Interruption of the Inferior Vena Cava with Azygos Continuation

steroids with gradual improvement in his pulmonary status during the following three days. He was discharged from the hospital the 19th day after surgery. During the ensuing two years he developed progressive angina. Repeat coronary arteriography showed complete occlusion of the saphenous vein bypass to the anterior descending branch, but preservation of patency in the bypass to the right coronary artery. Cardiac output and left ventricular enddiastolic pressure remained the same as before operation. Pulmonary function evaluation 19 months after discharge revealed a moderate restrictive ventilatory defect (Table 2), normal arterial blood oxygenation and a slight decrease in diHusing factor. Chest x-ray findings were unremarkable. The patient was again operated upon on March 18, 1971, and another graft to the anterior descending branch was performed (pump time 138 min). The postoperative course on this occasion was again complicated by an episode of the adult respiratory distress syndrome. This time the treatment was promptly initiated on the third day after operation (steroids, furosemide and gentamycin ) with improvement in 72 hours. Neither tracheostomy nor endotracheal intubation was necessary. He was discharged on April 4, 1971. Repeat pulmonary function evaluation carried out in April, 1972, one year after the second episode of adult respiratory distress syndrome (Table 2) showed no changes from the previous evaluation. Chest x-ray examination at this time was interpreted as normal. DISCUSSION

Of the small but significant number of patients with the adult respiratory distress syndrome who survive pulmonary injury, most are said to develop pulmonary fibrosls." The mechanism of this process is not understood at present. Administration of high concentrations of oxygen, intercurrent infection, or a combination of both," have been suggested as possible causes for this interstitial fibrosis. The patient described here is one of 14 cases recently reported from our institution who developed the syndrome after cardiopulmonary bypass! and he stands out as having developed it twice. On both Table 2-Pulmonary Function Studies alter Each Epi.ooe 01 the "Re.piratory Distre•• Syndrome"

19 months after 12 months after first episode second episode

VC (% predicted)

75

76

FEV 1.0 (% of VC)

81

82

MVV (% predicted)

86

84

Pa02 (mm Hg)

83

84

Pac02 (mm Hg)

34

37

pH

7.41

DLCoSS (ml/min/mm Hg)

VC FEV 1.0 MVV P8.02 Pac02

DLCoSS

15

7.47 13.8

= vital capacity = forced expiratory volume in 1.0 second

=

maximal voluntary ventilation

=

arterial carbon dioxide tension diffusing capacity of the lungs by steady state technique (predicted 17 ml/miu/mm Hg)

= arterial oxygen tension

=

CHEST, 65: 4, APRil, 1974

occasions he received concentrations of oxygen in the inspired air in excess of 60 percent and the course was complicated by Gram-negative bacterial infections of the respiratory tree. In this particular patient, these facts mitigate against their being an important etiologic factor in the development of interstitial pneumonia since he recovered completely. Other factors, including genetic and immunologic considerations, may be more important, although the present state of knowledge is insufficient to corroborate such an assumption. No clinical, physiologic or radiologic signs of pulmonary damage, one and three years after recovery from the syndrome, have been observed. Longterm studies of similar patients to the one reported here should help us understand the natural history of survivors of the adult respiratory distress syndrome and add valuable information to an understanding of the mechanisms of lung injury and healing in interstitial pneumonias and fibrosis. ACKNOWLEDGMENT: I wish to express my appreciation to Drs. Thomas o. Centsch, Parry B. Larsen and Ernest A. Traad for making the above patient available to me. REFERENCES

1 Ashbaugh DC, Bigelow DB, Petty TL: Acute respiratory distress syndrome in adults. Lancet 2:319-323, 1967 2 Respiratory diseases. Task force report on problems, research approaches, needs: Respiratory distress syndrome 167-180. Department of HEW publication No. (NIH) 73432, October 1972 3 Huber G, LaForce M, Mason R: Impairment and recovery of pulmonary antibacterial defense mechanisms after oxygen administration. J Clin Invest 49:47a, 1970 4 Llamas R, Forthman HJ: Respiratory distress syndrome in the adult after cardiopulmonary bypass. A successful therapeutic approach. JAMA 225: 1183-1186, 1973

Interruption of the Inferior Vena Cava with Azygos Continuation* Mario Bernal-Ramirez, M.D.,oO Hurst B. Hatch, Ir., M.D., F.C.C.P.,t and P. Jeffrey Bower, M.D.o O

A case of an inferior vena caval drainage anomaly presented as a right hOar density on plain chest roentgenogram. The benign nature of this shadow was established by angiography. Clues to its presence are reviewed and appropriate diagnostic steps are illustrated.

