Asunto(s)
Síndrome del Nevo Basocelular/genética , Adulto , Síndrome del Nevo Basocelular/diagnóstico por imagen , Síndrome del Nevo Basocelular/patología , Cromosomas Humanos Par 9/genética , Estudios de Seguimiento , Humanos , Cariotipificación , Masculino , Maloclusión/diagnóstico por imagen , Maloclusión/genética , Mandíbula/diagnóstico por imagen , Polimorfismo Genético/genética , Prognatismo/diagnóstico por imagen , Prognatismo/genética , Prognatismo/patología , Radiografía Panorámica , Piel/patologíaRESUMEN
PURPOSE: A competent velopharyngeal mechanism is essential for the production of intelligible speech and the secondary procedure of the posterior pharyngeal flap may be necessary in some patients to achieve this goal. The purpose of this study was to examine short and long-term results between the Sanvenero-Rosselli and Hogan's modification of the cranially based velopharyngoplasty. METHODS: During a 20-year period, 17 patients underwent secondarily a posterior pharyngeal flap to treat velopharyngeal incompetence. All the patients were treated in the same institution and under the supervision of the same stuff surgeon. Nine patients were operated on according to the Sanvenero-Rosselli method and eight according to Hogan's modification, which consists of the preparation of two mucosal flaps on the dorsal side of the soft palate. Both mucosal flaps are sutured at the base of the pharyngeal flap and cover its raw surface. RESULTS: Comparing the post-operative condition of the patients in two groups to their pre-operative state, it was observed that speech and comprehensibility were significantly improved, but Hogan's technique produced better speech results. CONCLUSIONS: According to our limited experience, additional surgical effort to create mucosal flaps on the nasal surface of the soft palate in Hogan's technique is compensated for by the reliable and substantial advantages that this technique holds, compared to Sanvenero-Rosselli's original proposition.
Asunto(s)
Procedimientos de Cirugía Plástica/métodos , Insuficiencia Velofaríngea/cirugía , Estudios de Seguimiento , Humanos , Paladar Blando/cirugía , Faringe/cirugía , Inteligibilidad del Habla/fisiología , Colgajos Quirúrgicos/cirugía , Resultado del TratamientoRESUMEN
INTRODUCTION: Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome, is an autosomal dominant inherited disorder which is characterized by the presence of multiple basal cell carcinomas, maxillary keratocysts, and musculoskeletal anomalies. CASE REPORT: We present a case of a patient suffering from Gorlin-Goltz syndrome who developed an intraosseous basal cell carcinoma associated with a recurrent maxillary keratocyst. To our knowledge, this is the first case of malignant transformation of a keratocyst into a basal cell carcinoma described in the literature. CONCLUSION: This case highlights the importance of careful histologic examination of keratocysts excised in patients suffering from Gorlin-Goltz syndrome.