Achieving equity in childhood cancer represents a global challenge. In Africa, where retinoblastoma has high mortality and morbidity, strengthening research capacity is crucial in developing clinical guidelines from local evidence. This review identified the scope of retinoblastoma research conducted in Africa. A systematic search identified English-language studies on retinoblastoma in Africa, published between January 1, 2003 and May 15, 2025. Studies were excluded if retinoblastoma was not the primary focus or if Africa was not the main study location. Data collected included journal information, author affiliations, study type, country, purpose, and results. Quantitative findings were summarized with descriptive statistics, while qualitative findings were narratively synthesized. Of the 1546 citations retrieved, 89 met the inclusion criteria. Single-country studies (n = 85) represented 20 of 54 (37%) African countries, while four multi-country studies increased representation to 43 countries (80%). Most studies were clinical observational (55/89, 62%). Of the 89 studies, 49 (55%) were authored solely by researchers in Africa, but studies which included foreign authors tended to be published in journals with higher journal impact factors (p<0.001). The growth of retinoblastoma research in Africa reflects both expanding local research capacity and increased international collaboration. However, limited experimental research and basic science studies point to opportunities to strengthen the local evidence base needed to inform clinical guidelines for improving retinoblastoma outcomes in Africa.
Pseudopapilledema caused by optic disc drusen (ODD) mimics the appearance of papilledema and usually presents as a diagnostic challenge. A young boy with known Chiari malformation type 1 (CM-1) was referred to the pediatric ophthalmology clinic for eye assessment to exclude papilledema due to elevated intracranial pressure (ICP). Despite the ophthalmic examination revealing bilateral optic disc elevation, multimodal imaging techniques such as fundus autofluorescence, optical coherence tomography (OCT), and B-scan ultrasonography are recommended to confirm the distinction between bilateral ODD causing pseudopapilledema and papilledema secondary to elevated ICP. Accidental coexistent papilledema mimickers like ODD need to be considered in patients with CM-1 before making a diagnosis of papilledema to avoid unnecessary invasive procedures. There was no evidence that the presence of ODD excludes the possibility of future optic nerve head changes due to elevated ICP. The multidisciplinary consensus decided on annual ophthalmology follow-ups using multimodal imaging to detect any subtle optic nerve head changes.
Background/aimLaser photocoagulation is less invasive than chemotherapy (systemic, intra-arterial or periocular) and brachytherapy. We studied the safety and efficacy of laser as primary monotherapy for discrete retinoblastoma with well-defined borders and attached retina.MethodsA single-institution retrospective non-comparative review (2004–2018) of discrete retinoblastoma tumours managed with primary laser (532 or 810 nm wavelength, 0.5–1 s duration and power titrated until desired tumour whitening). Efficacy was evaluated by tumour long-term stability avoiding non-laser therapies. Safety was evaluated by frequency of laser-related complications and uncontrollable tumour progression.ResultsEligible were 112 tumours in 55 eyes of 44 patients. Laser monotherapy (median 2 sessions) achieved initial remission in 95/112 (85%) tumour. Initial encircling only laser photocoagulation was associated with tumour progression (9/11, one tumour had vitreous seeding) compared with direct or combined photocoagulation techniques (0/94 and 0/7 tumours, respectively, p<0.001). Direct laser had no vitreous seeding, haemorrhage or injury to vital structures. Tumour recurrences developed in 52/112 (46%) tumour but repeat laser achieved long-term stability, except five tumour recurrences that required invasive therapy. Receiver operating characteristic analysis identified threshold largest basal diameter of 3 disc diameters (DD) for successful laser monotherapy, where 92/106 (87%) of tumours ≤3 DD and 0/6>3 DD achieved long-term stability with laser monotherapy (p<0.001). Overall, 35/55 (64%) eyes and 24/44 (55%) patients achieved long-term stability with laser monotherapy. No eye was enucleated for uncontrollable tumour progression.ConclusionsDiscrete retinoblastoma ≤3 DD can be effectively and safely managed with laser monotherapy, sparing a significant proportion of patients/eyes from more invasive therapies.
