Case Report Refractory Hepatic Hydrothorax: A Rare Complication of Systemic Sclerosis and Presinusoidal Portal Hypertension Gary A. Abrams , 1 Robert Chapman, 1 and Samuel R. W. Horton 2 1 Gastroenterology & Liver Center, Greenville Health System, University of South Carolina School of Medicine Greenville, Greenville, SC, USA 2 Department of Pathology, Greenville Health System, University of South Carolina School of Medicine Greenville, Greenville, SC, USA Correspondence should be addressed to Gary A. Abrams; gabrams@ghs.org Received 19 February 2018; Accepted 22 March 2018; Published 30 April 2018 Academic Editor: Julio M. F. Chebli Copyright © 2018 Gary A. Abrams et al. Tis is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. We report on a rare case of refractory hepatic hydrothorax in an individual with Scleroderma/CREST syndrome and noncirrhotic portal hypertension. Portal pressure measurements revealed a normal transjugular hepatic venous portal pressure gradient, mild pulmonary hypertension, and an unremarkable liver biopsy except for mild sinusoidal dilation. Pulmonary hypertension, cardiac diastolic dysfunction, and chronic kidney disease were determined to be the causes of his refractory pleural efusions and ascites. Over the year, he underwent 50 thoracenteses and 20 paracenteses averaging 10–12 liters/week. Repeat pulmonary evaluation determined his pulmonary pressures to be normal and a secondary review of the “unremarkable” liver biopsy noted mild venous outfow obstruction and possibly Nodular Regenerative Hyperplasia (NRH). Repeat portal pressures indirectly and directly confrmed the existence of presinusoidal portal hypertension that has been associated with NRH. A transjugular intrahepatic portal systemic shunt (TIPS) was placed and he has not required thoracentesis or paracentesis over the past 18 months. 1. Introduction Idiopathic noncirrhotic portal hypertension (INCPH) has many etiologies, but a common denominator is vascular resistance at various locations that include the intrahepatic sinusoidal and presinusoidal as well as extrahepatic portal and hepatic veins [1]. Schistosomiasis is a common worldwide illness and the most frequent cause of INCPH [2]. Nodular Regenerative Hyperplasia (NRH) was frst described in 1959 [3]; however this is a histological diagnosis that can ofen be overlooked. We describe a complicated rare case of refrac- tory right-sided pleural efusions and ascites due to NRH and presinusoidal portal hypertension that was successfully treated with a transjugular intrahepatic portal systemic shunt (TIPS). 2. Case Report A 59-year-old Caucasian male was referred to our liver center for refractory right-sided pleural efusion and abdominal ascites. His history is signifcant for Scleroderma/CREST syndrome and chronic kidney disease (CKD). He had 12 paracenteses in 2015 and starting from February 2016 was undergoing thoracentesis 3 times weekly (about 8-9 liters/week) and a single weekly paracentesis up to 5 liters. Medications included Spironolactone 50 mg and Furosemide 20 mg, which were limited dosages due to CKD. In February 2016, prior to our visit, he underwent a liver transplant evaluation: Na 138 mg/dL, Cr 4.4 mg/dL, eGFR 31 mL/min, INR 1.0, Hb 13 g/dL, Platelets 342 T/mm 3 , TB 0.5 g/dL, AlkPhos 278 IU/L, ALT/AST 46/40 IU/L, albumin 2.7 mg/dL, negative viral serology, ANA 1 : 320, SMA and AMA neg- ative, C282Y/H63D, and MELD-Na score 14. Abdominal ultrasound revealed a heterogeneous liver and ascites. A thoracentesis demonstrated a SAAG 1.9 and total protein 3.3 gm/dL suggesting posthepatic portal hypertension. A right heart catheterization was notable for RA 5mmHg, PA 31/15 mm/Hg, mean 22 mmHg, normal Echo LV function, and grade 1 diastolic dysfunction. At this juncture, the etiology of his presumed cirrhosis had not been determined and a liver biopsy with portal pressures was to be scheduled but he wanted a second opinion and presented to us in March. Hindawi Case Reports in Hepatology Volume 2018, Article ID 2704949, 4 pages https://doi.org/10.1155/2018/2704949