RESUMO
Malignant peripheral nerve sheath tumors are extremely rare tumors arising in peripheral nerves. Only 17 cases involving the trigeminal nerve have ever been reported. These tumors have a very poor prognosis and very high rates of recurrence and metastases. Their recommended treatment involves complete tumor resection followed by radiation. This can be problematic in the head and neck region. We present a clinical case involving a 33-year-old female patient presenting with a slow-growing, exophytic mass of the anterior maxilla. Incisional biopsy and subsequent histological examination revealed a diagnosis of a malignant peripheral nerve sheath tumor. Surgical resection involved a complete maxillectomy, rhinectomy, and resection of the upper lip and aspects of the left and right cheeks. Reconstruction of the subsequent defect incorporated the placement of four zygomatic oncology implants to aid in retention of a facial prosthesis. These implants, however, were subsequently lost; and an anatomical model of the hard tissues was manufactured via 3D printing. This model was used to design and manufacture a titanium frame (customized implant) for the patient. The frame was then fixated and secured intraoperatively with 21 cortical screws. A maxillary denture and silicone facial prosthesis were also made to fit onto this frame. This is the first known case where additive manufacturing, via the use of rapid prototyping and 3D printing, was employed to manufacture a facial prosthesis.
Assuntos
Modelos Anatômicos , Neoplasias de Bainha Neural/cirurgia , Impressão Tridimensional , Desenho de Prótese , Adulto , Bochecha , Feminino , Humanos , Lábio/cirurgia , Maxila/cirurgiaRESUMO
The aim of this article is to publish a literature review and report on two new cases of Gordon syndrome (GS), a rare syndrome documented to have an autosomal dominant inheritance pattern or to occur sporadically; it is characterized by camptodactyly, cleft palate, and talipes equinovarus. We report two exceptional cases of GS where both patients were also diagnosed with congenital myopathy, and one developed malignant hyperthermia. These are the first two cases reported where patients were diagnosed with both GS and congenital myopathy or where GS is associated with malignant hyperthermia.
Assuntos
Artrogripose/diagnóstico , Artrogripose/cirurgia , Fissura Palatina/diagnóstico , Fissura Palatina/cirurgia , Pé Torto Equinovaro/diagnóstico , Pé Torto Equinovaro/cirurgia , Deformidades Congênitas da Mão/diagnóstico , Deformidades Congênitas da Mão/cirurgia , Artrogripose/genética , Fissura Palatina/genética , Pé Torto Equinovaro/genética , Diagnóstico Diferencial , Feminino , Deformidades Congênitas da Mão/genética , Humanos , Lactente , Recém-NascidoRESUMO
We describe an extremely rare type of isolated cleft of the hard palate, which extends the published classification of the Type III cleft.
