An aberrant right subclavian artery (ARSA) is the most common aortic arch anomaly, but only 19 previous cases of ARSA-esophageal fistula have been reported. Six patients have survived their bleeding episode. We describe the case of a 44-year-old woman who developed massive hemoptysis. Laryngoscopy, bronchoscopy, head and neck angiogram, and median sternotomy did not reveal what was presumed initially to be a tracheoinnominate fistula. Contrasted CT showed an anomalous subclavian artery posterior to the esophagus. Given the technical challenge of approaches for this pathology, the patient was unfit for open surgical repair. Therefore, endovascular covered stent grafts were deployed spanning the segment of the subclavian artery in continuity with the esophagus, via a right brachial artery approach. Unfortunately, the patient died after successful placement of the grafts.
Artery-esophageal fistulae are rare but can cause massive and life-threatening hemorrhage. Prompt diagnosis and treatment are mandatory if the patient is to be saved, since these fistulae are fatal in a majority of cases. These fistulae most often develop secondary to prior thoracic operations, infection, neoplasm, foreign body, or radiotherapy and typically involve a fistula between the esophagus and the adjacent descending thoracic aorta [
A 44-year-old woman with a history of gastroesophageal reflux disease and hypertension presented with worsening shortness of breath. She was found to have pneumonia and required intubation. She developed multiple complications, including sepsis, adult respiratory distress syndrome, acute renal failure, intensive care neuropathy, cardiac arrhythmias, and cardiac arrest. Approximately three weeks after hospitalization, a tracheostomy tube was placed. She then developed intra-abdominal abscesses after a gastrostomy tube became dislodged. She underwent exploratory laparotomy with abscess drainage and closure of the gastrostomy and was maintained on total parenteral nutrition.
Four months after admission to the hospital, a nasogastric tube was placed and enteral nutrition was started. Sixteen days later, she experienced an episode of substantial bleeding, and it was not clear whether this came from the airway or oropharynx. Her hematocrit was 20 percent, platelets were 396,000, and blood urea nitrogen was 13 mg/dL. She was resuscitated with packed red blood cells, platelets, cryoprecipitate, and fresh frozen plasma; selective carotid arteriography of the head and neck, direct laryngoscopy, and bronchoscopy were unremarkable.
Three days later, she again experienced severe bleeding, which was initially felt to have originated from her airway. She was taken to the operating room by the vascular surgery team with the preoperative impression that she had a tracheoinnominate fistula. Median sternotomy revealed a right carotid artery arising directly from the aortic arch with no innominate or right subclavian artery in the anterior mediastinum. No artery to trachea fistula was identified and a provisional diagnosis of aberrant right subclavian artery with subclavian to esophageal fistula was made given the observation of the absence of the subclavian and innominate arteries in the usual location. The sternotomy was closed and a CT of the chest was performed, which demonstrated the anomalous right subclavian artery without aneurysmal degeneration (Figures
Sequential CT scan images show origin of right subclavian artery from the proximal descending thoracic aorta ((a), black arrow) and coursing posterior to the esophagus ((b), black arrow).
(a) Intraoperative angiogram performed in an AP projection through a sheath positioned in the right axillary artery from a right brachial approach. The right subclavian artery is seen (wide arrow) as is the left subclavian artery (narrow arrow). (b) Intraoperative image in the same projection after deployment of a balloon expandable covered stent at level of esophageal-arterial communication. The arrow indicates the position of the covered stent.
The presence of an aberrant right subclavian artery (ARSA) is the most common aortic arch anomaly, present in 0.2–2.5% of the population [
Despite the relative frequency of ARSA, our case of ARSA-esophageal fistula is only the twentieth reported case of ARSA-esophageal fistula, which has involved both aneurysmal and nonaneurysmal anomalous arteries. None of the patients who developed an ARSA-esophageal fistula associated with an aneurysmal artery have survived, and none of the first ten reported cases of nonaneurysmal ARSA-esophageal fistula survived their bleeding episode. Hemorrhage was fatal in fourteen of these twenty patients, bringing the mortality rate in reported cases to 70%.
