Canad. Med. Ass. J.May 24 and 31. 19K9. Case Reports: "Downhill" Esophageal Varices 961

'DownhilP5 Esophageal Varices in SuperiorVena Caval Obstruction

N. M. SHEINER, F.R.C.S.|C| and M. J. PALAYEW, F.R.C.P.fC], Montreal

VARICES of the esophagus, a common clinicalentity, usually occur at the lower end of the

esophagus; here they are almost always due toportal hypertension. However, they can also ap¬pear in the proximal esophagus, the so-called"downhill" varices.1,2 This rare type occurs incases of superior vena caval obstruction andrepresents part of the collateral venous systemby which blood is diverted from the upper to thelower part of the body.

It is the intent of this report to add two cases

of "downhiir varices to those reported in theliterature and to increase awareness of a condi¬tion which is probably commonly associated withsuperior vena caval obstruction. Although gastro¬intestinal hemorrhage did not occur in thesecases, it has been reported by others,5' 6* 8- 9 and"downhiir varices must be considered in thedifferential diagnosis of gastrointestinal bleeding.Case 1..Mrs. M.G., a 35-year-old white woman,

had a radical mastectomy for adenocarcinoma(Stage II) of the right breast in March 1964. Abilateral oophorectomy was performed during thesame hospital admission. The patient was well untilJanuary 1966, when carcinoma recurred in themedial portion of the mastectomy incision. This re¬

currence and the immediately adjacent area of thechest wall were resected. At the time, directextension of the tumour into the right side of themediastinum was found. The postoperative course

was complicated by infection in the skin incision andrecurrent pneumothorax; eventually the latter was

treated successfully by the insertion of multiplechest tubes. Following her recovery from thesecomplications, irradiation of the chest wall andmediastinum was carried out.

The patient was examined repeatedly in thetumour clinic and appeared relatively well untilMay 1966, when she complained of headache,dizziness, tightness of the neck and shortness ofbreath; edema and cyanosis of the face, neck andupper extremities rapidly followed. There was no

hemoptysis or gastrointestinal bleeding.On physical examination there was extensive

edema of the tissues of the face and neck withmarked periorbital swelling. The veins of the neckwere distended and the superficial veins of the arms

and upper thorax were dilated and prominent. Therewas no hepatomegaly, splenomegaly or clinical evi-

Fron.i the Departments of Cardiovascular Surgery andRadiology, Jewish General Hospital. Montreal, Quebec.Reprint requests to: Dr. N. M. Sheiner or Dr. M. J.Palayew, Jewish General Hospital. 3755 Cote Ste. Cathe-rine Road, Montreal 2G, Quebec.

Fig. 1..Case 1. Barium examination of the proximalesophagus demonstrates the large varices.

dence of portal hypertension. The remainder of thephysical examination revealed no abnormalities.

Radiographic examination demonstrated largevarices in the proximal portion of the esophagusbut none in the distal half (Figs. 1 and 2). Asuperior vena cavogram demonstrated obstruction ofthe superior vena cava and the left innominate vein(Fig. 3). The azygos vein did not fill. There were

prominent collaterals in the mediastinum.The patient was given 100 mg. of dromostanolone

propionate* by intramuscular injection three timesweekly, with temporary improvement in her symp¬toms. She died on November 9, 1966. Permissionfor an autopsy was not granted.

Case 2..A.L., a 34-year-old white man, was ad¬mitted to the Jewish General Hospital in October1965 complaining of a sensation of "fullness" of thehead and neck for the previous three weeks. Thisoccurred most often on arising and was aggravatedby stooping. He also noted that his neck veins were

*Dn>n>an .101 i Lilly and Company,

962 Case Reports: "Downhill" Esophageal Varices Canad. Med. Ass. J.May 24 and 31, 1969. vol. 100

Fig. 2..Case 1. The distal esophagus appears normal.

prominent, and his lips and ears were cyanotic andthat he was unable to button his shirt collar. Hegave no history of cough, hemoptysis, respiratory in-

#*. t0®m~®.

Fig. 3..Case 1. A venograph reveals the obstruction ofthe superior vena cava and left innominate vein. Theextensive collateral network is apparent.

