Rehabilitation of pediatric retinoblastoma patients with ocular prostheses and their subsequent modifications: A 15-year retrospective study.

PURPOSE: Enucleation is a common treatment modality performed for pediatric retinoblastoma patients, and the resultant defects are reconstructed using an ocular prosthesis. The prostheses are modified or replaced periodically, as the child develops due to orbital growth and patient-error. The purpose of this report is to evaluate the replacement frequency of prostheses in the pediatric oncologic population. METHODS: A retrospective review was completed by the two senior research investigators, of patients that had ocular prostheses fabricated following enucleation of their retinoblastoma from 2005 to 2019 (n = 90). Data collected from the medical records of the patient included the pathology, date of surgery, date of prosthesis delivery, and the replacement schedule of the ocular prosthesis. RESULTS: During the 15-year study period, 78 enucleated observations (ocular prosthesis fabricated) were included for analysis. The median age of the patients at the time of delivery of their first ocular prosthesis was calculated to be 2.6 years (range 0.3-18 years). The median time to the first modification of the prosthesis was calculated to be 6 months. The time to modification of the ocular prosthesis was further stratified by age. CONCLUSION: Pediatric patients require modification of their ocular prostheses throughout their growth and development period. Ocular prostheses are reliable prostheses with predictable outcomes. This data is helpful to set an expectation among the patient, parent, and provider.

Participation in an occupational therapy referral program for children with retinoblastoma.

PURPOSE: Because retinoblastoma typically arises at a very young age, children are particularly vulnerable to vision impairment, associated developmental delays, and functional limitations. Limited information is available describing developmental delay and functional limitations in this population, necessitating supportive services including rehabilitation. The aims of this study were to describe the participation of children with newly diagnosed retinoblastoma in an occupational therapy program that identifies children in need of rehabilitation services. We also identify indications for referral to rehabilitation services among children with newly diagnosed retinoblastoma and enumerate the likelihood of these children receiving the recommended services. METHODS: Twenty-two children participated in longitudinal occupational therapy assessments during the first year after diagnosis. RESULTS: We recommended 1 or more types of rehabilitation services for 16 of 22 (72.7%) participants. Twelve of 16 (75%) received services. CONCLUSIONS: The results of this pilot study indicate that implementing a prospective occupational therapy-screening program is feasible and results in identification and initiation of therapy services in some children with retinoblastoma. Developmental screenings and follow-up of children with retinoblastoma is strongly recommended.

[Retinoblastoma: inventory in Mali and program to develop early diagnosis, treatments and rehabilitation]. / Le rétinoblastome: état des lieux au Mali et programme d'aide au diagnostic précoce, aux traitements et à la réhabilitation.

Retinoblastoma (RB) is a rare embryonic tumour that represents 1/16,000 births in France. In Mali, a study showed the characteristics of a hospital series of cases seen in Bamako in the Pediatric Oncology Unit of Gabriel Touré Teaching Hospital and in the Tropical Ophthalmology Institute of Africa (IOTA) between January 2005 and June 2007. Median age was 4 years versus 2 years in France for unilateral disease. Near two third of children with RB had extra-ocular extension at diagnosis, which is now exceptional in France. Only 11% were bilateral versus 35% in France. Cure rate was around 50%, but it is estimated only on the cases arriving in Bamako and with at least 20% lost of follow-up. Cure rate is over 95% in France within an exhaustive register. RB appears as an exemplary tumor and rapid improvements could be obtained in low-income countries with relatively limited means. This is why, the Alliance mondiale contre le cancer (AMCC), the Institut Curie in Paris, which is the reference center in France for RB, and teams in Bamako were proposing a program to help the development of early diagnosis, treatments, including eye preservation, and rehabilitation of children with RB in sub-Saharan Africa in collaboration with the Groupe franco-africain d'oncologie pédiatrique (GFAOP). The official launching of this program was held in Bamako November 4, 2011 for Mali and the surrounding regions. After this first experience, this program is now implemented in other countries.

Rehabilitation after surgical treatment for retinoblastoma: ocular prosthesis for a 6-month-old child.

Maxillofacial prosthetic (MFP) rehabilitation can be especially challenging in a young, precooperative, or behaviorally compromised child presenting with an enucleated eye. Retinoblastoma is the most common intraocular malignancy in childhood and is one of the most common pediatric cancers. Treatment consists of enucleation (or removal of the entire globe) followed by placement of orbital implants. Unrestored anopthalmic sockets exhibit growth retardation and can lead to facial disfigurement. This report describes the challenges faced during rehabilitation of a 6-month-old girl with an anophthalmic socket due to enucleation for retinoblastoma. The objective of the MFP team was to provide a custom-built, acrylic ocular prosthesis in as comfortable and atraumatic manner as possible. The case was a success and underscores the value of a multidisciplinary dental approach for the treatment of children with very special needs.

