Durr-e-Sabih ( Atomic Energy Medical Centre, Nishtar Medical College and Hospital, Multan. )
Sher Mohammed Khan Swati ( Atomic Energy Medical Centre, Nishtar Medical College and Hospital, Multan. )
Zahida Sabih ( Atomic Energy Medical Centre, Nishtar Medical College and Hospital, Multan. )
Budd Chiari Syndrome is a rare disease and can be difficult to diagnose. Most of the investigations to diagnose this disease are invasive. Liver scintiscanning is not only non-invasive, it also shows a typical pattern in this condition. Good correlation exists between Liver scanning and other procedures.
A case of Budd Chiari Syndrome with typical clinical and scan findings is described (JPMA 34: 375, 1984).
Symptomatic occlusion of hepatic veins, Budd Chiari Syndrome, is an infrequently diagnosed condition1. Clinically it presents with abdominal pain and rapidly aácumulating ascites. The commonest causes are polycythaemia rubra vera, nocturnal haemoglobinuria, hepatic webs, generalized thrombotic tendency, oral contraceptives and secondary hepatic involvement from hypernephromas. The cause is not clear in 50 70% of the cases1,2. The investigations used to diagnose this condition include hepatic scintigraphy, inferior venacavography, hepatic vehography, direct hepatography, splenoportagraphy and liver biopsy. A case with typical clinical and liver scan findings is reported here.
A 45 year old male presented with rapidly accumulating ascites. There was no history of previous jaundice, melaena or haemetemesis. On examination the patient had ascites, the abdomen was tense with fluid. No mass could be felt and there was no evidence of chronic liver dysfunction like spider naevi palmer erythema, gynaecomastia and testicular atrophy. The chest radiograph showed a normal heart size and clear lung fields. There was no calcification in the pericardium. Ascitic fluid was haemorrhagic out there was no evidence of malignant cells.
Scintiscan showed an enlarged, low lying medially displaced liver with a triangular midline area of increased uptake which was posteriorly oriented in the right lateral view. Spleen was not enlarged but there was increased uptake by the spine and ribs (Fig 1 and 2).
A diagnosis of hepatic vein obstruction was made and the patient was advised further investigations. Unfortunately the patient did not return and could not be followed up.
Hepatic venography and hepatic biopsy with typical findings of centrizonal venous congestion, haemorrhage and necrosis in the presence of normal right atrial and jugular venous pressures correlates well with radiocolloid scanning. Both hepatic venography and biopsy are relatively invasive procedures and not easily available in our setting. Hepatic scintiscanning on the other hand though technologically a more sophisticated investigation, is easily available in or near almost all large medical centres of the country and is almost totally non-invasive.
The typical findings of radionuclide imaging are diminished peripheral activity in the right and left lobe with a triangular midline area of normal or increased uptake3. Posteriorly oriented as seen in the right lateral view2 . this central localization may be the predominant feature or it may be accompanied by a patchy uptake by the rest of the liver. Normally the area of maximum uptake is to the right of the midline. This scan finding is suggestive enough to warrant a first diagnosis of Budd Chiari Syndrome3. The dimple sign has also been mentioned, appearing as an exaggeration of the normally present defect in the mid portion of the superior border of the liver due to the imprint of the two hepatic veins4, a’defect in this area is usually seen due to the cardiac imprint and the presence of this finding in a previously normal liver scan when associated with a normal cardiac shadow on the chest radiograph and central localization may increase the specificity of the scan4. The reason for this localization has been attributed to the caudate lobe of the liver which is not totally dependent upon the hepatic veins for drainage and has direct drainage channels into the inferior vena cava. This means more efficient flow to the lobe is maintained while the rest of the liver flow is compromised in hepatic vein obstruction. This ‘sparing’ along with the hypertrophy which this lobe undergoes in this pathology, as evidenced by inferibr vénacavography and autopsy2 is responsible for the increased uptake seen in the scintiscan.
The ‘hot area’ is not an exclusive finding seen only in Bucid Chiari Syndrome. Other pathologies described to give this appearance include haemangioma5, focal nodular hyperplasia and hepatic adenoma6 and abscess7 though these hot areas are not confined to any location. A picture similar to Budd Chiari Syndrome is seen in constrictive pericarditis and advanced cirrhosis but the distribution of colloid is different being largely perihilar without a prominent midline uptake and with more irregularity throughout the liver and with a more intense splenic uptake2.
In our patient the diagnosis of hepatic vein obstruction was suggested by the history, clinical examination, laboratory and scintiscan findings.
Liver scanning is easily available in this country and the diagnostic yield of this infrequently diagnosed condition might improve with greater awareness of scan findings in this pathology. This is especially relevant because treatment of Budd Chiari Syndrome is different from cirrhosis with the option of surgery being a very important consideration.
1. Parker, R.C.G. Occlusion of the hepatic veins in man. Medicine, 1959; 38: 369.
2. Tavill, A.S., Wood, E.J., Kreel, L., Jones, E.A., Gregory, M. and Sherlock, S. Budd-Chiari syndrome; correlation between hepatic scintigraphy and the clinical, radiological and pathological findings in nineteen cases of hepatic venous outflow obstruction. Gastroenterology, 1975; 68 : 509.
3. Meindok, H. and Langer, B. Liver scan in Budd Chiari Syndrome. J.Nucl. Med., 1976; 17: 365.
4. Gooneratne, N.S., West, T.E. and Frantz, G.M. “Dimple sign’ in hepatic vein thrombosis. Br. J. Radiol., 1979;52: 584.
5. Volpe, J.A. and Johnston, G.S. ‘Hot’ hepatic hemeangionca; a unique radiocolloid-concentrating liver scan lesion. J. Sur. Oncol., 1970; 2: 373.
6. Salve, A.F., Schiller, A., Athenasoulis, C., Galdabini, J. and McKusick, K.A. Hepatoadenoma and focal nodular hyperplasia; pitfalls in radiocolloid imaging . Radiology, 1977; 125 : 451.
7. Chayez, Z., Koenigsberg, M. and Freeman, L. The hot hepatic abscess. J. Nuclear Med., 1974; 15 : 305.