“Downhill” Varices of the Esophagus*

"Downhill" Varices of the Esophagus* BENJAMIN FELSON, M.D., F.C.C.P.** AND ALFRED

P.

LESSURE, M.D.***

Cincinnati, Ohio

T

There were no masses palpable in the neck. Physical examination of the heart, lungs, and abdomen was normal. The liver and spleen were not enlarged. There was no ascites. The lower extremities showed bilateral varicose veins with stasis ulcers. Routine laboratory studies and liver function tests were all normal. The venous pressure was 29 em. of water in the right arm and 40 em. in the left. Venous pressure in a dilated vein in the right anterior chest wall was 32 em. Arm-totongue circulation time was 35 seconds. Histoplasmin and tuberculin skin tests were both strongly positive. Needle biopsy of the liver only showed mild congestion. Chest fluoroscopy and barium swallow revealed extensive varices involving the lower three-fourths of the esophagus (Fig. I). No mediastinal masses or calcification were observed. The heart appeared normal. Oral cholecystography was normal. Serial right brachial venography showed obstruction at the junction of the subclavian and innominate veins, with opacification of many collateral veins of the right thoracic wall (Fig. 2). The superior vena cava did not fill, nor did the azygos system or esophageal veins. However, the study was not prolonged sufficiently to demonstrate the collateral channels all the way to the heart. Percutaneous splenoportography revealed an intrasplenic pressure of 14 to 16 em. of water (normal up to 20 em.) . The splenic and portal veins appeared normal and there were no esophageal varices or other collateral vessels demonstrated (Fig. 3). An attempt was made to determine the direction of blood flow in the esophageal varices, utilizing balloon tamponade in conjunction with a barium esophagram. This study was technically unsatisfactory, to state it mildly. Thoracotomy was not performed because of the stable clinical course. The discharge diagnosis was fibrous mediastinitis, probably caused by histoplasmosis: with superior vena caval obstruction and "downhill" varices. She had periodic examinations over the next five years. During this time she was admitted to the hospital several times for surgical treatment of the leg ulcers and for management of hypertensive heart disease with congestive failure. Gastrointestinal bleeding did not occur. There was no change in the roentgen appearance of

HE PRESENCE OF ESOPHAGEAL VAR-

ices is not always indicative of intraor extrahepatic portal vein obstruction. Esophageal varices of obscure derivation are encountered fairly often at necropsy and may even be the source of severe bleeding. 14 There is yet another cause of varices: superior vena caval obstruction. The collateral flow which bypasses the obstructed cava may involve the esophageal veins and produce varicosities. We have applied the term «downhill, varices to denote this entity, and believe that it occurs with fair frequency. Since the symptoms of the vena caval occlusion, or of the underlying disease causing it, usually dominate the clinical picture, the esophagus is seldom carefuly studied and the varices remain undetected. The purpose of this report is to emphasize the occurrence of esophageal varices from retrograde flow in the esophageal veins, to review its mode of development, and to indicate some of its causes. CASE REPORT

J. W., a 46-year-old Negro woman, was admitted to the Cincinnati General Hospital in April, 1958, for the evaluation of engorgement of the veins of the anterior chest wall which had been present for approximately 15 years. There had been no hematemesis, stridor, or dysphagia. There was no history of alcoholism or of jaundice or other symptoms of hepatic disease. Blood pressure was 130/80 mm.Hg in both arms. Edema of the face, especially the eyelids, and marked distention of the superficial veins of the right side of the neck, anterior chest wall, and upper abdomen were observed. Mild exertion and stooping caused intense facial cyanosis. *From the Department of Radiology, University of Cincinnati College of Medicine. **Professor and Director, Department of Radiology, University of Cincinnati College of Medicine. ***Formerly Resident and Fellow in Radiology, University of Cincinnati College of Medicine. Now at the Welborn Clinic, Evansville, Indiana.

740

Volume 46, No. 6 December 1964

DOWNHILL VARICES OF ESOPHAGUS

the esophageal varices or in the clinical signs of compression of the superior vena cava.

