Incidence and pathogenesis of transient cheiro-oral syndrome after surgical revascularization for moyamoya disease.

BACKGROUND

There are no reports that denote transient cheiro-oral syndrome (COS) after surgical revascularization for moyamoya disease.

OBJECTIVE

To clarify the incidence and pathogenesis of transient COS after surgical revascularization for moyamoya disease.

METHODS

This study included 21 patients who underwent superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis and indirect bypass because of Moyamoya disease. Their medical records were evaluated to identify clinical features of postoperative transient COS. The findings on MRI, magnetic resonance angiography, and single-photon emission computed tomography were also analyzed.

RESULTS

Transient COS developed in 8 (22.9%) of 35 operated hemispheres, or in 6 (28.6%) of 21 patients between 3 and 20 days after surgery. Most of the COS were associated with mild weakness of the ipsilateral face and hand. Simultaneous radiological studies detected no findings of cerebral infarct or postoperative hyperperfusion. STA-MCA anastomosis was patent in all patients. However, their disease stage more frequently progressed owing to considerable blood flow via STA-MCA anastomosis, and basal moyamoya vessels disappeared or diminished in patients with transient COS rather than in those without.

CONCLUSION

Transient COS after surgical revascularization for moyamoya disease is not rare. Bypass flow through STA-MCA anastomosis may stimulate a rapid progression of disease stage and diminish basal moyamoya vessels, causing transient COS within 3 weeks after surgery.