PURPOSE:To highlight prevalence, spectrum of anomalies, and outcome of ophthalmic involvement in PHACES syndrome (posterior fossa malformations, infantile hemangiomas, arterial, cardiac, eye, and sternal anomalies).METHODS:A retrospective, noncomparative, single-institution observational case series of children with PHACES was conducted from 2000 to 2019. Data on ocular presentations, interventions and visual outcomes were collected. Primary outcome measures were the frequency and spectrum of ocular involvement. Secondary outcomes were final visual acuity, long-term ocular sequelae, and frequency of surgical interventions.RESULTS:A total of 43 infants had PHACES, of whom 29 (67%) had periocular infantile facial hemangiomas (IFH) and 6 (14%) had primary ocular anomalies that were always ipsilateral to the IFH. Five patients (12%) met ocular PHACES-specific diagnostic criteria, including optic nerve (3), retinal vascular (1) and lenticular (2) anomalies. Non-PHACES-specific abnormalities were Peters anomaly (1), persistent pupillary membranes (2), dysmorphic optic nerves (1), and iris/choroidal hemangiomas (2). IFH-related periocular abnormalities were frequent: ptosis (29), proptosis (9), strabismus (6). Surgery was required in 8 of the 29 children: (strabismus [6], entropion [2], ptosis [2], and optical iridectomy [1]), all of whom had orbital/conjunctival hemangioma (P = 0.03). Final visual acuity (follow-up, 8.7 years) ranged between 20/20 and 20/80 in 26 of 29 patients. All patients with visual acuity worse than 20/200 (3/29 [10%]) had structural anomalies.CONCLUSIONS:Two-thirds of infants with PHACES have periocular IFH causing vision compromising complications of amblyopia and strabismus. Structural ocular anomalies exist in 1 of 7 patients and are always ipsilateral to the IFH. Long-term ophthalmic monitoring and management is required, and the majority of patients obtain good visual outcomes.
OBJECTIVE:The primary aim of this study was to identify the frequency of death, metastasis, enucleation, and use of external beam radiation therapy (EBRT) among retinoblastoma patients. The secondary aim was to determine whether any events were associated with suboptimal clinical management to identify areas for clinical care improvement.METHODS:Patients diagnosed with retinoblastoma between January 1, 2000, and December 31, 2015, at The Hospital for Sick Children were included. Medical records of eligible patients underwent a comprehensive 2-part review. First, a chart review collected diagnostic details, treatment course, and occurrence of 4 events: death, metastasis, use of EBRT, and enucleation. Next, events were reviewed in detail, and a multidisciplinary committee reached consensus on cases managed suboptimally.RESULTS:The study included 209 patients (292 eyes). There were 8 deaths, 11 metastases, 177 enucleations (143 primary, 34 secondary), and 8 uses of EBRT. Thirteen patients were reviewed by the multidisciplinary committee, which confirmed that 5 of these patients had events associated with suboptimal clinical management. Three patients developed metastases leading to death (misdiagnosis and mismanagement of trilateral retinoblastoma [1], parental refusal of enucleation [1], and inaccurate histopathology after primary enucleation [1]). One patient developed extraocular extension related to scleral invasion following aggressive focal therapy. One patient underwent secondary enucleation for a Group B eye related to mismanagement of a treatment complication.DISCUSSION:Deaths, metastases, and enucleations with documented instances of suboptimal care highlighted a need to enhance medical team and patient communication, histopathology interpretation, laser treatment guidelines, and trilateral retinoblastoma management. Routine clinical audit of retinoblastoma management can identify areas for clinical practice change.