Diagnosis of the ARSA-esophageal fistula is challenging. In most cases, esophagoscopy cannot be used to assist in diagnosis of acute hemorrhage because of the extreme volume of hematemesis [
Management of patients with bleeding from an anomalous subclavian artery to esophagus fistula is very challenging since bleeding in these patients presents as abrupt, rapidly exsanguinating hematemesis, and the etiology is from an unusual and unexpected source. Sentinel bleeding was reported in four of the twenty patients with ARSA-esophageal fistula, including our patient. In most patients with nonaneurysmal ARSA-esophageal fistula, presentation is abrupt, massive arterial bleeding for several days to weeks after placement of a nasogastric or endotracheal tube [
Initial management can involve the use of a Sengstaken-Blakemore tube, which can be used to temporize the bleeding while the patient is stabilized, as in patients with any artery-esophageal fistula. In actively bleeding patients, right thoracotomy is recommended by most physicians to treat the problem. This results in appropriate exposure of both the anomalous artery and esophagus. In general, infants do not require revascularization of the right arm, because of excellent collateral circulation [
In patients who are at high surgical risk and not likely to survive a thoracotomy, or those individuals with a limited life expectancy, arteriography with endovascular stent grafting may be used. Inman et al. advocated placing stents to span the width of the posterior esophagus overlapping anomalous artery on both the distal and proximal ends [
Associated risk factors for the development of ARSA-esophageal fistulization have varied (Table
Reported cases of anomalous subclavian artery-esophageal fistulae.
Study | Age (years) | Sex | Pathology | Cause | Predisposing factors | Time lag after intubation (days) | Outcome | Treatment | Sentinel bleed |
---|---|---|---|---|---|---|---|---|---|
Lynn, 1969 | 57 | M | Aneurysm | Aneurysm | Fatal | ||||
Reynes, 1976 | 72 | F | Aneurysm | Aneurysm | Fatal | Yes | |||
Merchant et al., 1977 | 17 | F | Recovery after cesarian section | NGT | 9 | Fatal | |||
Livesay et al., 1982 | 25 | M | Head trauma | ETT/NGT | 13 | Fatal | |||
Jungck and Puschel, 1983 | 6 | M | Multiple trauma | ETT/NGT | 42 | Fatal | |||
Belkin et al., 1984 | 27 | M | ENT cancer | NGT | 60 | Fatal | |||
Edwards et al., 1984 | 36 | F | Subarachnoid hemorrhage | NGT | Corticotherapy | 27 | Fatal | Yes | |
Gosset et al., 1985 | 72 | F | Recovery after aortic repair | ETT/NGT | Corticotherapy, infection, surgery | 30 | Fatal | ||
Guzzeta et al., 1989 | 0.4 | F | Recovery after heart surgery | NGT | Survived | ||||
Kulling, 1989 | M | Aneurysm | Fatal | ||||||
Ikeda et al., 1991 | 9 | M | Recovery after heart surgery | ETT/NGT | Surgery | Fatal | |||
Stone, 1990 | M | Fatal | |||||||
Hirakata et al., 1991 | 55 | M | Esophageal cancer | NGT | Surgery, irradiation, cancer | 44 | Survived | ||
Miller et al., 1996 | 11 | F | Intracerebral hemorrhage | ETT/NGT | 17 | Survived | Surgery | No | |
Minyard and Smith, 2000 | 39 | F | Head trauma | NGT | Fatal | ||||
Feugier et al., 2003 | 24 | M | Multiple trauma | ETT/NGT | Deceleration syndrome | 31 | Survived | Surgery | |
Eynden, 2006 | 9 | F | Esophageal stent | Caustic ingestion in Down syndrome patient with subsequent esophageal fissures and mediastinitis | Survived | Surgery | Yes | ||
Inman et al., 2008 | 63 | M | Salivary bypass tube | Chemotherapy and radiation, then salvage total laryngectomy and pharyngectomy | Fatal | Endovascular stent | Yes | ||
Magagna et al., 2008 | 73 | F | Laryngectomy, then chemotherapy and radiation therapy | Survived | Stent | Yes | |||
Current study | 41 | F | NGT, tracheostomy tube | Corticotherapy, sepsis | Fatal | Stent | Yes |
It is, therefore, logical to recommend avoiding prolonged nasogastric or endotracheal intubation in patients with known aberrant right subclavian artery. Feugier et al. have suggested screening of intensive care patients before long term placement of nasogastric tubes [
Nasogastric tubes should be removed and gastrostomy performed in patients with ARSA on imaging [
The course of an anomalous subclavian artery usually brings it posterior and contiguous with the esophagus. Although usually asymptomatic, the presence of prolonged nasogastric intubation may predispose an erosion of the esophagus into the adjacent artery leading to an arterial-esophageal fistula. Subclavian esophageal fistulae do not always present with sentinel bleeding. In stable patients, CT with contrast, angiography, and endoscopy may be useful in diagnosis and may reveal an otherwise unsuspected source. Once a diagnosis is made, bleeding from an ARSA-esophageal fistula may be temporized with a Sengstaken-Blakemore tube. Open surgical repair remains the gold standard of treatment, although endovascular stent grafts may be important to control life-threatening bleeding. The presence of a nasogastric tube is associated with development of these fistulae; therefore, prolonged NG tube placement should be carefully considered in patients with known anomalous right subclavian arteries.
This work was presented as a poster at the American Bronchoesophagological Association, Combined Otolaryngologic Spring Meeting in Chicago, IL, in April 2011.
The authors declare that they have no conflicts of interest.