Comment : The findings were characteristic of obstruction of the superior vena cava. The normal splenoportogram, normal liver function tests, and absence of significant abnormality on liver biopsy were strong evidence that the esophageal varices were not of the usual portal obstruction variety. Recently, we encountered two additional cases of "downhill" varices. In each patient, the superior vena caval obstruction, apparent clinically, was attributed to mediastinal invasion or metastases from a histologically verified carcinoma of the lung. Neither showed evidence of gros..<~ or occult gastrointestinal bleeding. Barium studies in both demonstrated varices confined to the upper half of the esophagus (Fig. 4) . In both patients, bilateral brachial venography demonstrated superior caval obstruction. In one, the dilated esophageal veins actually filled with contrast and the flow was clearly shown to be in a caudal direction (Fig. 5) . DISCUSSION

Four principal routes of collateral venous flow in superior vena caval obstruction are

FIGURE

1:

741

generally described: the azygos, the vertebral, the internal mammary, and the lateral thoracic." However, a fifth undoubtedly exists-the portal route. All these pathways eventually lead to the inferior vena cava. The veins of the esophagus form a plexus on its outer surface. The lower part of this plexus anastomoses with the coronary vein of the portal system while its upper portion drains into the azygos and thyroid veins.• Connections with the internal mammarv via the mediastinal and pericardia! vein~ also exist. Thus, the lower esophageal veins join the portal with the azygos system, and the upper ones connect the azygos with the cervical and internal mammary veins. Theoretically, esophageal varices might develop from blockage of any of these systems, or even from obstruction of the inferior vena cava. With superior caval obstruction, varices mav occur whether the block lies above or bel~w the entrance of the azygos vein. If the obstruction lies above this point, some of the venous flow from the head and neck may traverse the esophageal veins on its way to the azygos vein and superior cava. In this event the varices should always be limited to the upper esophagus. In obstruc-

J.W. Esophagrams showing varices of the lower three-fourths of the esophagus.

742

Diseases of the Chest

FELSON AND LESSURE

TABLE 1-EsoPHAOEAL V AJttcu PROM SuPERIOR VENA CAVAL

Authon

hraelski and

Year

Diasnooil

Location of Esophageal Varices

y,

1932 Large superior med-

Upper

2 Templeton'

1944 Mediastinal fibrosis

Entire

Weinberg'

1949 Carcinoma of lung

Upper~

Simchowitz'

iastinal tumor

4

Upper~

5

Middle~

6

Middle~ Lower~?

Method of DemonAnsiography

strating Varices

Not performed

X-ray

Obstructed

superior cava

X-ray; esophagoscopy

Not performed

Necropsy

1955 Ligation of superior vena cava for trau·

1959 Mediastinallibroois (histoplasmosis)

Necropsy study; no

Necropsy study; no

clinical details

..

Necropsy study; no

clinical details

Upper~

Obstructed

X-ray

Entire

Not performed

Esophagoocopy Hemoptysis Caval obstruction confirmed at operation

Not performed

X-ray: esophagoocopy

Yes

Varices disappeared after thyroidectomy

X-ray

No

Varices improved after

varices shown

X-ray ; esoph-

agoscopy

Yes

Unproved

X-ray

No

y,

superior cava

Upper,_, Obstructed

Retrostemal thyroid

superior cava

Esophageal

13 Snodgrass and Mellinkoff"

1961 Mediastinal fibrosis

14 Mikkeloen"

1963 Carcinoma of thyroid Upper

y,

Patent azygos vein

Upper

y,

Not performed

Carcinoma of lung

15

Entire

y, y, Upper Y.

16 17 18

Mediastinal fibrosil

(osseous venogram)

X~ray;

Yes

esoph.

agoscopy

No

Varices discovered

four yean postop.

thyroidectomy

Varices dilappeared

after irradiation

Upper

X-ray

No

Upper

X-ray

No

Obstructed

X-ray ;

y.,

Not performed

X-ray

No

X-ray

Hemoptysis "Hemorrha'-e, presumably

Upper

19 20

Necropsy study ; no

clinical details

clinical details

12

adenoma

x~ray

Unproved; caval obstruc· tion for ten yean

Necropsy study; no

Upper

adenoma

No histologic proof;

very large masa on

clinical details

11 Sundermann and 1960 Retrostemal thyroid Kimmerer11

Remarka

No

matic A-V fistula

I 0 Salyer, •I 111. •

Bleeding No

8 Garrett and Gall' 1953 Hodgkin's Disease 9 MartorelP

OBSTRUCTION

y,

superior cava

En tift'

Bleeding site not found

at necropsy

necropsy

hemoptysis; ' caval ob-

atructlon for seven yean

21

Adenocarcinoma, primary site?

