Budd–Chiari syndrome complicating hepatic sarcoidosis: A rare association

Laamrani Fatima Zahra,Saoud Aida

doi:10.5348/101068z01sa2019cr

Abstract

Introduction: The Budd–Chiari syndrome is an obstruction of the hepatic venous drainage. It is not common as a complication of hepatic sarcoidosis but the association is possible. We report a case of Budd–Chiari syndrome complicating a hepatic sarcoidosis. Case Report: A 32-year-old female patient with a history of mediastino-pulmonary, hepatic, and splenic sarcoidosis since her young age, that stopped her medication, was readmitted at our Emergency Department for recurrent threatening hematemesis. The oesogastroduodenal fibroscopy found esophageal varices. Biological assessment found a cytolysis and cholestasis. Hepatic Doppler ultrasound showed nodular bumpy hepatomegaly, with outflow obstruction of the hepatic veins, and a laminated aspect of the inferior vena cava that remains permeable. Abdominal computed tomography (CT) showed multinodular liver and spleen, multiple lymphadenopathies, and a lack of individualization of hepatic veins. Chest CT showed an interstitial syndrome associated with bilateral mediastinal and hilar adenopathies. Viral serologies B and C, and the search for thrombophilia were negative. Hepatic sarcoidosis was previously confirmed by liver biopsy, with a negative tuberculosis testing and a high level of angiotensin-converting enzyme. All these assessments led to the diagnosis of Budd–Chiari syndrome complicating hepatic sarcoidosis. Conclusion: Budd–Chiari syndrome is thus an exceptional complication, but possible in case of hepatic sarcoidosis. Therefore, it is worthwhile to look for it in case of any worsening of hepatic sarcoidosis, and to suggest possible sarcoidosis in case of Budd–Chiari syndrome.

Highlights

The Budd–Chiari syndrome is an obstruction of the hepatic venous drainage
Budd–Chiari syndrome is a heterogeneous group of clinical conditions presenting with hepatic venous outflow obstruction, which can be located at any level from the small hepatic veins to the junction of the inferior vena cava with the right atrium [3]
It is classified as being primary if flow is obstructed due to an endoluminal aberration, or secondary if the flow is obstructed by compression or invasion of a lesion outside the hepatic venous outflow track [3]

Summary

INTRODUCTION

Sarcoidosis is an inflammatory disorder categorized by the presence of noncaseating granulomas, which has the ability to affect multiple organs of the body [1, 2]. Budd–Chiari syndrome is a heterogeneous group of clinical conditions presenting with hepatic venous outflow obstruction, which can be located at any level from the small hepatic veins to the junction of the inferior vena cava with the right atrium [3]. It is classified as being primary if flow is obstructed due to an endoluminal aberration, or secondary if the flow is obstructed by compression or invasion of a lesion outside the hepatic venous outflow track [3]. The search for thrombophilia, namely dosage of protein C, protein S, antithrombin III, nocturnal paroxysmal hemoglobinuria, antiphospholipid syndrome, Leiden’s factor V mutation, and the prothrombin gene mutation, was negative

CONCLUSION

DISCUSSION