PURPOSE:To determine the risk of patients with an early diagnosis of heritable retinoblastoma being diagnosed with TRb (or pineoblastoma) asynchronously in a later stage and its effect on screening.METHODS:We updated the search (PubMed and Embase) for published literature as performed by our research group in 2014 and 2019. Trilateral retinoblastoma (TRb) patients were eligible for inclusion if identifiable as unique and the age at which TRb was diagnosed was available. The search yielded 97 new studies. Three new studies and eight new patients were included. Combined with 189 patients from the previous meta-analysis, the database included 197 patients. The main outcome was the percentage of asynchronous TRb in patients diagnosed before and after preset age thresholds of 6 and 12 months of age at retinoblastoma diagnosis.RESULTS:Seventy-nine per cent of patients with pineoblastoma are diagnosed with retinoblastoma before the age of 12 months. However, baseline MRI screening at time of retinoblastoma diagnosis fails to detect the later diagnosed pineal TRb in 89% of patients. We modelled that an additional MRI performed at the age of 29 months picks up 53% of pineoblastomas in an asymptomatic phase. The detection rate increased to 72%, 87% and 92%, respectively, with 2, 3 and 4 additional MRIs.CONCLUSIONS:An MRI of the brain in heritable retinoblastoma before the age of 12 months misses most pineoblastomas, while retinoblastomas are diagnosed most often before the age of 12 months. Optimally timed additional MRI scans of the brain can increase the asymptomatic detection rate of pineoblastoma.
Cholera caused by Vibrio cholerae O139 could reemerge, and proactive development of an effective O139 vaccine would be prudent. To define immunoreactive and potentially immunogenic carbohydrate targets of Vibrio cholerae O139, we assessed immunoreactivities of various O-specific polysaccharide (OSP) related saccharides with plasma from humans hospitalized with cholera caused by O139, comparing responses to those induced in recipients of a commercial oral whole-cell killed bivalent (O1 and O139) cholera vaccine (WC-O1/O139). We also assessed conjugate vaccines containing selected subsets of these saccharides for their ability to induce protective immunity using a mouse model of cholera. We found that patients with wild-type O139 cholera develop IgM, IgA, and IgG immune responses against O139 OSP and many of its fragments, but we were able to detect only a moderate IgM response to purified O139 OSP-core, and none to its fragments, in immunologically naive recipients of WC-O1/O139. We found that immunoreactivity of O139-specific polysaccharides with antibodies elicited by wild-type infection markedly increase when saccharides contain colitose and phosphate residues, that a synthetic terminal tetrasaccharide fragment of OSP is more immunoreactive and protectively immunogenic than complete OSP, that native OSP-core is a better protective immunogen than the synthetic OSP lacking core, and that functional vibriocidal activity of antibodies predicts in vivo protection in our model but depends on capsule thickness. Our results suggest that O139 OSP-specific responses are not prominent following vaccination with a currently available oral cholera vaccine in immunologically naive humans and that vaccines targeting V. cholerae O139 should be based on native OSP-core or terminal tetrasaccharide. IMPORTANCE Cholera is a severe dehydrating illness of humans caused by Vibrio cholerae serogroup O1 or O139. Protection against cholera is serogroup specific, and serogroup specificity is defined by O-specific polysaccharide (OSP). Little is known about immunity to O139 OSP. In this study, we used synthetic fragments of the O139 OSP to define immune responses to OSP in humans recovering from cholera caused by V. cholerae O139, compared these responses to those induced by the available O139 vaccine, and evaluated O139 fragments in next-generation conjugate vaccines. We found that the terminal tetrasaccharide of O139 is a primary immune target but that the currently available bivalent cholera vaccine poorly induces an anti-O139 OSP response in immunologically naive individuals.
OBJECTIVE:To determine the role of iodine-125 plaque radiotherapy (IPR) as a secondary treatment for localized (solitary or multiple) residual (partially regressed) or recurrent (regrowth after ≥6 months stability) retinoblastoma in the era of systemic and/or regional chemotherapy. DESIGN:A single-institute retrospective, noncomparative, interventional case series managed between July 2014 and June 2019. PARTICIPANTS:Thirteen consecutive eyes of 12 patients with 14 residual or recurrent retinoblastoma tumors treated with IPR. Patients who had to follow up <1 year post-IPR were excluded except for those who had enucleation. METHODS:Data collected included pre-IPR treatments, tumor characteristics at IPR, and post-IPR anatomical outcome (local tumor control and globe salvage) and functional outcome (radiation complications). RESULTS:Local tumor control was achievable in 12 of 14 tumors. Local recurrences were observed in 2 of 5 tumors that exhibited fish-flesh regression after IPR (p = 0.04). Globe salvage was possible in 11 eyes (12 tumors). Only 2 eyes were legally blind and the remaining 9 eyes had vision >20/125. Radiation-induced complications included radiation retinopathy (4/11), radiation papillopathy (1/11), diffuse vitreous hemorrhage (4/11). Eyes with fish-flesh-regressed tumours tended to show more complications, but were statistically insignificant (p = 0.09, Fisher exact test). There was no association of time to IPR (early <6 months vs late >6 months) with occurrence of tumor recurrence or complications (p > 0.05). CONCLUSION:IPR offers satisfactory local tumor control and globe salvage in localized recurrent/residual retinoblastoma. Fish-flesh tumor regression after IPR should be closely monitored for further recurrences.
