Rupture of the splenic arterial aneurysm due to Behçet's disease

E. Dolar, H. Uslusoy, M. Kiyici, S. Gurel, S. G. Nak, M. Gulten, A. Zorluoglu1, H. Saricaoglu2 and F. Memik

Departments of Gastroenterology, 1 General Surgery and 2 Dermatology, Uludag University Medical Faculty, Bursa, Turkey

Correspondence to: E. Dolar, Department of Gastroenterology, Medical Faculty, Uludag University, 16059 Bursa, Turkey. E-mail: mkiyici{at}uludag.edu.tr

SIR, We present a patient with Behçet's disease who developed intra-abdominal haemorrhage due to rupture of a splenic artery aneurysm. The patient underwent urgent open surgical intervention. Aneurysmectomy and splenectomy were performed successfully; the patient recovered completely and was discharged on the sixth postoperative day. Our case is the first splenic artery aneurysm and its rupture due to Behçet's disease in the literature.

Behçet's disease was described by Hulusi Behçet, a Turkish dermatologist. The aspects of the disease are oral and genital aphthous ulcers and iridocyclitis. But it is now evident that the disease affects many systems, such as the central nervous system, the eyes, the skin and the locomotor, gastrointestinal, cardiovascular and genitourinary systems [1]. The aetiology of the disease is not clear yet. The disease has been found to be associated with HLA-B51 [1]. The prevalence is high in the Far-Eastern and Mediterranean countries, such as Japan, China, Iran and Turkey.

Arterial complications represent a fatal course and thus are very important in Behçet's disease [2, 3]. The approach to the diagnosis and treatment of aneurysms in Behçet's disease has been discussed. Furthermore, cerebral, carotid, subclavian, aortic (thoracic and abdominal), pulmonary, brachial, ulnar, iliac, renal, femoral and popliteal arterial aneurysms have been reported [4]. The case presented here is the first reported case of splenic artery aneurysm and its rupture due to Behçet's disease in the literature.

On the second day of admission, the patient was diagnosed with acute abdomen secondary to rupture of the aneurysm. Urgent upper abdominal ultrasonography revealed a 15 x 5 x 5 cm haematoma between the spleen and portal hilus, adjacent to the aneurysm of the splenic artery. The patient had urgent surgical intervention, and aneurysmectomy plus splenectomy was performed. The patient was discharged uneventfully on the sixth postoperative day.

The patient was a 46-yr-old Turkish man who had an 11-yr old history of Behçet's disease with ocular and neurological involvement. On the admission day, epigastric tenderness was found by physical examination. Blood pressure was 120/80 mmHg, the pulse rate 80/min and fever 38.8°C. The first laboratory findings were as follows: white blood cells 15 000/mm3, haemoglobin 10.3 g/dl, haematocrit 30.3%, platelets 263 000/mm3, ESR 103 mm/h and CRP 143 mg/dl. Nine aphthous ulcers on the corpus and antral mucosa of the stomach were observed on gastroscopy. A 5 x 5 cm mass was detected near the spleen by abdominal ultrasonography. Abdominal CT (Fig. 1) and portal system Doppler ultrasonography revealed that the mass was an aneurysm of the splenic artery.



View larger version (108K):
[in this window]
[in a new window]
 
FIG. 1. CT scan demonstrating ruptured splenic artery aneurysm (white arrow).

 
The incidence of vascular complications in Behçet's disease has varies widely, from 8 to 60% [5]. Four different vascular complications have been described: arterial occlusion, arterial aneurysm, venous thrombosis and variceal formation [5, 6]. The basic mechanism in the pathogenesis of all vascular complications is ‘vasculitis’. A number of consecutive immunological processes, inflammatory cell infiltration, impairment of endothelial cell function and coagulation disorders result in thrombosis, leading to collateral venous circulation. Furthermore, vena cava superior occlusion leads to downhill oesophageal varices [5, 7].

Venous involvement is more frequent (15–25%) than arterial lesions (1.5–3%) [7, 8]. The prevalences of arterial aneurysms and arterial occlusions are similar to each other in most studies (1–2%) [4, 5, 8].

Splenic arterial aneurysms can occur in atherosclerosis, mycotic infection, blunt abdominal trauma, essential hypertension, portal hypertension, chronic pancreatitis, diabetes, polyarteritis nodosa, arterial dysplasia, pregnancy and liver transplant patients. These conditions must also be considered in the differential diagnosis.

