Enucleation For Retinoblastoma Research Articles

Anophthalmic socket in retinoblastoma: Exploring complications and risk factors in a tertiary centre in Malaysia.

To evaluate the complications of anophthalmic socket in retinoblastoma patients at a tertiary centre in Malaysia. Retrospective study. Patients who underwent enucleation for retinoblastoma were reviewed from 2004-2020. Details were recorded, including demographics, diagnosis, surgical techniques, implant types, additional therapies, and complications. Of 250 patients with retinoblastoma managed over the period, the anophthalmic sockets of 160 eyes who underwent enucleation were analysed. The mean age at enucleation was 2.03 years (26 days to 9.18 years). The follow-up periods after enucleation range from 5 days to 16.83 years. Porous polyethylene (Medpor) implants were used in 135 patients (84.4%), as were Bioceramic in 9, glass balls in 7, acrylic in 7, dermis fat grafts in 1, and silicone implants (Aurosphere) in 1. The overall complications in our study were 28.8%. Complications seen in the study included implant exposure (12.5%), shallow inferior fornix (10.6%), granuloma formation (3.1%), discharge (2.5%), implant migration (1.9%), ptosis (0.6%), and orbital dystopia (0.6%). Implant exposure is solely found in Medpor, more common in those with donor sclera caps, and exposure times range from 28 days to 11.42 years. The suturing of the Tenon and conjunctiva in separate layers significantly reduced the rate of implant exposure. Six out of seven radiation patients had shallow inferior fornixes. Long-term post-enucleation complications were not uncommon. Luckily, most had good outcomes, with a few needing surgical intervention. Meticulous suturing technique on the Tenon and conjunctival layer is essential to prevent implant exposure.

A comparison of high risk pathological features between primary and secondary enucleation for retinoblastoma.

To compare the risk and pattern of High-Risk Pathologic Features (HRPF) in retinoblastoma between primary and secondary enucleation. A retrospective analysis of 121 eyes from 118 patients who underwent enucleation at the King Hussein Cancer Center (KHCC) Amman, Jordan, between November 2009 and January 2020. Demographic information, tumor stage, time from diagnosis-to-enucleation, results of pathology, metastasis, and mortality were retrieved. Patients in the secondary group (49/121 eyes, 40%) were considerably younger at diagnosis (p = 0.0014), had bilateral disease (p = 0.0001), and had less-progressed disease at presentation (p = 0.016) compared to the primary enucleation. Primarily enucleated eyes were more-likely to have massive choroidal invasion (p = 0.0315) and post-laminar optic nerve invasion (p = 0.027), in spite of the finding that the overall prevalence of HRPF was similar between the two groups (35.5 percent vs. 37.5 percent; p = 0.585). The likelihood of anterior chamber invasion, was considerably higher in secondary enucleated eyes (p = 0.013). We evaluated primary and secondary enucleation for each subgroup (D and E) of the International Intraocular Retinoblastoma Classification (IIRC) and found the prevalence of HRPF was comparable (p = 0.58, 1.0, respectively). The difference in time between diagnosis-to-enucleation in secondary enucleation did not predict HRPF (p = 0.50). There was no discernible difference between primary and secondary enucleated eyes in terms of metastasis or survival (p = 0.156 and 0.44, respectively). Systemic chemotherapy has the ability to reduce the extent of tumor expansion that has been pathologically identified. Primary and secondary enucleated eyes are comparable in low metastatic risk only when strict examination and management guidelines are followed.

Polyester 5–0 suture for porous implant placement after retinoblastoma enucleation: analysis of 120 sockets

BackgroundTechniques used to suture the rectus muscle to the implant can influence the implant-related complications which is still a major problem following retinoblastoma enucleation. The goals of this work were to report the efficacy among patients with retinoblastoma who underwent enucleation followed by porous implant placement with the rectus muscles sutured with 5–0 polyester suture.MethodsThis was a retrospective study of consecutive patients with retinoblastoma who underwent primary enucleation and porous implant placement with the rectus muscles tagged and sutured to the implant with polyester 5–0 suture. All the patients were followed up for a minimum of 2 years. The main outcome measure was implant exposure. The secondary efficacy measures were other implant-related complications.ResultsBetween May 2016 and December 2018, a total of 120 patients (120 eyes) underwent primary enucleation and porous implant placement were included. Postoperatively, 10/120 (8.3%) eyes developed exposure or conjunctival granuloma. Exposure was the most common postoperative complication (7/10, 70.0%). There were no cases of implant extrusion, migration, or infection.ConclusionsPolyester 5–0 sutures are successful in patients with retinoblastoma who underwent enucleation followed by porous implant placement. Complications are minimal. Polyester 5–0 sutures were not associated with unacceptable complications in this pediatric population.

