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Spontaneous Regression Of Budd-chiari Syndrome (hepatic Venous Occlusion) In A Young Female

Hultcrantz et al., 1987Other/Unknown

Acta Medica Scandinavica 221(5): 1987; 503-507

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Abstract

Serial hemodynamic and angiocardiographic studies demonstrated the spontaneous closure of an atrial septal defect in a 3-year-9-month-old white boy, the presence of which was confirmed in the second year of life. A possible myocarditis in the first year of life was considered to be potentially instrumental in the closure of this defect. Although similar observations have been made by others, to our knowledge this is the first such case, particularly at this age, reported in the literature.

Case Details

Personal Characteristics

18-year-old girl, previously healthy, swedish, no history of drug treatment including contraceptive steroids

Clinical Characteristics

Sudden onset with massive ascites, markedly impaired general condition, right-sided abdominal pain, extensive ascites and splenomegaly, right-sided pleural effusion, slight anemia, slightly elevated serum transaminases, raised alkaline phosphatases, slightly prolonged prothrombin time

Remission Characteristics

Clear improvement after two weeks, no ascites after four weeks, normalization of all liver function tests after four weeks, withdrawal of all diuretics after three months, complete recovery after 11 years

Treatment & Mechanisms

Proposed Remission Mechanisms

Spontaneous dissolution of widespread thrombi of the hepatic veins

Clinical Treatment

Conservative treatment with laparocentesis and diuretics, laparotomy, removal of ascites, liver biopsy, angiograms of the caval and hepatic veins and of the celiac artery

Additional Notes

This case is unusual since previously reported cases have had high mortality rates and, in surviving cases, operative procedures or large doses of diuretics have been required to control ascites.