BUDD-CHIARI SYNDROME DUE TO INFERIOR VENA CAVA OBSTRUCTION

Transcription

1 GASTROENTEROLOGY Copyright 1968 by The Williams & Wilkins Co. Vol. 54, Nc;. (j Printed in U.S.A. CASE REPORTS BUDD-CHIARI SYNDROME DUE TO INFERIOR VENA CAVA OBSTRUCTION M I, C~ EH DAVIS, L M.D., ROBERT PETERS, M.D., AND TELFEH REYKOLDS, M.D. Departments of Medicine and Pathology of the University of Southern California School of Medicine and the Los Angeles County General Hospital, Los Angeles, California The Budd-Chiari syndrome is an uncommon disorder caused by obstruction to hepatic venous outflow and associated clinically with ascites, abdominal pain, hepatomegaly, edema, and occasionally jaundice. It is usually ascribed to thrombosis of the hepatic veins, to their obstruction by tumor or cyst, or to unexplained fibrous obliteration of the veins or their orifices. In recent years there have been a number of reports of the Budd Chiari syndrome caused by a fibrous diaphragm or membrane occluding the inferior vena cava above the entrance of the right hepatic vein.1-6 In an article in this issue of Gastroenterology (page 1070), Yamamoto and colleagues describe 19 patients with this lesion. 7 Recognition of vena caval block by a membrane is important because of its potential relief by surgery. The patient described in' the present report has a remarkable paucity of symptoms and findings and is being presented to emphasize that this lesion must be considered in all patients with a Budd-Chiari syndrome, whether or not there are clinical signs of vena caval block. Case Report The patient, C. G., is a 31-year-old Caucasian housewife who was referred to the Hepatic Serv- Received February 1, Accepted February 12, Address requests for reprints to: Dr. Telfer Reynolds, 2025 Zonal A venue, Los Angeles, California The authors wish to thank Dr. Konstantin Sparkuhl for referring the patient and Drs. Mordecai Halpern and A. Franklin Turner of the University of Southern California Radiology Department for performing the inferior vena cavagram and preparing the X-ray sketches ice of the University of Southern California Medical School for evaluation of possible liver disease. Three years previously she had an illness lasting 2 months characterized by anorexia and fatigue that was diagnosed as viral hepatitis. Tender hepatomegaly and questionable scleral icterus were noted by her physician, although laboratory tests were within normal limits (serum bilirubin, 0.3 mg per 100 ml; cephalin cholesterol flocculation, 1+; thymol turbidity, 2 units; alkaline phosphatase, 8 King-Armstrong units). She was treated with bedrest at home. Since then she has had occasional episodes of mild abdominal pain and has noted intermittent mild ankle edema, for which she was given diuretics. Her health has otherwise been good. She had taken oral contraceptives for the past 7 years and has had scanty menstrual flow for the past 6 months. She was admitted to a local hospital on September 14, 1967, with a 24-hr history of cramping abdominal pain and fullness. On examination a tender liver edge was palpable 3 em below the right costal margin. There was no ascites or edema. The pain subsided but recurred again for brief periods during the next 2 weeks. Laboratory tests were as follows: total serum bilirubin, 1.3 mg per 100 ml; direct bilirubin, 0.9 mg per 100 ml; albumin, 3.6 g per 100 ml; globulin, 3.0 g per 100 ml; alkaline phosphatase, 1.9 Bessey-Lowrey (B-L) units; and Bromsulpha Ie in retention, 19% at 45 min. Barium meal revealed a questionable duodenal ulcer. A liver scan showed only slight hepatomegaly. Liver biopsy was performed because of the hepatomegaly and the prior history of "hepatitis." It showed marked centrolobular congestion in some areas. Other areas had centrolobular collagen with fibrin in the sinusoids at the edge of the collagenous scarring (fig. 1). The interpretation was "probable Budd-Chiari syndrome." The patient was then referred to the University of Southern California Medical School for further evaluation.