Ocular prosthetics in children-Possibilities and challenges

One of the greatest challenges for ocularists is prosthetic fitting in children, especially in children with congenital anomalies such as clinical anophthalmia or functionless (blind) microphthalmia. The most frequent reason for prosthetic fitting in children is acondition following enucleation for retinoblastoma, followed by trauma and congenital pathologies. The standard treatment after enucleation or evisceration begins intraoperatively with the selection of an suitable implant and the use of aconformer at the end of the operation to shape the prosthetic cavity. An initial prosthesis can be fitted 4weeks postoperatively, with afinal fitting taking place 3months later. If iatrogenic scarring or scarring due to an infection of the prosthetic cavity occurs, the approach of the ocularist must be appropriately adapted with the use of modified prosthesis shapes and shorter treatment intervals. Surgical options include scar excision and oral mucosa or amniotic membrane transplantation. Congenital anomalies require the shortest treatment intervals and even more so for anophthalmia than for microphthalmia. The strategy is characterized by simultaneous stimulation of the soft tissue of the ocular adnexa as well as the bony orbit. As self-inflating hydrogel expanders are no longer available, conservative prosthetic treatment is the only option. Close cooperation between child/parent, ocularist and ophthalmic plastic surgeon is the best prerequisite for agood long-term treatment outcome.

Chemotherapy induced juvenile dermatomyositis: a novel presentation- a case report

BackgroundIdiopathic connective tissue disease juvenile dermatomyositis (JDM) is characterised by inflammatory myositis and distinctive skin abnormalities. Only a few cases of Dermatomyositis (DM) owing to chemotherapy used to treat cancer have been reported, despite the fact that the link between DM and cancer in adults is widely known. We describe the case of a female, age 14, who experienced DM as a side effect of chemotherapy following enucleation for retinoblastoma. We also discussed our patient's likely pathophysiology of JDM after treatment.Case presentationA 14-year-old female came to our facility complaining of trouble walking and bluish-black discoloration on her neck, elbows, forehead, and knees that had been present for eight months. The patient had undergone enucleation of the left eye due to retinoblastoma, followed by 40 cycles of radiation therapy and 13 cycles of chemotherapy with Cyclophosphamide, Etoposide, Carboplatin, Vincristine, and Dactinomycin. Her serum LDH and CPK levels were high, and she tested positive for ANA. The muscle biopsy was consistent with the changes of DM. When electromyography was performed, it revealed tiny, fibrillating, polyphasic motor unit potentials and sharp, positive waves that were suggestive with DM. A diagnosis of JDM was made after taking into account the symptoms, biochemical data, muscle biopsy, and electromyography results. The patient's symptoms started to get better once methotrexate and oral corticosteroids were started.ConclusionThis case report emphasises the value of ongoing observation after cancer chemotherapy because specific cutaneous and muscle symptoms may lead paediatricians to consider the possibility of chemotherapy-induced JDM, which is uncommon in young patients.

Bilateral enucleation for retinoblastoma: A study of 14 patients

PURPOSE:To study the clinical profile of patients who underwent bilateral enucleation for retinoblastoma (RB).METHODS:Retrospective study of 14 cases.RESULTS:Of >3000 RB cases, 14 (<1%) underwent bilateral enucleation for treatment of RB. The mean age at diagnosis of RB was 26 months (median, 24 months; range, 5–72 months). All patients had bilateral RB at presentation. Intraocular RB was evident in 23 (82%) eyes and orbital tumor extension was noted in 5 (18%) eyes. Based on the International Classification of Intraocular RB, tumors were classified as Group B (n = 1; 4%), D (n = 4; 14%), or E (n = 14; 50%) at presentation. Based on the International RB Staging System, tumors were classified as Stage 1 (n = 23; 82%) or Stage 3 (n = 5; 18%). Two patients (four eyes with intraocular RB) had undergone prior treatment before presenting to us and thus could not be classified. Primary treatment included systemic chemotherapy (n = 27; 96%) or enucleation (n = 1; 4%). Five patients were lost to follow-up for a mean duration of 15 months (median, 12 months; range, 7–24 months) during treatment and presented with the orbital extension of RB in one (n = 4; 29%) or both (n = 1; 7%) eyes. Secondary enucleation was performed in 27 (96%) eyes. Over a mean follow-up period of 49 months (median, 29 months; range, 3–340 months), there was no evidence of metastasis and 1 (7%) child died due to pneumonia.CONCLUSION:Bilateral enucleation is rare in the treatment of RB. Advanced tumor presentation or noncompliance to treatment necessitates bilateral enucleation.

