Aberrant Right Subclavian Artery-Esophageal Fistula in 20-Year-Old with VATER Association

Abstract

Aberrant right subclavian artery (ARSA), the most common aortic arch abnormality, occurs in approximately 0.5 to 1.8% of the general population, with prevalence of up to 25% in those with esophageal atresia. Although ARSA is often asymptomatic, a fistulous tract into esophagus may develop with prolonged nasogastric tube placement or endotracheal intubation and lead to potentially fatal hematemesis. We present a first case of ARSA-esophageal fistula in a 20-year-old woman with VATER association in the absence of an esophageal anomaly and review 28 cases of ARSA-esophageal fistula reported in the literature to date. Requiring nasogastric and endotracheal tube placement for approximately 4 months, the patient had a prolonged hospital course and died after sudden hematemesis. An autopsy demonstrated an ARSA-esophageal fistula and no other source of upper gastrointestinal bleeding. In patients with esophageal atresia requiring prolonged placement of an endotracheal or nasogastric tube, a screening imaging study and corrective surgery may be indicated. Although the mortality rate is still high, timely recognition and repair of ARSA-esophageal fistula appear to be improving. Given the potentially prolonged latency for its development with occasional presence of heralding symptoms, increased awareness may facilitate surgical intervention to prevent a catastrophic exsanguination.

Aberrant Left Subclavian Artery-Esophageal Fistula in a Patient with a Prolonged Use of Nasogastric Tube: A Case Report and Literature Review

“…Thus, the prolonged use of NG and/or ET tubes in a patient with a retroesophageal aberrant artery or other aortic arch anomalies forming a vascular ring may make the patient susceptible to arterial-esophageal fistula formation. A literature search showed a total of 40 documented cases of aberrant subclavian artery and esophageal fistulae ( Table 1 ) [ 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 ]. Among those cases, a long-term use of NG and/or ET tube was thought to be the main cause of fistula formation in at least 18 cases.…”

Upper Gastrointestinal Bleeding From Aberrant Right Subclavian Artery-Esophageal Fistula

“…The most common etiology for development of ARSA-esophageal fistula appeared to be secondary to compression, friction, or pressure necrosis from recent instrumentation with an endotracheal tube, nasogastric tube, or tracheostomy tube, seen in 14 of the 33 summarized cases. 14,15,17,19,21,22,24,27,29,31,[38][39][40][41] Fistula development from prior placement of esophageal stent was noted in 5 cases. 34,36,42,43 Other reported causes for fistula formation included placement of a salivary bypass tube and a possible consequence of gastric pull-up surgery.…”