Crohn’s and Behçet’s Disease Association Presenting with Superior Vena Cava Thrombosis

Abstract

A 21-year-old male patient with a 6-year history of Crohn's disease presented with shoulder pain and edema in the neck and bilateral supraclavicular fossa. Color Doppler ultrasonography and computed tomography scan revealed bilateral thrombosis of the external jugular veins, brachiocephalic veins, and superior vena cava. Other etiologies were appropriately ruled out and the thrombosis was initially attributed to Crohn's disease. In the follow-up, the patient developed recurrent, painful orogenital ulcers and acne vulgaris-like skin eruptions. HLA tissue typing was HLA B-51. The diagnosis of Crohn's disease associated with Behçet's disease was made. Although there are several case reports describing such an association, a Medline research revealed that this was the first presentation of such an association with thrombosis. Thrombosis in unusual sites may reflect the uncommon association of Crohn's disease with hypercoagulable conditions such as Behçet's disease.

Thromboembolism in Inflammatory Bowel Disease: An Insidious Association Requiring a High Degree of Vigilance

“…Unusual sites of venous thrombosis in IBD include cerebral sinus, [28][29][30] retinal vein, [31][32][33][34] portal venous system, 10,[35][36][37] vena cava, [38][39][40] and hepatic vein. [41][42][43][44] Clinical articles on abdominal venous and arterial thromboembolism were systematically reviewed in 2009.…”

Thrombosen der Vena cava inferior – Ursachen und therapeutische Implikationen

“…Am häufigsten kommen Thrombosen der unteren Extremitäten und Viszeralvenenthrombosen vor; es sind aber vereinzelt auch Thrombosen der Vena cava superior bzw. inferior bei CED-Patienten beschrieben worden [21][22][23]. Neben der entzündlichen Aktivität tragen Dehydratation, Infektionen, zentralvenöse Zugangswege, Immobilität, Hospitalisierung und operative Eingriffe zum erhöhten Thromboserisiko bei.…”

Intra-Abdominal Venous and Arterial Thromboembolism in Inflammatory Bowel Disease

“…72 Thrombosis of the superior vena cava may occasionally manifest as superior vena cava syndrome, with severe reduction of venous return from the head, neck, and upper extremities. 62 Two cases of thrombosis of the superior vena cava and its tributaries have been described in patients with CD. 62,63 Diagnosis of thrombosis of the vena cava and hepatic vein may be achieved on the basis of CT scan and color Doppler ultrasound imaging.…”

“…62 Two cases of thrombosis of the superior vena cava and its tributaries have been described in patients with CD. 62,63 Diagnosis of thrombosis of the vena cava and hepatic vein may be achieved on the basis of CT scan and color Doppler ultrasound imaging. For Budd-Chiari syndrome, magnetic resonance, percutaneous transhepatic venography, and liver biopsy may help confirm the diagnosis.…”

“…66 Most of the authors described resolution of the thrombosis with systemic anticoagulant therapy. 58,62,63,67,71,74 Further therapeutic options reported in patients with IBD were: surgical thrombectomy/embolectomy for isolated inferior vena cava thrombosis in a patient with UC, 72 and mesoatrial-shunt from the SMV to the right atrium in a patient with UC with Budd-Chiari syndrome and thrombotic occlusion of the inferior vena cava. 70 Report of a patient with UC with acute Budd-Chiari syndrome, portal, and splenic vein thrombosis that required liver transplantation was found during the literature search.…”