Gain of Chromosome 6p Correlates with Severe Anaplasia, Cellular Hyperchromasia, and Extraocular Spread of Retinoblastoma.

PurposeGain of chromosome 6p has been associated with poor ocular survival in retinoblastoma and histopathologic grading of anaplasia with increased risk of metastatic spread and death. This study examined the correlation between these factors and other chromosomal abnormalities as well as results of whole genome sequencing, digital morphometry, and progression-free survival.DesignRetrospective cohort study from 2 United States tertiary referral centers.ParticipantsForty-two children who had undergone enucleation for retinoblastoma from January 2000 through December 2017.MethodsStatus of chromosomes 6p, 1q, 9q, and 16q was evaluated with fluorescence in situ hybridization, the degree of anaplasia and presence of histologic high-risk features were assessed by ocular pathologists, digital morphometry was performed on scanned tumor slides, and whole genome sequencing was performed on a subset of tumors. Progression-free survival was defined as absence of distant or local metastases or tumor growth beyond the cut end of the optic nerve.Main Outcome MeasuresCorrelation between each of chromosomal abnormalities, anaplasia, morphometry and sequencing results, and survival.ResultsForty-one of 42 included patients underwent primary enucleation and 1 was treated first with intra-arterial chemotherapy. Seven tumors showed mild anaplasia, 19 showed moderate anaplasia, and 16 showed severe anaplasia. All tumors had gain of 1q, 18 tumors had gain of 6p, 6 tumors had gain of 9q, and 36 tumors had loss of 16q. Tumors with severe anaplasia were significantly more likely to harbor 6p gains than tumors with nonsevere anaplasia (P < 0.001). Further, the hematoxylin staining intensity was significantly greater and that of eosin staining significantly lower in tumors with severe anaplasia (P < 0.05). Neither severe anaplasia (P = 0.10) nor gain of 6p (P = 0.21) correlated with histologic high-risk features, and severe anaplasia did not correlate to RB1, CREBBP, NSD1, or BCOR mutations in a subset of 14 tumors (P > 0.5). Patients with gain of 6p showed significantly shorter progression-free survival (P = 0.03, Wilcoxon test).ConclusionsGain of chromosome 6p emerges as a strong prognostic biomarker in retinoblastoma because it correlates with severe anaplasia, quantifiable changes in tumor cell staining characteristics, and extraocular spread.

Health related quality of life of patients treated with bilateral enucleation for retinoblastoma.

Bilateral enucleation is rarely used to manage retinoblastoma (Rb). We present the health-related quality of life (HQL) and the associated factors among individuals who underwent bilateral surgical enucleation for retinoblastoma. Patients were interviewed via telephone in January 2020. A retinoblastoma registry was used to select patients who underwent bilateral enucleation/exenteration during 33 years. Data included age, gender, literacy, occupation, marital status, and health issues. Patients were asked eight questions on HQL. The responses were graded as 0-10. The percentage proportion of the HQL score was correlated to the determinants. Twenty-one out of 24 participants were interviewed (median age, 23 years). Fourteen participants answered the HQL question themselves, and for 7, their parents answered. Twenty patients (95%) had an ocular prosthesis. The median HQL score was 58 (out of a maximum score of 80) (interquartile range (IQR): 49; 70; minimum, 0 and maximum, 74). The HQL score was positively correlated to: self-reporting than parents reporting (Mann Whitney U (MW) p = 0.05); among students versus individuals in other occupations (MW p = 0.03); interval between eye removal and interview (p = 0.02). Age at enucleation of the second eye (p = 0.001), students (p < 0.001), and self-responders (p < 0.001) were independent predictors of a high HQL score. HQL of individuals having bilateral eye enucleation for retinoblastoma was reasonably good and positively correlated to self-reporting, learning as students to cope and age at 2nd eye removal. Anaplasty services to improve cosmetics seem to benefit such disabled persons.