Retinoblastoma is the most common pediatric intraocular cancer. Rarely, it may develop in adults, with different clinical and imaging characteristics that make the diagnosis a challenge. We present a case of a white retinal tumor in a 42-year-old woman that progressed slowly over 3 years and on enucleation an undifferentiated tumor was found without a conclusive diagnosis. Molecular analysis identified RB1 pathogenic variant that confirmed retinoblastoma diagnosis in this discordant clinicopathologic presentation of the tumor.
Abstract Purpose: Optical coherence tomography (OCT) improved retinal assessment that is comparable to histopathologic sections. In retinoblastoma, OCT was reported to detect fovea-tumor relation, diagnose subclinical (new, residual and recurrent tumors), and suspect tumor spread into the optic nerve. We hypothesized that strategic incorporation of OCT into retinoblastoma management will improve outcomes and managing retinoblastoma at the subclinical level (clinically undetectable) provides means for less treatment burden. Methods: A single-center, noncomparative retrospective review of all eyes with subclinical retinoblastoma was conducted. Any eye that had an OCT-guided procedure, whether diagnostic or therapeutic, utilizing OCT software was included. OCT-guided management included projecting embedded OCT software tools (scan cube size and direction, multiple scan frames, localizing and measuring vertical and horizontal calipers, enhanced depth imaging frames, and/or red free fundus photograph) on a separate colored fundus image to guide management to improve visual, life, and eye salvage outcomes. Results: We used OCT-guided strategies in 83 children (114 eyes). Diagnostic strategies included posterior pole screening for invisible retinoblastoma (37 eyes), topographic localization of invisible tumors (15 eyes) or subclinical recurrence (29 tumors), topographic localization of fovea-tumor relation (31 eyes), and mapping of potential choroidal or optic nerve invasion to determine subsequent management (2 eyes each). Therapeutic strategies included laser photocoagulation for perifoveal residual (26) and invisible (11) tumors and verification of photocoagulation adequacy for invisible (11) new and recurrent (11) tumors. These procedures were scored for their influence to changed therapy. Conclusion: Detailed OCT assessment improves retinoblastoma management and facilitates precision therapy to preserve vital visual structures (e.g., the center of vision), detect subtle subclinical tumors (new or recurrent), allow administration and adequacy of laser treatment, and map subclinical high-risk features. Developing OCT-guided strategies with full technical description facilitates reproducibility and allows precision therapy to be a reality. Citation Format: Sameh E. Soliman, Cynthia VandenHooven, Leslie MacKeen, Brenda Gallie. Optical coherence tomography improves subclinical retinoblastoma management [abstract]. In: Proceedings of the AACR Special Conference on the Advances in Pediatric Cancer Research; 2019 Sep 17-20; Montreal, QC, Canada. Philadelphia (PA): AACR; Cancer Res 2020;80(14 Suppl):Abstract nr A68.