V

ascular malformations must be considered in the diHerential diagnosis in patients with chest x-ray film evidence of hilar masses. The relative ease of diagnoses (by angiography) and the major consequence of incomplete diagnosis (thoracotomy! and/or death-) , require an awareness of their presentation. We report an °From the Department of Internal Medicine, Sections on 00 Cardiology and tPulmonary Diseases, Ochsner Clinic and Ochsner Foundation Hospital, New Orleans, La. Reprint requests: Dr. Bower, Alton Ochsner Medical Foundation, 1514 JeDerson Highway, New Orleans 70121

INTERRUPTION OF INFERIOR VENA CAVA 469

FICamE 1. Anterior-posterior projection demonstrating right hilar mass. asymptomatic pati ent with a right hilar ma ss on ches t xray film and inferior vena cava int erruption with az ygos continua tion documented by angiogra phy . This entity (i nferior ven a cava int erruption with azygos continuation ) was previou sly described in a variety of wa ys: absent inferior vena cava, anomalous inferior vena cava with azygos drainage, abse nce of the hepatic segment of th e inferior vena cav a, persisten ce of th e subcardinal vein, per sisten ce of the sup raca rdina l ve in, and continuation of th e postcardinal ve in .' That all of th ese term s describe th e sa me entity is confusing. The ano ma ly de scribed herein consi sts simply of inte rru p tion of th e normal course of the inferior vena ca va above th e level of th e renal veins (and below th e level of th e hepatic veins ) and drainage into the azygos system through a nastomotic channels established in the primitive emb ryo. The hepatic veins drain directly into the right a trium. A similar malformation with inferior vena ca va int erruption and hemi azygos drainage has also been descr ibed. A complete and excellent system of precise an atomic ca te goriza tion of infer ior ven a caval drainage patterns was d escrib ed b y Testelli.' The az ygos ve in norm all y has a pathway parallel to th e right border of th e sp ine and curve s anteriorly at th e level of th e fourth and fifth th ora cic vertebrae (T. -T ,,) to en te r th e superior vena cav a. The hem iaz ygos vein norm all y run s parallel to th e left border of the sp ine and at th e level of th e eighth and n inth th ora cic vertebrae (T, -T ,,) cross es th e sp ine to join th e azygos vein. ' The ante rior porti on of th e azy gos a rch, near its junction with th e supe rior vena cava, may be recogn ized on ante roposter ior ( AP) roentgen ogram s of th e chest as an oval sha dow lying imm ediately lat eral to th e right main bronchus a nd just superi or to th e right upper lobe bronchus." The size of the az ygos a rch is va riab le but shoul d not excee d 6 to 7 mm in its maximal diameter on an AP x-ra y film of the chest with the patient erect." Dilatation of th e azygos vein may occur on a congen ital basis or be

470 BERNAL·RAMIREZ, HATCH, BOWER

FIGURE 2. Planigram through right hilum at 11 em. due to increased blood flow or increased filling pressure on th e right sid e of th e heart." The combination of dil at at ion of th e a zygos arch on AP ches t x-ray film made with th e patient in th e erect position and absence of th e sha dow of the inferior ve na cava on a lateral ches t x-ray film" should suggest th e di agnosis of int erruption of th e inferior vena cava with azygos con tinua tion . C ASE R EPORT

An asymptomatic 46-year-old man entered the Ochsner Foundati on Hospital for evaluation of a right hilar density demonstrated on routine chest x-ray 111m ( Fig 1). The only significant medical history consisted of mild diabetes mellitus and hypertension controlled with diet. Physical examination, including careful cardiovascular evaluation, was within normal limits. Findings on electrocardiogram were within normal limits. Routine laboratory findings revealed no abnorm alities, except for a fasting blood sugar level of 150 mg/100 ml (n ormal 70-110 mg/100 ml ). Planigrams through the right hilum ( Fig 2) bett er defined the right hilar shadow. The

FIGURE 3. "Cand y-cane" configuration in patients with interruption of inferior vena cava with azygos continuation.

