Original ArticleVascular Anomalies Associated with Esophageal Atresia and Tracheoesophageal Fistula
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
References (28)
- et al.
The significance of right aortic arch in repair of esophageal atresia and tracheoesophageal fistula
J Pediatr Surg
(1977) - et al.
The effect of a right-sided aortic arch on outcome in children with esophageal atresia and tracheoesophageal fistula
J Pediatr Surg
(2006) - et al.
The management of oesophageal atresia in neonates with right-sided aortic arch
J Pediatr Surg
(2000) - et al.
Esophageal atresia and tracheoesophageal fistula: surgical experience over two decades
Ann Thorac Surg
(1997) - et al.
Esophageal atresia associated with a rare vascular ring and esophageal duplication diverticulum: a case report and review of the literature
J Pediatr Surg
(2012) - et al.
Aortic arch anomalies associated with long gap esophageal atresia and tracheoesophageal fistula
J Pediatr Surg
(1997) - et al.
Double aortic arch associated with esophageal atresia and tracheoesophageal fistula
J Pediatr Surg
(1989) - et al.
Novel approaches for the treatment of the aberrant right subclavian artery and its aneurysms
J Vasc Surg
(2008) - et al.
Dysphagia lusoria: clinical aspects, manometric findings, diagnosis, and therapy
Am J Gastroenterol
(2000) - et al.
Follow-up of surgical correction of aortic arch anomalies causing tracheoesophageal compression: a 38-year single institution experience
J Pediatr Surg
(2009)
Surgical treatment of vascular rings: the Mayo Clinic experience
Mayo Clin Proc
Vascular ring abnormalities: a retrospective study of 62 cases
J Pediatr Surg
Oesophageal atresia
Orphanet J Rare Dis
The thoracic and abdominal aorta
Cited by (31)
Esophageal Surgery in Neonates: Esophageal Atresia, Gastroesophageal Reflux, and Other Congenital Anomalies
2023, Principles of NeonatologyStructural Anomalies of the Gastrointestinal Tract
2023, Avery's Diseases of the NewbornAnatomy and embryology of tracheo-esophageal fistula
2022, Seminars in Pediatric SurgeryCitation Excerpt :ARSA forms an incomplete vascular ring and as such is frequently asymptomatic and often undiagnosed as it is not visualized in the operative field. However, extrinsic posterior compression can lead to symptoms, particularly where the esophagus and trachea have reduced underlying rigidity, leading to dyspnea, recurrent cough, aspiration, and exacerbation of dysphagia.20 Fistula formation between aberrant vessels and the esophagus after stent or NG tube placement has been reported so whilst incidence is rare, recognition of these anatomical variants is important.
Advances in the Surgical Management of Esophageal Atresia
2021, Advances in PediatricsCitation Excerpt :Before closure, a chest tube or closed suction drain is frequently placed in case an anastomotic leak develops. Approximately 5% of infants with EA/TEF have a right-sided aortic arch (RAA), which can complicate exposure and repair of the EA/TEF [19,20,31,32]. Controversy exists regarding the optimal side to perform a thoracotomy for repair of the EA/TEF in patients with an RAA.
Evaluation of pulmonary complications and affecting factors in children for repaired esophageal atresia and tracheoesophageal fistula
2021, Respiratory MedicineCitation Excerpt :Moreover, major vascular anomalies and vascular rings are more common in patients with EA-TEF. Complications such as dyspnea, dysphagia, and aspiration pneumonia may increase depending on the type of malformations and degree of compression on the esophagus and trachea [16]. Therefore, the diagnosis of vascular anomalies with accompanying malformations (right aortic arch (RAA) and aberrant left subclavian artery (ALSA); RAA with mirror image; left aortic arch and aberrant right subclavian artery (ARSA); and double aortic arch) and genetic anomalies ((VACTERL spectrum (Vertebral, Anorectal, Cardiac, TracheoEsophageal, Renal and Limb), microcephaly, micrognathia, pyloric stenosis, duodenal atresia, a single umbilical artery, and anomalies of the genitourinary, respiratory and gastrointestinal systems) by flexible laryngoscopy, echocardiography and upper gastrointestinal series, genetic consultation etc. in the preoperative period can reduce postoperative early and late complications [17,18].
Aberrant Right Subclavian Artery to Esophageal Fistula: A Rare Case and Its Management
2020, Annals of Thoracic Surgery
The authors declare no conflicts of interest.