Invisible retinoblastoma tumors are now detected with screening for retinal tumors in at-risk neonates (those inheriting RB1 pathogenic alleles from affected parents) using handheld OCT. Laser photocoagulation is challenging, requiring exact localization of a tumor invisible to indirect ophthalmoscopy and standard imaging. We describe OCT-guided localization and photocoagulation of these invisible tumors with 1-year follow-up.Retrospective, noncomparative, single-institutional, observational case series.Children with any clinically invisible retinoblastoma tumor that was detected on OCT posterior pole screening.OCT revealed round homogeneous invisible tumors within the inner nuclear layer. Software calipers placed beside anatomic retinal landmarks (branched/curved vessels, fovea, or optic disc) mapped the tumor location and extent. A single laser (532 nm) burn flagged the location, and OCT evaluated the tumor-laser burn relationship; laser treatment was then continued in the correct location. Post-laser OCT ensured complete treatment.Accuracy (frequency of geographic miss and skip areas), effectiveness (recurrence rate), and burden (scar size and characteristics at final follow-up) of laser treatment.Eleven new invisible posterior pole tumors in 7 eyes of 5 children were treated by this technique. Localization and tumor-laser burn relationships were accurate in 11 of 11 tumors (100%, 95% confidence interval [CI], 49.9-100), and all showed swelling and hyper-reflectiveness of the tumor in post-laser OCT. Two photocoagulation sessions (2 weeks apart) were sufficient to successfully manage 9 of 11 tumors (82%, 95% CI, 37.4-100) with resulting permanent flat scars. One tumor (9%, 95% CI, 0.2-50.6) developed OCT-detected subclinical recurrences within 3 months, treated by 1 laser session. No treatment scar showed gliosis, foveal involvement, or retinal traction at 1-year follow-up. Scar expansion occurred in 1 tumor (9%, 95% CI, 0.2-50.6), and all scars (100%, 95% CI, 49.9-100) showed pigmentary changes.The OCT-guided localization and photocoagulation technique is valuable in achieving precision results in managing invisible new retinoblastoma tumors. This technique shows a potential to improve outcomes of secondary prevention screening for retinoblastoma.
Background The risk of tumor spread is the main concern while deciding primary treatment in advanced unilateral retinoblastoma, which presents later with an intact normal eye. Enucleation was recommended as a radical treatment but with newer treatment options such as systemic and intra-arterial chemotherapy. Ocular salvage could be tried in group D eyes with smaller tumors and visual potential. Aim The aim was to assess the histopathologic metastatic risk in primary vs secondary enucleated (trial salvage) unilateral group D eyes. Design Retrospective, noncomparative, single-institution observational case series. Patients and methods All primary and secondary enucleated unilateral group D eyes (June 2012 to December 2018) managed at the pediatric ocular oncology unit, Ophthalmology Department, University of Alexandria. Clinical, radiological, histopathologic, and treatment data were collected. Clinicopathologic correlation was performed to identify the frequency of high-risk histopathologic features (HRHF). Statistical analysis All analyses were performed using Microsoft Excel 2013 software and SPSS software version 17.0. Results Twenty-six eyes were included. Sixteen were primary enucleated and none showed HRHF while 10 were secondary enucleated after failed trial salvage. Timely enucleated secondary cases (6/10) showed no HRHF while delayed secondary enucleation (2/4) was significantly associated with HRHF (P=0.02). All cases are alive with no metastasis after a median follow-up of 5 years. Conclusion Primary and timely secondary enucleation for unilateral group D retinoblastoma eyes are comparable in low metastatic risk and effectivity of tumor control. Controlled discussed trial ocular salvage is safe conditioned by strict timely termination, if necessary.
Oligosaccharides equipped with amine-containing linkers can be conjugated to carrier proteins using squaric acid chemistry. In a two-step process, a squarate derivative of such oligosaccharide is formed first, which is followed by its reaction with a protein carrier. Monitoring of the conjugation reaction is achieved by SELDI-TOF-MS or MALDI-TOF-MS. This experimentally simple procedure yields desired glycoconjugates in high yields and with reproducible hapten-protein ratios.
Accurate diagnosis of retinoblastoma is crucial to develop a proper treatment plan, especially in limited resource settings. Retinal imaging facilitates the exchange of clinical findings to assist management.
Invisible retinoblastoma tumors are now detected by prenatal identification of RB1 pathogenic variants inherited from affected parents, followed by early delivery with screening for retinal tumors using handheld optical coherence tomography (OCT). Laser photocoagulation is challenging, requiring exact localization of an invisible tumor. We describe an OCT-guided localization and photocoagulation technique and its preliminary outcome.