22

Carcinoma of lung

Upper y,

X-ray Not stated

No

X·ray

X-ray

23

X-ray

Hodgkin's

24 25 Ottoand

Kurtzman••

1964 Mediastinal fibrosil

26 27 28 Felson and Leuure

Carcinoma of lung 1964 Mediastinal fibrosis

Enti~

Obstructed

X-ray

No

Upper~

Esophageal

varices shown

X-ray; esoph· agoocopy

No

Y.

Esophageal

X-ray

No

Obstructed

X-ray

No

Obstructed

X-ray

No

Esophageal

X-ray

No

Upper

superior cava

Enti~

varices shown

superior cava ; normalapleno-

portography

29 30

Carcinoma of lung

Upper

y,

Upper Y,

superior cava varices shown

Unproved Unproved; probable caval

obstruction for &ve yean

Unproved ; caval obstruction

for 15 yean

Volume 46, No. 6

December 1964

DOWNHILL VARICES OF ESOPHAGUS

tion of the cava below the azygos entry, flow in the esophagus veins is toward the portal system. If varices occur they may either be confined to the upper portion or involve the entire esophagus, depending on the quantity of shunted blood, the lateral connections with other collateral systems, and the duration of the obstruction. These are the same factors that apply to the development of lower esophageal varices in portal hypertension. We were able to collect only 30 cases of esophageal varices attributable to superior vena caval obstruction, including our own three cases. 1' 1 ' 4 ' 7•11 These are listed chronologically in Table I. Howell's case of superior mediastinal obstruction with esophageal varices was excluded because the patient also had hepatic cirrhosis with portal hypertension. 14 HeUendall's case of malignant thyroid tumor with varices was excluded because of inadequate documentation.11 Occasional mention has been made of the presence of varices in superior caval obstruction without citing case materiat.'•·n Despite the relatively few cases, the fact that Mikkelsen'• encountered 11 cases in a few years and Kurtzman 13 and ourselves each found three cases in a relatively short time span seems to indicate that "downhill" varices are not rare. We initially believed that long-standing vena caval obstruction was necessary for the development of "downhill" varices and, therefore, that it could be caused only by benign conditions, such as chronic mediastinal fibrosis. However, malignant disease, specifically carcinoma of the lung, is apparently a more frequent cause. The etiology of the superior vena caval obstruction in the 30 cases was as follows: carcinoma of the lung in 15, chronic mediastinal fibrosis in seven, retrostemal thyroid tumor in three (malignant in one), Hodgkin's disease in two, and surgical ligation of the superior cava, metastatic adenocarcinoma, and mediastinal mass of undetermined nature in one each. Most of the bronchogenic carcinomas in which the histology was described were of the anaplastic variety.

743

The location of the varices in the esophagus in these cases is of special importance. In 16 of the 17 tumors in which the barium esophagram was described, the lower esophagus was spared. In the remaining case ( 7, Table 1 ) it was not clear whether the entire esophagus was involved or only its lower third. In the patient who had a high ligation of the superior cava four years earlier ( 9, Table 1 ) , the varices were also confined to the upper esophagus. On the other hand, in six of the seven cases of mediastinal fibrosis, the varices involved the entire esophagus. In one of these, the varices were distinctly more prominent in the upper half. The duration of the me-

FIGURE 2: (Upper) J. W. Right brachial venogram demonstrating obstruction at the junction of the subclavian and innominate veins and collateral flow through dilated veins in the thoracic wall. FIGURE 3: (lower) J. W. Normal percutaneous splenoportogram.

744

FELSON AND LESSURE

diastinal disease in four of the six was known and ranged from five to 15 years. In the remaining case the varices were confined to the upper esophagus and the duration of the obstruction was not known. Although the case material is small, the different distribution of the varices in the tumor cases when compared to those with fibrosis suggests a relationship between the duration of the caval obstruction and the downward extension of the varices. The level of caval obstruction in relation to the entry of the azygos vein could be postulated in only five cases. In four, the patient with ligation of the upper portion of the superior vena cava and the three with thyroid tumor, high obstruction was assumed (confirmed by osseous venography in one). In all four, the lower esophagus was spared. This confirms our belief that in high obstruction the lower esophagus should be free of varices. The one case of mediastinal fibrosis in which operation was

