Downhill Varices: Report of a Case 29 Years After Resection of a Substernal Thyroid Gland

Vol. 73, No. 2

73:345-348, 1977 Copyright © 1977 by the American Gastroenterological Association

GASTROENTEROLOGY

Printed in U.S A.

DOWNHILL VARICES: REPORT OF A CASE 29 YEARS AFTER RESECTION OF A SUBSTERNAL THYROID GLAND JEFFREY J. SoROKIN, M.D.,

G.

Moss, M.D., AND

C.

F.A.C.P.,

STEPHEN M. LEVINE, M.D.,

F.A.C.P.,

EDWARD

MILLER BIDDLE, M.D.

Sections of Gastroenterology and Endocrinology of the Department of Medicine and the Department of Diagnostic Radiology of The Cooper Medical Center, Camden, New Jersey

A case of varices of the entire esophagus in a patient who had undergone resection of a substernal thyroid gland 29 years previously is reported. Dilated esophageal veins, varices, may serve as collateral channels between the portal and systemic systems. When the superior vena cava is obstructed, blood from the upper extremities and head is returned to the heart via downhill varices. Obstruction of the superior vena cava proximal to the azygos vein results in varices of the upper esophagus. Obstruction distal to the azygos vein, as in this patient, results in varices of the entire esophagus. Portacaval shunt has no place in the management of these patients. Downhill varices should be considered in any patient with superior vena caval obstruction from any cause. Esophageal varices are most frequently associated with portal hypertension. Sporadic reports have noted varices of the upper esophagus in patients with superior vena caval obstruction in the absence ofliver disease. 1- 8 Varices of the upper esophagus have also been associated with thyroid disease.9-:11 The purpose of this paper is to report a case of varices of the entire esophagus in a patient who had undergone resection of a substernal thyroid gland 29 years previously. Case Report This 46-year-old woman was admitted to the Cooper Medical Center on January 18, 1976, with the chief complaint of chest pain. She described the pain as an ache in the center of the chest radiating around to the right side and into the right arm. It was apparently unrelated to position, exertion, eating, or anxiety. Review of hospital records revealed complaints of pain in the right side of the neck in 1972. This woman had a subtotal thyroidectomy and resection of a substernal goiter in 1947. She has been euthyroid since the time of surgery but has noted some edema of her face, arms, and breasts. Except for a thrombophlebitis of the left leg in 1972 and 1973, her medical history is otherwise unremarkable. She has been on no medications, and has had no weight loss, no history of jaundice, or history of liver disease; there was no alcoholism, hepatotoxin exposure, dysphagia, or odynophagia. Physical examination revealed a healthy-looking woman 157.5 em tall weighing 63.2 kg with a blood pressure of 115/70, a pulse of 72 beats per min, and a temperature of 37°C. The skin was normal with no spider angiomata. The head, eyes, ears, nose, and throat were normal. There was no lymphadeReceived September 13, 1976. Accepted March 5, 1977. Address requests for reprints to: Dr. Jeffrey J. Sorokin, 1210 Brace Road, Cherry Hill, New Jersey 08034. The authors wish to thank Biruta Liepa, M.D., for her invaluable assistance in translating the German literature.

nopathy. There was a well healed horizontal scar on the neck extending vertically over the upper one-third of the sternum. The chest, heart, and breasts were normal. The abdominal examination did not reveal hepatosplenomegaly or abdominal collateral veins. The rectum and genitalia were normal. There was some tenderness in the left thigh. The neurological examination was normal. The following studies were obtained and were normal: SMA/12, bilirubin, Venereal Disease Research Laboratories test, urinalysis, complete blood count, prothrombin time, triglycerides, a 1-antitrypsin, a-fetoprotein, bromosulfophthalein, electrocardiogram, thyroid uptake and scan (to the level of the xyphoid), liver scan, T3, T4, and thyroid-stimulating hormone. Chest X-ray was read as showing two wire sutures in the upper sternum and mild pulmonary fibrosis. An upper gastrointestinal series showed multiple serpiginous cobblestonelike filling defects throughout the esophagus (fig. 1). Esophagogastroduodenoscopy revealed esophageal varices from the upper esophagus (18 em from the incisors) to the gastroesophageal junction. The stomach and duodenum were normal. A superior vena cavogram (fig. 2) was performed by injecting 30 ml of Renografin into both arms · through superficial veins. Neither the innominate vein nor the superior vena cava filled . Collateral veins were seen extending up into the neck on both sides, and a large left internal mammary vein was visualized. Opacifications compatible with esophageal varices were seen in continuity with the large internal mammary vein. The conclusion of this study was that the patient had an obstruction of the superior vena cava involving the azygos vein. The patient was discharged on analgesics and is being followed, with no real improvement in her symptoms.

Discussion The veins of the esophagus drain into the inferior thyroid, azygos, hemiazygos, and gastric veins, thereby 345

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CASE REPORTS

FIG. 1. Barium swallow demonstrating esophageal varices .

