Glaucoma and Cornea Surgery Outcomes in Peters Anomaly

Dolezal, Kara A.; Besirli, Cagri G.; Sugar, Alan; Moroi, Sayoko E.; Bohnsack, Brenda L.

doi:10.1016/j.ajo.2019.08.012

Cited by 27 publications

(47 citation statements)

References 36 publications

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“…However, many of the other forms of glaucoma represented in this study such as Axenfeld-Rieger syndrome, Peters Anomaly, microphthalmia, and aniridia are well-known to be refractory to both medications and angle surgery. Only a handful of studies have been able to access outcomes in these especially rare diseases [26,27]. More common forms of pediatric glaucomas such as primary congenital glaucoma and glaucoma following cataract surgery are often amenable to angle surgery and only the more complicated and difficult cases progress to trabecular bypass surgery.…”

Section: Discussionmentioning

confidence: 99%

Ologen augmentation of Ahmed glaucoma drainage devices in pediatric glaucomas

2021

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Background Limited data exists on the effectiveness of the collagen matrix, Ologen, on increasing Ahmed glaucoma valve (AGV) success in childhood glaucomas. Methods Ocular examination and surgical details of pediatric patients who underwent AGV placement ± Ologen augmentation between 2012 and 2020. Complete success was defined as intraocular pressure (IOP) between 5 and 20 mmHg without glaucoma medications and additional IOP-lowering surgeries. Qualified success was defined as above, except IOP control maintained with or without glaucoma medications. Results Twenty-two eyes of 16 patients underwent AGV placement of which 6 eyes had Ologen-augmentation (OAGV) and 16 eyes had conventional surgery (CAGV). Average age was 6.4 ± 5.1 years with 4.2 ± 2.5 follow-up years. There was no difference in age, number of previous surgeries, and preoperative IOP and glaucoma medications. At final follow-up, success rate was 100% (5 eyes complete, 6 eyes qualified) in the OAGV group compared to 31% (0 eyes complete, 5 eyes qualified) in the CAGV group. One and two-year survival rates were 100% for OAGV compared to 62 and 38% for CAGV. Postoperative IOP was significantly lower at 1-month and final follow-up (p = 0.02) as was the number of glaucoma medications at 3, 6, 12-months and final follow-up (p < 0.05) in the OAGV group. Conclusions Ologen-augmentation increased the success and survival rates of AGVs in childhood glaucomas. Further, Ologen mitigated the hypertensive phase and decreased medication dependency. Longer follow-up with a greater number of eyes is required to fully evaluate the effectiveness of OAGV.

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Section: Discussionmentioning

confidence: 99%

Ologen augmentation of Ahmed glaucoma drainage devices in pediatric glaucomas

2021

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“…In severe cases, the lens is also adhered to the cornea, which can cause corneal staphylomas (Alallah et al, 2020;Chang et al, 2020;Katz et al, 2020;Kletke et al, 2020;Samara and Eldaya, 2020). In addition, cataract, microphthalmia, and aniridia (iris absence or hypoplasia) are commonly associated with PA. Glaucoma complications due to a closed angle configuration and abnormal trabecular meshwork and aqueous outflow tract formation are observed in approximately two-thirds of cases and often requires multiple surgeries to obtain intraocular pressure control (Ozeki et al, 2000;Gould and John, 2002;Sowden, 2007;Harissi-Dagher and Colby, 2008;Dolezal et al, 2019). Although most cases are isolated to the eye, systemic findings can include craniofacial anomalies, congenital heart defects, and developmental delay (Dolezal et al, 2019).…”

Section: Peters Anomaly (Omim 604229)mentioning

confidence: 99%

“…Notably, mutations in Pax6 are more typically associated with aniridia, a panocular congenital disease characterized by foveal hypoplasia, optic nerve hypoplasia, limbal stem cell deficiency, and varying degrees of iris hypoplasia (reviewed in Lim et al, 2017;Sannan et al, 2017;Syrimis et al, 2018;Wawrocka and Krawczynski, 2018;Lima Cunha et al, 2019;Lee et al, 2020;Tripathy and Salini, 2020). However, phenotypic variation of the same Pax6 gene mutation has shown both aniridia and PA within one family (Wang et al, 2018), and PA has been associated with aniridia in more than 10% of cases (Dolezal et al, 2019).…”

Section: Peters Anomaly (Omim 604229)mentioning

confidence: 99%

The Ocular Neural Crest: Specification, Migration, and Then What?