FIGURE

4:

Diseases of the Chest

performed showed obstruction below the entrance of the azygos. The entire esophagus was involved by varices. Experience indicates that in most cases of bronchogenic carcinoma with superior caval syndrome the obstruction extends below the azygos junction. Hence, as implied earlier, in low obstruction the lower esophagus may or may not be free of varices. In ten cases, brachial venography was performed and in every instance obstruction was demonstrated. Usually the upper extent of the obstruction was demonstrated in one or both innominate or subclavian veins. However, the collateral pattern indicated that the cava itself was also occluded, usually below the azygos entry. In four instances, the esophageal varices themselves were visible. They filled from above and the contrast material in them flowed caudally. In the frontal projection, esophageal veins may be confused with vertebral veins. However, the position of the vertical, linear,

R.S. Esophagram showing varices in the upper half of the esophagus.

Volume 46, No. 6

December 1964

DOWNHILL VARICES OF ESOPHAGUS

tortuous vessels just to the left of the midline and the lack of transverse components indicates esophageal varices. Lateral angiography, as performed in the case of Snod~ and Mellinkoff," obviates this problem. There was evidence of bleeding from the esophageal varices in four cases, one each with mediastinal fibrosis, bronchogenic carcinoma, ligation of the superior vena cava, and thyroid adenoma. In two additional cases of mediastinal fibrosis, hemoptysis occurred. In 15 other cases, bleeding did not occur. Tests of liver function were normal in the few cases in which they were recorded. Necropsy details were seldom available, but in two cases the liver, spleen, and portal veins were recorded as normal. Removal of a retrostemal thyroid adenoma resulted

745

in complete disappearance of the esophageal varices in one case and improvement in another ( 11 and 12, Table 1). The vena caval obstruction and the esophageal varices disappeared following irradiation in a case of bronchogenic carcinoma ( 15, Table 1 ). From the foregoing it is evident that the " downhill" mechanism of development of esophageal varices is real and that it occurs with fair frequency. If searched for in every instance of superior caval syndrome, we believe esophageal varices will be turned up with surprising frequency. Conversely, whenever esophageal varices appear to be confined to the upper segment of the esophagus, superior caval obstruction should be suspected. However, it should be reemphasized that "downhill" varices may involve the lower esophagus as well, especially when the superior caval obstruction has been present for years. Although, as stated earlier, a number of cases of idiopathic esophageal varices have been reported, evidence of subclinical superior caval obstruction should also be sought in every instance of unexplained varices. Awareness that "downhill" varices can occur should lead to the discovery of many additional cases, since superior vena caval obstruction is such a common condition. AcKNOWLEDGMENT : Our sincere thanks go to the Evansville Institute for Continuing Medical Education and Research for assistance in obtaining essential reference materials. We are also grateful for the information supplied us by Dr. Raymond S. Kurtzman of Detroit, and Dr. William J. Mikkelsen of Seattle.

2

3

4

FIGURE 5: Bilateral brachial venography. The esophageal varices (arrow) and azygos vein have filled with contrast medium. Serial films demonstrated caudal flow in these vessels.

5

REFERENCES GAKR.ETT, N., ]R., AND GALL, E. A.: "Esophageal Varices without Hepatic CirrhosiS," A .M .A . Arch. Path., 55:196, 1953. WEINBERG, T.: "Observations on the Occurrence of Varices of the Esophagus in Routine Autopsy Material," Amer. f . Clin. Path., 19: 554, 1949. RAcx, F. J., MINCJts, J. R., AND SIMEONE, F. A.: "Observations on Etiology of Esophageal Varices," AM.A . Arch, Surg., 65:422, 1952. SALYER, J. M., HAKRisoN, H. N., WINN, D. F., ]R., AND TAYLOR, R. R.: "Chronic Fibrous Mediastinitis and Superior Vena Caval Obstructions due to Histoplasmosis," Dis. Chest, 35: 364, 1959. LIEBOWITZ, H . R. : Bleeding Esophageal Varices, Portal Hypertension, C. C Thomas, Springfield, Ill., 1959, p. 479.

