Survival after aberrant right subclavian artery–esophageal fistula: Case report and literature review

CASE REPORTS

Survival after aberrant right subclavian artery-esophageal fistula: Case report and literature review R o n n a G. Miller, M D , Daniel K. Robie, M D , Susan L. Davis, M D , D e n t o n A. Cooley, M D , WiUiam J. Klish, M D , M a r k D. Skolkin, M D , D e b r a L. Kearney, M D , and T o m Jaksic, M D , P h D , Houston, Tex. Development of a fistula between an aberrant right subclavian artery and the esophagus is a rare cause of heretofore fatal hematemesis. We report the first known survivor of this devastating complication of the most common aortic arch anomaly. Intraoperative esophagogastroduodenoscopy, intraesophageal balloon tamponade, and arteriography were the keys to successful management. This lesion should be suspected in the setting of bright red, "arterial" hematemesis. Prolonged nasogastric a n d / o r endotracheal intubation should be avoided in patients with a known aberrant right subclavian artery or other aortic arch anomaly. (J Vasc Surg 1996;24:271-5.)

Arterioesophageal fistula is a rare, typically fatal complicafion o f intrinsic cardiothoracic disease, surgery, or infecfion, and usually occurs in older adults. In the pediatric age group, foreign-body ingestion is the most frequent cause.:-4 The most c o m m o n vessel involved is the thoracic aorta, 5-7 as first reported by Dubreuil in 1818.4 An even rarer and more devastating variant is a fistula between an aberrant right subclavian artery and the esophagus. Although an aberrant right subclavian artery is present in 0.5 to 1.8% of the population, s:2 only 10 previous case reports of this heretofore lethal complication have been published (Table I). :3-22 We present the following unique case as the first documented survival o f a patient with an aberrant right subclavian arteryFrom the Cora and Webb Mading Department of Surgery (Drs. Miller, Robie, and ]aksic), Department ofPediatrics (Drs. Davis, Klish, and Jaksic), and Department of Pathology (Dr. Kearney), Baylor College of Medicine and Texas Children's Hospital; and the Department of Cardiovascular Surgery (Dr. Cooley) and Department of Radiology (Dr. Skolkin), St. Luke's Episcopal Hospital and TexasHeart Institute. Presented at the Forty-seventhAnnual Meeting of the Southwestern Surgical Congress, San Antonio, Tex., April 23-26, 1995. Reprint requests: Tom Jaksic,MD, PhD, Department of Pediatric Surgery, Texas Children's Hospital, 6621 Fannin MC 3-2325, Houston, TX 77030-2399. Copyright © 1996 by The Society for Vascular Surgery and International Societyfor CardiovascularSurgery, North American Chapter. 0741-5214/96/$5.00 + 0 24/4/71436

esophageal fistula, along with recommendations for successful diagnosis and management. CASE REPORT An 11-year-old girl required prolonged endotracheal (14 days) and nasogastric (17 days) intubation after craniotomy for intraventricular hemorrhage. On the seventeenth day after surgery, an abrupt onset oftorrential, bright red hematemesis with shock necessitated massive resuscitation, transfusion, and immediate transfer to the operating room. Upper gastrointestinal endoscopy, performed while the patient was being draped, localized the bleeding to a gastroesophageal source. Laparotomy and gastrotomy were performed, with temporary packing of the gastroesophageal junction, which excluded the stomach as the bleeding source. Oropharyngeal packing likewise excluded this region. Cardiac arrest necessitated resuscitative left thoracotomy and median sternotomy for direct right atrial volume infusion. Esophagotomy suggested the esophagus to be the site ofhemorrhage. Intraesophageal balloon tamponade with a 30F Foley catheter positioned at the bleeding point controlled the hemorrhage and allowed the patient to be stabilized hemodynamically. The balloon then was reinflated with contrast material, and a subsequent chest radiograph demonstrated the balloon to be at the level of the aortic arch, which was suggestive ofa vascular ring anomaly. Formal arch arteriography through a right femoral approach then was performed on the fluoroscopy table in the operating room by orte of the authors (M.D.S.). This procedure demonstrated a previously unsuspected aberrant 271

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Table I. Clinical summary o f l 0 cases ofaberrant right subclavian artery-esophageal fismla Reference

Sex

Age (yr)

Diagnosis

ETT

NG

Lynn 13 Reynes et al) 4 Merchant et al. is Livesay et od.16 Belkin et al. 17 Edwards et al) 8 Gossot et al) 9 Kullniß ° Stone et al. 21 Ikeda et al. 22 Present case

M F F M M M F M M M F

57 72 17 25 27 74 72 66 72 9 11

Aneurysm o f ARSA Aneurysm o f ARSA Status after Caesarian section Status after head injury Head and heck cancer Aneurysm o f ARSA Status after aortic graft Aneurysm o f ARSA Status after TAA/CCA-SCA bypass Status after Fontan Status after craniotomy

N N N Y N N Y N Y Y Y

N N Y Y Y N Y N ?Y Y Y

Days with NG

9 13 60 30 ? "long" 17

Outcome Died Died Died Died Died Died Died Died Died Died Lived

ETF, Endotracheal tube; NG, nasogastric tube; ARSA, aberrant right subclavian artery; TAA/CCA-SCA bypass, thoracic aortic aneurysm/common carotid artery-subclavian artery bypass.

