Orbital exenteration: Institutional review of evolving trends in indications and rehabilitation techniques.

PURPOSE: To determine the changes in indications for orbital exenteration over 20 years and to assess its impact on patient survival. Evolving techniques of rehabilitation of the orbit in our institution were also evaluated. METHODS: This was a retrospective review of hospital records of patients who underwent orbital exenteration from 1995 to 2015 in a tertiary care center. Data extracted included primary location of the tumor, preoperative treatments, interval between initial diagnosis and exenteration, status of surgical margins, presence of metastatic disease, and postoperative survival. The types of prosthesis utilized over the years were also reviewed. Cox regression analysis was performed for categorical variables. Kaplan-Meier analysis was used to estimate post-exenteration survival. RESULTS: Over a 20-year period, orbital exenteration was performed on 100 orbits of 100 patients. The mean age was 39.4 years (range: 2 months to 90 years). The most common indications among 98 malignant causes were retinoblastoma, squamous cell carcinoma, basal cell carcinoma, extraocular extension of uveal melanoma, and conjunctival melanoma. Postoperative survival was significantly related to age and tumor location but independent from gender, surgical margin, histopathological diagnosis, previous treatment modality, and preoperative interval. In the whole cohort, 1-year and 5-year survival rates were 97% and 84%, respectively. CONCLUSIONS: Exenteration appears to be life-saving in children with orbital extension of retinoblastoma. While patients exenterated for malignant eyelid tumors have the best chance of survival, those with orbital extension of uveal melanoma and adenoid cystic carcinoma of the lacrimal gland have the worst prognosis.

Resultados del analisis genético de los pacientes con retinoblastoma resistente a quimioterapia sistémica / Genetic analysis results of patients with a retinoblastoma refractory to systemic chemotherapy

OBJETIVO: Analizar las alteraciones genéticas identificadas en el gen RB1 en los pacientes con retinoblastoma no respondedores a quimioterapia sistémica. MÉTODOS: Se seleccionó a un total de 115 pacientes con retinoblastoma con análisis genético realizado, 40 de los cuales habían recibido quimioterapia sistémica; 29 de ellos presentaban una enfermedad bilateral. Estudio descriptivo y retrospectivo. Consideramos no respondedores a los pacientes que finalmente son enucleados. RESULTADOS: Los pacientes con mutaciones del tipo deleción son los que presentan menor conservación del globo ocular (Chi-cuadrado de Pearson; p = 0,055). Los pacientes que presentan una alteración del tipononsense-frameshift tienen más posibilidades de conservar el globo ocular. De los 3 pacientes que han experimentado enucleación bilateral, 2 de ellos presentaban deleciones y uno alteración del tipomissense. El análisis de supervivencia (curva de Kaplan-Meier) evidencia que los pacientes con mutaciones del tipo deleción son los que presentan mayor resistencia a la quimioterapia, son los que sufren mayor tasa de enucleaciones y en un menor periodo de tiempo (Log Rank [Mantel-Cox] con nivel de significación de p = 0,053); son, además, los que se asocian con mayor tasa de bilateralidad. CONCLUSIONES: Los pacientes con un genotipo que presente mayor resistencia a la quimioterapia deben ser evaluados más estrechamente y tratados con varias armas terapéuticas de forma precoz. Los pacientes que presentan deleciones en el gen RB1 presentan mayor riesgo de quimiorresistencia. Es probable que otras alteraciones genéticas distintas de las del gen RB1 puedan tener relación con la agresividad del tumor o la resistencia al tratamiento

OBJECTIVE: To analyze the genetic alterations identified in the RB1 gene in retinoblastoma patients who do not respond to systemic chemotherapy. METHODS: A genetic analysis was performed on 115 patients with retinoblastoma, 40 of whom had received systemic chemotherapy, and 29 of them had bilateral disease. Descriptive and retrospective study. Non-responders were considered as patients who are finally enucleated. RESULTS: Patients with deletion type mutations are those with less preservation of the eyeball (Pearson Chi-square,P=.055). Patients with an impaired nonsense-frameshift type are more likely to preserve the eyeball. Of the 3 patients who had undergone bilateral enucleation, 2 of them had deletions and one missense alteration. Survival analysis (Kaplan-Meier curve) shows that patients with deletion type mutations are more resistance to chemotherapy, are suffering higher rates of enucleation, and for a shorter period of time (log rank [Mantel-Cox] with a significance level of P=.053), which are also associated with increased rate of being bilateral. CONCLUSIONS: Patients with a genotype show increased resistance to chemotherapy should be evaluated more closely and treated with various therapeutic weapons early. Patients that have deletions in the RB1 gene are at increased risk of chemoresistance. It is likely that other genetic alterations other than RB1 gene may be related to tumor aggressiveness and treatment resistance

A unique method of retaining orbital prosthesis with attachment systems - a clinical report.