Primary orbital polymethylmethacrylate implant following primary enucleation for retinoblastoma: a study of 321 cases

ABSTRACT Purpose: To evaluate the outcome of primary orbital polymethylmethacrylate (PMMA) implant following the primary enucleation for retinoblastoma. Methods: Retrospective study of 321 retinoblastoma patients who underwent unilateral enucleation and PMMA implant for retinoblastoma by myoconjunctival technique. Outcome measures included implant centration and extrusion. Results: The mean age at the time of enucleation of patients with retinoblastoma was 35 months (median, 30 months; range, <1 to 449 months). After primary enucleation, primary orbital PMMA implant was used in all cases. The mean diameter of implant was 18 mm (median, 18 mm; range, 12–20 mm) and the mean horizontal diameter of the socket conformer was 24 mm (median, 24 mm; range, 18 mm–26 mm). Post-enucleation and implant, seven (2%) patients underwent orbital external beam radiotherapy owing to microscopic extrascleral tumor extension or tumor infiltration of optic nerve transection. Over a mean follow-up period of 40 months (median, 34 months; range, 4–129 months), implant migration was noted in 28 (9%) patients, implant extrusion in 9 (3%), and implant exposure in 5 (2%), and contracted socket in 5 (2%) patients including grade 1 contraction in 3 (1%), grade 2 in 1 (<1%), and grade 4 in 1 (<1%) patient. Implant exchange for an improved prosthesis fit was performed in 4 (1%) cases. Stable customized ocular prosthesis was achieved in all but one patient. Conclusion: Primary orbital PMMA implant following primary enucleation for retinoblastoma is associated with minimal complications and provides acceptable cosmetic outcomes.

Next-Generation Sequencing of Retinoblastoma Identifies Pathogenic Alterations beyond RB1 Inactivation That Correlate with Aggressive Histopathologic Features

To determine the usefulness of a comprehensive, targeted-capture next-generation sequencing (NGS) assay for the clinical management of children undergoing enucleation for retinoblastoma. Cohort study. Thirty-two children with retinoblastoma. We performed targeted NGS using the UCSF500 Cancer Panel (University of California, San Francisco, San Francisco, CA) on formalin-fixed, paraffin-embedded tumor tissue along with constitutional DNA isolated from peripheral blood, buccal swab, or uninvolved optic nerve. Peripheral blood samples were also sent to a commercial laboratory for germline RB1 mutation testing. Presence or absence of germline RB1 mutation or deletion, tumor genetic profile, and association of genetic alterations with clinicopathologic features. Germline mutation or deletion of the RB1 gene was identified in all children with bilateral retinoblastoma (n= 12), and these NGS results were 100% concordant with commercial germline RB1 mutation analysis. In tumor tissue tested with NGS, biallelic inactivation of RB1 was identified in 28 tumors and focal MYCN amplification was identified in 4 tumors (2 with wild-type RB1 and 2 with biallelic RB1 inactivation). Additional likely pathogenic alterations beyond RB1 were identified in 13 tumors (41%), several of which have not been reported previously in retinoblastoma. These included focal amplifications of MDM4 and RAF1, as well as damaging mutations involving BCOR, ARID1A, MGA, FAT1, and ATRX. The presence of additional likely pathogenetic mutations beyond RB1 inactivation was associated with aggressive histopathologic features, including higher histologic grade and anaplasia, and also with both unilateral and sporadic disease. Comprehensive NGS analysis reliably detects relevant mutations, amplifications, and chromosomal copy number changes in retinoblastoma. The presence of genetic alterations beyond RB1 inactivation correlates with aggressive histopathologic features.

High-risk retinoblastoma based on age at primary enucleation: a study of 616 eyes.

