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1.
Int Ophthalmol ; 39(7): 1437-1443, 2019 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-29916121

RESUMEN

PURPOSE: To compare visual dysfunction between very preterm-born (VPB) children with no retinopathy of prematurity (no-ROP) at 6-10 years of age and age- and sex-matched full-term-born controls. METHODS: This is an observational, prospective study that included 30 children, 6-10 years of age, born ≤ 32 weeks of gestation, with no-ROP, and 30 age- and sex-matched full-term-born controls, conducted from January 2015 until August 2015. All children underwent complete ophthalmic evaluation. Main outcome measures include visual functions (best corrected visual acuity (BCVA), color vision, and stereoacuity), ocular alignment, refractive errors, and the presence of amblyopia and nystagmus. RESULTS: Mean BCVA of the right eyes was 0.04 ± 0.08 logMAR for VPB children and 0.02 ± 0.05 logMAR for the full-term children (P = 0.075). Mean BCVA for the left eyes was 0.07 ± 0.09 logMAR for VPB children and 0.02 ± 0.05 logMAR for the full-term children (P = 0.014). Refractive errors were slightly higher though not statistically significant in VPB children compared to full-term children (P = 0.125). The incidence of myopia and hypermetropia was 16.7 and 40%, respectively, in VPB children and 10 and 23.3%, respectively, in full-term children. Anisometropia found only in VPB children with an incidence of 16.7%. Amblyopia found in 10% of VPB children compared to 3.3% in full-term children. Strabismus was found equally in 10% of each group. CONCLUSION: VPB children with no-ROP are at an increased risk of developing decreased BCVA at least in one eye and anisometropia compared to age-matched full-term controls.


Asunto(s)
Visión de Colores/fisiología , Percepción de Profundidad/fisiología , Recien Nacido Extremadamente Prematuro , Trastornos de la Visión/fisiopatología , Agudeza Visual/fisiología , Estudios de Casos y Controles , Niño , Femenino , Estudios de Seguimiento , Edad Gestacional , Humanos , Masculino , Estudios Prospectivos
2.
Int Ophthalmol ; 35(6): 833-42, 2015 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-25701045

RESUMEN

The aim of this study was to investigate the presence of increased macular or retinal nerve fiber layer thickness (RNFLT) in amblyopic eyes, find if the increased macular or RNFLT is related to the lack of response in amblyopic eyes, and to explore whether the increased central macular thickness (CMT) in amblyopic eyes is purely related to the hyperopia. This is a prospective descriptive study. CMT and peripapillary RNFLT were measured by spectral-domain optical coherence tomography to evaluate 60 patients with unilateral-treated amblyopia (median age 11.00 year). Patients were divided into two groups: 33 patients in recovered amblyopia group and 27 patients in persistent amblyopia group. The mean CMT in the recovered group was 247.31 (±23.4) versus 246.8 (±32.7) µm (p = 0.95) for the persistent group. The mean peripapillary RNFLT was 99.13 (±12.1) versus 99.9 (±14.9) µm (p = 0.85) for the persistent group. In anisometropic amblyopia, there was no significant difference in CMT and RNFLT in either group. Also there was no relation between the type of refractive error and CMT or RNFLT. There was no significant difference in CMT and RNFLT in amblyopic eyes for both the recovered amblyopia group and the persistent amblyopia group to explain the lack of response in persistent amblyopic eyes. Additionally there was no relation between the type of refractive error and CMT or peripapillary RNFLT.


Asunto(s)
Ambliopía/patología , Fibras Nerviosas/patología , Retina/patología , Células Ganglionares de la Retina/patología , Adolescente , Niño , Preescolar , Estudios Transversales , Femenino , Humanos , Hiperopía/patología , Mácula Lútea/patología , Masculino , Estudios Prospectivos , Errores de Refracción/patología , Tomografía de Coherencia Óptica , Agudeza Visual
3.
Clin Ophthalmol ; 14: 113-118, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32021075

