Presence of cataract in patients treated for retinoblastoma at the national institute of pediatrics in Mexico (2011-2021).

PURPOSE: The goal of this study is to describe the presence of secondary cataract in patients with retinoblastoma treated at the National Institute of Pediatrics of Mexico (INP) over the past 10 years. METHODS: This was a single center observational, retrospective and descriptive study. We included all eyes diagnosed with retinoblastoma and cataract between June 2011 and June 2021. RESULTS: In total, 833 records of patients diagnosed with Retinoblastoma at the National Institute of Pediatrics during the period between June 2011 and June 2021 were reviewed. Out of all of them, only 14 developed cataract (1.6%). The median age at retinoblastoma diagnosis was 10.5 months (Rank: 6-13 months), and the median age at cataract diagnosis was 51.5 months (Rank: 25-73 months). The majority (13, 92.9%) of the patients had bilateral involvement. 42% of the eyes were Stage D according to the international classification of retinoblastoma. Cryotherapy was applied in 57.1%, intravitreal chemotherapy in 85.7%, radiation therapy in 42.6%, and only 7.1% of cases were treated with intra-arterial chemotherapy. CONCLUSIONS: The presence of cataract in patients with retinoblastoma is a rare but important entity impacting the development of vision in children and detection of intraocular tumors. These probably occur late as a result of the multiple treatments to which the children have been subjected, without being able to determine in this study which is the risk factor most associated with the development of this pathology.

Bloodstream infection by Rhodococcus corynebacterioides in a pediatric patient diagnosed with high-risk retinoblastoma.

Rhodococcus is a pathogen that is known to cause infections in animals and humans, mainly in cases of immunocompromised patients. A case of a pediatric cancer patient suffering from a bloodstream infection caused by Rhodococcus corynebacterioides was described in this work. Gram positive rods were isolated from blood cultures. The target bacterium was identified using a combination of biochemical tests, the MALDI-TOF mass spectrometry technique, and the analysis of the 16S rRNA sequence. Moreover, an antimicrobial susceptibility test was performed using the E-test. The isolated bacterium was identified as R. corynebacterioides. The 3-year-old patient was successfully treated with vancomycin and meropenem. This is the first published report of R. corynebacterioides in a pediatric patient diagnosed with retinoblastoma that developed a bloodstream infection. R. corynebacterioides should be considered among the opportunistic infectious agents affecting pediatric cancer patients.

Subsequent malignant neoplasms in the pediatric age in retinoblastoma survivors in Argentina.

BACKGROUND: Retinoblastoma survivors in low- and middle-income countries are exposed to high-intensity treatments that potentially place them at higher risk of early subsequent malignant neoplasms (SMNs). METHODS: We followed 714 (403 [56.4%] nonhereditary and 311 [43.5%] hereditary) retinoblastoma survivors diagnosed from August 1987 to December 2016, up to the age of 16 years. We quantified risk of SMNs with cumulative incidence (CI) and standardized incidence ratios (SIR) analysis. Multivariate regression Cox model was used to determine the association of treatments and risk of SMNs. RESULTS: Median follow-up was of 9 years (range: 0.18-16.9) and 24 survivors (3.36%) developed 25 SMNs (n = 22 hereditary, n = 2 nonhereditary). SMNs included sarcomas (osteosarcomas, Ewing sarcomas, rhabdomyosarcomas; n = 12), leukemias (n = 5), and central nervous system tumors (CNS; n = 3). All cases of acute myeloid leukemia (AML) and most of Ewing sarcomas occurred within 5 years of retinoblastoma diagnosis. The type of SMN was the main indicator of mortality (five of five patients with leukemias, six of 12 with sarcomas, and zero of three with CNS tumors died). Compared to the general population, radiation increased the risk of Ewing sarcoma in hereditary survivors by 700-fold (95% CI = 252-2422.6) and chemotherapy increased the risk of AML by 140-fold (95% CI = 45.3-436). The CI of SMNs for hereditary survivors was 13.7% (95% CI = 8.4-22.1) at 15 years. CONCLUSION: Retinoblastoma survivors from Argentina are at higher risk of developing SMNs early in life compared to the general Argentinean population, especially those treated with radiation plus chemotherapy. AML and Ewing sarcoma presented within 5 years of retinoblastoma diagnosis are associated with chemotherapy and radiation exposure.

SOLITARY RETINAL CAPILLARY HEMANGIOMA IN A PATIENT WITH BILATERAL CHORIORETINAL COLOBOMA.