2 June 1968 CASE REPORTS II-±:3 FIG. 1. First liver needle biopsy, showing a segment of sublobular vein to the right with fibrous obliteration of the centro lobular area. The recognizable liver to the left has intrasinusoidal fibrin (hematoxylin and eosin, X 150). On admission she appeared to be in excellent health (fig. 2). Blood pressure was 110/ 70 mm Hg, pulse rate was 70 per min, and temperature was 98.6 F. The heart and lungs were normal to examination. A firm, slightly tender liver edge was palpable 4 cm below the right costal margin. There was no evidence of dilated abdominal Yeins, ascites, edema, splenomegaly, jaundice, or spider angiomata. The urinalysis was normal. The hematocrit was 48%; white blood count, 7600 cells per mm 3 ; serum urea nitrogen, 13 mg per 100 ml; albumin, 4.9 g per 100 ml; globulin, 2.5 g per 100 ml; alkaline phosphatase, 4.5 B-L units; total bilirubin, 0.8 mg per 100 ml; serum glutamic oxaloacetic transaminase, 38 units; serum glutamic pyruvic transaminase, 24 units; prothrombin activity, 90% ; and Bromsulphalein retention, 7% at 30 min. Chest X-ray was normal. Hepatic vein catheterization was attempted via an antecubital vein but the catheter could not be passed into the inferior vena cava from the right atrium (fig. 3). The next day the inferior vena cava was catheterized percutaneously from the right femoral vein. Serial biplane X-rays after contrast media injection revealed a complete block in the vena cava just above the orifice of the right hepatic vein (fig. 4). The right hepatic vein was widely patent but the left was not visualized. The right hepatic and caval blood was rerouted by an extensive collateral system into a large azygos vein (fig. 5). Pressure in the inferior vena cava was 11 mm Hg above the externally estimated position of the right atrium (5 cm below the sternal angle with the patient FIG. 2. Patient C. G. with no demonstrable physical abnormalities except for (costal margins and liver edge are outlined in ink). h eapt om eal g~

3 1144 CASE REPORTS Vol. 54, No. (j FIG. 3. Contrast media injection through a cardiac catheter in the right atrium. The catheter could not be passed into the inferior vena cava. supine). A second liver biopsy was performed, again showing centrolobular fibrosis surrounded by severe sinusoidal congestion (fig. 6). Repeat barium meal failed to show the previously suspected duodenal ulcer and did not demonstrate esophageal varices. Esophagoscopy showed no evidence of varices. A liver scan with radioactive colloidal gold indicated mild hepatomegaly with no filling defects and no appreciable splenic uptake of radioactivity. Discussion In 1959, Parker reviewed the literature on Budd-Chiari syndrome, finding 149 cases with autopsy confirmation and adding 18 cases from the records of The London Hospital. s Although stenosis of the vena cava accompanied hepatic vein occlusion in about one-third of the cases, there wa5 only 1 patient (case 9 in Parker's series) with a clear description of a fibrous membrane occluding the vena cava above the right hepatic vein orifice. In contrast, the Japanese literature contains many references to such a lesion. Kimura and colleagues in 1963 reported 6 cases of their own and referred to 24 additional cases in a total of 119 with the Budd-Chiari syndrome reported in the Japanese literature. 1 In this issue, Yamamoto and colleagues describe 19 patients with thi:=

4 June 1968 CASE REPORTS 1145 _Hep_opc Vein -', ~ ~ /,,,, Diaphragms theter B FIG. 4. Contrast media injected through a catheter in the inferior vena cava. The right hepatic vein is widely patent; the vena cava is occluded immediately above the hepatic vein orifice. lesion seen between 1951 and 1967 and indicate that it was the sole cause for the Budd-Chiari syndrome seen in their surgical department during that period. 7 Our hepatic service has recognized the disorder once previously, in a 34-year-old Chinese male. The lesion is not exclusively Oriental, however. In addition to Parker's case, there are American patients with similar pathology reported by Schaffner and colleagues,2 Bennett,9 vvatkins and Fortin,4 and Lam et a1. 5 The same lesion has been described in 5 African patients 6 and 111 a New Zealander.lo Etiology is uncertain, although a congenital origin, related to the closure of the ductus venosus, has been suggested by Kimura et au Rossall and Caldwell ll and KibeI and Marsden 12 interpreted seemingly similar membranes in their cases as persistent eustachian valves. If the lesion is congenital, then deterioration of the compensating collateral circulation in adult life would seemingly be required to account for the delayed appearance of clinical manifestations. There are two recent reports of development of a Budd-Chiari syndrome in women taking oral contraceptives. 13,14 Although we question the importance of the association, our patient had been taking oral contraceptives for 7 years. We did not demonstrate thrombosis, although this might have developed in some of the small branches of the right hepatic vein or in the collateral vessels around the vena caval block, preceding the episode of abdominal pam. The diagnosis of a vena caval diaphragm should be considered in all patients with evidence of the Budd-Chiari syndrome. Although most patients will have visible abdominal collateral veins to suggest vena caval block, this is not a necessary finding as indicated by our patient and 1 of

