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1.
Eur J Ophthalmol ; : 11206721241253305, 2024 May 06.
Artículo en Inglés | MEDLINE | ID: mdl-38710194

RESUMEN

PURPOSE: To study the outcomes of diode laser transscleral cyclophotocoagulation (TSCPC) with trans-corneal transillumination using a novel low-cost torchlight method in refractory glaucoma. METHODS AND ANALYSIS: This prospective interventional study included patients with refractory glaucoma who underwent TSCPC with trans-corneal transillumination (TSCPC-TI) using a novel low-cost torchlight method. Patients completing a minimum 6-month follow-up were analyzed. They were compared to a historical control group of patients who underwent TSCPC without transillumination (TSCPC-No TI) at 6-month follow-up period. We analyzed the mean laser energy delivered, post-laser intraocular pressure (IOP) reduction, number of antiglaucoma medications (AGM), the requirement of retreatment and complications of the procedure in both groups. RESULTS: 32 eyes of 29 patients comprised the TSCPC-TI group and were compared with 39 eyes of 37 patients in the TSCPC-No TI group. The TSCPC-TI group required lower energy than the TSCPC-No TI group (46.15 ± 22.8 Vs 80.65 ± 56.1 J p < 0.001). At 6-month follow-up, the TSCPC-TI group required lesser AGM for IOP control (2.33 ± 1.02 vs 3.02 ± 1.32 p = 0.01). There was a significantly reduced dependence of oral acetazolamide in the TSCPC-TI group at 6 months follow-up (15.6% vs 41% p = 0.03%). The success and response rates were 71.8% Vs 23.1%; p < 0.0001 and 87.5% Vs 51.2%; p = 0.001 significantly high in the TSCPC-TI group. The TSCPC-No TI group had a significantly high failure rate (12.5% Vs 48.2% p = 0.001). Hypotony (n = 1) and phthisis (n = 2) were noted TSCPC-No TI group. CONCLUSIONS: TSCPC with transillumination with a low-cost torchlight resulted in a more efficient and effective cycloablation than TSCPC without transillumination.

2.
J Glaucoma ; 2024 May 06.
Artículo en Inglés | MEDLINE | ID: mdl-38709205

RESUMEN

PRCIS: Ab interno bleb revision using the Grover and Fellman spatula offers potential benefits in improved intraocular pressure control and spare the conjunctiva for enhanced bleb management. PURPOSE: This study aimed to evaluate the safety and effectiveness of Ab-Interno Bleb Revision (AIBR) for treating failed trabeculectomy blebs. METHODS: This non-comparative case series enrolled glaucoma patients with failed trabeculectomy blebs, who underwent AIBR and were followed up for one year. Eligible patients received subconjunctival Mitomycin C 0.1 mL of 0.2 mg/mL (20 µg) five days before the AIBR, performed using the Grover and Fellman spatula. The primary outcome measures were intraocular pressure (IOP) and the usage of IOP-lowering medications one year after the procedure. Secondary outcomes included the procedure's failure rate and complications. Complete success was defined as achieving an IOP between 5-21 mmHg without medication, while qualified success required additional medical treatment. RESULTS: The study comprised 23 eyes from 23 patients, having a mean age of 59.66±14.93 years. Preoperatively, the mean IOP was 23.54±10.7 mmHg, and the mean anti-glaucoma medication requirement was 3.99±0.65, both significantly reduced to 15.7±6.9 mm Hg (P=0.009) and 1.26±1.2 (P <0.001) at one year, respectively. Overall, 19 eyes (82.6%) achieved success (Complete success: 39.2%, Qualified success: 43.4%), while in 4 (17.4%) patients the procedure failed (IOP >21 mmHg). 5 patients had transient hyphema, but there were no sight-threatening complications such as hypotony or choroidal detachment. CONCLUSION: Ab-Interno Bleb Revision (AIBR) is a safe, effective, and minimally invasive intervention for managing failed trabeculectomy blebs. By eliminating the need to reopen the conjunctiva, this technique offers a promising alternative for the treatment of this challenging condition.

