Infants with esophageal atresia and right aortic arch: Characteristics and outcomes from the Midwest Pediatric Surgery Consortium

on behalf of the Midwest Pediatric Surgery Consortium

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Purpose: Right sided aortic arch (RAA) is a rare anatomic finding in infants with esophageal atresia with or without tracheoesophageal fistula (EA/TEF). In the presence of RAA, significant controversy exists regarding optimal side for thoracotomy in repair of the EA/TEF. The purpose of this study was to characterize the incidence, demographics, surgical approach, and outcomes of patients with RAA and EA/TEF. Methods: A multi-institutional, IRB approved, retrospective cohort study of infants with EA/TEF treated at 11 children's hospitals in the United States over a 5-year period (2009 to 2014) was performed. All patients had a minimum of one-year follow-up. Results: In a cohort of 396 infants with esophageal atresia, 20 (5%) had RAA, with 18 having EA with a distal TEF and 2 with pure EA. Compared to infants with left sided arch (LAA), RAA infants had a lower median birth weight, (1.96 kg (IQR 1.54–2.65) vs. 2.57 kg (2.00–3.03), p = 0.01), earlier gestational age (34.5 weeks (IQR 32–37) vs. 37 weeks (35–39), p = 0.01), and a higher incidence of congenital heart disease (90% vs. 32%, p < 0.0001). The most common cardiac lesions in the RAA group were ventricular septal defect (7), tetralogy of Fallot (7) and vascular ring (5). Seventeen infants with RAA underwent successful EA repair, 12 (71%) via right thoracotomy and 5 (29%) through left thoracotomy. Anastomotic strictures trended toward a difference in RAA patients undergoing right thoracotomy for primary repair of their EA/TEF compared to left thoracotomy (50% vs. 0%, p = 0.1). Side of thoracotomy in RAA patients undergoing EA/TEF repair was not significantly associated with mortality, anastomotic leak, recurrent laryngeal nerve injury, recurrent fistula, or esophageal dehiscence (all p > 0.29). Conclusion: RAA in infants with EA/TEF is rare with an incidence of 5%. Compared to infants with EA/TEF and LAA, infants with EA/TEF and RAA are more severely ill with lower birth weight and higher rates of prematurity and complex congenital heart disease. In neonates with RAA, surgical repair of the EA/TEF is technically feasible via thoracotomy from either chest. A higher incidence of anastomotic strictures may occur with a right-sided approach. Level of evidence: Level III.

Original languageEnglish (US)
Pages (from-to)688-692
Number of pages5
JournalJournal of pediatric surgery
Volume54
Issue number4
DOIs
StatePublished - Apr 2019

Fingerprint

Esophageal Atresia
Thoracic Aorta
Pediatrics
Incidence
Thoracotomy
Birth Weight
Heart Diseases
Research Ethics Committees
Patient Rights
Gestational Age
Pathologic Constriction
Cohort Studies
Thorax
Retrospective Studies
Demography
Newborn Infant

Keywords

  • Anastomotic stricture
  • Complications
  • Esophageal atresia
  • Right aortic arch
  • Thoracotomy