RESUMEN

PURPOSE: To assess residents' performance of phacoemulsification surgery and determine which steps of the procedure are most difficult to learn, and to measure rate of intraoperative complications. DESIGN: This was a prospective observational study. METHODS: Phacoemulsification surgery was divided into steps and each step was given a proficiency grade by the attending consultant. All intraoperative complications were recorded and analyzed. RESULTS: 200 cases performed by the Eastern Province ophthalmology program residents were evaluated. The most commonly encountered difficulty factors were hard nucleus (20.7%), small pupil (12.6%), and white cataract (10.3%). Capsulorhexis, nucleus disassembly and removal, and cortex removal were the most difficult steps to learn. General complication rate was 17.5%, and posterior capsular rupture was the most common complication (40%). Proficiency more than 90% of the time in each step was noted in residents with prior experience of more than 40 cases, except for nucleus disassembly. CONCLUSION: The study showed that nucleus disassembly remained the major obstacle in the residents' exponential learning curve of phacoemulsification surgery. Majority of complications occurred at level of capsulorhexis and cortical removal steps.

4.
Saudi J Med Med Sci ; 7(2): 110-113, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31080392

RESUMEN

A 54-year-old male presented to our ophthalmology clinic with a diagnosis of the right central retinal vein occlusion with macular edema. The patient was treated with dexamethasone intravitreal implant. Three days after the implant insertion, the patient experienced pain, redness and blurred vision in the same eye. With a provisional diagnosis of acute endophthalmitis, a combination of ceftazidime 2.25 mg/0.1 ml and vancomycin 1 mg/0.1 ml was injected intravitreally. One week after the treatment, endophthalmitis signs and symptoms subsided. This report presents a case of endophthalmitis following dexamethasone intravitreal implant, with a favorable outcome after treatment with intravitreal antibiotic injection without removal of the implant.

5.
Clin Ophthalmol ; 13: 707-714, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31114148

RESUMEN

Purpose: The study aims to describe the patterns of diabetic macular edema (DME) and their association with visual acuity using optical coherence tomography (OCT). Patients and methods: This is a retrospective observational study with chart review of patients with DME including both type 1 and 2 diabetics seen between January 2015 and January 2016. Main Outcome Measures: Type of diabetes, diabetes duration, best-corrected visual acuity, DME pattern, central macular thickness (CMT), and stage of diabetic retinopathy. DME was classified based on OCT scans into: sponge-like diffuse retinal thickness (SLDRT), cystoids macular edema (CME), and sub-retinal fluid (SRF). Results: 227 eyes (144 patients) were included. The SLDRT represented 67.84%, CME 19.82%, and presence of SRF 2.20%. OCT scan from 21 patients (22 eyes) displayed more than a single pattern. The CMT and visual acuities varied depending on the DME morphologic patterns. SLDRT was associated with the least affected mean visual acuity of 0.2±0.21. SRF signified the worst mean visual acuities. Increase in CMT significantly correlated with reduced visual acuity (P=0.005). A statistically significant positive correlations between diabetic stage-high risk proliferative diabetic retinopathy (PDR) and severe non-proliferative diabetic retinopathy (NPDR)-with the CMT (P=0.050) and (P=0.021) respectively, were observed. A significant positive correlation between the duration of diabetes, age and type 1 diabetes with visual acuity in LogMAR (P=0.003), (P=0.03), and (P=0.0005) respectively. Conclusions: SLDRT was the most common morphological subtype of DME patterns and increasing retinal thickness impaired the visual acuity. Older ages, longer diabetic duration and type 1 diabetes are considered significant risk factors for visual acuity impairment. The study also suggests that there is a significant correlation between the DME patterns and visual acuity.

6.
Saudi J Ophthalmol ; 32(3): 246-249, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-30224892

RESUMEN

A 4-month old healthy infant was brought by her parents to the emergency department with bloody tears of three days duration. There was also intermittent yellowish discharge since birth and a history of flu-like symptoms a week prior to presentation. Extensive investigations revealed no infection or other possible etiologies. The patient was treated with antibiotic eye drops and her condition resolved within a three-four days. In the literature, 15 cases with haemolacria of undermined source were reviewed; the median age of onset (12 years), bilateral involvement and female gender were more commonly encountered, and the most common associated illnesses were headache and epistaxis. The condition is self-limiting and spontaneous resolution is seen in majority of cases. Idiopathic haemolacria is a rare condition that can be presumed in patients presenting with bloody tears when all work-up turns to be negative. The condition is self-limiting with spontaneous resolution.