CHEST, 65: 4, APRIL, 1974

clinical impression was that the right hilar mass was due to azygos vein dilatation. Right heart catheterization and venous angiography were performed to define the mass lesion and exclude associated heart disease. Percutaneous . catheterization via the right femoral vein demonstrated the characteristic course of vena caval drainage in inferior vena caval interruption with azygos continuation (Fig 3) (the catheter courses parallel to the right side of the spine, posterior to the heart, then turns anteriorly and inferiorly to enter the right atrium : so-called "candy-cane" configuration). Pressures in the right side of the heart were normal . There was no evidence of left-to-right or right-to-left shunt. An inferior vena cava angiogram (Fig 4) was obtained utilizing 40 ml of 30 percent diatrizoate sodium and 60 percent diatrizoate meglumine (Hypaque-M, 90 percent) injected under pressure. Subsequently, a superior vena cava angiogram (Fig 5) was obtained utilizing 30 ml of the same contrast medium injected under pressure through a catheter introduced into a left basilic vein . The superior vena cava and the azygos continuation of the inferior vena cava appear to enter the right atrium as separate channels (see arrow s, Fig 4, 5). Figure 6 represents an artistic composite of the two angiograms demonstrating a more lateral and anterior entry of the azygos continuation of the inferior vena cava and a more medial and inferior entry of the superior vena cava into the right atrium. The patient tolerated the procedure well and was discharged after counseling in reference to his chest xray film. DISCUSSION

FiGURE 4. Inferior vena cava angiogram. Retrocardiac course of inferior vena cava-azygos system (lower arrows) . Course of azygos into right atrium (upper arrows). Note smooth inferior margin of right atrium and absence of inferior vena cava from its usual location.

Interruption of the inferior vena cava with azygos vein continuation is unusual. It is reported to occur in 0.6 percent of cases of congenital heart disease." Associated cardiac malformations generally give rise to cyanosis and include atrioventricular canal defects, cor biloculare, large atrial septal defects, and pulmonary valve stenosis. There is also a frequent association with dextrocardia or dextroversion." The true incidence of this anomaly in the absence of associated cardiac defects would be difficult to determine. Untreated, its benign nature is testified to by its presence at autopsy in a 91-year-old patient."

Azygo s

FIGURE 5. Superior vena cava angiogram. Course of superior vena cava into right atrium (arrows) . Compare with Figure 4. Note shadow lateral and parallel to superior vena cava : azygos continuation.

CHEST, 65: 4, APRIL, 1974

FIGURE 6. Artistic composite R. AIM demonstrating more lateral and anterior entry of azygos continuation of inferior vena cava and more medial and inferior entry of superior vena cava into right atrium. Arrows indicate separate portals of entry.

- Inferior

""= Vena
INTERRUPTION OF INFERIOR VENA CAVA 471

Clues to the presence of this anomaly can be appreciated on routine chest roentgenograms made with the patient in erect AP and lateral positions as previously discussed; Further support for the diagnosis may be obtained by chest fluoroscopy with the patient in the upright position. Increase in size of the perihilar shadow during the Muller maneuver and decrease in size of the shadow during the Valsalva maneuver!" lends support to the diagnosis of azygos vein dilatation . Venous angiography is essential for proof, particularly in patients with associated cardiac disease. Proper diagnosis eliminates the need for unnecessary thoracotomy and allows for appropriate planning for operation on those patients in whom the azygos vein might be sacrificed, or cardiopulmonary bypass could be used . Our report is the third documented case of inferior vena cava interruption with azygos continuation wherein the azygos enters the right atrium as a separate channeltt -!" rather than into the superior vena cava as is usually the case. ACK;\;OWLEDGME;\;T : We wish to express our appreciation to Mr. George Atkins, photographer, and Mrs. Suzanne Clark, medical illustrator, for their assistance in preparation of the illustrations. REFERENCES