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

Cite this

@article{94b727f1aea1475398301265f130a242,
title = "Infants with esophageal atresia and right aortic arch: Characteristics and outcomes from the Midwest Pediatric Surgery Consortium",
abstract = "Purpose: Right sided aortic arch (RAA) is a rare anatomic finding in infants with esophageal atresia with or without tracheoesophageal fistula (EA/TEF). In the presence of RAA, significant controversy exists regarding optimal side for thoracotomy in repair of the EA/TEF. The purpose of this study was to characterize the incidence, demographics, surgical approach, and outcomes of patients with RAA and EA/TEF. Methods: A multi-institutional, IRB approved, retrospective cohort study of infants with EA/TEF treated at 11 children's hospitals in the United States over a 5-year period (2009 to 2014) was performed. All patients had a minimum of one-year follow-up. Results: In a cohort of 396 infants with esophageal atresia, 20 (5{\%}) had RAA, with 18 having EA with a distal TEF and 2 with pure EA. Compared to infants with left sided arch (LAA), RAA infants had a lower median birth weight, (1.96 kg (IQR 1.54–2.65) vs. 2.57 kg (2.00–3.03), p = 0.01), earlier gestational age (34.5 weeks (IQR 32–37) vs. 37 weeks (35–39), p = 0.01), and a higher incidence of congenital heart disease (90{\%} vs. 32{\%}, p < 0.0001). The most common cardiac lesions in the RAA group were ventricular septal defect (7), tetralogy of Fallot (7) and vascular ring (5). Seventeen infants with RAA underwent successful EA repair, 12 (71{\%}) via right thoracotomy and 5 (29{\%}) through left thoracotomy. Anastomotic strictures trended toward a difference in RAA patients undergoing right thoracotomy for primary repair of their EA/TEF compared to left thoracotomy (50{\%} vs. 0{\%}, p = 0.1). Side of thoracotomy in RAA patients undergoing EA/TEF repair was not significantly associated with mortality, anastomotic leak, recurrent laryngeal nerve injury, recurrent fistula, or esophageal dehiscence (all p > 0.29). Conclusion: RAA in infants with EA/TEF is rare with an incidence of 5{\%}. Compared to infants with EA/TEF and LAA, infants with EA/TEF and RAA are more severely ill with lower birth weight and higher rates of prematurity and complex congenital heart disease. In neonates with RAA, surgical repair of the EA/TEF is technically feasible via thoracotomy from either chest. A higher incidence of anastomotic strictures may occur with a right-sided approach. Level of evidence: Level III.",
keywords = "Anastomotic stricture, Complications, Esophageal atresia, Right aortic arch, Thoracotomy",
author = "{on behalf of the Midwest Pediatric Surgery Consortium} and Lal, {Dave R.} and Gadepalli, {Samir K.} and Downard, {Cynthia D.} and Minneci, {Peter C.} and Michelle Knezevich and Chelius, {Thomas H.} and Rapp, {Cooper T.} and Deborah Billmire and Steven Bruch and {Carland Burns}, R. and Deans, {Katherine J.} and Fallat, {Mary E.} and Fraser, {Jason D.} and Julia Grabowski and Ferdynand Hebel and Helmrath, {Michael A.} and Hirschl, {Ronald B.} and Rashmi Kabre and Jonathan Kohler and Landman, {Matthew P.} and Leys, {Charles M.} and Mak, {Grace Z.} and Ostlie, {Daniel J.} and Jessica Raque and Beth Rymeski and Saito, {Jacqueline M.} and {St. Peter}, {Shawn D.} and {von Allmen}, Daniel and Warner, {Brad W.} and Sato, {Thomas T.}",
year = "2019",
month = "4",
doi = "10.1016/j.jpedsurg.2018.08.002",
language = "English (US)",
volume = "54",
pages = "688--692",
journal = "Journal of Pediatric Surgery",
issn = "0022-3468",
publisher = "W.B. Saunders Ltd",
number = "4",

}

Infants with esophageal atresia and right aortic arch : Characteristics and outcomes from the Midwest Pediatric Surgery Consortium. / on behalf of the Midwest Pediatric Surgery Consortium.

In: Journal of pediatric surgery, Vol. 54, No. 4, 04.2019, p. 688-692.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Infants with esophageal atresia and right aortic arch

T2 - Characteristics and outcomes from the Midwest Pediatric Surgery Consortium

AU - on behalf of the Midwest Pediatric Surgery Consortium

AU - Lal, Dave R.

AU - Gadepalli, Samir K.

AU - Downard, Cynthia D.

AU - Minneci, Peter C.

AU - Knezevich, Michelle

AU - Chelius, Thomas H.

AU - Rapp, Cooper T.

AU - Billmire, Deborah

AU - Bruch, Steven

AU - Carland Burns, R.

AU - Deans, Katherine J.

AU - Fallat, Mary E.

AU - Fraser, Jason D.

AU - Grabowski, Julia

AU - Hebel, Ferdynand

AU - Helmrath, Michael A.

AU - Hirschl, Ronald B.

AU - Kabre, Rashmi

AU - Kohler, Jonathan

AU - Landman, Matthew P.

AU - Leys, Charles M.

AU - Mak, Grace Z.

AU - Ostlie, Daniel J.

AU - Raque, Jessica

AU - Rymeski, Beth

AU - Saito, Jacqueline M.

AU - St. Peter, Shawn D.

AU - von Allmen, Daniel

AU - Warner, Brad W.

AU - Sato, Thomas T.

