The impact of monocular vision on motor function and quality of life in survivors of retinoblastoma.

BACKGROUND: Monocular vision has been found to have a negative effect on children's motion processing and motor functions. Yet, knowledge of motor function of survivors of retinoblastoma (RB) with monocular vision (due to enucleation, for example) is limited. This study examined motor function and its relationship to visual-related and health-related quality of life (HRQOL) in survivors of RB with monocular vision. PROCEDURE: Parents of 27 survivors of RB, who underwent an enucleation of one eye resulting in monocular vision, and of 21 typically developing children between the ages of 6 and 12, were administered questionnaires relating to their children's motor function (DCDQ), as well as vision-related function (CVFQ) and HRQOL (PedsQL). RESULTS: Of the 27 survivors of RB, 7 (25.6%) were found to have difficulties in motor functions, compared with 1 (4.8%) child in the control group. The difficulties were faced mainly in daily function requiring control during movement, including jumping, running, and ball playing. Additionally, significant correlations were found between motor functions and children's QOL. Finally, survivors of RB with monocular vision were found to have lower QOL, specifically physical- and school-related QOL. CONCLUSION: Survivors of RB who have monocular vision have a higher rate of decreased motor function and lower QOL. These results point to a need for ongoing assessment of survivors of RB to allow timely detection of motor deficits and to institute appropriate therapeutic interventions.

[ADVANCES IN THE TREATMENT OF RETINOBLASTOMA AT HADASSAH IN THE LAST THREE DECADES].

INTRODUCTION: Retinoblastoma (RB) is a malignant tumor presenting in the eyes of infants and children, which endangers life, the eye and vision. The treatment of RB has undergone marked changes in recent years, and great progress has been made in our ability to preserve eyes. Over the last three decades most Israeli patients with RB have been treated in the National Specialty Ocular Oncology Service at the Hadassah-Hebrew University Medical Center in Jerusalem. AIMS: To describe advances in the primary treatment of RB with an emphasis on eye-preserving treatments. METHODS: The study included a retrospective cohort of patients who were diagnosed and treated at our center over the last three decades. Review of patients' records was approved by the Hadassah IRB. RESULTS: From 1988 to 2014 we diagnosed 290 children (138 girls - 47.6%). The mean age at diagnosis (±SE) was 18.1±1.2 months, median 12.5 months. RB was unilateral in 55.6% of the cases, bilateral in 41.3% and unilateral multifocal in 3.1%. There was an even distribution of disease severity (IRB grouping). Since the advent of IV chemotherapy (IVC) there has been a decrease in the rate of eye enucleation from ~90% to ~30% of the children until the year 2000 with a stable rate thereafter. In the years 1990-2000 there was an increase followed by a decrease in the use of primary external beam radiotherapy (EBRT), and a parallel small increase in the use of brachytherapy from the mid '90s until today. The recently introduced novel treatments - intravitreal (IVitC) and intra-arterial chemotherapy (IAC) - were used as a complimentary treatment to IVC, and not yet as a single primary modality until 2014. CONCLUSIONS: IVC replaced the need to enucleate in most of the cases, but 30% of children still require a primary enucleation. DISCUSSION: IVC usually requires additive treatments (thermal-cryotherapy, trans-pupillary thermotherapy - TTT, brachytherapy and/or local chemotherapy - IVitC and IAC) and with the use of multi-modal therapy many eyes can be preserved. In the period reported in the current manuscript, the use of IAC as a primary treatment approach was only used in isolated cases. In Summary, There have been significant advances in our ability to save eyes, and the field continues to progress.

The coping experience of parents of a child with retinoblastoma-malignant eye cancer.

The authors explored 12 couples' coping with their children's diagnosis and treatment of retinoblastoma using a semistructured interview, with qualitative, descriptive, narrative-interpretative analysis. Findings showed that the parents' experienced increased distress with the physician's first suspicion that something was seriously wrong. Distress was ameliorated when they arrived at a specialty treatment center but increased as they tackled treatment decisions. Distress decreased again after they consented to enucleation but increased after hospital discharge. The parents' strength, their ability together and individually, to separate and split between cognition and emotion contributed to coping. Parents need support from a multidisciplinary staff and parents who coped with retinoblastoma.

Participation in daily activities and quality of life in survivors of retinoblastoma.

PURPOSE: To assess the participation and health-related quality of life (HRQOL) of survivors of childhood retinoblastoma (RB). PATIENTS AND METHODS: Parents of 46 survivors of childhood RB between the ages of 2-18 were administered questionnaires relating to their children's participation (CFFS) and HRQOL (CHQ and PedsQL) and children were administered the PedsQL. Results of the HRQOL were compared to population-based norms. RESULTS: The overall QOL of survivors of RB was similar to that of age norms. However, parents' rating of their children's general and emotional health was lower than that of age norms, and survivors reported lower QOL related to school. Survivors of bilateral RB participated less in daily activities and had lower emotional QOL compared to those with unilateral RB, and parents of children who had an eye enucleated reported that their children had lower self-esteem. The level of participation was related to the perceived QOL. CONCLUSION: Our results indicate that children who are survivors of RB have an overall QOL that is similar to their age-peers. However, subgroups of survivors appear to have unique difficulties that require continued follow-up and intervention. Focus should be placed on their participation in daily activities both in the community and at school.