3.
Indian J Ophthalmol ; 2024 May 20.
Artículo en Inglés | MEDLINE | ID: mdl-38770604

RESUMEN

PURPOSE: To study the morphological and histological characteristics of the fibrous capsule formed around a novel biodegradable glaucoma drainage device (GDD) implant made of oxidized regenerated cellulose (ORC) after 1 and 3 months of its implantation. METHODS: It was a prospective, interventional, preclinical experimental study using New Zealand white rabbits. All animals underwent GDD implantation with a novel biodegradable device. The device's body was made of ORC and attached to a silicone tube, connecting the body to the anterior chamber. Histopathology (hematoxylin and eosin/Masson's trichrome staining) and immunohistochemistry (Alpha-Smooth Muscle Actin expression) characteristics of the bleb formed around the novel device were noted at 1 and 3 months. RESULTS: One month post implantation, the biodegradable material produced a significant foreign body type of reaction evidenced by the exuberant infiltration by macrophages, lymphocytes, and multinucleated giant cells. The granulomatous response subsided by 3 months with disorganized collagen deposition on Masson's trichrome staining. The silicone tube was an internal control, and histopathology demonstrated well-organized collagen deposition around it at 3 months. Immunohistochemistry for α-smooth muscle actin also demonstrated more myofibroblast transformation at the site of the tube than the biodegradable implant. CONCLUSIONS: Our results indicate that the tissue response around a biodegradable GDD was different from the response to conventional devices. A diffuse, loose vascularized mesh was observed to develop, which may be more compatible with sustained IOP control over a longer period, in contrast to the usual thick and dense capsule formed around the non-biodegradable devices.

4.
Indian J Anaesth ; 68(5): 480-485, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38764961

RESUMEN

Background and Aim: Sevoflurane, a preferred anaesthetic for children, exhibits a dose-dependent reduction in intraocular pressure (IOP). However, consensus is lacking regarding optimal end-tidal sevoflurane concentration for safe IOP measurement. This study aimed to identify the concentration at which IOP measurement could be attempted without inducing movements in paediatric patients after inhalational induction. Methods: Two paediatric groups (1-12 months and 12-36 months) with glaucoma undergoing examination under anaesthesia were recruited. After induction with 8% sevoflurane and 100% oxygen, the first child had an end-tidal sevoflurane concentration maintained at 2% for 4 min, followed by IOP measurement. Success was defined as 'no movement', and subsequent concentrations (adjusted in 0.2% steps) were determined using the Dixon and Massey method based on the previous patient's responses. Results: The study included 75 children. The effective concentration of sevoflurane causing 'no movement' during IOP measurement in 50% of the study population for successful IOP measurement was 1.98% (95% confidence interval [CI] 1.63, 2.17, P = 0.017) for 1-12 months group and 0.55% (95% CI 0.39, 0.66, P = 0.002) for 12-36 months group. Probit regression analysis yielded effective concentration of sevoflurane causing 'no movement' during IOP measurement in 95% of the study population values of 2.47% (95% CI 2.24, 4.58, P = 0.017) for 1-12 months group and 0.94% (95% CI 0.78, 1.57, P = 0.002) for 12-36 months group. Conclusion: In paediatric patients, a higher end-tidal sevoflurane concentration of 2% is needed for IOP measurement in 1-12 months age group compared to 0.5% required in 12-36 months age group, achieving success in 50% of the study population.