Williams

Bohnsack

2020

Front. Cell Dev. Biol.

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During vertebrate embryonic development, a population of dorsal neural tube-derived stem cells, termed the neural crest (NC), undergo a series of morphogenetic changes and extensive migration to become a diverse array of cell types. Around the developing eye, this multipotent ocular NC cell population, called the periocular mesenchyme (POM), comprises migratory mesenchymal cells that eventually give rise to many of the elements in the anterior of the eye, such as the cornea, sclera, trabecular meshwork, and iris. Molecular cell biology and genetic analyses of congenital eye diseases have provided important information on the regulation of NC contributions to this area of the eye. Nevertheless, a complete understanding of the NC as a contributor to ocular development remains elusive. In addition, positional information during ocular NC migration and the molecular pathways that regulate end tissue differentiation have yet to be fully elucidated. Further, the clinical challenges of ocular diseases, such as Axenfeld-Rieger syndrome (ARS), Peters anomaly (PA) and primary congenital glaucoma (PCG), strongly suggest the need for better treatments. While several aspects of NC evolution have recently been reviewed, this discussion will consolidate the most recent current knowledge on the specification, migration, and contributions of the NC to ocular development, highlighting the anterior segment and the knowledge obtained from the clinical manifestations of its associated diseases. Ultimately, this knowledge can inform translational discoveries with potential for sorely needed regenerative therapies.

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“…Due to the difference in the phenotypic presentation in patients with PA, as well as co-existing glaucoma in many of these patients, the visual prognosis in children with PA is highly variable; studies show anywhere from 10% to 54% of patients achieving visual acuity (VA) better than 20/200. 4 , 5 Although variability in functional outcomes is often attributed to the severity of disease, the difference in keratoplasty techniques, frequency, and severity of graft rejection, and the presence of glaucoma has also been suggested as a potential confounder. 4–6 …”

Section: Introductionmentioning

confidence: 99%

“… 4 , 5 Although variability in functional outcomes is often attributed to the severity of disease, the difference in keratoplasty techniques, frequency, and severity of graft rejection, and the presence of glaucoma has also been suggested as a potential confounder. 4–6 …”

Section: Introductionmentioning

confidence: 99%

Visual Outcomes in Pediatric Patients with Peters Anomaly

Fouzdar-Jain

Ibrahim

Reitinger

et al. 2021

OPTH

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Purpose This study aimed to evaluate the visual outcomes of pediatric patients diagnosed with Peters anomaly (PA) in a tertiary eye care center. Methods This was a retrospective study undertaken at a single academic center. Clinical records of pediatric patients diagnosed with PA from 2000 to 2017 were reviewed. Parameters recorded included visual acuity (VA), intraocular pressure (IOP), presence of glaucoma, and surgical procedures performed. The impact of PA type on final visual acuity levels was analyzed using generalized estimation equation models. Results Twenty-one eyes from 11 patients were analyzed. At the final visit, 9 eyes (42.8%) had a VA ≥20/200, while 12 (57%) had <20/200, ranging from 20/250 to no light perception. Of the study cohort, 15 (71.5%) eyes had type I, while six (28.5%) had type II PA. Glaucoma was present in 20 eyes (95%) at the final visit. One eye had dense central leucoma and microphthalmos where IOP could not be measured accurately. Seventeen eyes (81%) underwent at least one intraocular surgery, while the average number of surgeries per eye was 2.9 ± 2.5 (range=0– 9). The type of PA beta(se)=0.53 (0.41), p = 0.20] was not found to be a significant risk factor for worse VA. Conclusion Severe visual impairment develops in the majority of patients with PA. The type of anomaly does not appear to be associated with a worse visual function in patients with PA. Glaucoma develops in a high percentage of this patient population, and thus close monitoring of IOP in this patient population is crucial.

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Glaucoma and Cornea Surgery Outcomes in Peters Anomaly

Cited by 27 publications

References 36 publications

Ologen augmentation of Ahmed glaucoma drainage devices in pediatric glaucomas

Ologen augmentation of Ahmed glaucoma drainage devices in pediatric glaucomas

The Ocular Neural Crest: Specification, Migration, and Then What?

Visual Outcomes in Pediatric Patients with Peters Anomaly

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