Behavioural functioning of retinoblastoma survivors.

OBJECTIVE: To assess behavioural problems in retinoblastoma (RB) survivors. METHODS: This population-based cross-sectional study included 148 RB survivors (8-35 years), registered in the Dutch national RB register. Survivors and parents were asked to fill in behavioural questionnaires. Prevalence rates were computed, based on both self-reports and proxy reports. One-sample T-tests were applied to analyse differences compared with healthy reference samples. Multiple regression analyses were performed to identify predictors for behavioural problems within the RB sample. RESULTS: Between-group differences varied across informants and across age groups. Parents reported significantly elevated total problem behaviour in 30% of their offspring (aged 8-17 years); this against 9% in adolescents (12-17 years) and 12% in adults (18-35 years) based on self-report. Parental reports showed significantly elevated rates of (1) internalising problems in boys and (2) somatic complaints in both girls and boys. Self-reports indicate significantly lowered levels of (1) externalising problems in adolescent and adult women and (2) thought problems in female adolescents and in adult men. Especially survivors who suffered hereditary RB, who had undergone more intensive treatment, and who came from a single-parent family were identified to be at most behavioural risk. CONCLUSION: Perception of severity and the nature of behavioural problems seem to differ between beholder, and to vary between age groups, if not between life stages. Health professionals should be aware that especially those who are confronted with hereditary RB and who subsequently undergo intensive treatment, and who grow up in broken families, run the risk of developing behavioural difficulties.

Maxillofacial prosthodontics for the pediatric patient: "an eye-opening experience".

The following case report describes the expanding role of pediatric dentists in treating children with craniofacial pathology. Retinoblastoma is the most common intraocular malignancy in childhood and is approximately the tenth most common pediatric cancer in the United States. Treatment consists of enucleation, or removal of the entire globe followed by placement of orbital implants. Un-restored anopthalmic sockets exhibit growth retardation and can lead to facial disfigurement. Maxillofacial prosthetic (MFP) rehabilitation can be especially challenging in younger, pre-cooperative or behaviorally compromised children and requires the skills and participation of a pediatric dental specialist as part of the MFP team. The following case report involving a 3 yr-old girl with retinoblastoma describes such challenges. The objective of the maxillofacial prosthetic team was to provide custom-built, acrylic, bilateral ocular prostheses in as comfortable and atraumatic manner as possible. The case was a success and underscores the value of a multidisciplinary dental approach for the treatment of children with very special needs.

Mothers' perceptions of children's quality of life following early diagnosis and treatment for retinoblastoma (Rb).

We describe the Quality of Life (QoL) and IQ of survivors of retinoblastoma (Rb), both in relation to the normal population and between subgroups of Rb patients differing in relative risk (i.e. unilateral vs. bilateral disease). The sample included 54 children (28 males, age-range 8-16 years) and their mothers. Mothers completed standardized questionnaires to report their own QoL and that of their child. Children completed a brief IQ test. Compared with population norms, mothers reported lower levels of QoL for their child on total QoL and for sub-scales measuring Physical and Psychosocial function. Mothers reported their own QoL to be comparable or higher than norms on all but one of eight sub-scales (energy/vitality). Compared with population norms, children with no visual impairment scored in the normal range for tasks measuring Verbal IQ, but below the mean on tasks measuring Performance IQ. According to their mothers, survivors of Rb have excellent school attendance and take part in most school activities. However, based on standardized questionnaire, they show compromised QoL. We consider that excellent survival rates in Rb are matched with good QoL according to mothers' report.

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

[Bilateral rehabilitation with an implant-anchored orbital prosthesis]. / Doppelseitige Rehabilitation mit einer implantatfixierten Orbitaepithese.

From the aesthetic point of view, a patient can be completely rehabilitated after the loss of an eye with the insertion of an artificial eye made of glass. If the delicate structures of the eyelids have been severely damaged, however, or if the eye socket does not provide adequate retention for an eye prosthesis, rehabilitation becomes more difficult, and sometimes cannot be achieved with a cosmetically satisfactory result. In such cases, a facial prosthesis offers an alternative solution. Stable retention can be ensured with craniofacial implants. Single-sided rehabilitation by this method is quite common, but bilateral treatment is a rarity. We report on the prosthetic rehabilitation of a patient after exenteration on both sides due to retinoblastomas.