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ITALIAN JOURNAL OF MAXILLOFACIAL SURGERY
A Journal on Maxillofacial Surgery
Official Journal of the Italian Society of Maxillofacial Surgery
Rivista Italiana di Chirurgia Maxillo-Facciale 2001 December;12(3):187-93
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
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.