1 Magbitang MH, Hayford FC , Blake JM : Dilated azygos vein simulating mediastinal tumor: Report of case. N Engl J Med 263 :598-600, 1960 2 Effler DB, Green AE, Sifers EC : Anomaly of vena cava inferior: Report of fatality after ligation. JAMA 146: 13211322, 1951 3 Anderson RC, Adams P Jr. Burke B: Anomalous inferior vena cava with azygos continuation (infrahepatic interruption of the inferior vena cava). J Pediatr 59 :370-383, 1961 4 Testelli MR : Continuacion hemiacigos de la vena cava inferior. Descripcion de una variedad con situs inversus del corazon. Arch Inst Cardiel Mex 36 :690-697, 1966 5 Testelli MR, Cutierrez-Fuster E, Soni J : Continuacion hemiacigos a seno coronario de la vena cava inferior con vena cava superior bilateral. Arch Inst Cardiol Mex 38 :88-102, 1968 6 Heitzman ER : Radiologic appearance of the azygos vein in cardiovascular disease. Circulation 47 :628-634, 1973 7 Flei schner FG, Udis SW: Dilatation of the azygos vein : Roentgen signs of venous engorgement. Am J Roentgenol Radium Ther Nucl Med 67 :569-575, 1952 8 Heller RM, Dorst JP , James AE Jr, et al : A useful sign in the recognition of azygos continuation of the inferior vena cava. Radiology 101 :519-522, 1971 9 Dwight T: Absence of the inferior vena cava below the diaphragm. J Anat Physiol35-37, 1901 10 Stauffer HM, LaBree JW, Adams FH : The normally situated arch of the azygos vein: Its roentgenologic identification and catheterization. Am J Roentgenol Radium Ther Nucl Med 66 :353-360, 1951 11 Downing DF : Absence of the inferior vena cava . Pediatrics 12:675-680, 1953 12 Kjellberg SR, Mannheimer E, Rudhe U, et al: Diagnosis of Congenital Heart Disease (ed 2) . Year Book, Chicago, 1959, p 12

472 O'BRIEN, FORSMAN, WILTSE

Early Onset Sarcoidosis with Pulmonary Function Abnormalities* Leo E. O'Brien, M.D ., Patricia]. Forsman , M.D ., and Hobart E. Wiltse, M.D.

A four-year-old boy with sarcoidosis is described. His clinical features led to the diagnosis of cutaneous and synovial sarcoidosis and tissue biopsies confirmed the clinical impression. Pulmonary involvement was documented by pulmonary function studies. Treatment with adrenocorticosteroids resulted in regression of the disease process. he onset of sarcoidosis prior to the age of four years T appears to be rare. Only 13 documented cases have

been reported previously.' :" The early clinical symptoms in the very young child with sarcoidosis differ from those seen in older children and adults . The characteristic features are a distinctive rash, polyarticular arthritis, severe uveitis and absence of roentgenographic or laboratory evidence of disease.' Data on pulmonary function studies in the very young child with sarcoidosis are lacking. Reported herein are the findings , including pulmonary function studies in a child with onset of his disease prior to four years of age. CASE REPORT

The patient is a four -and-one-half-year-old white boy who was apparently well until , at age two and one-half years, his mother noted a progressive, non pruritic, pink, maculopapular rash covering most areas of his body. The lesions were unresponsive to topical steroid therapy. Since the age of three and one-half year s the boy has had intermittent ankle and knee swelling without subjective complaints. Physical examination revealed a four-and-one-half-year-old boy who was 100 cm tall (third percentile) and weighed 14.2 kg (third percentile) . A follicular, keratotic, mildly erythematous rash with clusters of small, oily, yellow papules was °From the Department of Medicine and the Derartment of Pediatrics, University of Nebraska College 0 Medicine, Omaha, Neb . Reprint requests: Dr . O'Brien, University of Nebraska Medical Center, Omaha 68105

I

FIGURE 1, 2. Cutaneous involvement.

CHEST, 65: 4, APRil, 1974