TOPIC:To determine the age up to which children are at risk of trilateral retinoblastoma (TRb) developing, whether its onset is linked to the age at which intraocular retinoblastomas develop, and the lead time from a detectable pineal TRb to symptoms. CLINICAL RELEVANCE:Approximately 45% of patients with retinoblastoma-those with a germline RB1 pathogenic variant-are at risk of pineal TRb developing. Early detection and treatment are essential for survival. Current evidence is unclear regarding the usefulness of screening for pineal TRb and, if useful, the age up to which screening should be continued. METHODS:We conducted a study according to the Meta-analysis of Observational Studies in Epidemiology guidelines for reporting meta-analyses of observational studies. We searched PubMed and Embase between January 1, 1966, and February 27, 2019, for published literature. We considered articles reporting patients with TRb with survival and follow-up data. Inclusion of articles was performed separately and independently by 2 authors, and 2 authors also independently extracted the relevant data. They resolved discrepancies by consensus. RESULTS:One hundred thirty-eight patients with pineal TRb were included. Of 22 asymptomatic patients, 21 (95%) were diagnosed before the age of 40 months (median, 16 months; interquartile range, 9-29 months). Age at diagnosis of pineal TRb in patients diagnosed with retinoblastoma at 6 months or younger versus older than 6 months were comparable (P = 0.44), suggesting independence between the ages at diagnosis of intraocular retinoblastoma and pineal TRb. The laterality of intraocular retinoblastoma and its treatment were not associated with the age at which pineal TRb was diagnosed. The lead time from asymptomatic to symptomatic pineal TRb was approximately 1 year. By performing a screening magnetic resonance imaging scan every 6 months after the diagnosis of heritable retinoblastoma (median age, 6 months) until 36 months of age, at least 311 and 776 scans would be required to detect 1 case of asymptomatic pineal TRb and to save a single life, respectively. CONCLUSIONS:Patients with retinoblastoma are at risk of pineal TRb developing for a shorter period than previously assumed, and the age at diagnosis of pineal TRb is independent of the age at diagnosis of retinoblastoma. The GRADE (Grading of Recommendations Assessment, Development and Evaluation) level of evidence for these conclusions remains low.
Purpose: Attempted eye salvage for unilateral (cT2b/group D) retinoblastoma may risk tumor spread compared with primary enucleation. Identification of clinical features predictive of low histopathologic risk support safe trial salvage. Design: Retrospective, noncomparative single-institutional observational case series. Participants: Children with unilateral cT2b/group D retinoblastoma managed with primary enucleation at the Hospital for Sick Children, Toronto, Canada, January 2008 through February 2018. Methods: Data included clinical features (intraocular pressure, optic nerve obscuration, macular involvement, tumor seeding, and serous retinal detachment [RD] >1 quadrant), timing to enucleation, histopathologic features, and follow-up. Main Outcome Measures: Primary outcome was low-risk (LR; pT1/pT2) versus high-risk (HR; pT3/pT4) histopathologic features with clinicopathologic correlations. Secondary outcomes were positive predictive (probability that certain clinical features would predict LR histopathologic features) and negative predictive values (probability that absence of these clinical features would predict HR histopathologic features). Results: Thirty-eight eyes were eligible and showed vitreous seeding and normal intraocular pressure. The median diagnosis to enucleation interval was 4 days (range, 0-14 days). Histopathologic analysis diagnosed 4 (10.5%) HR and 34 (89.5%) LR eyes. High-risk eyes demonstrated massive choroidal invasion (4/38) or trans-scleral, extraocular, and postlaminar optic nerve invasion (1/38). Clinical findings included macular involvement (31/38), complete optic nerve obscuration (27/38), and RD (28/38). The proportion of eyes with HR histopathologic features was 13% (4/31; 95% confidence interval [CI], 1 %-25%) with macular involvement, 15% (4/27; 95% CI, 1 %-28%) with complete optic nerve obscuration, and 14% (4/28; 95% CI, 1 %-27%) with RD. The predictability of LR histopathologic features was 100% with macular sparing (7/7; 95% CI, 47%-100%), optic nerve visibility (10/10; 95% CI, 63%-100%), and less than 1 quadrant of RD (10/10; 95% CI, 63%-100%). In 1 child lacking all 3 clinical LR predictive features with HR histopathologic features (pT3a), metastases developed and the patient died; other children are alive and well (mean follow-up, 65 months). Conclusions: Presence of macular sparing, optic nerve visibility, less than 1 quadrant of RD, or a combination thereof predicted LR histopathologic features at primary enucleation, suggesting safe trial eye salvage. No clinical sign predicted HR histopathologic features. (C) 2019 by the American Academy of Ophthalmology
