Vascular Anomalies Associated with Esophageal Atresia and Tracheoesophageal Fistula

doi:10.1016/j.jpeds.2015.01.038

The Journal of Pediatrics

Volume 166, Issue 5, May 2015, Pages 1140-1144.e2

https://doi.org/10.1016/j.jpeds.2015.01.038 Get 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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Esophageal pathologies in the newborn period include esophageal atresia (EA), gastroesophageal reflux, and other congenital anomalies such as stenosis, duplication cysts, and vascular rings. The presentation of esophageal pathology in the newborn includes both upper gastrointestinal symptoms and respiratory symptoms, and the diagnosis can be challenging at times. EA with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus and requires surgical correction in all cases. Although the management of short-gap EA has become quite successful during the past several decades, there continues to be significant morbidity as infants grow into childhood. To date, the surgical management of EA has been based largely on individual surgeon experience, preference, and training, with wide variation occurring nationally in certain aspects of care. However, recent multicentered registries have begun to elucidate the optimal aspects of EA/TEF management, and prospective studies will continue to move the field forward to improve outcomes. Similarly, the surgical management of other esophageal pathologies in the neonatal period is benefiting from increasing evidence and understanding of those disorders.
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Anatomy and embryology of tracheo-esophageal fistula
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Anomalies in tracheo-esophageal development result in a spectrum of congenital malformations ranging from, most commonly, esophageal atresia with or without trachea-esophageal fistula (EA+/-TEF) to esophageal web, duplication, stricture, tracheomalacia and tracheal agenesis. Despite the relative frequency of EA, however, the underlying etiology remains unknown and is likely due to a combination of genetic, epigenetic and environmental factors. In recent years, animal models have dramatically increased our understanding of the molecular and morphological processes involved in normal esophageal development during the key stages of anterior-posterior regionalization, dorsal-ventral patterning and morphogenic separation. Moreover, the use of animal models in conjunction with increasingly advanced techniques such as genomic sequencing, sophisticated live imaging studies and organoid models have more recently cast light on potential mechanisms involved in EA pathogenesis. This article aims to unravel some of the mysteries behind the anatomy and embryology of EA whilst providing insights into future directions for research.
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Citation Excerpt :
Five percent of patients with EA have a right-sided arch, which may complicate exposure during the repair [19]. In addition, patients with EA can have other vascular anomalies, including double aortic arch, circumflex aorta, and/or aberrant right subclavian arteries, which, if suspected, can be further evaluated with a computed tomography angiogram or cardiac MRI [8,20,21]. Other commonly associated anomalies include those of the musculoskeletal system, genitourinary tract, intestine, spine, and craniofacial locations (see Table 1).
Evaluation of pulmonary complications and affecting factors in children for repaired esophageal atresia and tracheoesophageal fistula
2021, Respiratory Medicine
Recurrent pulmonary infections, wheezing and stridor due to swallowing dysfunction, esophageal dysmotility, gastroesophageal reflux, tracheomalacia and bronchomalacia are frequently seen complications after esophageal atresia and tracheo-esophageal fistula (EA-TEF) surgeries. This study aimed to investigate the frequency and causes of respiratory problems and to evaluate the factors that affect respiratory morbidity in patients who had undergone EA-TEF repair in a tertiary referral center.
Preoperative and postoperative records of patients with EA, TEF + EA and isolated EA were examined retrospectively. Accompanied diseases and swallowing dysfunction symptoms were questioned. Bronchoalveolar lavage results were investigated if the patient had flexible bronchoscopy.
A total of 71 children with EA were included in the study, and seven patients who did not have follow-up after surgery were excluded. 46 of the 64 patients continue regular follow-up visits in our department. Male sex, primary EA repair in another center, EA type C, accompanying genetic anomalies, severe tracheomalacia, late per oral feeding (1 year after surgery), and severe GER were found to cause significantly higher incidence of coughing, recurrent wheezing, recurrent pneumonia, and bronchiectasis despite surgical and medical treatments (p = 0.048, p = 0.045, p = 0.009, p = 0.029, p = 0.025).
Even if anatomical anomalies are corrected by surgery in patients who underwent EA repair, precautions can be taken for GERD, laryngotracheomalacia, and swallowing dysfunction, and effective pulmonary rehabilitation can be initiated with early multidisciplinary approach before the development of respiratory tract symptoms.
Aberrant Right Subclavian Artery to Esophageal Fistula: A Rare Case and Its Management
2020, Annals of Thoracic Surgery
A 29-year-old woman underwent esophageal stent placement after developing esophageal stenosis in the setting of tracheoesophageal fistula repair in childhood. The patient developed hemoptysis from an esophageal to aberrant right subclavian artery fistula; this was managed with several staged procedures involving arterial stent placement, carotid-to-subclavian bypass, and aberrant subclavian artery ligation. The patient then underwent pericardial patch repair of her perforated esophagus. This case illustrates the importance of understanding congenital anatomy and frequent associations, such as tracheoesophageal fistula and aberrant right subclavian artery; furthermore, it demonstrates the importance of multidisciplinary care for complex cases.

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: The authors declare no conflicts of interest.

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Original ArticleVascular Anomalies Associated with Esophageal Atresia and Tracheoesophageal Fistula

Objective

Methods

Results

Conclusion

Section snippets

Methods

Results

Discussion

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

Ann Thorac Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Vasc Surg

Am J Gastroenterol

J Pediatr Surg

Mayo Clin Proc

J Pediatr Surg

Oesophageal atresia

Orphanet J Rare Dis

The thoracic and abdominal aorta

Original Article
Vascular Anomalies Associated with Esophageal Atresia and Tracheoesophageal Fistula