Combined Aniridia Ring Implantation and Cataract Surgery in an Axenfeld–Rieger Syndrome: A UBM Report

Guerriero, Silvana; L'Abbate, Milena; Tegola, Maria Gabriella La; Alessio, Giovanni; Sborgia, Giancarlo

doi:10.1097/icl.0b013e3182050472

Cited by 12 publications

(9 citation statements)

References 10 publications

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“…Consistently, 33 out of 45 ASD patients were younger than 20 years old in our study. Glaucoma secondary to ASD can be caused by the following mechanisms: (1) open-angle glaucoma due to maldevelopment of the trabecular meshwork and Schlemm's canal, and (2) ACG due to high insertion of the iris root over the trabecular meshwork or secondary PAS [34][35][36][37]. Apparently, since patients with extensive PAS and closed anterior chamber angle were included, our patients all belong to the second category of mechanisms.…”

Section: Number Of Etiologiesmentioning

confidence: 99%

Etiologies and clinical characteristics of young patients with angle-closure glaucoma: a 15-year single-center retrospective study

Gao

Wang

Chen

et al. 2021

Graefes Arch Clin Exp Ophthalmol

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Purpose To investigate the etiologies and the clinical characteristics of angle-closure glaucoma (ACG) patients younger than 40 years old in Chinese. Methods Inpatients with diagnosis of ACG and diagnosed age younger than or equal to 40 years old, who were admitted in Eye, Ear, Nose, and Throat Hospital Fudan University from 2002 to 2017, were included in this retrospective non-comparative case series. The underlying causes and clinical features for all the patients were analyzed by comprehensive review of medical charts. Results A total of 298 patients (463 eyes) met the criteria, including 153 females (51.3%) and 145 males (48.7%); the mean age was 25.6 ± 13.0 years. Primary angle-closure glaucoma (PACG), uveitis, and anterior segment dysgenesis (ASD) were the top three etiologies in our patients, which accounted for 32.6%, 20.3%, and 15.1% of the total patients respectively. PACG mainly occurs after 30 years of age and ASD is the top reason of ACG in patients younger than 20 years old. Other known etiologies include iridocorneal endothelial syndrome, neovascular glaucoma, nanophthalmos, retinitis pigmentosa, spherophakia, bestrophinopathy, persistent fetal vasculature, iridociliary cysts, congenital retinoschisis, Marfan’s syndrome, retinopathy of prematurity, familial exudative vitreoretinopathy, congenital retinal folds, Coat’s disease, and neurofibromatosis. Conclusions We described the uncommon presentation of ACG in Chinese young patients. Although unusual, most of the etiologies could be identified. Therefore, more careful and comprehensive examinations are needed for early detection and timely treatment for young ACG patients.

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Section: Number Of Etiologiesmentioning

confidence: 99%

Etiologies and clinical characteristics of young patients with angle-closure glaucoma: a 15-year single-center retrospective study

Gao

Wang

Chen

et al. 2021

Graefes Arch Clin Exp Ophthalmol

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“…In our case, the occurrence of displacement of the iris implant due to a contraction of the capsular bag 10 months after the surgical procedure, leads to persistent inflammation, and constant rise in IOP measurements, which required removal of one of the rings with glaucoma valve surgery in the late post-operative period. Similarly, Gerreiro et al, 16 described a contraction of the capsular bag, 8 months after cataract phacoemulsification and aniridic ring implantation (Morcher Aniridia rings 50 D) a patient with Axenfeld-Rieger syndrome. UBM examination showed contraction of the capsular bag, which appeared wrinkled, fibrous, and thickened.…”

Section: Discussionmentioning

confidence: 85%

Combined cataract phacoemulsification and aniridia endocapsular rings implantation in a patient with bilateral congenital aniridia and cataract: A case report

González-Salinas

Pena-Guani

2016

Saudi Journal of Ophthalmology

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The objective of this article was to determine the long-term outcome of surgical treatment in a patient with bilateral congenital aniridia and congenital cataracts. The patient was treated by cataract removal and implantation of a single piece IOL in both eyes. Two aniridia rings were also implanted in the capsular bag. The best-corrected visual acuity (BCVA), intraocular pressure (IOP), stability of the intraocular lens (IOL) and subjective glare reduction were measured for two years after the surgery. After 10 months, the IOP in the left eye had increased to 26 mmHg despite the fact that anti-glaucoma medication was added. Therefore, the patient was scheduled for an Ahmed valve implantation. These results suggest that good visual outcomes can be achieved in patients with bilateral congenital aniridia and cataracts. Nevertheless, the IOPs must be continuously monitored, and glaucoma screening performed to prevent further complications.

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“…Five eyes without the prior ocular disease (except ARS) or surgical history developed irreversible endothelial decompensation in our study. Endothelial decompensation had been found in several cases with ARS [8, 13,20]. However, in these cases, the damage to endothelial membrane could be explained by surgery or long-standing glaucoma.…”

Section: Discussionmentioning

confidence: 99%

Axenfeld-Rieger syndrome：A novel histopathologic finding associated with corneal abnormalities

Dai

Peng

et al. 2022

Preprint

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Background: Axenfeld-Rieger syndrome (ARS) is a rare kind of anterior segment dysgenesis (ASD). The most common ocular features of ARS are posterior embryotoxon and iris hypoplasia, while some patients may manifest as corneal opacity and edema. However, the current understanding of how ARS affects the cornea is still incomplete. This study reports a novel histopathological finding of Axenfeld-Rieger syndrome (ARS), complicating corneal abnormalities, including congenital corneal opacity and irreversible endothelial decompensation.Methods: This retrospective study included 6 eyes of 3 ARS patients who underwent keratoplasty for irreversible endothelial decompensation from May 2016 to January 2019. No eye had a history of surgery or glaucoma. We reviewed the data of epidemiology, clinical manifestations and pathological examinations.Results: Five eyes developed irreversible endothelial decompensation, among which 4 were born with corneal opacity. One eye exhibited transparent cornea but showed a continuous loss of endothelial cells in the absence of surgery and glaucoma thereafter. Anterior segment optical coherence tomography photographs showed that anterior synechia existed in the area with corneal opacities, where we found the interlayer splitting of the Descemet membrane inserted by hypoplastic iris and a basement membrane-like structure under a light microscope. Conclusions: Anterior synechia might be associated with corneal abnormalities in ARS patients. The novel histopathologic finding revealed the internal relation between anterior segment dysgenesis and would help explore the inner mechanism of corneal abnormalities in ARS.

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Combined Aniridia Ring Implantation and Cataract Surgery in an Axenfeld–Rieger Syndrome: A UBM Report

Abstract: Ultrasound biomicroscopy is a useful noninvasive diagnostic means to picture anatomic details before and after surgery; it suggested that capsular bag shrinking caused migration of the aniridic rings, 8 months after surgery.

Cited by 12 publications

References 10 publications

Etiologies and clinical characteristics of young patients with angle-closure glaucoma: a 15-year single-center retrospective study

Etiologies and clinical characteristics of young patients with angle-closure glaucoma: a 15-year single-center retrospective study

Combined cataract phacoemulsification and aniridia endocapsular rings implantation in a patient with bilateral congenital aniridia and cataract: A case report

Axenfeld-Rieger syndrome：A novel histopathologic finding associated with corneal abnormalities

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