Diminution of the orbital contents post-surgical removal of a malignant tumor can have a severe psychological impact on the patient in terms of function and esthetics. Therefore, esthetic remedy should be planned subsequently, since tumor obliteration precedes cosmetic concern. A convenient option for successful rehabilitation in such patients is a simple, user-friendly, removable orbital prosthesis. Retention of the prosthesis is one of the key factors for the successful rehabilitation. Spectacle frame, conformers, adhesives, osseointegrated implants, magnets or buttons have been used to impart retention to the prosthesis. The use of semi precision attachments in maxillofacial prostheses is limited to the osseointegrated prostheses. This case report describes a conventional spectacle frame technique, to retain the silicone orbital prosthesis using two different types of stud attachments viz., dalla bona and O-ring attachment systems.

Prosthetic rehabilitation of a patient with enucleated eye - a case report.

BACKGROUND: The loss of an eye has a crippling effect on the psychology of the patient, making rehabilitation process challenging for both doctor and patient. OBJECTIVE: To report a case of anophthalmic socket with prosthetic rehabilitation in a cost effective manner. CASE: A 32-year-old female presented with anophthalmic socket for prosthetic rehabilitation. A custom made ocular prosthesis was implanted successfully. CONCLUSION: The custom made ocular prosthesis is simple, affordable and can be carried out in a small clinical set-up and provides a superior natural appearance.

Surgical and prosthetic considerations to rehabilitate an ocular defect using extraoral implants: a clinical report.

This clinical report shows the use of extraoral implants to rehabilitate an ocular defect, focusing the surgical and prosthetic procedures. Using local anesthesia and a surgical template obtained from the diagnostic wax ocular pattern, two cylinder dental implants were strategically placed in the lateral aspect of the right infraorbital region. Four months later, an acrylic framework including two spherical magnets was made using plastic UCLA abutments. After casting laboratory steps, a customized silicon prosthetic appliance was fabricated from the diagnostic wax ocular pattern and attached to the Co-Cr framework, observing its profile and seating aspects. The patient was satisfied with the treatment result, due to the retention, esthetics, and adhesive-free method to anchor his ocular prostheses.

Multiple retentive means for prosthetic restoration of a large facial defect - a case report.

A 70-year-old man who suffered from extensive extra and intraoral defects was rehabilitated with a prosthesis using multiple retaining means. The treatment was performed in two parts: externally involving the construction of an episthesis supported only by the remaining intact boundaries of the defect and retained by mini-dental implants and spectacle frame with a modified ear hook; and intraorally by an acrylic resin obturator to restore the function of the hemi-sectioned hard and soft palate. The episthesis was securely retained with minimal movement and/or dislodgment of the prosthesis during function. Multiple retentive techniques may be used to fix heavy external prostheses as an alternative to conventional implants or biological adhesives.

Rehabilitación ocular de un niño de 2 años en el Centro de Rehabilitación de la Cara y Prótesis Bucomaxilofacial / A 2 years-old boy's ocular rehabilitation in the Face and Buccomaxillofacial Denture Rehabilitation Center

Se presenta el caso de un niño de 2 años, rehabilitado con prótesis ocular en el Centro de Rehabilitación de la Cara y Prótesis Bucomaxilofacial de la provincia Santiago de Cuba, donde fue operado y tratado con radioterapia por presentar retinoblastoma. La conducta terapéutica mejoró la apariencia física del paciente y contribuyó a su adecuado desarrollo biológico, psicológico y social

Rehabilitación ocular de un niño de 2 años en el Centro de Rehabilitación de la Cara y Prótesis Bucomaxilofacial / A 2 years-old boy's ocular rehabilitation in the Face and Buccomaxillofacial Denture Rehabilitation Center

