RESUMO
PURPOSE: Orbital exenteration is a disfiguring surgical procedure that requires thoughtful reconstruction. PATIENTS AND METHODS: We analyzed all cases of orbital exenteration done in our department since 1998. RESULTS: A total of 26 patients had undergone surgery during our study period, and 27 exenterations have been conducted since 1998. The reconstruction was performed in 20 patients using an ipsilateral temporal muscle flap. One patient underwent reconstruction using a temporalis fascia flap and 6 using free flaps. A total of 11 patients received postoperative radiotherapy. Data collection revealed 6 patients with an ocular prosthesis. The prosthesis had either been glued or mounted onto glasses. CONCLUSIONS: Analyzing our results and the published data, we found that the reconstruction mode influences prosthetic rehabilitation. Thus, we have identified 2 categories. In the first category, postoperative radiotherapy will be required and coverage with a flap will be needed. In the second category, postoperative radiotherapy is not indicated and thus the healing time might be extended in favor of more space for better retention of the prosthetic rehabilitation.
Assuntos
Neoplasias Faciais/cirurgia , Exenteração Orbitária/reabilitação , Procedimentos de Cirurgia Plástica/métodos , Neoplasias Cutâneas/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinoma Basocelular/cirurgia , Carcinoma de Células Escamosas/cirurgia , Olho Artificial , Neoplasias Palpebrais/cirurgia , Fáscia/transplante , Feminino , Seguimentos , Retalhos de Tecido Biológico/transplante , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/patologia , Neoplasias Orbitárias/secundário , Radioterapia Adjuvante , Estudos Retrospectivos , Músculo Temporal/transplante , Adulto JovemRESUMO
We report a family with a rare association of congenital bilateral cataract and cleft palate that has not to our knowledge been previously reported. The lineage has unveiled abnormalities over four generations affecting 21 people with congenital bilateral cataract, with or without cleft palate. The transmission seems autosomal dominant. Four brothers and sisters presented with this defect, and a fifth sister is healthy. The mother has facial dimorphism, congenital bilateral cataract, submucous cleft palate, clinodactyly, and scoliosis. The propositus' karyotype was normal. Array comparative genomic hybridisation (CGH) analysis showed an interstitial amplification in Xp21.1, found in the mother, in all the affected siblings but one, and in the healthy girl. Thisl association is not rare and has been reported in over 50 syndromes but rarely in familial observations. Based on the genetic-clinical discordance we wonder about the deleterious impact of the Xp21.1 amplification that might be a copy number polymorphism.
