Abstract
Microcephalic osteodysplastic primordial dwarfism type II (MOPD II) is a rare genetic syndrome characterized by extremely small Stature and microcephaly, and is associated in 25% of patients with intracranial aneurysms and moyamova disease. Although aneurysmal subarachnoid hemorrhage and stroke are leading causes of morbidity and death in these patients. MOPD II is rarely examined in the neurosurgical literature. The authors report their experience with 3 patients who presented with MOPD II, which includes a patient with 8 aneurysms (the most aneurysms reported In the literature). and the first report of a patient with both moyamoya disease and multiple aneurysms. The poor natural history of these lesions Indicates aggressive microsurgical and/or endovascular therapy Microsurgery, whether for aneurysm clip placement or extracranial-intracranial bypass. is challenging due to tight surgical corridois and diminutive arteries in these patients. but is technically feasible and strongly indicated when multiple aneurysms must be treated or cerebral revascularization is needed (DOI. 10.3171/2009.6.PEDS08137)