Histologic examination revealed focal pressure necrosis of the resected artery, with neutrophil infiltration, granulation tissue formation, and polymicrobial bacterial overgrowth, with no evidence ofvasculitis.

DISCUSSION The incidence ofaberrant right subclavian artery, the most c o m m o n aortic arch anomaly, is 0.5% to 1.8%. 8-~2 There is a marked female predominance (65% to 72%). 23 The aberrant right subclavian artery forms an incomplete vascular ring as it courses toward the right axilla posterior to the esophagus in 80% o f cases (as in our case; Fig. 3), between the esophagus and trachea in 15%, and anterior to the trachea in 5%.24, 25

Fig. 1. Intraoperative arch aortogram demonstrates aberrant right subclavian artery (solid arrows) coursing from its origin at distal aortic arch toward right axilla. The inflated intraesophageal balloon (open arrow) is also seen (right posterior oblique projection). right subclavian artery (Fig. 1), as weil as contrast extravasation consistent with a fistula between the anomalous vessel and the esophagus (Fig. 2). The involved segment of the aberrant vessel was ligated and resected via right thoracotomy, and the esophagus was repaired primarily. Mediastinal and pleural drains placed at the completion of the procedure were removed on the seventh day after surgery, when contrast studies confirmed esophageal integrity. At the most recent follow-up, 2 years after surgery, the patient is completelyweil and has returned to school and exträcurricular activities, including cheerleading. The only sequela of the prolonged hospitalization is a mild left hemiparesis resulting from the intraventricular hemorrhage that was present before the gastrointestinal hemorrhage.

Development o f a fistula between the aberrant subclavian artery and the esophagus is a rare, previously lethal complication ofthis anomaly. The abnormal anatomic proximity to the esophagus or trachea likely renders the aberrant right subclavian artery vulnerable to extrinsic compression and pressure necrosis by indwelling nasogastric or endotracheal t u b e s 15'17'26-28 o r by vascular conduits. 19,21,22 The pathophysiologic events may involve occlusion and thrombosis o f the vasa vasorum, leading to vessel wall infarction and eventual wall dissolution. ö Alternatively, Gable et al.29 have proposed that ischemia and bacterial invasion o f the vessel wall are the most important etiologic factors. One or more o f these factors are likely to account for most o f the 10 previously reported cases. For example, four were elderly patients (mean age, 67.3 years) with aneurysmal degeneration o f the aberrant right subclavian artery. Apparent spontaneous aneurysm erosion into the esophagus resulted in fatal hematemesis in all f o u r . 13'14'18'2° Among the remaining six previous cases, threc patients had undergone cardiovascular surgery with conduit placement 19'2~'22 and three had an unrelated primary diseasc, ls-~7 Four

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Fig. 2. Intraoperative arch aortogram with intraesophageal balloon deflated demonstrates massive extravasation of contrast medium (arrows) from the aberrant right subclavian artery into the esophagus (anteroposterior projecuon). of these six latter patients had had prolonged cndotracheal intubation, and all six had had prolonged nasogastric intubation (at least 9 to 60 days). All six of these cases were fatal. Our case most closely resembles that reported by Livesay e t al. 16 in that the patient had a noncardiothoracic primary disease process and had prolongcd nasogastric and endotracheal intubation, which implicates pressure necrosis as a major etiologic factor. When it occurs, an aberrant right subclavian artery-esophageal fistula causes abrupt, rapidly exsanguinating hematemesis characterized by bright red arterial bleeding. Warning signs typical of more than halfofpatients with aortoesophageal fistula,3° such as chcst pain and "sentinel" bleeding, occurred in only one of the previous reported cases 1+and did not occur in our case. Previously unsuspected aberrant right subclavian artery-esophageal fistulas are rarely diagnosed before autopsy. 5 Upper gastrointestinal contrast studies, computed tomography, and magnetic resonance imaging are of no value in the face of exsanguinating bleeding. As in our case, esophagogastroduodenoscopy (EGD) may help to exclude other causes of bleeding and may reveal the site of the fistula. 31 Among the 10 previously reportcd cases, endoscopy was not performed in five, 13,14'19'20'22 confirmed an esophageal source of bleeding in two, ~7,21 and was nondiagnostic in three. 15,16,~sThese results are similar

