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Rivista Italiana di Chirurgia Maxillo-Facciale 2001 December;12(3):187-93

Copyright © 2002 EDIZIONI MINERVA MEDICA

language: English

Surgical treatment of facial dysmorphism in a patient with Werdnig-Hoffmann spinal muscular atrophy

Polastri F., Asperio P., De Gioanni P. P., Daneo M., Margaglia E., Austa M.

From the Asti Civil Hospital Maxillo-Facial Surgery Division Maxillo-Facial Surgeon School of Specialization in Maxillo-Facial Surgery Turin University, Turin, Italy


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The ­case pre­sent­ed ­here is that of a ­patient suf­fer­ing ­from Type IIIa Werdnig-Hoffmann atro­phy, ­with seri­ous ­and inval­i­dat­ing ­facial dys­mor­phism ­that ­caused dif­fi­cul­ty in pho­na­tion, mas­ti­ca­tion ­and res­pi­ra­tion, ­but ­with no par­tic­u­lar psy­cho­log­i­cal defi­cien­cy. In ­this ­type of ­patient, improve­ment of ­the sto­mat­og­nath­ic ­system ­and relat­ed func­tions is ­vital ­for ­social rein­ser­tion. The ­patient pre­sent­ed at ­age 20 ­years ­with ­the spe­cif­ic ­request ­for ­improved mas­ti­ca­tion ­and ­speech. The seri­ous­ness of ­the mal­for­ma­tion ­made nec­es­sary a ­two-­stage orthognathodontic sur­gi­cal oper­a­tion ­with ­two dif­fer­ent tech­niques: ­the ­first ­stage of ­the oper­a­tion ­entailed short­en­ing ­the man­dible ­through a mod­i­fied Digman tech­nique (extrac­tion of ­the ­first infe­ri­or ­molars). The sec­ond sur­gi­cal ­stage con­sist­ed of a Le Fort I oste­ot­o­my of ­the max­il­la ­with pos­te­ri­or bilat­er­al impact­ing ­and man­dib­u­lar retrac­tion ­after Obwegeser osteotomy. The plan­ning ­and exe­cu­tion phas­es ­are illus­trat­ed togeth­er ­with ­the ­results ­achieved. The ­case pre­sent­ed ­may ­appear to be an ­extreme ­case in ­which sur­gery ­may be ­seen as exces­sive, con­sid­er­ing ­the ­subject’s ­life pros­pects. In ­our opin­ion, ­though, ­even hand­i­capped ­patients ­must be eval­u­at­ed ­from ­the cephal­o­met­ric stand­point ­and if nec­es­sary sub­ject­ed to sur­gery of ­the orthog­nath­o­log­i­cal ­type, to cor­rect anom­a­lies of ­the ­facial skele­ton. In ­our ­view a cor­rect ther­a­peu­tic ­approach ­may ­improve ­the qual­ity of ­life ­for ­such per­sons ­and ­enable ­social inclu­sion ­that ­would oth­er­wise be com­pro­mised.

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