Fig. 4..Case 2. A tomogram demonstrates the widen¬ing of the superior mediastinum.

fection or weight loss. His symptoms became pro¬gressively worse and he complained of dizziness.On physical examination the patient was a well-

developed man displaying a dusky, cyanotic faceand neck and periorbital edema. The external jugu-lar veins were large and even in the upright positionmarkedly distended up to the angle of the jaw.There were prominent superficial veins over theneck, arms and upper thorax. The remainder of thephysical examination was unremarkable.A complete blood count, a coagulogram and a

bone marrow aspiration showed no abnormalities.The following laboratory investigations yielded re¬sults which were within normal limits: blood urea

nitrogen, creatinine, sugar (fasting and postcibal),serum electrolytes, blood calcium and phosphorus,conjugated and unconjugated bilirubin, acid andalkaline phosphatases, thymol turbidity and thymolflocculation, total blood proteins and protein electro¬phoresis, and serum chorionic gonadotrophin level.The histoplasmin skin test was positive; the inter-mediate-strength purified protein derivative (tuber¬culin) skin test was negative. The complement-fixation test for histoplasmosis was negative.A tomogram of the chest revealed widening of the

superior mediastinum (Fig. 4). Venography of thesuperior vena cava revealed obstruction of that veinand of both innominate veins without extensivecollateralization. The azygos vein was not visualized.A right scalene lymph node biopsy revealed hyper¬plasia of the lymph glands. Radiological examina¬tion of the esophagus performed at this time did notreveal any varices.A diagnosis of idiopathic mediastinal fibrosis was

made. The patient was advised to avoid stoopingand to elevate the head of his bed 10 inches on

Canad. Med. Ass. J.May 24 and 31, 1969, vol. 1 00 Case Reports: "Downhill" Esophageal Varices 963

Fig. 5..Case 2. Barium examination demonstrates ex¬tensive varices in both the proximal (left) and distal(right) esophagus.

wooden blocks. Moderate salt restriction was prac¬tised and hydrochlorotniazide, 50 mg., was givendaily. In the three-year follow-up period he hasimproved to the point where he is almost completelyasymptomatic. When the barium examination of theesophagus was repeated in October 1966, variceslimited to the proximal esophagus were demon¬strated, and at the last examination in July 1968they were evident throughout the length of theesophagus (Fig. 5). Superior vena cava veno-

graphy at that time again demonstrated the ob¬struction of the innominate veins and the superiorvena cava (Fig. 6). However, on this occasion there

Fig. 6..Case 2. A venograph demonstrates the largeazygos vein (>) which fills in a retrograde direction.The inferior hemiazygos vein (<.) is readily apparentand there is an extensive network of collaterals in themediastinum (< <).

was a massive conglomeration of veins in the medi¬astinum. The azygos vein itself was large and flowin this vein occurred in a retrograde direction.

DiscussionThe venous drainage of the esophagus con¬

sists of a plexus of veins which is situated inthe submucosa and which extends through itsentire length. This plexus communicates with a

peri-esophageal venous plexus which, in thecervical and superior mediastinal segments ofthe esophagus, drains into the innominate veinsand superior vena cava by way of the inferiorthyroid, the bronchial and the highest inter¬costal veins. Venous effluent from the mid-thoracic segment of the esophagus drains intothe azygos and hemiazygos veins, and in thisway reaches the superior vena cava. The veinsof the distal esophagus drain into the azygosvein and also into the portal vein via the leftgastric vein and the vasa brevia of the splenicvein. Communications also exist between theperi-esophageal plexus and the internal mam¬

mary veins via mediastinal and pericardialveins.3

In superior vena caval obstruction blood re¬

turns to the right atrium via an extensive systemof collateral pathways; these include the azygos,the vertebral, the lumbar, the internal mammary,the lateral thoracic and intercostal and the portalveins.4 If the junction of the azygos vein and thesuperior vena cava is not involved in the ob¬structive process, blood can return to thesuperior vena cava via the azygos system andconsequently will not be diverted into theportal system or into the inferior vena cava.

If this junction is obstructed, blood must bediverted through all the collateral channels intothe portal vein as well as directly into the in¬ferior vena cava.

