[Moyamoya disease among children. An analysis of a series in the Western world and literature review].

PATIENTS AND

METHODS

A total of 12 patients with moyamoya disease or syndrome with a mean age of 6 years were analyzed in a retrospective fashion.

RESULTS

Infarction was the most frequent presentation feature (8 out of 12 patients), 2 had epilepsy and 2 were incidental findings. Seven cases were classified as idiopathic while the other five were related to systemic illnesses. Diagnosis was initially made by magnetic resonance angiography in 9 cases, and conventional angiography in 3 cases. Mean follow-up is 5 years; six patients experienced clinical worsening of symptoms, while 6 cases remained clinically stable. However, all of them showed angiographic progression. Four patients underwent revascularization surgery. Two children died due to complications associated with moyamoya disease, and six have moderate handicaps.

CONCLUSION

Due to the fact moyamoya disease is not an indolent disorder and readily progress to cause complications, surgical revascularization should always be considered in the management of these patients.