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עמוד בית
Thu, 18.07.24

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February 2007
S. Nitecki, A. Bass

Background: Klippel-Trenaunay syndrome, a congenital disorder, is characterized by capillary malformation, varicosities and bony or soft tissue hypertrophy. Since there is no cure for this syndrome, treatment is directed towards secondary prevention of venous hypertension and preservation of functional integrity of the legs. Elastic stockings are the mainstay of treatment and are indicated in all cases. Surgery is reserved only for a few selected symptomatic patients, however the outcome is unsatisfactory in most cases, with recurrent pain, edema, poor cosmetic result and limb deformity. Ultrasound-guided foam sclerotherapy is a recently introduced minimally invasive ambulatory procedure for the treatment of chronic venous insufficiency. It was recently introduced to treat this disorder.

Objectives: To evaluate the efficacy of USFS[1] in the treatment of patients with Klippel-Trenaunay syndrome.

Methods: Seven patients diagnosed with Klippel-Trenaunay, with massive lower extremity involvement, were treated with USFS between October 2003 and October 2005. Sclerovein® (polidocanol, Resinag, Switzerland) 2–4% was used as the sclerosant. The signs, symptoms and overall patient satisfaction were assessed before, during and after the treatment.

Results: Patients' mean age was 26 years (range 15–54). The CEAP[2] clinical classification, with ascending severity ranging from 0 (no signs) to 6 (active venous ulcer), was C4 in 5 patients (71.5%) and C5 and C6 in one patient each. The average number of sessions was 14.5 (range 9–21). No major complications were encountered. All seven patients reported improvement in signs and symptoms. Five of the 7 patients (71%) were very satisfied with the cosmetic result.

Conclusion: USFS is an effective minimally invasive ambulatory technique, essentially pain-free and with excellent short-term results in patients with Klippel-Trenaunay syndrome (when the deep system is functional). Long-term results and larger study groups are warranted. 

[1] USFS = ultrasound-guided foam sclerotherapy

[2] CEAP = Clinical, Etiology, Anatomic, Pathophysiology

March 2004
S.S. Nitecki, A. Ofer, T. Karram, H. Schwartz, A. Engel and A. Hoffman

Background: Arterial involvement in Behçet's syndrome is rare. Aneurysms are common among the arterial lesions, affecting various arteries but mostly the abdominal aorta. Surgical interposition graft insertion is the treatment of choice for large aneurysms. However, vasculitis in these patients is the reason for the notorious surgical complications that result in up to 50% false aneurysms in anastomotic sites. Recently, endovascular repair for abdominal aortic aneurysms has been established.

Objectives: To learn more about vascular Behçet and, specifically, to compare the results of surgical treatment and endovascular repair of AAA[1] in patients with Behçet's syndrome.

Methods: We retrieved the medical records of all 53 patients with Behçet disease admitted to Rambam Medical Center during the years 1985 and 2001 and analysed the results and follow-up of open surgery versus endovascular repair of AAA in patients with known Behçet's syndrome.

Results: Of the 53 patients with Behçet's disease 18 had vascular manifestations (34%). AAAs were encountered in 8 patients (15%) and 5 were treated. Open surgery (group 1), under general anesthesia, lasted less than 3 hours with an average aortic clamping time of 34 minutes (range 26–41 min) after which the patients were transferred to the intensive care unit for 24–48 hours. Endovascular treatment (group 2), although lasting about the same time without the need for intensive care, necessitated contrast media and fluoroscopy. The length of hospital stay was considerably shorter for patients after endovascular repair compared to open surgery (3 days vs. 6 days). Combined mortality and morbidity was higher in patients who underwent open surgery compared to endovascular repair (one death, one major amputation and three anastomotic pseudoaneurysms compared to one temporary contrast-induced nephropathy).

Conclusions: Vasculo-Behçet patients with AAA are better candidates for endovascular treatment than atherosclerotic patients. Combined morbidity (especially anastomotic pseudoaneurysms) and mortality of Behçet patients after endovascular repair is considerably lower than after open surgery.

[1] AAA = abdominal aortic aneurysm

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