V ol. 73 , No . 2

forming a connection between the portal and systemic venous systems. Esophageal varices are present when these esophageal veins are dilated and serve as collateral channels between the portal and systemic systems. These channels usually serve to relieve portal obstruction in the liver and flow "uphill," i.e., blood flows from the portal vein through the coronary vein into the esophageal veins and into the superior vena cava via the azygos vein (fig. 3A). "Downhill varices" occur when blood is returned to the heart via the portal venous system. With obstruction of the superior vena cava and the azygos vein, blood from the upper extremities, head, and thorax may flow through the inferior thyroid veins and mediastinal collaterals into esophageal varices and then into the coronary vein to the portal vein through the liver to the hepatic vein and inferior vena cava to the heart. Several factors may influence the distribution ofthese varices which are collateral vessels. These vessels develop in response to an increase in venous pressure. The degree of increase in venous pressure varies with the rate of development of obstruction, as well as with the site of obstruction. 12 Thus, the duration of obstruction is an important developmental factor in the extent and location of the varices. Mediastinal fibrosis is more insidious in its progression than bronchogenic carcinoma, and varices are more extensive in the former . At the opposite extreme, acute occlusion of the vena cava during thoracic surgery may cause fatal cerebral edema within minutes . Varices of the upper esophagus may occur with obstruction of the inferior thyroid veins in patients with goiters, surgical resection ofthe thyroid gland, or recurrent thyroid enlargement.4 • 8• 9 If the superior vena cava is obstructed proximal to the azygos vein, blood may

Fra. 2. Superior venocavogram demonstrating obstruction of the superior vena cava and azygos vein with collateral flow into the neck (open arrows ) and a large left internal mamma ry vein (closed arrows).

Portal V.

Superior Vena Cava

(B)

I

(C)

FIG. 3. A , schematic diagram of "uphill" esoph ageal varices in portal hypertension; B, downhill varices of the upper esophagus with obstruction of the superior vena cava proximal to the azygos vein; C , downhill varices of the entire esophagus with obstruction of the superior vena cava and azygos vein. 8

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348

Vol. 73,No.2

CASE REPORTS

still return to the heart via the superior vena cava and the downhill varices will involve only the upper esophagus (fig. 3B). Collateral pathways include the internal mammary and vertebral and azygos veins. 13 When the azygos is obstructed as well, varices may involve the entire esophagus and blood will return to the heart via the portal system and the inferior vena cava (fig. 3C). Most reports of downhill varices have involved patients with superior vena caval obstruction due to bronchogenic carcinoma2• 3 • ~7 and idiopathic mediastinal fibrosis. L 3 • 6 • 7 Localized upper esophageal varices have been reported after resection of a retrosternal thyroid gland in the absence of vena caval obstruction. 9 • 10 In our patient, downhill varices involving the entire esophagus occurred because of obstruction of the superior vena cava and the azygos vein, presumably by fibrosing mediastinitis secondary to previous surgery. There were no clinical indications of coexistent portal hypertension in this woman whose physical examination and biochemical and radionucleotide studies were within normal limits. Idiopathic mediastinal fibrosis and congenital anomalies are also included in the differential diagnoses of superior vena caval obstruction. There have been reports of bleeding from downhill varices. 1• 14 Failure to recognize downhill varices as an entity separate from the more common variety seen with portal hypertension could result in unnecessary and fruitless portacaval shunt surgery. Awareness of this entity is the first step in its recognition. Downhill varices of the esophagus should be considered in any patient with superior vena caval obstruction, bronchogenic carcinoma, or thyroid gland enlargement. Downhill varices need not be limited to the upper esophagus, but they may involve the entire length of the esophagus. Surgery, if undertaken for massive hemorrhage , should

be designed to "shunt" blood to the right atrium through a "pseudoazygos" pathway. REFERENCES 1. Snodgrass RW, Mellinkoff SM: Bleeding varices of the upper

2. 3.

4. 5.

6.

7.

8.

9. 10. 11. 12. 13. 14.

esophagus due to obstruction of the superior vena cava. Gastroenterology 41:505-508, 1961 Mikkelsen WJ: Varices of the upper esophagus in superior vena caval obstruction. Radiology 81:945-948, 1963 Otto DL, Kurtzman RS: Esophageal varices in superior vena caval obstruction. Am J Roentgenol Radium Ther Nucl Med 92:1000-1012, 1964 Hauss WH, Pfeiffer K: Ubervarizen des oberen Oesophagusabschnittes. Munch Med Wochenschr 107:2282-2286, 1965 Subramaniam R, Madanagopalan N , Krishnan KT, et al: A case of anaplastic bronchogenic carcinoma with "downhill varices" of the esophagus. Dis Chest 51:545-549, 1967 Sheiner NM, Palayew MJ: "Downhill" esophageal varices in superior vena caval obstruction. Can Med Assoc J 100:961-964, 1969 Anderson IF, Dannheimer 1: A case of "downhill varices". S Afr Med J 44:1035-1037, 1970 Gerstenberg E, Taenzer V, Bachmann D, et al: Downhill Varizen: Oesophagusvarizen ohne portale Hypertension. Med Welt 22:1433-1436, 1971 Saudermann A, Kammerer J: Retrosternale Struma und Oesophagusverizen. Munch Med Wochenschr 102:2133-2137, 1960 Keiminger K, Kolb R: Symptomatische Oesophagusvaricen bei retrosternalen Strumen. Chirurg 43:277-279, 1972 Lagamann VK: Obere Oesophagusvarizen bei Struma. Fortschr Geb Roentgenstr Nuklearmed 118:440-445, 1973 Spencer, FC: Diseases of great vessels. In Principles of Surgery. Edited by SI Schwartz. New York, McGraw-Hill, 1969, p 715 Liebowitz HR: Bleeding Esophageal Varices: Portal Hypertension . Springfield, Ill, Charles C Thomas, 1959, p 479-481 Palmer ED: Primary varices of the cervical esophagus as a source of massive upper gastrointestinal hemorrhage. Am J Dig Dis 19:375-378, 1952