Visual Outcomes in Pediatric Patients with Peters Anomaly

Abstract

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.

Approach to childhood glaucoma: A review

“…The reported rate of glaucoma in this cohort varies, ranging from 50% to 70% but has been described as high as 95%. 20 Associated systemic abnormalities including cleft lip and palate, short stature, abnormal ears, and intellectual disability can occur in conjunction with PA and is referred to as Peters plus syndrome. While angle surgery may be considered in selected cases, results are poor compared to PCG.…”

Peter’s anomaly—A homeotic gene disorder

“…A management plan has to be instituted within the first year of life. For the mildest cases with small axial or paraxial opacity, pharmacological pupillary dilatation or optical sector iridectomy may be sufficient 1,2,6,16 . For larger opacity (>3 mm), a full thickness penetrating keratoplasty may be needed 1,2,17 .…”

“…1,2,6,16 For larger opacity (>3 mm), a full thickness penetrating keratoplasty may be needed. 1,2,17 Selective endothelial removal and posterior lamellar keratoplasty can be optimal in some cases. 18,19 Peripheral broad iridectomy and auto-rotational keratoplasty rather than allograft penetrating keratoplasty would be more optimal to establish functional vision in the type II anomalies.…”

“…Peter's anomaly is a rare congenital anomaly characterised by anterior segment dysgenesis involving any of the structures including the cornea, iris, lens, anterior and posterior chambers, ciliary body and the irido‐corneal angle, which contains the trabecular meshwork and Schlemm's canal 1 . Peter's anomaly was previously called kerato‐lenticular dysgenesis, primary mesodermal dysgenesis of the cornea, congenital anterior synechiae, posterior keratoconus or anterior chamber cleavage syndrome but is now thought as one of the subtypes of anterior segment dysgenesis (ASD) 2 . ASD disorders are developmental anomalies of the cornea, iris and lens including posterior embryotoxon, Axenfeld–Rieger syndrome, megalocornea, sclerocornea, posterior keratoconus, congenital glaucoma, hereditary endothelial dystrophy, aniridia and iris hypoplasia.…”

“…The association of Peter's anomaly with short stature, brachydactyly, dysmorphic facial features, cognitive deficits, malformed ears and cleft lip and palate is classified as Peter's plus anomaly. [1][2][3]…”