Diseases of the Chest

FELSON AND LESSURE

6 KAPLAN , B. : "Esophageal Varices," Medical Record, 154 : 176, 1941. 7 lsRAELSKI, M., AND SIMCHOWITZ, H. : "Rontgendiagnostic der Osophagusvarizen," Zeitschr. f. Laryng., Rhino/. & Otol., 22 : 208, 1932. 8 TEMPLETON, F. E.: X-ray Examination of the Stomach : a Description of the Roentgenologic Anatomy, Physiology, and Pathology of the Esophagus, Stomach, and Duodenum. Univ. of Chicago Press, Chicago, 1944, p. 437 . 9 MARTORELL, F. : " Varices del Es6fago por Hipertensi6n Caval Superior," Angiologia, 7:49, 1955. 10 SuNDERMANN, A., AND KAMMERER, J.: "Retrostemale Struma and Osophagusvarizen," Minchen M ed. W chschr., 102 : 2133, 1960. II SNOOORASS, R . w., AND MELLINKOFF, s. M .: "Bleeding Varices in the Upper Esophagus due to Obstruction of the Superior Vena Cava," Gastroenterology, 41:505, 1961. 12 MIKKELSEN, W. J. : "Varices of the Upper Esophagus in Superior Venal Caval Obstruction," Radiology, 81 :945, 1963.

13 OTTo, D. L., AND KuRTZMAN , R. S.: " Esophageal Varices in Superior Vena Caval Obstruction ," Amer. j. Roent., Rad. Ther. and Nuc. Med ., 92 : 1000, 1964. 14 HowELL, D. S.: "Circulatory Manifestations of Obstruction of the Superior Vena Cava in a Patient with Portal Hypertension," Rhode Island Med. ]., 33:659, 1950. 15 HELLENDALL, quoted by LOTHEISSEN, G.: Medizin . Klinik, 32:589, 1936. 16 KAUFMAN , E., translated by REIMANN, S. P. : Patholofy for Students and Practitioners, P. Blaluston's Son & Co., Philadelphia, 1929, p. 633. 17 FISCHER, W., IN HENKE, F., AND LUBARSCH, 0 ., Handbuch der Speziellen Pathologischen Anatomie und Histologie. Vol. IV, Part 1. Julius Springer, Berlin, 1926, p. Ill. For reprints, please write Dr. Felson, Department of Radiology, General Hospital, Cincinnati 29.

BALANCED DRAINAGE OF PNEUMONECTOMY SPACE Balanced drainage of the pneumonectomy space catastrophic bleeding. Disadvantages are minimal. Balanced drainage does not Impair emclency of the has many advantages to recommend Its routine use. cough mechanism. It can maintain the mediastinum In a physiologically optimal position and can etfect satisfactory drainage I..AFOaBT, E. G. AND BoYD, T . F.: " Balanced Drainage of the In the presence of Infection or contamination. It Pneumonectomy Space," S11rg., Gy•"· 0/11111,, 118:1051, permits prompt and often llfe-savlng recognition of 1964.

••tl

ALCOHOLISM AND PULMONARY TUBERCULOSIS IN THE DEPARTMENT OF CALAIS HOSPITALS The author presents a study of 122 patients hosbeing encountered with Increasing frequency, the pitalized for treatment of pulmonary tuberculosis author states that patients so amtcted are dlmcult to who were also alcoholics. Each case was studied rehabllltate. from the psychologic, sociologic, pathologic and therapeutic aspects. It was found that almost always BELBENOIT, S.: " Lcs Tuberculeux Pulmonaircs Alcooliqucs the alcoholism preceded the pulmonary tuberculosis. en Mcl ieu Hospitalicr dans lc Department du Pas·de·Cala is," In discussing these combined disorders, which are Rn. Je Till> . tl P"'"·· 28 : 117, 1964.

METABOLIC AND ENDOCRINE COMPLICATIONS ENCOUNTERED DURING ANTITUBERCULOSIS THERAPY ministering vitamin 8 8 when treating patients with The authors present a meticulous study of the INH. A comprehensive bibliography of !541 related metabolic and endocrine complications encountered articles Is Included. In antituberculosis chemoth.erapy with Isoniazid. BaouET, G. AND MAitCHE, ] .: "Lcs Complications d 'Ordre PAS, streptomycin, viomycin, kanamycin, ethionaMetabolique et Endocrinien Produites par Ia Chimiotherapie mide, pyrazinamide and the sulfonamldes and thloAntibacillaire, " Rt~ . Jt Td. tl 28 :,, 1964. semlcarbazones. They stress the Importance of ad-

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