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ITALIAN JOURNAL OF MAXILLOFACIAL SURGERY

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Rivista Italiana di Chirurgia Maxillo-Facciale 2002 April;13(1):45-9

language: English

Mandibular distraction osteogenesis using external devices in the Pierre Robin sequence. Report of a case and review of the literature

Bertolini F., Brevi B., Baj A., Bianchi B., Sesenna E.

Chair ­and Division of Maxillo-Facial Surgery, General Hospital, Parma


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The Pierre Robin Sequence (­PRS) is an anom­a­ly con­sist­ing of microg­nath­ia ­with glos­sop­to­sis, res­pir­a­to­ry obstruc­tion, ­and con­se­quent feed­ing dif­fi­cul­ties. It ­can be ­present as an iso­lat­ed anom­a­ly or ­may rep­re­sent ­part of a syn­drome. Patients ­with ­PRS ­may ­have res­pir­a­to­ry obstruc­tion short­ly ­after ­birth or dur­ing ­the fol­low­ing ­weeks. These symp­toms grad­u­al­ly sub­side as man­dib­u­lar ­growth ­and ­improved con­trol of ­the ­tongue mus­cu­la­ture devel­op. The ­goal of treat­ment is to ­avoid per­ma­nent trach­e­os­to­my ­and gav­age feed­ing. Many con­ser­va­tive and sur­gi­cal ther­a­peu­tic modal­ities ­have ­been pro­posed. Mild cas­es ­are ­often man­aged con­ser­va­tive­ly by plac­ing ­the lit­tle ­patient in an adapt­ed ­bed, hold­ing ­the ­head in ­true ­prone posi­tion ­and/or ­using gav­age feed­ing ­when ­oral feed­ing is ­not tol­er­at­ed. However, ­when ­severe res­pir­a­to­ry dis­tress ­ensues ­despite posi­tion­al treat­ment, sur­gi­cal inter­ven­tion to ­relieve ­the obstruc­tion is man­da­to­ry. The tech­nique of dis­trac­tion in ­adult ­patients ­has ­been mod­i­fied in ­infants ­with ­upper air­way obstruc­tion ­and obstruc­tive ­sleep ­apnea, ­such as in ­the ­PRS, to per­mit an imme­di­ate sag­it­tal advance­ment of ­the man­dible ­and ­the ­tongue. We ­present ­the ­case of a 3-­month-­old ­patient affect­ed by ­PRS ­who under­went exter­nal man­dib­u­lar dis­trac­tion oste­o­gen­e­sis to ­treat ­the ­upper air­way obstruc­tion ­with a ­good func­tion­al out­come.

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