Document Detail

Multiple intracranial aneurysms and moyamoya disease associated with microcephalic osteodysplastic primordial dwarfism type II: surgical considerations.
MedLine Citation:
PMID:  19877776     Owner:  NLM     Status:  MEDLINE    
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 moyamoya 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 corridors and diminutive arteries in these patients, but is technically feasible and strongly indicated when multiple aneurysms must be treated or cerebral revascularization is needed.
James S Waldron; Steven W Hetts; Jennifer Armstrong-Wells; Christopher F Dowd; Heather J Fullerton; Nalin Gupta; Michael T Lawton
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of neurosurgery. Pediatrics     Volume:  4     ISSN:  1933-0715     ISO Abbreviation:  -     Publication Date:  2009 Nov 
Date Detail:
Created Date:  2009-11-02     Completed Date:  2009-11-17     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101463759     Medline TA:  J Neurosurg Pediatr     Country:  United States    
Other Details:
Languages:  eng     Pagination:  439-44     Citation Subset:  IM    
Department of Neurological Surgery, University of California, San Francisco, California 94143-0112, USA.
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MeSH Terms
Abnormalities, Multiple
Anastomosis, Surgical
Brain / pathology
Cerebral Angiography
Dwarfism / complications,  pathology,  surgery*
Intracranial Aneurysm / complications,  pathology,  surgery*
Magnetic Resonance Angiography
Microcephaly / complications,  pathology,  surgery*
Moyamoya Disease / complications,  pathology,  surgery*
Neurosurgical Procedures*
Subarachnoid Hemorrhage / surgery
Tomography, X-Ray Computed
Treatment Outcome

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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