An Overview of Retinoblastoma and Enucleation in Pediatric Patients.

Retinoblastoma is a rare malignancy of the eye affecting children, most commonly four years old and younger. Although chemotherapy and radiation treatment aim to spare the eye, in some cases, enucleation (ie, removal of the eye) is required to prevent cancer metastases or recurrence. Enucleation procedures are primarily performed at specialty institutions and may involve the surgical placement of an implant in the orbit of the eye. Unique perioperative considerations are required because of the age of the child at the time of the diagnosis and procedure and the involvement of a parent or caregiver who will be caring for the child postoperatively. This article presents an overview of retinoblastoma and enucleation and discusses the care and management of the unique patient population undergoing enucleation.

Manejo multidisciplinario en retinoblastoma: experiencia en 37 ojos / Multidisciplinary management of retinoblastoma: Experience in 37 eyes

OBJETIVOS: Reportar los resultados del manejo multidisciplinario de pacientes con retinoblastoma, incluyendo supervivencia global, tasa de enucleación y éxito de la quimiorreducción sistémica. MÉTODOS: Estudio retrospectivo 27 pacientes (37 ojos) con diagnóstico de retinoblastoma, tratados por un equipo multidisciplinario en el Hospital San Juan de Dios. Se incluyeron información demográfica, características clínicas, supervivencia, tratamiento local y sistémico. Se realizó además un subanálisis de tratamiento con quimioterapia intraarterial (QIA). RESULTADOS: Catorce pacientes (52%) fueron de sexo masculino. La mediana de edad al ingreso fue de 8 meses (0,16-90). La mediana ± desviación estándar de seguimiento fue de 33 ± 21 meses. Diez casos (37%) se diagnosticaron después de los 15 meses de edad, con una mediana de 35 meses (24-90). En 17 (63%) pacientes fueron retinoblastomas unilaterales, mientras 10 (37%) tuvieron retinoblastoma bilateral. La leucocoria, aislada o asociada a otros signos, fue el motivo de consulta más frecuente (63%). La tasa global de enucleación fue del 57% (n = 21), siendo el tratamiento primario en 15 (55%) pacientes. La tasa de enucleación en retinoblastoma unilateral fue del 76,5% y en retinoblastoma bilateral del 60% de un ojo y el 10% de ambos. Diecisiete (63%) pacientes recibieron quimioterapia sistémica (media de ciclos: 5,3 ± 2,1). El éxito global de la quimiorreducción sistémica y la terapia focal fue del 68%. Tres pacientes fueron tratados con QIA como terapia de rescate, logrando controlar el tumor en 2 pacientes a 6 meses de seguimiento, siendo los primeros casos en Chile. La supervivencia fue del 100%. CONCLUSIÓN: El manejo multidisciplinario del retinoblastoma permite una supervivencia y una morbilidad comparable con la literatura internacional

OBJECTIVE: To report the results of the multidisciplinary management of patients with retinoblastoma, including survival, enucleation rate, and systemic chemoreduction success. METHODS: A retrospective study was conducted on 27 patients (37 eyes) diagnosed with retinoblastoma, and treated by a multidisciplinary team in San Juan de Dios Hospital. Demographic information, clinical characterization, survival, local and systemic treatments were included in the analysis. Patients treated with intra-arterial chemotherapy (IAC) were also reviewed. RESULTS: The study included14 male patients (52%). The median of age at presentation was 8 months (0.16-90). The median follow-up time was 33 ± 21 months. The diagnosis was made in 10 (37%) cases after 15 months old, with a median of 35 months (24-90). 17 (63%) patients had unilateral retinoblastoma, and 10 (37%) bilateral retinoblastoma. Leukocoria, isolated or associated with other signs, was the most frequent reason for referral (63%). Global enucleation rate was 57% (n = 21), being the primary treatment in 15 (55%) patients. Enucleation rate in unilateral retinoblastoma was 76.5%, and for bilateral retinoblastoma, it was 60% for one eye and 10% for both. Systemic chemotherapy was prescribed in 17 (63%) patients, with a mean number of cycles of 5.3 ± 2.1. The overall success of chemoreduction and focal therapy in order to avoid external radiotherapy and/or enucleation was 68%. Three patients were treated with IAC as a salvage therapy, controlling the tumor in 2 patients at 6 months of follow-up. These are the first cases reported in Chile. Survival rate was 100%. CONCLUSION: Multidisciplinary management of retinoblastoma led to a survival rate and morbidity comparable with international reports

Undergoing enucleation of the eye. Part 2: postoperative care.

In this, the second of a 2-part article, the author discusses the postoperative care of patients who undergo enucleation of the eye. Part one explored aspects of preoperative care of enucleated patients. In the immediate period following surgery, patients require support and advice regarding pain, nausea and simple care of the eye socket. Apart from the intrinsic postoperative demands of the surgery, there are also the difficulties and challenges associated with providing psychological support and helping patients cope with the loss of an eye. Ultimately, the patient can overcome any initial problems and eventually be able to wear a custom-made prosthesis, which will hopefully allow the patient to move on with their lives. As in the first part, the author will use both referenced material and anecdotal references (upon personal reflection) to analyze the ongoing postoperative care and support of these patients.

Undergoing enucleation of the eye. Part 1: preoperative considerations.

