Elsevier

The Journal of Pediatrics

Volume 166, Issue 5, May 2015, Pages 1140-1144.e2
The Journal of Pediatrics

Original Article
Vascular Anomalies Associated with Esophageal Atresia and Tracheoesophageal Fistula

https://doi.org/10.1016/j.jpeds.2015.01.038Get rights and content

Objective

To report the incidence of congenital vascular anomalies in a cohort of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) while describing the clinical presentation, diagnosis, and consequences, and to evaluate the diagnostic value of esophagram in diagnosing an aberrant right subclavian artery (ARSA).

Methods

All patients born with EA/TEF between 2005 and 2013 were studied. Preoperative echocardiography reports, surgical descriptions of primary esophageal repair, and esophagrams were reviewed retrospectively.

Results

Of the 76 children born with EA/TEF included in this study, 14 (18%) had a vascular malformation. The incidence of a right aortic arch (RAA) was 6% (5 of 76), and that of an aberrant right subclavian artery (ARSA) was 12% (9 of 76). RAA was diagnosed in the neonatal period by echocardiography (4 of 5) or surgery (1 of 5), and ARSA was diagnosed by echocardiography (7 of 9) or later on the esophagram (2 of 9). Respiratory and/or digestive symptoms occurred in 9 of the 14 patients with vascular malformation. Both long-gap EA and severe cardiac malformations necessitating surgery were significantly associated with vascular anomalies (P < .05). The sensitivity of the esophagram for diagnosing ARSA was 66%, the specificity was 98%, the negative predictive value was 95%, and the positive predictive value was 85%.

Conclusion

ARSA and RAA have an incidence of 12% and 6% respectively, in patients with EA/TEF. A computed tomography angioscan is recommended to rule out such malformations when stenting of the esophagus is indicated, before esophageal replacement surgery, and when prolonged (>2 weeks) use of a nasogastric tube is considered.

Section snippets

Methods

This study was a retrospective review of all patients born with EA/TEF between January 2005 and October 2013 and followed at Sainte Justine Hospital. The study was approved by the local Institutional Review Board.

Patients were excluded from the analysis if they had been operated on elsewhere or if death occurred in the first week of life. Data were collected using standardized data abstraction forms. The abstracted information included the following patient-level variables: sex, birth weight,

Results

A total of 86 patients with EA/TEF born between January 2005 and October 2013 were seen at Sainte Justine Hospital. Ten children had insufficient data or follow-up, including 3 children who died in the first week of life and 7 children who underwent surgery elsewhere (Figure 2; available at www.jpeds.com.)

Seventy-six patients (36 females; 47%) met the inclusion criteria. The median birth weight was 2530 g (range, 1065-4400 g), and median gestational age was 37 weeks (range, 29-41 weeks). Seven

Discussion

We report that the overall incidence of ARSA and RAA is 18% in children who undergo surgery for EA/TEF. Long-gap EA and severe cardiac malformations requiring surgery are both significantly associated with vascular anomalies. We also show that the clinical consequences are variable, ranging from no symptoms to severe respiratory problems and dysphagia. The diagnostic yield of routinely used techniques–preoperative cardiac ultrasound and esophagram–is not optimal.

Congenital vascular anomalies

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    The authors declare no conflicts of interest.

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