To study the high-risk histopathology features of retinoblastoma based on age at primary enucleation. Retrospective study of 616 patients. The mean age at presentation and primary enucleation for retinoblastoma was 34 months (median, 28 months; range, <1-455 months). Of these cases, 128 (21%) were aged ≤1 year, 149 (24%) were in the age group of 1-2 years, 117 (19%) in 2-3 years, 104 (17%) in 3-4 years, and 118 (19%) were >4 years of age at the time of enucleation. Bilateral retinoblastoma (34%; p < 0.0001) and buphthalmos (20%; p < 0.0001) were more common in children ≤1 year of age. Anterior chamber pseudohypopyon (15%; p < 0.0001) and vitreous seeds (53%; p < 0.0001) were more common in children aged >4 years. Based on 8th edition American Joint Committee on Cancer staging system, pT3 was less common in children ≤1 year of age (13%; p < 0.001). Based on histopathology, 38% patients had high-risk features including 24% children aged ≤1 year, 42% in the age group of 1-2 years, 34% in 2-3 years age group, 45% in 3-4 years age group, and 48% patients were >4 years of age. Post-laminar optic nerve infiltration (6%; p = 0.02) and massive choroidal infiltration (9%; p = 0.04) was least common in children ≤1 year of age. Over a mean follow-up period of 52 months (median, 36 months; range, <1-218 months), systemic metastasis and death occurred in 9% patients despite adjuvant systemic chemotherapy. The predominant high-risk histopathology feature of retinoblastoma varies with age at primary enucleation.

Milia en plaque

The aim of this 8-year retrospective review was to determine the clinical significance of gadolinium-enhanced magnetic resonance imaging (MRI) findings in retinoblastoma patients after enucleation, particularly the presence of abnormal contrast enhancement of the transected optic nerve.Retrospective chart review.A review was done on 88 patients with retinoblastoma undergoing 90 enucleations between January 2008 and December 2015.These patients underwent 233 MRI scans: 90 preoperative and 143 postoperative that were included for review.The primary outcome measure assessed was abnormal MRI findings in the preoperative and postoperative MRI scans, specifically enhancement of the optic nerve and correlations between abnormal MRI findings and clinical outcomes for the 88 patients.On the preoperative MRI, 4 optic nerves out of 90 scans showed positive enhancement. Fifty orbits had ≥1 postoperative MRI. Overall, 41 of 50 orbits (82%) of enucleated patients demonstrated postoperative contrast enhancement on MRI after enucleation, at a mean interval of 10 months after surgery. The percentage of MRI scans with optic nerve enhancement was 77% from 0 to 6 months after enucleation and 68% at >24 months after surgery. Postenucleation optic nerve enhancement did not correlate with preoperative optic nerve enhancement, chemotherapy administration, or the presence of optic nerve invasion on histopathology. No child required an orbital biopsy. None of the 88 patients were found to have subsequent orbital or metastatic disease at the last clinical follow-up visit (average, 29 months; range, 1–71).Optic nerve contrast enhancement on follow-up MRI after enucleation for retinoblastoma seems to be a common, benign radiographic finding; none of the patients in this series developed extraocular tumor relapse. The presence of postenucleation enhancement on MRI did not correlate with preoperative chemotherapy or the presence of optic nerve invasion on histopathology. Based on our findings, intervention for isolated optic nerve enhancement on MRI is not indicated in the absence of other abnormal clinical or radiographic signs. A prospective trial with a validated radiographic grading system would be helpful to clarify the MRI features to differentiate orbital recurrence from benign postoperative enhancement.

Racial, Ethnic, and Socioeconomic Disparities in Retinoblastoma Enucleation: A Population-Based Study, SEER 18 2000-2014

To determine the effect of race, ethnicity, and census tract-level composite socioeconomic status (SES) on retinoblastoma enucleation. This study augments Truong and associates, providing multivariate analyses combining sociodemographic and clinical characteristics with more accurate SES measures. We hypothesized that children from nonwhite, Hispanic, and lower socioeconomic backgrounds would have increased adjusted odds of enucleation. Retrospective cohort analysis. Multicenter population-based study using the Surveillance, Epidemiology, and End Results (SEER) 18 Registries. Children aged 18 years and younger diagnosed with retinoblastoma between 2000 and 2014. Subjects were identified using International Classification of Diseases-Oncology (ICD-O) site and morphology codes. Enucleation odds ratios (ORs) and 95% confidence intervals (CIs). Analysis of 959 retinoblastoma patients revealed that 70.8% were enucleated. Adjusted analyses showed associations between enucleation and Asian (OR 2.00, CI 1.08-3.71) or black (2.42, 1.41-4.16) race, Hispanic ethnicity (1.69, 1.16-2.46), and low SES (1.68, 1.09-2.58). Significantly increased enucleation risk was associated with older age at diagnosis (age 1-2 years 2.55, 1.80-3.61; >2 years 4.88, 2.57-9.25), unilateral disease (5.00, 3.45-7.14), and advanced stage (regional 4.71, 2.51-8.84; distant 3.15, 1.63-6.08). No interactions were observed between race, ethnicity, SES, and stage at diagnosis. Enucleation rates decreased over time across all racial, ethnic, and socioeconomic groups. Children from nonwhite, Hispanic, and lower socioeconomic backgrounds are more likely to receive enucleation. These associations are independent of stage of diagnosis, suggesting larger systemic disparities in retinoblastoma care. The origin of these differences requires further study and attention by clinicians and policy makers.