PURPOSE: To report a case showing a very rare association of chorioretinal coloboma and retinal capillary hemangioma in a previously healthy patient. METHODS: Observational case report. RESULTS: A 21-year-old woman presented at our clinic for vision screening. She had a history of bilateral chorioretinal coloboma with amblyopia in her left eye. Her best-corrected visual acuity was 20/25 in her right eye and 20/200 in her left eye. On slit-lamp examination, iris coloboma was observed in the left eye. Fundus evaluation showed inferior chorioretinal coloboma in both eyes, and an elevated, round, and orange-red retinal lesion with feeder vessels localized in the midperipheral region of the right eye. The patient was diagnosed as having solitary retinal capillary hemangioma and underwent argon laser therapy for treating the tumoral lesion. Diagnostic studies were negative for von Hippel-Lindau disease. This is the first reported case of solitary retinal capillary hemangioma associated with bilateral chorioretinal coloboma. CONCLUSION: Chorioretinal coloboma is a congenital defect of the eye caused by improper closure of the embryonic fissure. Retinal capillary hemangioma is a vascular retinal tumor that may occur sporadically or as part of the von Hippel-Lindau syndrome. We report a rare association of chorioretinal coloboma and retinal capillary hemangioma in a previously healthy patient.

Preserving vision in retinoblastoma through early detection and intervention.

The most frequent neoplasm of the eye in children is retinoblastoma. It is a rare disease with an average incidence of 1 in 14,000 to 18,000 live births. The incidence is greater in developing countries. Early detection may allow widespread use of eye-sparing treatments, minimizing the morbidity of this disease. Although leukocoria is usually the first sign that parents notice especially when a flash photograph is taken, the diagnosis is not performed since this presentation is not known by health care providers and the public. Improving public awareness and knowledge of the early signs may lead to early diagnosis, which can save more eyes and lives. Advances in both the basic science and clinical applications of new therapies continue to emerge for this rare cancer. We expect that all conservative treatments will be available worldwide and consequently more children will be cured and maintain the vision.

Descolamento da retina após tratamento do retinoblastoma / Rhegmatogenous retinal detachment after succesful treatment of retinoblastoma

OBJETIVO: Avaliar a apresentação de descolamento de retina nos pacientes tratados de retinoblastoma no Centro Infantil Dr. Domingos Boldrini - Campinas (SP), Brasil. MÉTODOS: Estudo retrospectivo de 220 pacientes submetidos a tratamento clínico e cirúrgico de retinoblastoma, no período de janeiro de 1978 a dezembro de 2008. RESULTADOS: Encontramos dois pacientes com descolamento de retina após tratamento de retinoblastoma sem atividade tumoral, corrigidos com sucesso pelo método cirúrgico de cerclagem+criopexia. CONCLUSÃO: É possível a correção de descolamento secundário de retina nos pacientes portadores de retinoblastoma, sem atividade tumoral.

PURPOSE: To evaluate the characteristics and evolution of the retinal detachment developed after successful treatment of retinoblastoma. METHODS: A retrospective analysis of 220 consecutive patients treated for retinoblastoma from january 1978 to december 2008 disclosed two patients who developed retinal detachment during the follow-up. RESULTS: Retinal detachment was found in two patients several months after the treatment of retinoblastoma. Both were managed by surgery with restoration of their monocular vision. CONCLUSION: Surgical management of retinal detachment is indicated to preserve visual function and quality of life in patients successfully treated for retinoblastoma.

Bilateral central retinal vein occlusion associated with multiple myeloma.

PURPOSE: To report a case of simultaneous bilateral central retinal vein occlusion (CRVO) associated with multiple myeloma. METHODS: A 65-year-old woman had sudden, painless loss of vision in both eyes for 20 days. Ophthalmologic examination revealed bilateral CRVO. Appropriate medical workup was conducted, and multiple myeloma was diagnosed as the underlying cause. RESULTS: Clinical support and chemotherapy effectively controlled paraprotein production, leading to improvement of both systemic and ocular alterations. CONCLUSIONS: Many conditions have been noted to be associated with CRVO. Based on a Medline search, this is the first report of simultaneous bilateral CRVO as the first manifestation of multiple myeloma, illustrating the need for a primary care ophthalmologist to be involved in the basic assessment for associated underlying diseases in retinal disorders.

Combined hamartoma of the retina and retinal pigment epithelium associated with neurofibromatosis type-1.

BACKGROUND: Retinal tumors are occasionally seen in patients with neurofibromatosis type-1 (NF-1). These are usually astrocytic hamartomas or angiomas. Combined hamartoma of the retina and retinal pigment epithelium are tumors also described in NF-1, but this association has not been definitively established yet. METHOD: We report on a child with NF-1, who presented a combined hamartoma of the retina and retinal pigment epithelium in both eyes. RESULTS: The diagnosis of bilateral combined hamartoma of the retina and retinal pigment epithelium in our patient was performed on the basis of the ophthalmoscopic appearance of the lesions. NF-1 was diagnosed following the current international clinical criterion, supplemented by neuroimaging findings. CONCLUSION: Despite the extreme rarity of this association, we believe that it is not coincidental, as the presence of a hamartomatous retinal lesion in a patient with a systemic hamartomatous neuroectodermic disease would be, at least, rational. Thus, NF-1 must be excluded in patients with combined hamartoma of the retina and retinal pigment epithelium.