Se presenta el caso de un niño de 2 años, rehabilitado con prótesis ocular en el Centro de Rehabilitación de la Cara y Prótesis Bucomaxilofacial de la provincia Santiago de Cuba, donde fue operado y tratado con radioterapia por presentar retinoblastoma. La conducta terapéutica mejoró la apariencia física del paciente y contribuyó a su adecuado desarrollo biológico, psicológico y social

Residual facial disfigurement after the ablative surgery of a lachrymal gland carcinoma: a clinical report of the prosthetic rehabilitation.

Facial disfigurement after ablative surgery of a massive adeno-carcinoma of the lachrymal gland is described. A rehabilitation with a maxillofacial prosthesis was proposed to restore the aesthetic appearance of the patient without inserting craniofacial implants. Retention of a maxillofacial prosthesis, that is not anchored to implants, depends on the use of adhesives or on mechanical devices like glasses. This clinic report describes a residual anatomic defect which allowed for the double choice of wearing the facial prosthesis both with or without glasses. A retentive backside of the prosthesis was developed to engage the facial defect undercuts, which enhanced retention when the patient used the skin glue without glasses. A unique foam silicone was utilized to reduce the weight of the prosthesis and to permit its retention only by skin adhesive.

Prótesis oculo-palpebral. A propósito de un caso clínico / Orbital prosthesis. A clinical case report

La prótesis maxilofacial comprende aquellos dispositivos artificiales que restauran una parte ausente de la cara, debido a tumores, traumatismos, o como resultado de una anomalía congénita. El objetivo de este artículo es la exposición de un caso clínico que precisaba la confección de una prótesis óculo-palpebral tras sufrir una pérdida de sustancia por causa oncológica. Este tipo de prótesis son de las más difíciles de elaborar con buenos resultados pues intentan reconstruir un órgano móvil con una prótesis estática. La prótesis maxilofacial tiene la finalidad de aumentar la calidad de vida del paciente y ayudar a la seguridad emocional de los mismos, quedando así rehabilitados para poder integrarse nuevamente en la sociedad (AU)

Computer-assisted maxillofacial prosthodontics: a new treatment protocol.

PURPOSE: This article presents a new treatment protocol in maxillofacial prosthodontics. MATERIALS AND METHODS: A case report of a patient suffering from an orbital defect after tumor resection is presented. To explain the treatment protocol, the procedure of fabricating an implant-retained orbital prosthesis for this patient is discussed. RESULTS: After tumor resection, implants were placed and a prosthesis was made based on both a CT scan and computer-supported technology. CONCLUSION: The integration of several existing techniques in and outside the field of maxillofacial prosthodontics led to a treatment protocol that was shown to be beneficial in the presented case and others.

Correction of the contracted eye socket and orbitozygomatic hypoplasia using postauricular skin flap and temporal fascial flap.

The authors corrected a contracted eye socket and orbitozygomatic hypoplasia simultaneously, secondary to previous surgery and radiotherapy. A one-stage surgical reconstruction was undertaken using both a postauricular skin flap and a temporal fascial flap, which were rotated for eye socket reconstruction and bone graft coverage. Hydroxyapatite was inserted as an onlay bone graft substitute to enlarge the orbitozygomatic region. The authors' experience with 12 patients resulted in contours that were satisfying and encouraging. f1

Intraocular cancers.

1. Retinoblastoma is the most common primary intraocular cancer of children. It occurs in unilateral and bilateral forms. 2. Approximately 40% of all cases are associated with a heritable defect in chromosome 13. 3. Malignant melanoma is the most common primary intraocular malignant tumor among adults. 4. Unlike retinoblastoma, primary intraocular malignant melanoma is almost exclusively a monocular, unifocal disease without hereditary transmission.

Flecks retiniano em linfome non-Hodgkin / Non-Hodgkin lymphoma and retinal flecks

Os autores relatam a presença de lesöes puntadas, brancas, sub-epitélio pigmentar da retina, em uma paciente portadora de linfoma non-Hodgkin. Chamam a atençäo para a associaçäo dos flecks esbranquiçados retinianos em pacientes com esta forma de linfoma