6.
J AAPOS ; 28(2): 103863, 2024 04.
Artículo en Inglés | MEDLINE | ID: mdl-38458600

RESUMEN

PURPOSE: To investigate the correlation between swept-source anterior segment optical coherence tomography (AS-OCT) and ultrasound biomicroscopy (UBM) in congenital corneal opacity (CCO). METHODS: All children with unilateral or bilateral congenital corneal opacities who underwent examination under anesthesia (EUA) and anterior segment optical coherence tomography (AS-OCT) imaging from January 1, 2022, to December 31, 2022, were included. Main outcome measures were corneal and anterior segment evaluation and correlation of UBM and AS-OCT findings. RESULTS: A total of 22 eyes of 15 patients were imaged using both technologies. The age at first EUA ranged from 11 days to 4 years. Different phenotypes were classified based on the clinical examination, UBM, and AS-OCT findings. Fourteen eyes were diagnosed with Peters anomaly, congenital corneal staphyloma was observed in 4 eyes, 2 eyes had coloboma, 1 eye had peripheral sclerocornea, and 1 eye was diagnosed with congenital primary aphakia. AS-OCT and UBM findings were closely correlated in 18 of 22 eyes (82%) but AS-OCT failed to provide detailed information in 4 eyes (18%) where UBM revealed more details. CONCLUSIONS: Although AS-OCT offers valuable preliminary data for initial assessment and counseling, it may not consistently provide precise assessments in all cases. Therefore, UBM should be considered for definitive evaluation.


Asunto(s)
Opacidad de la Córnea , Microscopía Acústica , Niño , Humanos , Recién Nacido , Microscopía Acústica/métodos , Tomografía de Coherencia Óptica/métodos , Opacidad de la Córnea/diagnóstico por imagen , Segmento Anterior del Ojo/diagnóstico por imagen , Córnea/diagnóstico por imagen
9.
Semin Ophthalmol ; 39(3): 235-241, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38404174

RESUMEN

PURPOSE: To evaluate the long-term efficacy of selective laser trabeculoplasty (SLT) in eyes with primary angle-closure (PAC) and primary angle-closure glaucoma (PACG) following a laser peripheral iridotomy (LPI). METHOD: In this prospective cross-sectional study, 45 eyes of 34 patients with PAC/PACG diagnosis, uncontrolled intraocular pressure (IOP), and visible pigmented trabecular-meshwork (TM) at least 180° on gonioscopy following a LPI were recruited. Following a detailed baseline ophthalmic evaluation, all eligible eyes underwent SLT, and the patients were examined on day1, at 1 week, 1-, 3-, and 6-months, and 1-, 2-, 3-, 4-, and 5-year subsequently. The main outcomes measured were IOP, number of IOP-lowering agents, and complications. RESULTS: The mean age of the cohort was 57.80 ± 6.44 years, the male-female ratio was 8:26, and 17 eyes were PACG, and 28 were PAC. The baseline IOP was 23.81 ± 1.78 mm Hg, and was significantly declined at all follow-ups (p < .0001). The cumulative probability of overall success was 91% and 84% at 2-, and 5-year, respectively. At 5-year SLT provided drug-freedom in 80% of PAC and 23% of PACG eyes. Six eyes had IOP spike at 1-week and two patients underwent repeat SLT after 1-year. No other complications, such as pain/discomfort, inflammation, an increase in peripheral anterior synechiae and cystoid-macular-edema, were noted. CONCLUSIONS: SLT appears a safe and cost-effective procedure in PAC/mild- moderate PACG eyes with uncontrolled IOP after laser iridotomy. The long-term effectiveness of SLT as adjuvant treatment was good, but need large sized randomized studies for more validation.


