Portal Vein Thrombosis in the Setting of Newly Diagnosed Cushing's Syndrome.

Alexandria D Mcdow,Carmel Fratianni,Michael G Jakoby,Anju Gurung,Rama Poola,Marc Garfinkel

doi:10.1177/2324709617703672

Abstract

The hallmark manifestations of Cushing’s syndrome (CS) are well known, but hypercoagulability is perhaps least recognized. Patients with CS are at increased risk of both spontaneous and postoperative thromboembolism, with the significant majority of events occurring in the lower extremity and pulmonary venous circulations. We present a case of portal vein thrombosis (PVT) occurring in the setting of newly diagnosed CS due to a left adrenal adenoma. Factor VIII activity was approximately 2.5-fold elevated, a known mechanism by which hypercortisolemia predisposes to venous thrombosis. Acute abdominal pain and fever responded well to unfractionated heparin and parenteral antibiotics, and CS was eventually cured by left adrenalectomy. No thromboembolic events have occurred since surgery. PVT is uncommon and usually occurs as a complication of primary or secondary hepatobiliary malignancies and cirrhosis. To the best of our knowledge, this is just the second reported case of PVT due to CS and the first published in the English language literature.

Highlights

Cushing’s syndrome (CS) is caused by chronic exposure to excess glucocorticoid, with endogenous CS due to elevated levels of cortisol
We present an unusual case of portal vein thrombosis (PVT) complicating CS caused by an adrenal adenoma
The combination of increased coagulation factors and decreased fibrinolytic capacity predisposes to venous thrombosis

Summary

Introduction

Cushing’s syndrome (CS) is caused by chronic exposure to excess glucocorticoid, with endogenous CS due to elevated levels of cortisol. Endogenous CS may be caused by corticotrope adenomas in the pituitary (Cushing’s disease), ectopic corticotropin (ACTH) production by peripheral tumors of neuroendocrine origin (eg, small cell carcinoma), or adrenal adenomas and carcinomas that autonomously overproduce cortisol. We present an unusual case of portal vein thrombosis (PVT) complicating CS caused by an adrenal adenoma. Neither cholescintigraphy nor right upper quadrant abdominal ultrasound showed evidence of cholecystitis, but the left portal vein was found to be occluded by ultrasonography. Abdominal CT revealed an incidental 3.8 × 3.2 cm left adrenal mass with radiographic features favoring adenoma including regular borders and homogeneous, low-density attenuation (−9 Houndsfield units) on noncontrast images (Figure 1B). PVT due to hypercoagulability caused by a cortisol hypersecreting left adrenal adenoma was diagnosed. Fever was felt to be a sign of pylephlebitis, so the patient was managed

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