Fig. 3. The most common (80%) anatomy of the aberrant right subclavian artery arising from the aortic arch distal to the left subclavian artery and coursing behind the esophagus toward the right arm. AA, Aortic arch; ARSA, aberrant right subclavian artery; T, trachea; E, esophagus; RCCA, right common carotid artery; LSA, left subclavian artery; LCCA, left common carotid artery; LPA, left pulmonary artery; LDA, ligamentum arteriosum/ductus arteriosus; LPA, left pulmonary artery.

to those obtaincd with EGD in the diagnosis ofother arterioesophageal fistulas, with a diagnostic sensitivity ofonly 38%. 32 Furthermore, EGD in this setting may provoke exsanguinating hemorrhage and should be performed only in the operating room with surgical and anesthesia support for immediate thoracotomy. 33 As in the case presented herein, intraoperative arteriography may be extremely useful in simultaneously diagnosing the source of bleeding and in delineating the anomalous vascular anatomy, assuming the patient can be satisfactorily stabilized for the procedure, and assuming the availability ofa portable C-arm and an expcrienced angiographer. When a known or unsuspected aberrant right subclavian artery erodes into the esophagus, survival depends on immediate, simultaneous diagnostic and therapeutic maneuvers, including vigorous resuscitation, surgical exploration, and artcriography. In our case, intraoperative upper gastrointestinal endoscopy cxcluded other bleeding sources, whereas intraesoph-

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ageal balloon tamponade at the level o f the aortic arch proved extremely valuable in controlling the massive hemorrhage while direct vascular control was achieved. Others have used a similar technique in the control o f this lesion 1~q7 and o f tracheoinnominate fistula, a4 Unlike in the elective setting, we r e c o m m e n d right thoracotomy as optimal exposure for both the arterial resection and the esophageal repair. In infants and children, the aberrant vessel may be ligated and divided because collateral circulation prevents "subclavian steal" syndrome. 35 40 In contrast, revascularization o f the right arm is required in adults, a7,41-43 In the absence o f massive mediastinal soiling, primary esophageal repair and drainage should be feasible, as in our case. The majority o f the 0.5 to 1.8% o f the population with an aberrant right subclavian artery remain asymptomatic, bnt the precise natural history o f the lesion is unlcnown. Godtfredsen et al.,44 in their series o f 11 conservatively managed symptomatic children with a variety ofvascular rings, reported that nine had become asymptomatic within 1 to 2 years ofdiagnosis, whereas the others had only mild esophageal compression (one infant) or sev¢re neuromuscular lesions (one infant). O n this basis, they advocated nonoperative management. Nevertheless, the prohibitive mortality rate (>90%) in the setting of arterioesophageal erosion argues against this conservative approach in symptomatic individuals and in favor o f elective surgical intervention, which can be accomplished with minimal morbidity and mortality rates.21,3°,45 Even though it is unlikely that a given individual with an aberrant right subclavian artery may require prolonged nasogastric or endotracheal intubation, the incidence o f late aneurysmal degeneration is unknown. Such aneurysm formation itself carries at least a 50% risk of death as a result o f hemorrhage, a7 which further supports a policy of p r o m p t surgical therapy, at least for symptomatic lesions. Moreover, ligation and division o f the aberrant right subclavian artery should be performed before correcfion o f any associated complex cardiac anomaly is u n d e r t a k e n Y An aberrant right subclavian artery is the most c o m m o n aortic arch anomaly. When symptomatic, elective surgical treatment should be undertaken. The aberrant vessel likely will not lead to a spontaneous arterioesophageal fistula unless it nndergoes aneurysmal dilatation (characteristic o f older adults), or nnless it undergoes pressure necrosis from an indwelling esophageal foreign body, such as a nasogastric tube, as in our case. Endotracheal tubes and vascular conduits also may contribute to pressure necrosis.

The present case o f aberrant right subclavian artery-esophageal fistula is tmique for several reasons. It is only the second reported pediatric case, the second youngest case, and, most importantly, the only known survivor. This is also the first report ofsuccessful temporary intraesophageal balloon tamponade in this setting. Saving the patient & m a n & a high index ofsuspicion in the setting o f " a r t e r i a l " hematemesis. To prevent the devastating consequences o f an aberrant right subclavian artery-esophageal fistula, we strongly reiterate the warning of McKeating et a l s and H e c k et al. 28 to avoid prolonged nasogastric and endotracheal intubation in patients known to have an aberrant right subclavian artery or other vascular ring anomaly. REFERENCES

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Submitted Sep. 29, 1995; accepted Dec. 18, 1995.