Asunto(s)
Glaucoma de Ángulo Cerrado , Terapia por Láser , Trabeculectomía , Humanos , Masculino , Femenino , Persona de Mediana Edad , Estudios Prospectivos , Estudios Transversales , Glaucoma de Ángulo Cerrado/cirugía , Presión Intraocular , Rayos Láser , Resultado del Tratamiento , Iris/cirugía , Iridectomía
10.
Am J Ophthalmol Case Rep ; 33: 101962, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38192606

RESUMEN

Purpose: To describe a novel technique for repair of rhegmatogenous retinal detachment in an eye with a previous non-valved glaucoma drainage device, the Aurolab Aqueous Drainage Implant (AADI). Observations: A 5-year-old child with bilateral primary congenital glaucoma presented with an inferior retinal detachment (RD) in the left eye. The left eye had a history of multiple surgical interventions including combined trabeculotomy and trabeculectomy done twice, AADI implantation and subsequently phacoaspiration with IOL implantation, 18 months prior to presentation. The left eye retinal detachment was managed by scleral buckling technique using the plate of the AADI as a buckling element without its explantation. Conclusions: AND IMPORTANCE: Management of retinal detachment in eyes with a pre-existing glaucoma drainage device (GDD) is uniquely challenging. Explantation of the GDD would likely result in intractable glaucoma post-operatively, requiring another surgery. Use of the trimmed plate of the GDD itself as the buckling element helped in settling the RD and preserving intraocular pressure control.

11.
Ophthalmol Glaucoma ; 7(1): 54-65, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-37454975

RESUMEN

OBJECTIVE: To report the profile of newly diagnosed childhood glaucoma using the Childhood Glaucoma Research Network (CGRN) classification, presenting over 1 year from across centers in India. DESIGN: Prospective observational multicentric study. SUBJECTS: Newly diagnosed children aged < 18 years diagnosed with childhood glaucoma according to CGRN criteria presenting between January and December 2019 to 13 centers across India. METHODS: All children underwent a comprehensive ocular examination, including examination under anesthesia for younger children, and were diagnosed with childhood glaucoma as per CGRN. Data were entered in a standard Excel chart. Refraction and visual acuity assessments were done when feasible. MAIN OUTCOME MEASURES: The profile of newly diagnosed childhood glaucoma in different parts of India and the severity of glaucoma at presentation. RESULTS: A total of 1743 eyes of 1155 children fulfilled the definition of glaucoma and were analyzed. Primary congenital glaucoma (PCG) comprised the single largest group (34.4%), most of which were infantile onset (19%). Neonatal-onset PCG comprised 6.2% of all glaucoma. Secondary glaucoma constituted 53.4% of all glaucoma, one-half of which were acquired conditions (28%), followed by isolated ocular anomalies (14.7%), glaucoma after cataract surgery (6.7%), and glaucoma with nonacquired systemic diseases (4.5%). Of the 1743 eyes with glaucoma, all 3 parameters for severity grading were available in 842 eyes, of which 501 (59.5%) eyes presented with mild, 320 (38%) with moderate, and 21 (2.5%) with severe glaucoma. Nearly one-third of the children (28.5%) were not brought back for follow-up after the initial treatment given. CONCLUSIONS: Our study has one of the largest numbers of consecutive children with glaucoma classified according to the CGRN classification. Despite a widely diverse population, the profile of childhood glaucoma was relatively uniform across India. Childhood glaucoma is a significant problem in India, primarily treated in tertiary care hospitals. The data presented may be the tip of the iceberg because we have only reported the children who reached the hospitals offering treatment for this challenging disease. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.


Asunto(s)
Glaucoma , Hidroftalmía , Niño , Humanos , Recién Nacido , Glaucoma/diagnóstico , Glaucoma/epidemiología , India/epidemiología , Presión Intraocular , Agudeza Visual , Lactante , Preescolar , Adolescente
12.
J Glaucoma ; 33(3): 211-217, 2024 Mar 01.
Artículo en Inglés | MEDLINE | ID: mdl-37671499