Arterial aneurysmal rupture is the most common cause of death in Behçet's disease [3]. Rupture may occur in 60% of arterial aneurysms and has a varying range of fatal outcome [7]. According to Urayama et al., the mortality rate in patients with arterial aneurysm is about 60% [9]. Furthermore, 50% of patients with pulmonary artery aneurysm, which is the form occurring most often, die after 10 months of haemoptysis [10].

Routine examination for vascular involvement includes palpation of the peripheral arteries and superficial veins, detecting impaired arterial perfusion and looking for evidence of superficial thrombophlebitis or deep-vein thrombosis, such as superior or inferior vena cava occlusion. The presenting symptoms are abdominal pain, abdominal distension and bleeding into the peritoneum, intestine and pancreatic duct. Imaging studies for all vascular involvement are plain abdominal X-ray, ultrasonography, Doppler ultrasonography, computed tomography with contrast, magnetic resonance imaging, magnetic resonance angiography, nuclear scanning, and digital subtraction angiography and venography.

Medical treatment is mainly with immunosuppressive drugs (chlorambucil, cyclophosphamide, azathioprine and cyclosporin) and corticosteroids in suitable patients.

Surgical therapy is indicated where there are non-regressive occlusions despite sufficient medical therapy, or large and ruptured aneurysms, or lesions resistant to medical agents [3, 8]. Because of the considerable tendency to rupture, surgical treatment should not be delayed in large aneurysms. To our knowledge, our case is the first splenic artery aneurysm and its rupture caused by Behçet's disease reported in the literature. The patient was treated successfully and recovered completely.

Arterial aneurysms are the major causes of death in Behçet's disease. For this reason, patients with Behçet's disease require close monitoring and routine examination for vascular involvement.

The authors have declared no conflicts of interest.

References

  1. Yazici H, Yurdakul S, Hamuryudan V, Fresko I. The vasculitides: Behçet's syndrome. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, eds. Rheumatology, 3rd edn. Barcelona: Elsevier, 2003;1665–9.
  2. Tuzun H, Besirli K, Sayin A et al. Management of aneurysms in Behçet's syndrome: an analysis of 24 patients. Surgery 1997;121:150–6.[CrossRef][ISI][Medline]
  3. Park JH, Chung JW, Joh JH et al. Aortic and arterial aneurysms in Behçet's disease: management with stent-grafts–initial experience. Radiology 2001;220:745–50.[Abstract/Free Full Text]
  4. Ko GY, Byun JY, Choi BG, Cho SH. The vascular manifestations of Behçet's disease: angiographic and CT findings. Br J Radiol 2000;73:1270–4.[Abstract/Free Full Text]
  5. Sagdic K, Ozer ZG, Saba D, Ture M, Cengiz M. Venous lesions in Behçet's disease. Eur J Vasc Endovasc Surg 1996;11:437–40.[CrossRef][ISI][Medline]
  6. Gebitekin C, Yilmaz M, Senkaya I, Saba D, Sagdic K, Ozer G. Fatal haemoptysis due to pulmonary artery aneurysm in Behçet's disease. Eur J Vasc Endovasc Surg 1997;13:233–6.[CrossRef][ISI][Medline]
  7. Tohme A, Aoun N, El-Rassi B, Ghayad E. Vascular manifestations of Behçet's disease. Eighteen cases among 140 patients. Joint Bone Spine 2003;70:384–9.[CrossRef][ISI][Medline]
  8. Saba D, Saricaoglu H, Bayram AS et al. Arterial lesions in Behçet's disease. Vasa 2003;32:75–81.[ISI][Medline]
  9. Hassikou H, Bono W, Bahiri R, Abir S, Benomar M, Hassouni NH. Vascular involvement in Behçet's disease. Two case reports. Joint Bone Spine 2002;69:416–8.[CrossRef][ISI][Medline]
  10. Greene RM, Saleh A, Taylor AK et al. Non-invasive assessment of bleeding pulmonary artery aneurysms due to Behçet disease. Eur Radiol 1998;8:359–63.[CrossRef][ISI][Medline]
Accepted 20 May 2005





This Article
Full Text (PDF)
All Versions of this Article:
44/10/1327    most recent
keh725v1
Alert me when this article is cited
Alert me if a correction is posted
Services
Email this article to a friend
Similar articles in this journal
Similar articles in ISI Web of Science
Similar articles in PubMed
Alert me to new issues of the journal
Add to My Personal Archive
Download to citation manager
Disclaimer
Request Permissions
Google Scholar
Articles by Dolar, E.
Articles by Memik, F.
PubMed
PubMed Citation
Articles by Dolar, E.
Articles by Memik, F.
Related Collections
Other Rheumatology