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Journal of Neurosurgical Sciences 2001 June;45(2):83-91


lingua: Inglese

Surgical treatment of moyamoya disease in pediatric age

Caldarelli M., Di Rocco C., Caglini P.

Institute of Neurosurgery, Section of Pediatric Neurosurgery, Catholic University Medical School, Rome, Italy


Background. Moyamoya is a pro­gres­sive occlu­sive cereb­ro­vas­cu­lar dis­ease ­which ­affects the dis­tal inter­nal carot­id ­artery and its ­main branch­es, and is char­ac­ter­ized, ­from a clin­i­cal ­point of ­view, by a varie­ty of ischem­ic man­i­fes­ta­tions rang­ing ­from tran­sient ischem­ic ­attacks (TIA) to ­stroke. Although con­sid­ered ­until recent­ly as ­almost spe­cif­ic of Japanese pop­u­la­tion, it can ­also ­affect indi­vid­u­als of dif­fer­ent ori­gin, and ­there is an increas­ing inter­est for ­this ­rare dis­ease in Western med­i­cal lit­er­a­ture. Nonetheless ­papers deal­ing ­with moy­a­moya dis­ease in pedi­at­ric age are ­still ­very ­rare and ­almost exclu­sive­ly lim­it­ed to iso­lat­ed ­case ­reports. These con­sid­er­a­tions prompt­ed us to ­review our expe­ri­ence ­with the diag­no­sis and treat­ment of ­such unusu­al dis­ease.
Methods. Nine chil­dren (seven ­months to nine ­years of age) pre­sent­ed ­with clin­i­cal man­i­fes­ta­tions of occlu­sive cereb­ro­vas­cu­lar dis­ease (TIAs or ­stroke), epi­lep­sy, and/or psy­cho­mo­tor retar­da­tion. Neuroradiological inves­ti­ga­tion, and in par­tic­u­lar cere­bral angio­gra­phy, dem­on­strat­ed the clas­si­cal pat­tern of moy­a­moya dis­ease. Eight of ­these chil­dren under­went cere­bral revas­cu­lar­iza­tion uti­liz­ing the enceph­a­lom­yo­sy­nan­gio­sis (EMS) tech­nique, ­with a ­total of 14 oper­a­tions (six on ­both ­sides, two on one ­side).
Results. There was no mor­tal­ity nei­ther ­major mor­bid­ity relat­ed to sur­gery. One ­patient ­with ren­o­vas­cu­lar hyper­ten­sion, ­died at ­home approx­i­mate­ly six ­months ­after a suc­cess­ful oper­a­tion, prob­ably ­from cere­bral hem­or­rhage. In all the cas­es post­op­er­a­tive angio­gra­phy ­revealed a ­marked improve­ment in cere­bral cir­cu­la­tion. However, in ­spite of the appar­ent­ly nor­mal­ized cere­bral ­blood ­flow, clin­i­cal man­i­fes­ta­tions ­were affect­ed ­less sig­nif­i­cant­ly. In ­fact, ­there was ­only min­i­mal if any improve­ment in chil­dren oper­at­ed on ­with an ­already estab­lished hem­i­ple­gia; where­as improve­ment in ­motor abil­ity was ­obtained in ­those pre­sent­ing ­with hem­i­par­e­sis or TIAs, as ­well as in epi­lep­sy and psy­cho­mo­tor retar­da­tion.
Conclusions. On the ­grounds of our ­results we con­clude ­that cere­bral revas­cu­lar­iza­tion (by ­means of a sim­ple oper­a­tion ­like the EMS) in chil­dren ­with moy­a­moya dis­ease is worth­while, pro­vid­ed ­that the oper­a­tion be per­formed ­before irre­ver­sible cere­bral dam­age is estab­lished; how­ev­er, ­even ­under ­these cir­cum­stanc­es the oper­a­tion can at ­least ­stop the pro­gres­sion of the dis­ease.

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