Given the diagnosis of BCS, a TIPS procedure was performed with a resultant decrease of portal pressure gradient from 17 to 4 mm Hg. The patient's postprocedural hospital stay was uneventful, and he was eventually discharged on lifelong anticoagulation.
BCS is a rare disease that is well described in patients with underlying myeloproliferative disease, malignancy, and hypercoagulable states. Our diagnosis of BCS was based on (i) clinical evidence of postsinusoidal portal hypertension in the absence of cardiac or pericardial disease, (ii) hepatic venography with an occlusion of the middle and left hepatic vein, and (iii) a liver biopsy showing central vein occlusion and sinusoidal congestion and hemorrhage. A diagnosis of hepatic sarcoidosis was established via non-necrotizing granulomas seen on the liver biopsy with negative periodic acid-Schiff, Grocott methenamine silver, and alpha-fetoprotein stains as well as an elevated angiotensin-converting enzyme level of 97 U/L that is described to have a specificity of almost 90%.6 The proposed theory that associates BCS and hepatic sarcoidosis is that granulomas can lead to hepatic veins stenosis, resulting in venous stasis and extensive thrombotic occlusion.7
NRH may develop as a result of an underlying autoimmune disease such as hepatic sarcoidosis, and it was also described in few cases with BCS.3–5,8 To our knowledge, our patient is the first documented case presenting with BCS with a simultaneous diagnosis of hepatic sarcoidosis and NRH. We hypothesize that in our case, sarcoidosis was the primary pathology that led to BCS and portal hypertension with NRH likely a pathological consequence of hepatic sarcoidosis. Another possible contributing factor is the hypercoagulable state because workup showed that our patient had a mildly low functional protein C and antithrombin level although the levels of antithrombin are likely falsely positive, given that this value was obtained while the patient was on heparin infusion.9
Reviewing the literature, there are 8 case reports of adult patients diagnosed with hepatic sarcoidosis who were complicated by BCS.2,7,10–15 Of the 8 cases, 2 were treated surgically with a portosystemic shunt, 2 were treated with liver transplantation, 2 were treated with steroids and anticoagulation, 1 was treated with steroids alone, and 1 with an unstated course of treatment.2,7,10-15 Our case is the first documented case of an adult with BCS associated with hepatic sarcoidosis that was treated with a TIPS. Furthermore, 2 pediatric cases of BCS were described to be associated with hepatic sarcoidosis; 1 was treated with corticosteroids, whereas the other was initially treated with a TIPS, complicated by recurrent thrombosis which eventually required liver transplantation.16,17
BCS is often managed by restoring hepatic venous drainage, anticoagulation, treatment of portal hypertension complications, and treatment of the predisposing condition. If all fails, then liver transplantation is the last resort.1 On the other hand, hepatic sarcoidosis often does not require treatment except if symptomatic. Treatment usually consists of steroids, ursodeoxycholic acid, or immunosuppressive agents, and in rare cases, liver transplantation is warranted.18 To conclude, to our knowledge, we present the first case of BCS to be dually associated with hepatic sarcoidosis and NRH, which was successfully managed with a TIPS. Our case highlights the utility of the TIPS procedure in treating patients with BCS in general and those with a simultaneous diagnosis of sarcoidosis in particular.
Author contributions: T. Odah wrote the manuscript and is the article guarantor. A. Al-Khazraji and R. Idriss edited the manuscript. M. Morrow provided the pathological images and wrote the captions. M. Curry approved the final version.
Financial disclosure: None to report.
Informed consent was obtained for this case report.
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