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

language: English

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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