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

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,...

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Main Authors: Devon Michael Evanovich, Jue Teresa Wang, Benjamin Zendejas, Russell William Jennings, Dusica Bajic
Format: Article
Language:English
Published: Frontiers Media S.A. 2022-03-01
Series:Frontiers in Surgery
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fsurg.2022.799052/full
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author Devon Michael Evanovich
Devon Michael Evanovich
Jue Teresa Wang
Jue Teresa Wang
Benjamin Zendejas
Benjamin Zendejas
Benjamin Zendejas
Russell William Jennings
Russell William Jennings
Russell William Jennings
Dusica Bajic
Dusica Bajic
author_facet Devon Michael Evanovich
Devon Michael Evanovich
Jue Teresa Wang
Jue Teresa Wang
Benjamin Zendejas
Benjamin Zendejas
Benjamin Zendejas
Russell William Jennings
Russell William Jennings
Russell William Jennings
Dusica Bajic
Dusica Bajic
author_sort Devon Michael Evanovich
collection DOAJ
description 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 (n = 53) and premature infants (28–37 weeks of gestation; n = 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. Long-gap 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 short-gap vs. long-gap 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.
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spelling doaj.art-9dab86e0aa344a76aaf24665189b8d732022-12-22T01:41:21ZengFrontiers Media S.A.Frontiers in Surgery2296-875X2022-03-01910.3389/fsurg.2022.799052799052From the Ground Up: Esophageal Atresia Types, Disease Severity Stratification and Survival Rates at a Single InstitutionDevon Michael Evanovich0Devon Michael Evanovich1Jue Teresa Wang2Jue Teresa Wang3Benjamin Zendejas4Benjamin Zendejas5Benjamin Zendejas6Russell William Jennings7Russell William Jennings8Russell William Jennings9Dusica Bajic10Dusica Bajic11Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Boston, MA, United StatesTufts School of Medicine, Tufts University, Boston, MA, United StatesDepartment of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Boston, MA, United StatesHarvard Medical School, Harvard University, Boston, MA, United StatesHarvard Medical School, Harvard University, Boston, MA, United StatesDepartment of Surgery, Boston Children's Hospital, Boston, MA, United StatesEsophageal and Airway Treatment Center, Boston Children's Hospital, Boston, MA, United StatesHarvard Medical School, Harvard University, Boston, MA, United StatesDepartment of Surgery, Boston Children's Hospital, Boston, MA, United StatesEsophageal and Airway Treatment Center, Boston Children's Hospital, Boston, MA, United StatesDepartment of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Boston, MA, United StatesHarvard Medical School, Harvard University, Boston, MA, United StatesEsophageal 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 (n = 53) and premature infants (28–37 weeks of gestation; n = 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. Long-gap 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 short-gap vs. long-gap 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.https://www.frontiersin.org/articles/10.3389/fsurg.2022.799052/fullASAEALGEAmortalitytermterm-born infant
spellingShingle Devon Michael Evanovich
Devon Michael Evanovich
Jue Teresa Wang
Jue Teresa Wang
Benjamin Zendejas
Benjamin Zendejas
Benjamin Zendejas
Russell William Jennings
Russell William Jennings
Russell William Jennings
Dusica Bajic
Dusica Bajic
From the Ground Up: Esophageal Atresia Types, Disease Severity Stratification and Survival Rates at a Single Institution
Frontiers in Surgery
ASA
EA
LGEA
mortality
term
term-born infant
title From the Ground Up: Esophageal Atresia Types, Disease Severity Stratification and Survival Rates at a Single Institution
title_full From the Ground Up: Esophageal Atresia Types, Disease Severity Stratification and Survival Rates at a Single Institution
title_fullStr From the Ground Up: Esophageal Atresia Types, Disease Severity Stratification and Survival Rates at a Single Institution
title_full_unstemmed From the Ground Up: Esophageal Atresia Types, Disease Severity Stratification and Survival Rates at a Single Institution
title_short From the Ground Up: Esophageal Atresia Types, Disease Severity Stratification and Survival Rates at a Single Institution
title_sort from the ground up esophageal atresia types disease severity stratification and survival rates at a single institution
topic ASA
EA
LGEA
mortality
term
term-born infant
url https://www.frontiersin.org/articles/10.3389/fsurg.2022.799052/full
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