Accuracy of preoperative imaging in predicting optic nerve invasion in retinoblastoma: A retrospective study.

Purpose:Optic nerve invasion is an important cause of mortality in retinoblastoma. We aimed correlate preoperative imaging and surgical histopathology findings in enucleated eyes with retinoblastoma to determine the efficacy of preoperative imaging in predicting optic nerve invasion in retinoblastoma.Methods:A retrospective review of records of all patients undergoing primary enucleation for retinoblastoma at a tertiary eyecare system between March 2013 and December 2017 with all patients having undergone preoperative imaging, either CT scan or MRI. Data was analyzed statistically to determine the correlation between preoperative CT scan/MRI and histopathology.Results:Totally, 97 eyes of 97 patients were included in the study who underwent primary enucleation for unilateral retinoblastoma. The average age at presentation was 27.8 months with the chief complaint being leukocoria in all the cases. 14 patients (14.43%) had evidence of optic nerve involvement in preoperative imaging. 30 patients had optic nerve invasion on histopathology (laminar and retrolaminar). Spearman's rank correlation test revealed a significant correlation between MRI findings and HP and an insignficant correlation between CT findings and HP. The CT scan had a sensitivity of 20%, specificity of 88.89%, 50% positive predictive value and 66.67% negative predictive value. MRI had a sensitivity of 40%, specificity of 93.55%, positive predictive value of 66.67% and a negative predictive value of 82.86%.Conclusion:MRI showed significant moderate correlation with surgical histopathology for predicting optic nerve invasion in retinoblastoma whereas CT shows insignificant correlation with HPE. Therefore, we recommend MRI for predicting optic nerve invasion in cases of retinoblastoma.

Prospective study of ophthalmic artery chemosurgery (OAC) as alternative to enucleation in retinoblastoma.

10555Background: Over the past twenty-five years, the introduction of systemic chemotherapy combined to local treatments has enabled to dramatically increase globe retention for retinoblastoma (RB)...

Porous orbital implant after enucleation in retinoblastoma patients: indications and complications

ABSTRACTThis study aims to identify risk factors associated with complications in retinoblastoma patients following primary and secondary enucleations with porous implant placement.A retrospective case-control study was performed between 2010 and 2015. Data pertaining to subjects’ demographics, medical history, clinical, and pathological findings, implant characteristics and complications were collected.The analysis included 103 eyes of 101 patients age 27.8 ± 21.9 months undergoing enucleation for retinoblastoma. Postoperatively, 19/103 (18%) eyes developed exposure, extrusion, or hematoma requiring subsequent surgery. Exposure was the most common postoperative complication (12/19, 63%). Age at enucleation 24 months or younger, Hispanic ethnicity, female gender, and intravenous chemotherapy prior to enucleation were associated with increased odds of implant complications. In contrast, patients who were given intravitreal melphalan (IM), subtenons carboplatin (SC), or external beam radiation therapy (EBRT) did not demonstrate an increased risk of complications.In this cohort of retinoblastoma patients undergoing primary or secondary enucleation with porous implants, implant exposure was the most common postoperative complication. Our findings suggest that female gender, Hispanic ethnicity, age at enucleation 24 months or younger, and intravenous chemotherapy prior to enucleation may increase the risk of complications.

Loss of fundus view as an indication for secondary enucleation in retinoblastoma.

In this retrospective study, we evaluated loss of fundus view as an indication for secondary enucleation and associated histopathologic findings. Of 64 secondarily enucleated eyes, 24 were enucleated for loss of fundus view. Average time from loss of fundus view to enucleation was 4.7 months. Of these eyes, 22 had viable tumor cells on histopathology, but none had high-risk features. Loss of fundus view was a common indication for secondary enucleation after chemoreduction. Given the high prevalence of viable histopathologic tumor cells, enucleation for loss of fundus view should not be significantly delayed to decrease risk of high-risk tumor progression.

