Objectives Bilateral ocular ischaemic syndrome has rarely been reported as the initial presenting symptom of Takayasu arteritis (TA). To report the medical and surgical treatment of a Takayasu disease associated occlusion of left subclavian artery, brachiocephalic trunk and left carotid artery.
Methods A 16-year old lady with no known systemic illness presented to the ophthalmology department of a tertiary hospital in the locality with the complaints of an insidious onset of gradually progressive visual loss since 1 week. She also complained of giddiness, fainting and tinnitus. A general physical examination revealed feeble upper limb pulses and pulseless in the bilateral carotid artery. There was no audible murmur. Other systems were normal. On admission, her white blood cell count was 16,340/mm3, platelets 411 × 109/L, haemoglobin 12.2 gm/dL, her erythrocyte sedimentation rate was 13 mm/h, and CRP 3.21 mg/L. The carotid and vertebral arteries’ Doppler showed bilateral carotid artery occlusion with normal vertebral arteries, and computed tomography of her head did not demonstrate any abnormalities. Computed tomography Angiography (CTA) and digital subtraction Angiography (DSA) demonstrated occlusion of the proximal segment of the left subclavian artery, brachiocephalic trunk and left carotid artery as well right vertebral artery stenosis.
Results Methylprednisolone (2 mg·kg-1·d-1) was administered intravenously the day after admission to suppress inflammatory arteritis. The patient received ascending aorto-bilateral carotid artery bypass on 5 Sep 2011, Postoperative steroid and immunosuppressive therapy were administered for this patient because of her erythrocyte sedimentation rate was 45 mm/h. Postoperative CTA one week after the procedure revealed good patency of the bypass grafts. The patient was followed up after 18 month, Her symptoms were gradually improved with a decrease in ESR and CRP.
Conclusions the pathogenesis of TA remains to be elucidated, It is now hypothesised that an unknown stimulus triggers the expression of the 65 kDa Heat-shock protein in the aortic tissue and induces the Major Histocompatibility Class I Chain-Related A (MICA) on vascular cells, The γδ T cells and NK cells play a more important role of this pathways . The brachiocephalic trunk is the most frequently involved site in TA, and multi-vessel lesions are common. Surgery should be avoided in the acute phase, for it was associated with a high morbidity of complication and reconstructions occluded . Bypass operation is optimal for brachiocephalic-vessel involvement in TA . Postoperative steroid therapy is strongly recommended for the patient associated with clinical and serological signs of an active phase. In conclusion, a combination of medical and surgical treatment is necessary for optimal management of TA.