Consequently in the presence of superior cavalobstruction (with or without azygos vein in¬volvement) blood flows through the collateralchannels in a retrograde direction, i.e. "down-hiH". The pressure in the mediastinal collateralsis considerably elevated and this elevated pres¬sure is transmitted to the loosely supported andvalveless veins in the submucosal esophagealplexus, resulting in varices. Since blood literallyflows in a downhill direction in these varices,there is validity to Felson's term, "downhiir'varices.1 In portal hypertension, blood flows inan upward direction through the lower eso¬

phageal veins to reach the azygos vein. Conse¬quently, esophageal varices that occur as a resultof portal hypertension can theoretically be re¬

ferred to as "uphill" varices.

964 CASE REPORTS: "DOWNHILL" ESOPHAGEAL VARIGES Canad. Med. Ass. J.May 24 and 31, 1969, vol. 100

In the first of our two patients the superiorvena caval obstruction appeared acutely andwas undoubtedly due to metastatic carcinomaof the breast. In the second patient the three-year follow-up period would appear to confirmthe benign nature of the obstructive process.The failure to demonstrate varices at the firstbarium examination of the esophagus in thissecond case is most likely related to the pro-gressive development of collateral channels,which, in the case of such an extensive network,presumably requires a relatively long period oftime. The initial radiography of the esophagusand the initial venography were performedshortly after the onset of symptoms, presumablybefore enough time had elapsed for the develop-ment of collaterals. Furthermore, the relief ofthe patient's symptoms appeared to correspondto the development of this collateral network.The location of the varices in superior vena

caval obstruction appears to be dependent ontwo factors-the site of the venous obstructionand its duration. Theoretically, if the azygosvein is not involved in the obstructive process,varices should be limited to the proximal portionof the esophagus, and this is borne out by thecases reported in the literature..' 6 In our firstpatient the azygos vein was not visualized onthe venogram and presumably was obstructed.In the second patient retrograde flow wasdemonstrated in the azygos vein, confirming thatits junction with the superior vena cava was in-vQlved. One would therefore expect that varicesin both patients could have been demonstratedalong the entire length of the esophagus, asthe azygos vein was obstructed in each. How-ever, in one patient (Case 1) the varices werelimited to the proximal esophagus and in theother (Case 2) they eventually were presentthroughout. The difference in the extent of in-volvement can be attributed to the secondfactor-the duration of the disease process inthe mediastinum. In the first patient the venousobstruction was due to malignant disease andthe patient survived for only six months after thediagnosis of superior vena caval obstruction wasmade. In almost all instances of superior venacaval obstruction due to malignant disease, evenif the azygos vein is involved, the lower eso-phageal veins are not affected, presumably be-cause the spread of the process to the entireesophagus requires a longer period of time andsuch patients do not live sufficiently long. Onthe other hand, in Case 2, as in other reportedinstances of esophageal varices due to idio-pathic mediastinal fibrosis where the diseaseprocess was benign and of long duration, varicesinvolved the entire length of the esophagus." 710

Varices are occasionally encountered in theesophagus without any apparent cause. Palmer'1described three cases of primary varices of thecervical esophagus in the absence of superiorcaval obstruction or portal hypertension. Hespeculated that the varices in this location weredue to the constricting action of the crico-pharyngeus muscle (which is normally in astate of tonic contraction) on abnormal caudalextensions of the hypopharyngeal plexus. Inthese three patients massive upper gastro-intestinal hemorrhage was attributed to thevarices.

Varices of the upper esophagus secondary tosuperior caval obstruction have been recognizedfor many years but have been infrequently re-ported. In 1929 Kaufmann'2 mentioned proximalesophageal varices in cases of "congestion of thesuperior vena cava as that which occurs frommalignant goitres", though he cited no specfficcases. In 1964 Felson and Lessure' collected 27cases from the literature and added three oftheir own. The cause of superior caval obstruc-tion in these cases was carcinoma of the lung in15, Hodgkin's disease in two, retrostemal thyroidtumour in three, metastatic carcinoma in one,mediastinal fibrosis in seven, surgical ligation ofthe superior vena cava in one and a mediastinalmass of undetermined origin in one. Although"downhill" varices are asymptomatic in mostinstances, four cases of bleeding attributed tothem have been reported.5' . 8, 9

It is likely that proximal esophageal varicesdo not constitute a rare entity, and they prob-ably occur in the majority of patients withsuperior caval obstruction who survive suffi-ciently long for the varices to form. Theirrecognition as a potential cause of gastroin-testinal bleeding in such patients depends on anunderstanding of the collateral venous pathways.

The authors would like to thank Dr. M. A. Gold forreferring the second patient.

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