Congenital Aniridia: Clinic, Genetics, Therapeutics, and Prognosis

Calvão-Pires, Pedro; Santos-Silva, Renato; Falcão‐Reis, Fernando; Rocha‐Sousa, Amândio

doi:10.1155/2014/305350

Cited by 23 publications

(34 citation statements)

References 33 publications

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“…Genetic conditions such as albinism and aniridia can arrest foveal pit formation during fetal development and maturation after birth 33,34. Sporadic developmental defects such as fovea plana similarly affect foveal pit morphology, often without impact on visual acuity 35,36.…”

Section: Discussionmentioning

confidence: 99%

Optical Coherence Tomography Findings After Childhood Lensectomy

Daniel

Dubis

MacPhee

et al. 2019

Invest. Ophthalmol. Vis. Sci.

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PurposeTo explore the impact of childhood lensectomy on posterior segment development.MethodsCross-sectional observational study at children's eye clinics at a tertiary referral center in London, UK. We included 45 children age 4 to 16 years with healthy eyes and 38 who had undergone lensectomy. We acquired posterior segment optical coherence tomography scans of both eyes. We used parametric and nonparametric tests in SPSS24 for the comparison of parameters between groups and within individuals; a P value less than 0.05 was considered significant. The main outcome measures were foveal pit depth and subfoveal choroidal thickness (CT). Secondary outcomes were inner and outer ring CT and photoreceptor layer parameters, macular and peripapillary retinal nerve fiber layer thickness.ResultsFoveal pit depth and subfoveal CT are significantly reduced in eyes that have undergone lensectomy compared with nonoperated eyes. Inner ring CT and outer ring CT are reduced. Foveal inner retinal layer thickness is increased. Mean inner retinal and outer nuclear layer thickness are not affected.ConclusionsChildhood lensectomy is associated with a reduction in developmental foveal pit deepening and lack of developmental thickening of the posterior choroid. Mechanical and optical disruption of foveal and subfoveal choroidal development may affect structural foveal development after childhood lensectomy.

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Section: Discussionmentioning

confidence: 99%

Optical Coherence Tomography Findings After Childhood Lensectomy

Daniel

Dubis

MacPhee

et al. 2019

Invest. Ophthalmol. Vis. Sci.

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“…Aphakic glaucoma is often associated with abnormal ocular development such as microcornea [ 12 ], anterior segment dysgenesis, and aniridia [ 32 , 33 ]. Microcornea is known to be an independent risk factor for aphakic glaucoma [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

Outcome of Pediatric Cataract Surgeries in a Tertiary Center in Switzerland

Ambroz

Töteberg‐Harms

Hanson

et al. 2018

Journal of Ophthalmology

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Purpose To determine and to analyze the outcome of pediatric cataract surgery. Methods A retrospective chart review of individuals aged up to 10 years who underwent cataract surgery between January 1, 2004, and December 31, 2014, at the UniversityHospital Zurich, Switzerland. Results 63 children (94 affected eyes) with bilateral (68/94) or unilateral (26/94) cataract were identified. Surgery was performed at a median age of 1.5 months (IQR: 1.3–2.6 months) for the aphakic group (45/94) and of 50.7 months (IQR: 38.0–78.4 months) for the IOL group (49/94). At the last follow-up visit (median 31.1 months, IQR: 18.4–50.2 months), visual acuity was better in bilateral than in unilateral cataract cases. Posterior capsular opacification (PCO) was diagnosed in 30.9% of eyes without a significant difference in the IOL and aphakic groups (p = 0.12). Aphakic glaucoma was diagnosed in 12/45 eyes at a median of 6.8 months (IQR 2.1–13.3 months) after surgery. Microcornea (5/12) and anterior segment anomalies (8/12) were associated with glaucoma development (p < 0.05). Conclusion Laterality and timing of surgery influence the outcome of pediatric cataract surgery. PCO was the most frequent postoperative complication. Aphakic glaucoma is often associated with ocular developmental abnormalities and a poor visual outcome.

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“…The incidence of congenital aniridia is 1 in 60,000. [11] Thus, the Saudi population could include as many as 325 cases of aniridia. [12] Aniridia following trauma has been documented; however, traumatic aniridia is rare and is caused by intraocular surgery in most cases.…”

Section: Discussionmentioning

confidence: 99%

Black diaphragm intraocular lens implantation in patients with aniridia

Alrashidi¹

2019

J Ophthalmic Vis Res

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Purpose: To review the management outcomes of black diaphragm intraocular (BDI) lens implantation in Arab patients with aniridia. Methods: Patients with aniridia undergone BDI lens implantation at our institution between 2013 and 2014 were included. Uncorrected visual acuity (UCVA), manifest refraction, and best-corrected visual acuity (BCVA) were evaluated before and 6 months and yearly after BDI lens implant surgery until the last visit. Intra- and postoperative complications were noted. Results: Our series comprised 14 patients (8 males) with aniridia. The median duration of follow-up was 30 months (25% quartile). Ocular parameters, refractive status, and vison were all significantly improved at the last follow-up compared to the preoperative values ( P < 0.05 for all comparisons). All patients reported a significant decrease in photophobia and glare. Postoperatively, 11 eyes (78%) gained 2 or more lines of UCVA. At the last follow-up, BCVA increased by 2 or more lines in all cases. Early postoperative complications included main wound leakage (one eye) and anterior chamber hyphema (one eye). Late (≥6 months) complications included corneal decompensation (one eye), failed penetrating keratoplasty graft (2 eyes), and subluxation of a scleral fixated BDI lens (one eye). Surgical interventions performed to manage complications included penetrating keratoplasty in 2 eyes with corneal decompensation and failed graft (one each), and re-suturing of a subluxated intraocular lens (one eye). Conclusion: BDI lenses seem to be a safe and effective iris prosthetic with intraocular lens combination surgery for patients with congenital or traumatic aniridia. Periodic evaluation and prompt management of complications are recommended.

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Congenital Aniridia: Clinic, Genetics, Therapeutics, and Prognosis

Cited by 23 publications

References 33 publications

Optical Coherence Tomography Findings After Childhood Lensectomy

Optical Coherence Tomography Findings After Childhood Lensectomy

Outcome of Pediatric Cataract Surgeries in a Tertiary Center in Switzerland

Black diaphragm intraocular lens implantation in patients with aniridia

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