Introduction: Moyamoya disease (MMD) is a progressive occlusive disease of the cerebral vasculature with particular involvement of the circle of Willis. The purpose of this study was to study the clinical profile and effectiveness of surgery in patients of Moyamoya disease.
Methods: The demographic profile, the clinical symptomatology was recorded. Diagnosis was confirmed by CT/ CTAngiography, MRI/ MR angiography and /or DSA. SPECT was done to assess brain perfusion. The effectiveness of revascularization procedures was studied in terms of improvement in clinical profile, angiographic changes, and neuropsychological assessment.
Results: 28 patients (32 sides) of Moya-moya disease have been operated. The mean age was 13.7 years. There were 3 adult patients whose presentation was with ICH. Rest all were children whose presentation was ischemia. Seizures were present in 12 patients (57.1%), focal deficit in 18 (85.7%), aphasia in2 (9.5%) and features of raised intra cranial pressure in 1 patient (4.7%) . Patients were investigated by DSA or MRA. SPECT studies were performed to detect areas of hypo-perfusion. STA-MCA anastomosis was done/attempted in all patients. The clinical progression of the disease was halted in almost all patients. One patient developed an additional ischemic event during surgery. Improvement in neuropsychological status was also observed especially in terms of increase in attention span and improved scholastic performance in school. Radiology showed regression in Moya Moya vessels in majority of the patients.
Conclusions: Surgical revascularization procedures were effective in halting the neurological progression of the disease and also resulted in neuropsychological and radiological improvement.
Patient Care: This will help in analyzing the impact of surgery in improving the neurological, radiological and neuropsychological picture of patients with Moyamoya disease
Learning Objectives: By the conclusion of this session, participants should be able to describe the effective of direct surgical revascularization in patients having Moyamoya disease