7.
Eur J Ophthalmol ; 27(6): 705-710, 2017 Nov 08.
Artículo en Inglés | MEDLINE | ID: mdl-28430330

RESUMEN

PURPOSE: To determine the long-term visual outcomes and risk factors for visual loss in children with primary congenital glaucoma (PCG) who underwent angle surgery over a 21-year period. METHODS: This was a retrospective study of patients who underwent trabeculotomy, trabeculectomy, or combined trabeculotomy-trabeculectomy for PCG. Visual acuity (VA), surgeries, and underlying cause of visual impairment were recorded and compared. Main outcome measures were final best-corrected good VA (20/20 to 20/50), moderate VA (<20/50 to 20/200), or poor VA (<20/200); age at surgery, sex, laterality, type of initial glaucoma surgery, and number of surgeries were recorded. RESULTS: Fifty-three eyes were eligible to be included in the study. The mean logMAR VA was 0.61 ± 0.57. A good VA was attained in 51%, moderate VA in 30%, and poor VA in 19%. The main cause of visual impairment with VA <20/50 was deprivation amblyopia (64%). There was no association between VA level and status of success (controlled with or without medications) (p = 0.202). The mean spherical equivalent of refraction was -4.47 ± 5.66; myopia was the predominant refractive error (74%) and astigmatism >2 D (40%). A statistically significant high myopic shift was more frequent in the visually impaired group. None of the studied factors was statistically associated with moderate to poor visual outcome (all p>0.05). CONCLUSIONS: A favorable VA outcome was achieved. Topical antiglaucoma medication has an adjuvant role in maintaining the success rate of surgery without risking the visual outcome. Corneal opacification and anisometropia were the cause of amblyopia.


Asunto(s)
Glaucoma/complicaciones , Baja Visión/etiología , Ambliopía/complicaciones , Anisometropía/complicaciones , Antihipertensivos/uso terapéutico , Astigmatismo/complicaciones , Niño , Preescolar , Femenino , Estudios de Seguimiento , Glaucoma/tratamiento farmacológico , Glaucoma/cirugía , Humanos , Presión Intraocular/fisiología , Masculino , Miopía/complicaciones , Miopía Degenerativa/complicaciones , Degeneración Retiniana/cirugía , Estudios Retrospectivos , Factores de Riesgo , Trabeculectomía/métodos , Agudeza Visual/fisiología
8.
Clin Interv Aging ; 12: 1313-1330, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-28860733

RESUMEN

BACKGROUND: Visual impairment in elderly people is a considerable health problem that significantly affects quality of life of millions worldwide. The magnitude of this issue is becoming more evident with an aging population and an increasing number of older individuals. OBJECTIVE: The objective of this article was to review the clinical and pathological aspects of age-related macular degeneration (AMD), diagnostic tools, and therapeutic modalities presently available or underway for both atrophic and wet forms of the disease. METHODS: An online review of the PubMed database was performed, searching for the key words. The search was limited to articles published since 1980 to date. RESULTS: Several risk factors have been linked to AMD, such as age (>60 years), lifestyle (smoking and diet), and family history. Although the pathogenesis of AMD remains unclear, genetic factors have been implicated in the condition. Treatment for atrophic AMD is mainly close observation, coupled with nutritional supplements such as zinc and antioxidants, whereas treatment of wet AMD is based on targeting choroidal neovascular membranes. CONCLUSION: Identification of modifiable risk factors would improve the possibilities of preventing the progression of AMD. The role of anti-vascular endothelial growth factor (anti-VEGF) agents has transformed the therapeutic approach of the potentially blinding disease "wet AMD" into a more favorable outcome.