RESUMEN

PRCIS: Trans-corneal transillumination (TI) aided transscleral cyclophotocoagulation (TSCPC) for intraocular pressure control of refractory pediatric glaucoma was found to be effective at 1 year. PURPOSE: To study 1-year outcomes of diode laser TSCPC with trans-corneal TI using a novel low-cost torchlight method in refractory pediatric glaucoma. METHODS: This prospective interventional study included children with refractory glaucoma who underwent TSCPC with the Oculight laser system (IRIS Medical Instruments) with trans-corneal TI (TSCPC-TI) using a novel low-cost torchlight method. Children completing a minimum 1-year follow-up were analyzed. They were compared with a historical control group of children who underwent TSCPC without TI (TSCPC-No-TI) at the 1-year follow-up period. We analyzed the mean laser energy delivered, post-laser intraocular pressure reduction, number of antiglaucoma medications (AGM), the requirement of retreatment and complications of the procedure in both groups. RESULTS: Forty-two eyes of 35 patients comprised the TSCPC-TI group and were compared with 31 eyes of 21 patients in the TSCPC-No-TI group. The TSCPC-TI group required lower energy than the TSCPC-No-TI group (24.7±7.8 J vs. 47.2±10.9 J, P <0.0001). Mean topical antiglaucoma drug requirement decreased from 2.9±0.08 before treatment to 0.66±0.8 at the end of 1 year in TSCPC-TI and from 2.6±1.0 before treatment to 0.6±0.77 in the TSCPC-No-TI groups, respectively ( P =0.15). There was a significantly reduced dependence of oral acetazolamide in the TSCPC-TI group at 1-year follow-up [5.7% vs. 61.9%, respectively ( P <0.001%)]. No adverse event of hypotony or choroidal detachment was noted in any group. CONCLUSIONS: TSCPC aided by TI with a low-cost torchlight for pediatric refractory glaucoma was found to be effective at 1 year in reducing intraocular pressure and the burden of medication.


Asunto(s)
Glaucoma , Presión Intraocular , Niño , Humanos , Coagulación con Láser/métodos , Láseres de Semiconductores/uso terapéutico , Estudios Prospectivos , Transiluminación/efectos adversos , Glaucoma/diagnóstico , Glaucoma/cirugía , Glaucoma/etiología , Cuerpo Ciliar/cirugía , Resultado del Tratamiento , Estudios Retrospectivos
13.
J AAPOS ; 27(4): 203.e1-203.e7, 2023 08.
Artículo en Inglés | MEDLINE | ID: mdl-37442537

RESUMEN

PURPOSE: To compare visual field indices in normal children and adults with similar retinal nerve fiber layer (RNFL) thickness on optical coherence tomography (OCT). METHODS: This cross-sectional study included 59 eyes of 59 normal children 6-18 years of age compared to normal adults. The children underwent visual field testing on the Humphrey Field Analyzer (HFA) and RNFL thickness measurement on OCT. Normal adults who had undergone OCT and visual field testing were retrieved from the OCT database. The mean deviation (MD) in each child was compared with the MD in RNFL thickness-matched eyes of normal adults. Children 6-11 years and 12-18 years were analyzed separately. RESULTS: Overall, the MD of children was significantly less than that of the normal adults despite having the same RNFL thickness (-2.42 ± 1.42 dB vs -1.61 ± 1.47 dB [P = 0.006]). When stratified by age, the difference in MD of children <12 years was significantly less than normal adults (-2.72 ± 1.5dB vs -1.53 ± 1.2 dB [P = 0.003) while in children >12 years, the difference did not reach statistical significance (-2.18 ± 1.2 vs -1.51 ± 1.32 [P = 0.12]). CONCLUSIONS: In our study cohort, children <12 years had lower visual field indices than adults. Their visual fields may appear worse than they would for an adult with the same RNFL thickness. Normal children >12 years of age seem to have a retinal sensitivity comparable to the adult normative database. It is worthwhile to consider the development of a separate pediatric normative database for the visual field assessment of children <12 years of age.


