASA评分相比,在预测疾病严重程度方面并无更大用处。过去十年生存率的提高表明可能需要评估这一独特患者群体的独特手术及围手术期风险。所呈现的研究结果也为未来对EA患儿预后的临床研究奠定了基础。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/452d/8959439/cd0fbf45eff1/fsurg-09-799052-g0001.jpg

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