PURPOSE:Invisible retinoblastoma tumors are now detected with screening for retinal tumors in at-risk neonates (those inheriting RB1 pathogenic alleles from affected parents) using handheld OCT. Laser photocoagulation is challenging, requiring exact localization of a tumor invisible to indirect ophthalmoscopy and standard imaging. We describe OCT-guided localization and photocoagulation of these invisible tumors with 1-year follow-up. DESIGN:Retrospective, noncomparative, single-institutional, observational case series. PARTICIPANTS:Children with any clinically invisible retinoblastoma tumor that was detected on OCT posterior pole screening. METHODS:OCT revealed round homogeneous invisible tumors within the inner nuclear layer. Software calipers placed beside anatomic retinal landmarks (branched/curved vessels, fovea, or optic disc) mapped the tumor location and extent. A single laser (532 nm) burn flagged the location, and OCT evaluated the tumor-laser burn relationship; laser treatment was then continued in the correct location. Post-laser OCT ensured complete treatment. MAIN OUTCOME MEASURES:Accuracy (frequency of geographic miss and skip areas), effectiveness (recurrence rate), and burden (scar size and characteristics at final follow-up) of laser treatment. RESULTS:Eleven new invisible posterior pole tumors in 7 eyes of 5 children were treated by this technique. Localization and tumor-laser burn relationships were accurate in 11 of 11 tumors (100%, 95% confidence interval [CI], 49.9-100), and all showed swelling and hyper-reflectiveness of the tumor in post-laser OCT. Two photocoagulation sessions (2 weeks apart) were sufficient to successfully manage 9 of 11 tumors (82%, 95% CI, 37.4-100) with resulting permanent flat scars. One tumor (9%, 95% CI, 0.2-50.6) developed OCT-detected subclinical recurrences within 3 months, treated by 1 laser session. No treatment scar showed gliosis, foveal involvement, or retinal traction at 1-year follow-up. Scar expansion occurred in 1 tumor (9%, 95% CI, 0.2-50.6), and all scars (100%, 95% CI, 49.9-100) showed pigmentary changes. CONCLUSIONS:The OCT-guided localization and photocoagulation technique is valuable in achieving precision results in managing invisible new retinoblastoma tumors. This technique shows a potential to improve outcomes of secondary prevention screening for retinoblastoma.
Retinoblastoma is a childhood retinal malignancy affecting approximately 9,000 new patients globally per year (1). Leukocoria and strabismus are the most common presenting signs. However, retinobla...
Background: Carbohydrate fatty acid esters are found in important biological molecules such as bile acid 1-O-acyl galacto and bacterial glycolipids. The synthesis of glycomimetics of those biomolecules is required to study their structure-activity relationship. Method: In this work a series of carbohydrate fatty acid esters were regioselectively prepared mainly via protection and deprotection and tin-mediated strategies of partially benzyl-protected sugars, followed by hydrogenolysis of the benzyl groups. Results: We report here the regioselective synthesis of galactose palmitoyl esters along with other fatty acyl galactose glycomimetics namely fucose and lactose fatty esters. 1-O-palmitoyl-, 1,6-di-Opalmitoyl-D-galactose and 1-O-palmitoyl-L-fucose derivatives were prepared via a low-temperature esterification of the partially benzyl-protected galactose and fucose derivatives, respectively, followed by hydrogenolysis of the benzyl groups. The regioselective synthesis of 3'-O-acyl-D-lactosides of palmitic and nitrobenzoic acids was accomplished using a dibutyltin oxide activation strategy of partially protected lactoside derivatives. Conclusion: The regioselective synthesis of galactose, fucose and lactose fatty acid esters as glycomimetics of biological molecules containing the 1-O-fatty acyl-D-galactose moiety is reported in this work.