Asunto(s)
Fibras Nerviosas , Campos Visuales , Adulto , Humanos , Niño , Estudios Transversales , Retina , Pruebas del Campo Visual/métodos , Tomografía de Coherencia Óptica/métodos
14.
BMJ Case Rep ; 16(1)2023 Jan 18.
Artículo en Inglés | MEDLINE | ID: mdl-36653044

RESUMEN

Rubinstein-Taybi syndrome, also known as broad thumb-hallux syndrome, is a rare autosomal dominant genetic disorder. This multiorgan syndrome is linked to a pathogenic mutation in the CREBBP or EBP300 genes.We present a patient with a hitherto unreported constellation of anterior segment abnormalities, including congenital glaucoma, congenital corneal keloid, cataract, and distinct facial and systemic features including a high-arched palate, low-set posteriorly rotated ears, Café-au-lait spots on the back, broad terminal phalanges of hands and feet, and bilateral cryptorchidism. The characteristic dysgenetic angle features and ultrasound biomicroscopic findings described in this case report show the occurrence of concomitant congenital keloid with glaucoma.Genetic testing revealed a heterozygous one-base pair duplication in exon 3 of the CREBBP gene (c.886dupC), a novel frameshift pathogenic mutation in the CREBBP gene that has not been previously reported in a clinical setting.


Asunto(s)
Glaucoma , Hidroftalmía , Queloide , Síndrome de Rubinstein-Taybi , Masculino , Humanos , Lactante , Síndrome de Rubinstein-Taybi/complicaciones , Síndrome de Rubinstein-Taybi/diagnóstico , Síndrome de Rubinstein-Taybi/genética , Mutación , Mutación del Sistema de Lectura , Proteína de Unión a CREB/genética
16.
Br J Ophthalmol ; 107(2): 227-233, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-34526297

RESUMEN

OBJECTIVE: To compare CYP1B1 and MYOC variants in a cohort of neonatal-onset (NO) and infantile-onset (IO) primary congenital glaucoma (PCG). METHODS: This prospective observational study included 43 infants with PCG (14 NO and 29 IO) presenting between January 2017 and January 2019 with a minimum 1-year follow-up. CYP1B1 and MYOC genes were screened using Sanger sequencing with in-silico analysis of the variants using Polymorphism Phenotyping v.2 and Protein Variation Effect Analyser platforms. Allelic frequency was estimated using Genome Aggregation Database (gnomAd). Disease presentation and outcome were correlated to the genetic variants in both groups. RESULTS: Babies with CYP1B1 mutations had more severe disease at presentation and worse outcomes. Six of 14 (42.8%) NO glaucoma and 5 of 29 (17.2%) IO harboured CYP1B1 mutations. Five of six babies in the NO group and three of five in the IO group harboured the variant c.1169G>A, [p.R390H]. They required more surgeries and had a poorer outcome. On in-silico analysis c.1169G>A, [p.R390H] scored very likely pathogenic. Two patients in the IO group who had the c.1294C>G, [p.L432V] variant had a good outcome. Five of 14 NO-PCG and 8 of 29 IO-PCG harboured the variant c.227G>A, [p.R76K] in the MYOC gene, which was scored benign by in-silico analysis, and was also found in 2 of 15 normal controls. CONCLUSIONS: Patients with CYP1B1 pathogenic variants had a poorer outcome than those without. We found more NO PCG babies with CYP1B1 mutations compared with IO PCG. This may be one of the reasons for NO PCG having a poorer prognosis compared with IO PCG.


Asunto(s)
Glaucoma , Humanos , Lactante , Recién Nacido , Citocromo P-450 CYP1B1/genética , Análisis Mutacional de ADN , Frecuencia de los Genes , Glaucoma/genética , Glaucoma/congénito , Mutación , Linaje , Estudios Prospectivos
17.
J Glaucoma ; 32(1): 65-67, 2023 01 01.
Artículo en Inglés | MEDLINE | ID: mdl-36001506

RESUMEN

Iatrogenic Cushing's syndrome is a life-threatening condition which requires an early diagnosis multidisciplinary approach. Despite reports of iatrogenic Cushing's syndrome following nasal or inhaled steroids, its development after topical betamethasone eye drops for a short duration is a less frequently reported or anticipated condition. We describe a 6-week-old infant who developed exogenous Cushing's syndrome following topical steroids after bilateral goniotomy for newborn glaucoma. The child was kept under observation and managed with a physiological dose of hydrocortisone. Parental counselling regarding the proper method of eye drop instillation and signs of steroid toxicity is of vital importance in all cases of pediatric ocular surgeries, especially where bilateral surgeries are undertaken.


