S2444 Diagnosis of a Grave Disease in a Seemingly Asymptomatic Woman: Budd-Chiari With Portal Vein Thrombosis in Setting of Polycythemia Vera

Abstract

INTRODUCTION: Vascular disorder of the liver are rare in the general population with increased prevalence in patients with cirrhosis and MPD. Up to 50 % of patients with BCS have an associated MPD while 10–15% are classified as idiopathic. Coincidence of BCS and PVT has been reported in 10–15% cases but rarely documented as the presenting finding of PV in the setting of an extensive splanchnic thrombosis. CASE DESCRIPTION/METHODS: A 35-year-old female with a past medical history of one spontaneous miscarriage presented with 1 week of epigastric pain and early satiety. On examination she had marked hepatosplenomegaly with diffuse dull percussion note and shifting dullness. On presentation, the patient was hemodynamically stable. Labs showed ALT 810, AST 499, Tb9.4, Db7.1, ALP 272, IINR 2.6, Albumin 2.9, Hemoglobin/Hct 14.3/42. CT of abdomen/pelvis showed hepatosplenomegaly, markedly enlarged left lobe of the liver, and diffuse ascites suggestive of PVT (Figure 1). Patient was started on heparin however course was complicated by worsening of symptoms, repeat imaging two days afterwards showed extension of the clot to involve hepatic vein and SMV thrombosis extending into the IVC with concerns for early signs of bowel necrosis. Patient underwent an emergent successful extended TIPS, IVC stent, and thrombectomy/thrombolysis of SMV and portal vein with no complications post procedure (Figure 2). Work-up showed positive hypercoagulability workup for JAK2 21%, no further mutations detected. Patient underwent bone marrow biopsy which confirmed the diagnosis of PV. Her symptoms improved over the course of 10 days, was transitioned to oral anti-coagulant. DISCUSSION: Acute PVT can sometimes remain with non-specific symptoms however, the extension of thrombosis is usually what depicts the clinical progression, in addition to use of systemic anti-coagulation which is seldom used in chronic forms of PVT. Several studies showed that JAK2 mutation to be found in almost 35% of all splanchnic vein thrombosis forms, and in 41-59% of patients with BCS. However, it is uncommon to see PV in young patients <40 years of age, in whom only a minority will develop BCS. In our case, the symptoms were imperceptible, yet the diagnosis was severe, which raises the possibility of likely a chronic picture of portal vein thrombosis or BCS. It was extremely challenging given the unclear symptom presentation and critical radiographic findings to provide adequate therapy to control extent of thrombosis without thrombolysis and TIPS.Figure 1.: CT scan of the abdomen pelvis in portal venous phase. (A) Coronal view showing pre TIPS hyper-enhancement of the caudate lobe (red). Thrombus in portal vein (orange). Ascites (blue). (B) After TIPS and IVC placement showing heterogeneous liver nutmeg (black/0 and resolution of ascites (blue).Figure 2.: A) Hepatic venogram showing Classical spider-web appearance of Budd-Chiari (blue arrow). B) percutaneous access of splenic vein doing portal venogram showing occluded/thrombosed portal vein (red arrow). C) stent extending from IVC to PV connected via splenic vein catheterization. D) TIPS (blue arrow) and IVC stent (red arrow).