Ann Thorac Surg 2006;81:358-360
© 2006 The Society of Thoracic Surgeons
Case report
Bleeding "Downhill" Varices: A Rare Complication of Intrathoracic Goiter
Eric L. R. Bédard, MD, MSc,
Jean Deslauriers, MD, FRCS(C)
*
Division de Chirurgie Thoracique, Centre de Pneumologie de L'Hôpital Laval, Sainte-Foy, Québec, Canada
Accepted for publication August 9, 2004.
* Address correspondence to Dr Deslauriers, Division de Chirurgie Thoracique, Centre de Pneumologie de L'Hôpital Laval, Sainte-Foy, Québec, G1V 4G5 Canada.
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Abstract
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We report a case of a 68-year-old woman with a 16-year history of a benign goiter. The patient presented with hematemesis and a clinically evident superior vena cava syndrome. Preoperative evaluation demonstrated upper esophageal varices and large posterior mediastinal goiter. The patient underwent successful resection of the mass with complete resolution of both the superior vena cava syndrome and "downhill" varices.
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Introduction
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The esophageal venous plexus is an important watershed network connecting the portal and systemic circulations. Lower esophageal varices, a commonly recognized complication of portal hypertension, are associated with a high mortality rate once they rupture. Upper esophageal varices, an uncommon diagnosis first described in 1964 [1], can provide an alternate route for venous return when systemic venous blood flow is obstructed. It may be possible to identify a surgically correctable cause of such "downhill" varices. We present a case of an intrathoracic goiter causing upper esophageal varices that resulted in upper gastrointestinal hemorrhage.
A 68-year-old woman was diagnosed with a benign goiter 16 years before presentation. After a prolonged asymptomatic period she was referred to our institution for investigation and treatment of an intrathoracic goiter after two episodes of upper gastrointestinal hemorrhage. The patient was admitted to our service symptomatic only for slight retrosternal discomfort. Physical examination revealed a slightly obese, well looking woman. No cutaneous signs of liver disease were observed. There was a large, nodular bilateral neck mass extending into the thoracic inlet. The patient had predominantly right-sided, dilated, tortuous collateral veins on the anterior chest extending away from the neck. The trachea was deviated to the right but no stridor was noted. The rest of the physical examination was unremarkable.
All laboratory data, including liver function tests were within normal range. Chest roentgenogram, thyroid scan, and computed tomographic scan (Fig 1) demonstrated a large intrathoracic goiter extending into the posterior mediastinum below the level of the carina. Barium swallow and esophago-gastro-duodenoscopy (Fig 2) showed upper esophageal varices. Venography demonstrated an extrinsic compression of the right innominate vein with filiform stenosis causing multiple dilated collaterals. The distal superior vena cava was filled by venous return from the deviated left innominate vein and the patent azygous system. Both these routes allowed drainage of the right-sided collateral veins including the distended esophageal plexus.
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Fig 1. Chest computed tomography showing a large superior mediastinal mass containing calcifications that extend below the level of the carina with associated dilated subcutaneous veins. The mass also displaces the esophagus (arrow).
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We used a combined cervico-sternotomy approach to excise a 6 x 10 x 12 cm mainly posterior mediastinal mass weighing 404 g. Histopathology confirmed our clinical diagnosis of an intrathoracic adenomatous goiter. The patient made an uneventful recovery. Esophago-gastro-duodenoscopy and venography were obtained 4 months after surgery revealing complete disappearance of the "downhill" varices with normalization of the upper trunk venous flow.
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Comment
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The esophagus is drained by both submucosal and peri-esophageal venous plexuses. The superior esophagus drains into the innominate veins through the inferior thyroid and bronchial and superior intercostal veins. Tributaries of the midthoracic esophagus drain into the azygous and hemi-azygous venous systems. The lower esophagus anastomoses with the portal system mainly through the coronary vein of the stomach and the short gastric veins which allows the esophageal venous plexus to shunt blood between the systemic and portal systems. The direction of blood flow being dictated by the venous pressure gradient.
Intrathoracic goiters cause a clinically evident superior vena cava syndrome in < 10% of patients [2]. As demonstrated by our patient, the right innominate vein is more often obstructed by an intrathoracic goiter given the relatively fixed aortic arch on the left [3]. When obstruction to venous flow occurs, blood returns to the right atrium through an extensive collateral venous network including the azygous, vertebral, esophageal, internal mammary, lateral thoracic and portal veins. The resulting increase in venous pressure in the upper esophageal plexus can lead to the development of upper esophageal varices. The caudal extension of these varices is dependant on the level and duration of the venous obstruction [4–6].
In a revue of the literature, Papazian and colleagues [5] found that only 7.6% of upper esophageal varices were associated with gastrointestinal hemorrhage. Thus it is much more common to bleed from varices due to portal hypertension than from those associated with the superior vena cava syndrome [4]. This discrepancy is partly explained by the normal liver function in patients with upper esophageal varices [6, 7]. Furthermore, these "downhill" varices are located in the upper esophagus where they are less exposed to the corrosive damage by refluxed gastric contents [6, 7]. Finally, lower esophageal varices associated with portal hypertension lie in the subepithelial venous plexus and are thus more vulnerable to rupture than the submucosal "downhill" varices that occur in the mid and upper esophagus [6, 7].
Upper gastrointestinal hemorrhage continues to present an important diagnostic and therapeutic challenge. Bleeding from upper esophageal varices should be suspected in any patient presenting with upper gastrointestinal bleeding and a history of intrathoracic goiter or superior vena cava syndrome. In the acute situation, this diagnosis is best confirmed by esophago-gastro-duodenoscopy allowing judicious use of banding, sclerotherapy, or balloon tamponade as temporizing measures. An intrathoracic goiter associated with bleeding "downhill" varices is an absolute indication for resection. A combined cervico-manubriotomy-sternotomy provides excellent exposure for vascular control and permits a safe resection.
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References
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- Felson B, Lessure AP. "Downhill" varices of the esophagus Dis Chest 1964;46:740-746.[Medline]
- Allo MD, Thompson NW. Rationale for the operative management of substernal goiters Surg 1983;94:969-977.
- Ulreich S, Lowman RM, Stern H. Intrathoracic goitera cause of the superior vena cava syndrome. Clin Radiol 1977;28:663-665.[Medline]
- Johnson LS, Kinnear DG, Brown RA, Mulder DS. "Downhill" esophageal varices Arch Surg 1978;113:1463-1464.[Abstract/Free Full Text]
- Papazian A, Capron JP, Rémond A, et al. Varices oesophagiennes supérieures Gastroenterol Clin Biol 1983;7:903-910.[Medline]
- Maton PN, Allison DJ, Chadwick VS. "Downhill" esophageal varices and occlusion of superior and inferior vena cava due to a systemic vasculitis J Clin Gastroenterol 1985;7:331-337.[Medline]
- Fleig WE, Stange EF, Ditschuneit H. Upper gastrointestinal hemorrhage from downhill esophageal varices Dig Dis Sci 1982;27:23-37.[Medline]
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Abstract
Introduction