Asunto(s)
Degeneración Macular/tratamiento farmacológico , Degeneración Macular/patología , Factores de Edad , Anciano , Antioxidantes/uso terapéutico , Suplementos Dietéticos , Conductas Relacionadas con la Salud , Humanos , Estilo de Vida , Degeneración Macular/diagnóstico , Anamnesis , Calidad de Vida , Factores de Riesgo , Factor A de Crecimiento Endotelial Vascular/antagonistas & inhibidores , Degeneración Macular Húmeda/tratamiento farmacológico , Degeneración Macular Húmeda/patología , Zinc
9.
Saudi Med J ; 38(1): 63-69, 2017 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-28042632

RESUMEN

OBJECTIVES: To determine the macular thickness in the eyes of healthy Saudi adults using spectral-domain optical coherence tomography (SD-OCT). Methods: This is a prospective, cross-sectional study, including 158 healthy participants between August and December 2015. Mean subject age was 29.9 ± 7.85 years old. All participants underwent full ophthalmic evaluation, including SD-OCT imaging, and axial length measurement. Data from the right eye were included. Mean retinal thickness was determined. Correlations between retinal thickness and gender, age, axial length, and spherical equivalence were analyzed. Results: Mean central retinal thickness was 244.76 ± 23.62 µm, mean axial length was 23.8 ± 1.062 mm (range: 20.5-29 mm) and mean spherical equivalent was -0.31 ± 1.75 diopters (D) (range: -5.50 to +4.25 D). Central subfield (CSF) thickness and foveal volume were significantly lower in women than in men (both p less than 0.001). Data from the various age groups did not show statistically significant differences in the CSF thickness (p=0.389) or foveal volume (p=0.341). A positive correlation between CSF thickness and axial length (p less than 0.001) was observed.  Conclusion: The normal macular thickness values in healthy Saudi individuals is different from that reported in other ethnic groups, as obtained by SD-OCT. Saudi men had thicker CSF than Saudi women and axial length was positively correlated to the central foveal thickness.


Asunto(s)
Mácula Lútea/anatomía & histología , Adolescente , Adulto , Estudios Transversales , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Arabia Saudita , Tomografía de Coherencia Óptica , Adulto Joven
10.
Acta Ophthalmol ; 94(2): 122-34, 2016 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-26249675

RESUMEN

Bleb-related infection (BRI) is one of the serious complications of glaucoma filtering surgery. This literature review is aimed at the evaluation of recent studies related to BRI. The review presented and discussed risk factors, and clinical and laboratory diagnosis emphasizing advances in diagnostic techniques to detect and distinguish the disease as well as to initiate immediate intensive antibacterial treatment. Clinical features of recognized prognostic factors of visual outcome were also presented. The studies showed that the prognosis of blebitis is usually good, unless infection has progressed to endophthalmitis that would signify a poorer prognosis. Despite prompt and intensive treatment of patients with bleb-related endophthalmitis, the outcomes remain unsatisfactory especially with virulent organisms and low initial visual acuity. It has been recommended that early detection and treatment of risk factors and thorough patient education are indispensable in ensuring best prognosis in post-trabeculectomy patients.


Asunto(s)
Endoftalmitis/etiología , Infecciones Bacterianas del Ojo/etiología , Cirugía Filtrante/efectos adversos , Infección de la Herida Quirúrgica/etiología , Endoftalmitis/epidemiología , Endoftalmitis/terapia , Infecciones Bacterianas del Ojo/epidemiología , Infecciones Bacterianas del Ojo/terapia , Glaucoma/cirugía , Humanos , Incidencia , Factores de Riesgo , Infección de la Herida Quirúrgica/epidemiología , Infección de la Herida Quirúrgica/terapia
11.
Saudi J Ophthalmol ; 30(1): 44-8, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-26949358

RESUMEN

PURPOSE: To determine the distribution of intraocular pressure (IOP) and its association with age, gender and refractive error in non-glaucomatous Saudi participants. DESIGN: Hospital-based cross-sectional observational study during Vision Day Screening Program. PARTICIPANTS: 458 participants living in the Al-Khobar, Saudi Arabia. METHODS: Recruited participants (aged 20 years or over) underwent a comprehensive questionnaire and ocular examination, including measurement of IOP with Perkins hand-held applanation tonometry and autorefraction. MAIN OUTCOME MEASURES: The distribution of IOP of either of the eyes (right or left eye by randomization) and associations with age, gender and refractive error. RESULTS: Median IOP was 15.0 (range: 6-28) mmHg in the total population. There is no significant difference between the overall IOP of male participants, median 15 (range: 6-28) mmHg and female participants, median 16 (range: 6-28) mmHg (p = 0.180). No statistically significant difference in IOP in relation to age comparing 20-45 years group to 46-69 years group was documented (p = 0.751). There was no statistically significant relationship between refractive error category and IOP (p = 0.405). Ocular hypertension with IOP > 21 mmHg was found in 8.7% of the participants. CONCLUSION: Variation in IOP by gender, age group and type of refractive error was not statistically significant. The observations need confirmation by study with larger sample representing Saudi population.