Structural changes of the macula and optic nerve head in the remaining eyes after enucleation for retinoblastoma: an optical coherence tomography study

BackgroundTo describe objectively the possible structural changes of the macula and optic nerve head in the free eyes of unilateral cured retinoblastoma patients and, also after enucleation using spectral domain optical coherence tomography.MethodsA cross sectional study involving 60 patients subdivided into three groups; 15 unilateral RB patients in whom enucleation was indicated as a sole treatment performed earlier in life [(study group (I)], 15 unilateral RB patients who had completely regressed disease with a preserved eye [(study group (II)] and 30 age and sex matched healthy controls. The remaining and free eyes in study groups and right eyes of control group had full ophthalmological examination, static automated perimetry and optical coherence tomography of the macula and optic nerve head.ResultsIn study group (II); a significant thinning of total macula, central fovea, ganglion cell layer (GCL), ganglion cell complex (GCC), and some sectors of outer nuclear layer (P- values ≤0.05) was found with no significant difference in peripapillary nerve fiber layer (pRNFL) thickness and optic nerve head parameters compared to the control group and the study group (I). A significantly thickened total macula, GCL, GCC, and pRNFL in study group (I) compared to study group (II). Thickened pRNFL was significantly correlated to standard automated perimetry pattern deviations. No significant difference was found between study group (I) and control group.ConclusionRetinoblastoma eyes characterized by thinning of central fovea, GCL, GCC compared to the control group. After unilateral enucleation, increased GCC and pRNFL thicknesses were detected compared to retinoblastoma group.

A Novel Method to Measure Artificial Eye Motility.

To measure objectively, reproducibly, and noninvasively artificial eye motility, a fundamental aspect in the (cosmetic) outcome of enucleation. A gaze- and pupil-tracking system, the "iView X," was implemented to measure horizontal eye motility. The system, with adjusted software, was tested with patients wearing 1 prosthetic eye after enucleation for retinoblastoma. Measurements were repeated 5 times in every patient and analyses were performed twice by 2 independent observers. Reproducibility was tested via linear mixed models. After the implementation of the method, more data were obtained, including more patients with a history of enucleation for the treatment of retinoblastoma for eye tracker measurement, and differences in motility percentages between implant types and sizes were analyzed via Mann-Whitney U tests. The intraclass correlation coefficient of the interobserver variable in the patient test group (n = 27, 6-53 years) was 0.98 and 0.96 for measurement of left gaze and right gaze, respectively. Intraobserver variation was <0.001. In the total of 58 included patients for comparative analysis, mean difference of prosthesis motility compared with the contralateral eye in abduction was 57.1% (range 3.2-91.5%); in adduction 65.8% (range 24.0-92.0%). No statistical differences were found between the different implant types and sizes. Motility measurements of the prosthetic eye in comparison to the contralateral eye using the iView X system are reproducible and reliable. This is, to the authors' knowledge, the first easy applicable, noninvasive, reproducible, and commercially available instrument to evaluate prosthesis motility. With the adjusted software program (freely available on request), a similar objective measurement can be performed worldwide. The implant size or type did not influence the outcome of the motility measurement; this finding deserves additional study.

Psychosocial factors influencing parental decision to allow or refuse potentially lifesaving enucleation in children with retinoblastoma

Objective:Retinoblastoma is the most common malignancy of the eye and ocular adnexa in the Philippines. It is curable when treated early, but delay in enucleation is common due to the parental refusal of surgery for varied reasons. The aim of this study is to identify the psychosocial barriers and facilitating factors for accepting versus refusing enucleation as treatment for retinoblastoma.Methods:This is a cross-sectional descriptive study utilizing structured interviews and a questionnaire. It was conducted at the Retinoblastoma Clinic of the Philippine General Hospital. A questionnaire using the Likert scale was constructed after performing key informant interviews and focus group discussions. It was pretested and revised before parents of patients with retinoblastoma were invited to participate in the study. Descriptive statistics, quantitative item analyses using inter-item correlations and item-total correlations was performed.Results:Factors that correlate with refusal to enucleate are the beliefs that cancer is a fatal illness, the fear of unacceptable esthetic outcome of the surgery, and the cost of treatment. Favorable factors include value of life, high regard for the opinion of medical practitioners, and appreciation of the efficacy of treatment.Conclusions:There are several favorable factors and barriers that health practitioners must consider in facilitating parental decision-making toward enucleation for retinoblastoma.