Asunto(s)
Síndrome de Cushing , Glaucoma , Trabeculectomía , Lactante , Recién Nacido , Humanos , Niño , Síndrome de Cushing/inducido químicamente , Síndrome de Cushing/diagnóstico , Síndrome de Cushing/cirugía , Glucocorticoides/efectos adversos , Presión Intraocular , Esteroides/efectos adversos , Enfermedad Iatrogénica
18.
Taiwan J Ophthalmol ; 13(4): 425-433, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-38249512

RESUMEN

The basic pathophysiology of all childhood glaucoma results from impaired outflow through the trabecular meshwork. Anterior Segment Dysgeneses (ASD) are a group of nonacquired anomalies associated with secondary developmental glaucoma, characterized by impaired development of the structures of the anterior segment. Many genes impact the development of the anterior segment. The cause of the development of the abnormalities is thought to be multifactorial. Molecular research has helped our understanding of the molecular basis of ASD and the developmental mechanisms underlying these conditions. Identifying the genetic changes underlying ASD has gradually led to the recognition that some of these conditions may be parts of a disease spectrum rather than isolated anomalies. The characterization of the underlying genetic abnormalities responsible for glaucoma is the first step toward developing diagnostic and screening tests, which could identify individuals at risk for disease before irreversible optic nerve damage occurs. It is also crucial for genetic counseling and risk stratification of later pregnancies. It also aids prenatal testing by various methods allowing for effective genetic counseling. This review summarizes various ocular and systemic conditions that result in secondary developmental glaucoma and provide an overview of the phenotypes, the diagnosis and principles of management of the various disorders.

19.
J AAPOS ; 26(6): 298.e1-298.e5, 2022 12.
Artículo en Inglés | MEDLINE | ID: mdl-36183996

RESUMEN

PURPOSE: To describe the presentation and treatment outcomes of a cohort of children with primary aphakia (PA). METHODS: Clinical photographs and ultrasound biomicroscopy (UBM) images of children presenting with sclerocornea and undetermined anterior segment dysgenesis between July 2017 and December 2020 were reviewed retrospectively. Children who had no crystalline lens visible on UBM were included. RESULTS: A total of 124 UBM images were captured for 124 children with cloudy corneas. Twelve children were identified with congenital primary aphakia: 5 had bilateral buphthalmos, 2 had buphthalmos in one eye and microphthalmos in the other, and 5 had bilateral sclerocornea-microphthalmia complex. All patients had a peculiar silvery-blue corneal appearance, with fine vascularization on the corneal surface. The overall corneal thickness was 409.1 ± 8.7 µm. The intraocular pressure (IOP) in eyes with glaucoma was 24.5 ± 7.3 mm Hg; in microphthalmic eyes, 11.4 ± 3.4 mm Hg (P <0.001). The raised IOP was treated with limited trans-scleral cyclophotocoagulation under transillumination and topical antiglaucoma medications. Children with glaucoma gained ambulatory vision with spectacles. CONCLUSIONS: Congenital primary aphakia has a characteristic clinical appearance and may present as buphthalmos or microphthalmos, depending on the extent of dysgenesis. Incisional surgery may result in phthisis because of ciliary body dysgenesis and unpredictable aqueous production.


Asunto(s)
Afaquia , Glaucoma , Hidroftalmía , Microftalmía , Niño , Humanos , Microftalmía/diagnóstico , Estudios Retrospectivos , Afaquia/diagnóstico , Presión Intraocular , Glaucoma/congénito
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