12.
Eur J Ophthalmol ; 26(6): 581-587, 2016 Nov 04.
Artículo en Inglés | MEDLINE | ID: mdl-27033964

RESUMEN

PURPOSE: To describe demographic criteria of primary congenital glaucoma (PCG) subjects and to evaluate the surgical outcomes of children who underwent angle surgery over a 20-year period. METHODS: This is a retrospective study of patients diagnosed with PCG who underwent trabeculotomy, trabeculectomy, or combined trabeculotomy-trabeculectomy (CTT). The surgery was considered to be successful if intraocular pressure (IOP) was 21 mm Hg or less with or without treatment. RESULTS: A total of 148 eyes (85 patients) were included in the study. The majority of the patients were younger than 6 months of age (78.9%), with a median age of 2 months. Sex distribution was relatively even between girls and boys (45:40) (53%/47%). Bilateral disease was seen in 63 patients (74%). The overall success rate was achieved in 80.4%. The majority of the eyes needed only one surgery (105 [70.9%]), 34 eyes (23.0%) needed 2 surgeries, and 7 eyes (4.7%) had 3 surgeries. There was no statistically significant difference in success rate between types of surgery and number of performed procedures. A progressive decline in success rate over time was evident, as success rate dropped from 96.6% at 5 months to less than 50% after 11 years of follow-up. CONCLUSIONS: Excellent IOP control can be achieved in a majority of patients with equally effective results from all 3 surgical procedures. The surgical outcome of PCG was more favorable in infants presenting before the age of 6 months. Adjuvant topical antiglaucoma medications can augment the surgical success rate.


Asunto(s)
Cuerpo Ciliar/cirugía , Hidroftalmía/cirugía , Coagulación con Láser , Trabeculectomía/métodos , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Hidroftalmía/fisiopatología , Lactante , Recién Nacido , Presión Intraocular/fisiología , Masculino , Estudios Retrospectivos , Tonometría Ocular , Resultado del Tratamiento
13.
Saudi J Ophthalmol ; 30(3): 180-184, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-28210179

RESUMEN

PURPOSE: The aim of this study was to determine and relate corneal biomechanical metrics with demographic, tomographic and refractive data in healthy Saudi people. DESIGN: Prospective, cross-sectional, observational study. PARTICIPANTS: The study included 215 normal Saudi adult individuals. METHODS: Corneal hysteresis (CH) and corneal resistance factor (CRF), Goldmann-correlated intraocular pressure (IOPg) and corneal-compensated intraocular pressure (IOPcc) were measured by ocular response analyzer. The central keratometry (CK), central corneal thickness (CCT), and anterior chamber depth (ACD) were measured using Pentacam system. The spherical equivalent of refraction (SER) was obtained by an Auto-refractometer. RESULTS: The mean CH was 11.16 ± 2.11, CRF was 11.07 ± 2.31 and IOP was 15.12 ± 3.5. Mean CK, ACD, CH and CRF were distinct among gender with the significant P values of 0.05, 0.006, 0.020, and 0.047 respectively. CRF was negatively correlated with ACD (r = -0.146, P = 0.032). A positive correlation was found between CRF and SER (r = 0.176, P = 0.010), CCT (r = 0.447, p = 0.000) and CH (r = 0.878 and p = 0.000). CH was negatively correlated with IOPcc (-0.433, p = 0.000). A positive correlation was found between CH and ACD (r = -0.14, p = 0.044), SER (r = 0.617, p = 0.014), CCT (r = 0.412, p = 0.000) and IOPg (r = 0.183, p = 0.007). CONCLUSION: This study demonstrated a distinct difference among gender values of corneal hysteresis and corneal resistance factor being higher in female Saudi subjects. CH and CRF values were higher in Saudi subjects than values in other populations. This may suggest the presence of ethnic differences in ocular parameters and support the importance of establishing population norms for corneal biomechanical parameters.