PY - 2019/4

Y1 - 2019/4

N2 - Purpose: Right sided aortic arch (RAA) is a rare anatomic finding in infants with esophageal atresia with or without tracheoesophageal fistula (EA/TEF). In the presence of RAA, significant controversy exists regarding optimal side for thoracotomy in repair of the EA/TEF. The purpose of this study was to characterize the incidence, demographics, surgical approach, and outcomes of patients with RAA and EA/TEF. Methods: A multi-institutional, IRB approved, retrospective cohort study of infants with EA/TEF treated at 11 children's hospitals in the United States over a 5-year period (2009 to 2014) was performed. All patients had a minimum of one-year follow-up. Results: In a cohort of 396 infants with esophageal atresia, 20 (5%) had RAA, with 18 having EA with a distal TEF and 2 with pure EA. Compared to infants with left sided arch (LAA), RAA infants had a lower median birth weight, (1.96 kg (IQR 1.54–2.65) vs. 2.57 kg (2.00–3.03), p = 0.01), earlier gestational age (34.5 weeks (IQR 32–37) vs. 37 weeks (35–39), p = 0.01), and a higher incidence of congenital heart disease (90% vs. 32%, p < 0.0001). The most common cardiac lesions in the RAA group were ventricular septal defect (7), tetralogy of Fallot (7) and vascular ring (5). Seventeen infants with RAA underwent successful EA repair, 12 (71%) via right thoracotomy and 5 (29%) through left thoracotomy. Anastomotic strictures trended toward a difference in RAA patients undergoing right thoracotomy for primary repair of their EA/TEF compared to left thoracotomy (50% vs. 0%, p = 0.1). Side of thoracotomy in RAA patients undergoing EA/TEF repair was not significantly associated with mortality, anastomotic leak, recurrent laryngeal nerve injury, recurrent fistula, or esophageal dehiscence (all p > 0.29). Conclusion: RAA in infants with EA/TEF is rare with an incidence of 5%. Compared to infants with EA/TEF and LAA, infants with EA/TEF and RAA are more severely ill with lower birth weight and higher rates of prematurity and complex congenital heart disease. In neonates with RAA, surgical repair of the EA/TEF is technically feasible via thoracotomy from either chest. A higher incidence of anastomotic strictures may occur with a right-sided approach. Level of evidence: Level III.

AB - Purpose: Right sided aortic arch (RAA) is a rare anatomic finding in infants with esophageal atresia with or without tracheoesophageal fistula (EA/TEF). In the presence of RAA, significant controversy exists regarding optimal side for thoracotomy in repair of the EA/TEF. The purpose of this study was to characterize the incidence, demographics, surgical approach, and outcomes of patients with RAA and EA/TEF. Methods: A multi-institutional, IRB approved, retrospective cohort study of infants with EA/TEF treated at 11 children's hospitals in the United States over a 5-year period (2009 to 2014) was performed. All patients had a minimum of one-year follow-up. Results: In a cohort of 396 infants with esophageal atresia, 20 (5%) had RAA, with 18 having EA with a distal TEF and 2 with pure EA. Compared to infants with left sided arch (LAA), RAA infants had a lower median birth weight, (1.96 kg (IQR 1.54–2.65) vs. 2.57 kg (2.00–3.03), p = 0.01), earlier gestational age (34.5 weeks (IQR 32–37) vs. 37 weeks (35–39), p = 0.01), and a higher incidence of congenital heart disease (90% vs. 32%, p < 0.0001). The most common cardiac lesions in the RAA group were ventricular septal defect (7), tetralogy of Fallot (7) and vascular ring (5). Seventeen infants with RAA underwent successful EA repair, 12 (71%) via right thoracotomy and 5 (29%) through left thoracotomy. Anastomotic strictures trended toward a difference in RAA patients undergoing right thoracotomy for primary repair of their EA/TEF compared to left thoracotomy (50% vs. 0%, p = 0.1). Side of thoracotomy in RAA patients undergoing EA/TEF repair was not significantly associated with mortality, anastomotic leak, recurrent laryngeal nerve injury, recurrent fistula, or esophageal dehiscence (all p > 0.29). Conclusion: RAA in infants with EA/TEF is rare with an incidence of 5%. Compared to infants with EA/TEF and LAA, infants with EA/TEF and RAA are more severely ill with lower birth weight and higher rates of prematurity and complex congenital heart disease. In neonates with RAA, surgical repair of the EA/TEF is technically feasible via thoracotomy from either chest. A higher incidence of anastomotic strictures may occur with a right-sided approach. Level of evidence: Level III.

KW - Anastomotic stricture

KW - Complications

KW - Esophageal atresia

KW - Right aortic arch

KW - Thoracotomy

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U2 - 10.1016/j.jpedsurg.2018.08.002

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