Atypical Clinical Presentation of Crohn's Disease with Superior Mesenteric Vein Obstruction and Protein-losing Enteropathy

Abstract

We herein report a 44-year-old man suffering from systemic edema due to protein-losing enteropathy (PLE) with superior mesenteric vein (SMV) obstruction and development of collateral veins, which subsequently proved to be a chronic result of thrombosis and a complication of Crohn's disease (CD). PLE was supposedly induced by both intestinal erosion and thrombosis-related lymphangiectasia, which was histologically proven in his surgically-resected ileal stenosis. Elemental diet and anti-TNFα agent improved his hypoalbuminemia after surgery. The rarity of the simultaneous coexistence of SMV obstruction and PLE and the precedence of these complications over typical abdominal symptoms of CD made the clinical course complex.

Polybacterial Iliopsoas Muscle Abscess as an Indication for Early Diagnosis of Crohn\'s Disease

“…Several cases of CD with atypical presentations have been reported: a case with poor systemic and gastrointestinal signs for CD other than fever [ 18 ], cases with lesions located in only the large intestine [ 19 , 20 ], and cases with atypical or rare extra-gastrointestinal signs of CD [ 21 – 25 ]. When lesions are located in only the large intestine, it can be difficult to distinguish CD from ulcerative colitis [ 19 ].…”

“…The clinical manifestations of a CD case localized to the appendix are similar to those of acute appendicitis, and a diagnosis of CD is possible only by histopathological examinations [ 20 ]. The reported atypical or rare extra-gastrointestinal signs of CD are as follows: complication of superior mesenteric vein obstruction and protein-losing enteropathy [ 21 ]; thrombosis in the portal vein and splenic vein, with diffuse abdominal lymphadenopathy requiring differentiation from malignant lymphoma [ 22 ]; acute intermittent porphyria [ 23 ]; and presacral abscess [ 24 , 25 ]. The present case was an atypical case of CD because of the lack of systemic and gastrointestinal signs other than fever.…”

Endovascular Stenting for Idiopathic Stenosis of the Superior Mesenteric Vein: A Case Report

“…Idiopathic SMV stenosis, where no clear causative factor is identifiable, remains a clinical rarity, with only one case reported in the literature [ 11 ]. The absence of predisposing factors such as a hypercoagulable state [ 12 , 13 ], malignancy [ 3 , 14 , 15 , 16 , 17 , 18 ], or inflammatory disease [ 19 , 20 , 21 ] in idiopathic cases necessitates a thorough diagnostic approach to exclude other potential causes.…”