Enucleation or evisceration of the eye is an uncommon but hugely life-changing surgical procedure for any patient. It may be necessary to remove the eye urgently due to trauma or tumour, or for some patients it may be the end of a long period of treatment and other surgical interventions that leads to removal of a blind, painful eye. No matter what the indications for surgery the care required to support and counsel these patients needs to start well before any surgical intervention. The author will outline and discuss some of the care implications associated with patients who undergo enucleation or evisceration surgery. Being an extensive topic the author has divided the subject into two articles. In this, the first of these articles, the author will discuss the preoperative considerations and in the second part the author will outline the postoperative care. The author works as an oculoplastic specialist nurse and has gained considerable experience in planning and facilitating the care for patients who have to undergo this surgery within a multidisciplinary team setting.

Providing compassionate care to the pediatric patient undergoing enucleation of the eye.

This article focuses on the care of the pediatric patient undergoing enucleation of the eye. It discusses the diagnoses related to the surgical procedure including blind, painful eye and retinoblastoma. It describes the nursing considerations such as impact on both patient and family, postoperative nursing care, emotional issues, teaching and follow-up required to best care for the patient undergoing this life-altering surgery.

Enucleación y evisceración ocular en el Hogar Clínica San Juan de Dios: Arequipa 1976-1996

Enucleación y evisceración ocular en el Hogar Clínica San Juan de Dios - Arequipa 1976-1996 es un trabajo retrospectivo longitudinal descriptivo para lo cual se revisó las historias clínicas de 40 pacientes sometidos a enucleación o evisceración en el período comprendido entre julio de 1976 a junio de 1996. Para la recolección de datos se confeccionó una ficha obteniéndose resultados que se registraron en cuadros de clasificación simple y distribución porcentual con aplicación de Ji cuadrado de acuerdo a objetivos. Se obtuvo los siguientes resultados: la causa principal de enucleación o evisceración fue retinoblastoma 12(30 por ciento) pacientes seguido de panoftalmitis 11(27.5 por ciento) pacientes, trauma ocular 7(17.5 por ciento) pacientes, glaucoma 4(10 por ciento) pacientes. Retino blastoma se presentó sólo en niños, panoftalmitis y trauma ocular casi similar en niños y adultos, glaucoma sólo en adultos. En retinoblastoma 9(75 por ciento) pacientes fueron del sexo femenino, en trauma ocular hubo 7(100 por ciento) pacientes varones, panoftalmitis se presentó en un porcentaje similar en hombres y mujeres encontrándose además a 3(75 por ciento) pacientes varones con el diagnóstico de glaucoma. La voloración de agudeza visual en ojo único sólo se realizó en 12(30 por ciento) pacientes. En historias clínicas de 30(75 por ciento) pacientes no hubo indicaciones al alta médica. Las patologías previscibles alcanzaron el 67.5 por ciento correspondiendo a panoftalmitis el mayor porcentaje (27.5 por ciento) el protocolo de patología ocular que llevó a enucleación o evisceración se esquematizó en un fluxograma de manejo condiderándose la patología encontrada en el presente trabajo no incluyéndose catarata complicada ni microftalmus por lo excepcional de éstas dos últimas patologías.

Hydroxyapatite eye implant.

Hydroxyapatite, a coral, provides an effective foundation for a prosthetic eye. With the implant, the prosthetic eye has a normal appearance, including movement. The infusion of blood into the implant prevents it from behaving as a foreign object in the patient's body.

Hydroxyapatite implants--a new trend in enucleation and orbital reconstructive surgery.

As treatment modalities evolve in ophthalmology one must assess and re-evaluate current methods of therapy in the care and treatment of the patient facing the loss of an eye. With the advent of Hydroxyapatite Orbital Implants, new challenges face the ophthalmic nurse in caring for the patient undergoing enucleation and reconstructive surgery. Integrated orbital implants are used to replace the volume of the orbit when the eye is removed due to enucleation, evisceration, or as secondary orbital implants. Various materials have been used in the past to replace orbital volume, with the most common being silicone or polymethylmethacrylate. Hydroxyapatite is a new orbital implant material which has unique interconnected porous matrix derived from marine corals with a mineral composition similar to bone. This orbital implant undergoes fibrovascular ingrowth by the patient's own tissue, becoming truly integrated and less likely to reject, migrate or extrude. The hydroxyapatite implant may be inserted with a variety of surgical techniques, all of which are more complex than standard enucleation implant techniques and have special considerations for the ophthalmic registered nurse.

Enucleation and the hydroxyapatite orbital implant.

Hydroxyapatite is a natural, porous material that allows the recipient's blood vessels and fibrous tissues to grow onto the implant, thus becoming a "living" portion of the orbital tissues. Natural eye movement, resistance to extrusions, rare complications, and flexibility in fitting the socket are the reasons oculoplastic surgeons are using the hydroxyapatite implant. Hydroxyapatite spheres are contraindicated in situations where other types of orbital implants are contraindicated; ie, severe trauma with possible orbital infection orbits that have poor vascularization and healing qualities, such as after irradiation, and with orbital infection.

Retinoblastoma.

Retinoblastoma, although a rare childhood cancer, is the most common primary malignant intraocular tumor of infancy and early childhood. In 75% of the cases, retinoblastoma is unilateral, and in 25% of the cases, retinoblastoma is bilateral. Presenting signs and symptoms of retinoblastoma, genetics, diagnostic tests, and their implications are reviewed in this paper. Treatment options for retinoblastoma (enucleation, external beam radiation, radioactive plaque, cryotherapy, photocoagulation, and chemotherapy) and their nursing care are discussed. Finally, second tumors such as osteosarcomas are discussed.