5 1146 CASE REPORTS Vol. 54, No.6 Vein... FIG. 5. Contrast media injected through a catheter in the inferior vena cava (lateral view). Collateral lumbar veins are visible as well as a large azygos vein, in the same plane as the vena cava, extending above t.he diaphragm. FIG. 6. The second liver biopsy, showing further centrolobular collagenosis depicted in the lower left comer. Surrounding the centro lobular obliteration the sinusoids are congested (Masson, X 125).

6 J une 1968 CASE REPORTS 1147 those (case 368) reported by Yamamoto and colleagues. 7 Ordinarily, ascites will be present to raise the suspicion of a Budd Chiari syndrome but, as indicated by our patient and case 368 of Yamamoto's, the clinical findings may be limited to hepatomegaly and right upper quadrant pain. Once suspected, vena caval block can be readily diagnosed by inferior vena caval catheterization and venography. Surgical therapy for this lesion is possible, as is emphasized in many of the reports. 1-7 Transcardiac m embranotomy would seem the procedure of choice if the membrane is not too thick. Long term results are unknown but maintenance of vena caval patency fora few months postoperatively has been demonstrated in several patients. 1-7 In 1 of the patients described by Rogers et al.,6 reclosure of the vena cava was found at autopsy 4 years after a successful transcardiac membranotomy. In our patient, because of her asymptomatic state, there has been considerable debate about the advisability of surgery at this time. We finally decided to discontinue oral contraceptives and to propose surgery if evidence of hepatic deterioration appears. Summary A patient is described with a Budd Chiari syndrome due to membranous obstruction of the inferior vena cava above the orifice of the right hepatic vein. Symptoms and findings were limited to right upper quadrant pain and mild hepatomegaly, and the Budd-Chiari syndrome was suspected only because of characteristic hepatic histology. REFERENCES 1. Kimura, C., H. Shirotani, M. Hirooka, M. Terada, K. Iwahashi, and S. Maetani Membranous obliteration of the inferior vena cava in the hepatic portion. J. Cardiov. Surg. 4: Schaffner, F., M. Gadboys, A. P. Safran, M. G. Baron, and A. H. Aufses Budd Chiari syndrome caused by a w eb i n the inferior v ena cava. Amer. J. Med. 42: Ohara,!., H. Ouchi, and K. Takahashi A bypass operation for occlusion of the hepatic i nferior vena cava. Surg. Gynec. Obstet. 117 : Watkins, E., Jr., and C. L. Fortin Surgical correction of a congenital coarctation of the inferior vena c ava. Ann. Su rg. 159 : Lam, C. R, E. Green, and H. Gale Transcardiac membranotomy for obstruction of the hepatic portion of the inferior v ena cava. Circulation 31: suppl. 1, Rogers, M. A., E. Chesler, L. DuPlessis, N. J affe, and E. Joubert Membranous obstruction of the hepatic segment of the inferior vena cava. Brit. J. Surg. 54: Yamamoto, S., Y. Yokoyama, K. T akeshige, and S. Iwatsuki Budd-Chiari syndrome with obstruction of the inferior vena cava. Gastroenterology 54: Parker, R G. F Occlusion of the hepatic veins in man. Medicine (BaIt.) 38 : Bennett,!., Jr A unique case of obstruction of the inferior vena c ava. Bull. Johns Hopkins Hosp. 87: Smith, L A case of congenital stenosis of the inferior vena cava w ith portal hypertension. Brit. J. Surg. 5 2: Rossall, R E., and R. A. Caldwell Obstruction of inferior vena cava by a persistent e ustachian v alve in a young adult. J. Clin. Path. 10: KibeI, M. A., and H. B. Marsden Inferior v ena caval and hepatic vein thrombosis : the Chiari syndrome in childhood. Arch. Dis. Child. 31: Ecker, J. A., J. E. McKittruk, a nd R M. F ailing Thrombosis of the hepatic veins. The Budd-Chiari syndrome; a possible link between oral contraceptives and thrombosis formation. Amer. J. Gastroent. 45: Sterup, K., and J. Mosbeck Budd Chiari syndrome after taking oral contraceptives. Brit. J. Med. 4: 660.