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Multiple intracranial aneurysms and moyamoya disease associated with microcephalic osteodysplastic primordial dwarfism type II: surgical considerations.
J Neurosurg Pediatr 2009; 4(5):439-44JN

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 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.

Authors+Show Affiliations

Department of Neurological Surgery, University of California, San Francisco, California 94143-0112, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

19877776

Citation

Waldron, James S., et al. "Multiple Intracranial Aneurysms and Moyamoya Disease Associated With Microcephalic Osteodysplastic Primordial Dwarfism Type II: Surgical Considerations." Journal of Neurosurgery. Pediatrics, vol. 4, no. 5, 2009, pp. 439-44.
Waldron JS, Hetts SW, Armstrong-Wells J, et al. Multiple intracranial aneurysms and moyamoya disease associated with microcephalic osteodysplastic primordial dwarfism type II: surgical considerations. J Neurosurg Pediatr. 2009;4(5):439-44.
Waldron, J. S., Hetts, S. W., Armstrong-Wells, J., Dowd, C. F., Fullerton, H. J., Gupta, N., & Lawton, M. T. (2009). Multiple intracranial aneurysms and moyamoya disease associated with microcephalic osteodysplastic primordial dwarfism type II: surgical considerations. Journal of Neurosurgery. Pediatrics, 4(5), pp. 439-44. doi:10.3171/2009.6.PEDS08137.
Waldron JS, et al. Multiple Intracranial Aneurysms and Moyamoya Disease Associated With Microcephalic Osteodysplastic Primordial Dwarfism Type II: Surgical Considerations. J Neurosurg Pediatr. 2009;4(5):439-44. PubMed PMID: 19877776.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Multiple intracranial aneurysms and moyamoya disease associated with microcephalic osteodysplastic primordial dwarfism type II: surgical considerations. AU - Waldron,James S, AU - Hetts,Steven W, AU - Armstrong-Wells,Jennifer, AU - Dowd,Christopher F, AU - Fullerton,Heather J, AU - Gupta,Nalin, AU - Lawton,Michael T, PY - 2009/11/3/entrez PY - 2009/11/3/pubmed PY - 2009/11/18/medline SP - 439 EP - 44 JF - Journal of neurosurgery. Pediatrics JO - J Neurosurg Pediatr VL - 4 IS - 5 N2 - 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. SN - 1933-0715 UR - https://www.unboundmedicine.com/medline/citation/19877776/Multiple_intracranial_aneurysms_and_moyamoya_disease_associated_with_microcephalic_osteodysplastic_primordial_dwarfism_type_II:_surgical_considerations_ L2 - https://thejns.org/doi/10.3171/2009.6.PEDS08137 DB - PRIME DP - Unbound Medicine ER -