14.
J Family Community Med ; 22(3): 158-62, 2015.
Artículo en Inglés | MEDLINE | ID: mdl-26392796

RESUMEN

PURPOSE: The purpose of this cross-sectional observational study was to determine the distribution and patterns of refractive errors, strabismus, and amblyopia in children seen at a pediatric eye care. MATERIALS AND METHODS: The study was conducted in a Private Hospital in Dammam, Kingdom of Saudi Arabia, from March to July 2013. During this period, a total of 1350 children, aged 1-15 years were seen at this Center's Pediatric Ophthalmology Unit. All the children underwent complete ophthalmic examination with cycloplegic refraction. RESULTS: Refractive errors accounted for 44.4% of the cases, the predominant refractive error being hypermetropia which represented 83%. Strabismus and amblyopia were present in 38% and 9.1% of children, respectively. CONCLUSIONS: In this clinic-based study, the focus was on the frequency of refractive errors, strabismus, and amblyopia which were considerably high. Hypermetropia was the predominant refractive error in contrast to other studies in which myopia was more common. This could be attributed to the criteria for sample selection since it was clinic-based rather than a population-based study. However, it is important to promote public education on the significance of early detection of refractive errors, and have periodic screening in schools.

15.
Ann Saudi Med ; 35(5): 387-93, 2015.
Artículo en Inglés | MEDLINE | ID: mdl-26506973

RESUMEN

BACKGROUND AND OBJECTIVES: Keratoplasty services in Saudi Arabia have progressed steadily in the past few decades. We sought to identify the leading indications and types of keratoplasty performed in the Eastern Province of Saudi Arabia over a six-year period and to compare these indications with published data. DESIGN AND SETTING: This was a retrospective descriptive analysis of the records of patients who underwent keratoplasty at four ophthalmology centers in the Eastern Province between 2008 and 2013. PATIENTS AND METHODS: All keratoplasty procedures were included in the analysis. The primary surgical indication and type of surgery were identified for each case. RESULTS: Keratoplasties included 570 penetrating keratoplasty, 217 deep lamellar keratoplasty, 80 triple procedures, 24 descemet stripping automated endothelial keratoplasty and 12 Boston keratoprosthesis implantations. The mean age of all patients was 28.8 years (range 14-72 years), and 58.9% of the patient were males. The lead.ing indication for keratoplasty was keratoconus 53.10%, bullous keratopathy 13.80%, corneal scarring 10.7%, regrafts 9.1%, and stromal dystrophies 4.9%. CONCLUSIONS: In this study, the leading indications for keratoplasty were keratoconus, bullous keratopathy, corneal scarring, regrafts and stromal dystrophies. A significant increasing trend for descemet's stripping automated endothelial keratoplasty surgeries was observed in spite of the number of cases.


Asunto(s)
Enfermedades de la Córnea/cirugía , Trasplante de Córnea/estadística & datos numéricos , Queratoplastia Endotelial de la Lámina Limitante Posterior/estadística & datos numéricos , Adolescente , Adulto , Anciano , Cicatriz/cirugía , Trasplante de Córnea/métodos , Queratoplastia Endotelial de la Lámina Limitante Posterior/métodos , Femenino , Humanos , Queratocono/cirugía , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Arabia Saudita , Adulto Joven
16.
Saudi J Ophthalmol ; 26(2): 229-34, 2012 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-23960997

RESUMEN

We report a family of three siblings followed between 2005 and 2011 with bilateral combined hamartoma of the retina and retinal pigment epithelium, with the age of diagnosis ranging from 7 to 13 years. The main reason for consultation was reduction of vision and squint. The diagnosis was determined based on the clinical findings on fundus examination: increased pigmentation at the macula with slightly elevated, gray-white macular lesion, tortuosity of perimacular blood vessels and glial epiretinal membrane. The elder brother was found to have left posterior subcapsular cataract. He was also confirmed to have neurofibromatosis type 1, the youngest sister fit in the diagnostic criteria for neurofibromatosis type 1, while the middle sister was presumed to have neurofibromatosis type 1. Follow-up showed stability of the retinal lesion in the three cases, with the progression to develop right posterior subcapsular cataract in the elder sister. This report is aimed to demonstrate that the occurrence of bilateral combined hamartoma of the retina and retinal pigment epithelium could raise the possibility of associated neurofibromatosis.

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