Surgical outcomes of Glaucoma associated with Axenfeld-Rieger syndrome

Abstract

Background The surgical management of glaucoma associated with Axenfeld-Rieger Syndrome (ARS) is poorly described in the literature. The goal of this study is to compare the effectiveness of various glaucoma surgeries on intraocular pressure (IOP) management in ARS. Methods Retrospective cohort study at a university hospital-based practice of patients diagnosed with ARS between 1973 and 2018. Exclusion criterion was follow-up less than 1 year. The number of eyes with glaucoma (IOP ≥ 21 mmHg with corneal edema, Haabs striae, optic nerve cupping or buphthalmos) requiring surgery was determined. The success and survival rates of goniotomy, trabeculotomy±trabeculectomy (no antifibrotics), cycloablation, trabeculectomy with anti-fibrotics, and glaucoma drainage device placement were assessed. Success was defined as IOP of 5-20 mmHg and no additional IOP-lowering surgery or visually devastating complications. Kaplan-Meier survival curves and the Wilcoxon test were used for statistical analysis. Results In 32 patients identified with ARS (median age at presentation 6.9 years, 0–58.7 years; median follow-up 5.4 years, 1.1–43.7 years), 23 (71.9%) patients were diagnosed with glaucoma at median age 6.3 years (0–57.9 years). In glaucomatous eyes (46 eyes), mean IOP at presentation was 21.8 ± 9.3 mmHg (median 20 mmHg, 4-45 mmHg) on 1.0 ± 1.6 glaucoma medications. Thirty-one eyes of 18 patients required glaucoma surgery with 2.2 ± 1.2 IOP-lowering surgeries per eye. Goniotomy (6 eyes) showed 43% success with 4.3 ± 3.9 years of IOP control. Trabeculotomy±trabeculectomy (6 eyes) had 17% success rate with 14.8 ± 12.7 years of IOP control. Trabeculectomy with anti-fibrotics (14 eyes) showed 57% success with 16.5 ± 13.5 years of IOP control. Ahmed© (FP7 or FP8) valve placement (8 eyes) had 25% success rate with 1.7 ± 1.9 years of IOP control. Baerveldt© (250 or 350) device placement (8 eyes) showed 70% success with 1.9 ± 2.3 years of IOP control. Cycloablation (4 eyes) had 33% success rate with 2.7 ± 3.5 years of IOP control. At final follow-up, mean IOP (12.6 ± 3.8 mmHg, median 11.8 mmHg, 7-19 mmHg) in glaucomatous eyes was significantly decreased (p < 0.0001), but there was no difference in number of glaucoma medications (1.6 ± 1.5, p = 0.1). Conclusions In our series, greater than 70% of patients with ARS have secondary glaucoma that often requires multiple surgeries. Trabeculectomy with anti-fibrotics and Baerveldt glaucoma drainage devices showed the greatest success in obtaining IOP control.

Ologen augmentation of Ahmed glaucoma drainage devices in pediatric glaucomas

“…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.…”

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

“…Most prominent are the ocular abnormalities that combine Axenfeld anomaly (posterior embryotoxon) and Rieger anomaly (iris hypoplasia), resulting in corectopia (irregular pupil), and pseudopolycoria (multiple pupils) ( Figure 4A ). Over 50% of affected individuals have glaucoma, which due to iris and trabecular meshwork abnormalities, often requires multiple surgeries to preserve vision ( Zepeda et al, 2020 ). In addition, individuals with ARS may have a distinctive craniofacial structure that includes telecanthus, maxillary hypoplasia, and broad flat nasal bridge, teeth abnormalities, such as oligodontia and microdontia, and congenital heart abnormalities.…”

Approach to childhood glaucoma: A review

“…31,32 Lifelong monitoring is required as 50% of patients with ARA and up to 70% of patients with ARS have been reported to develop glaucoma. 33 The association of Axenfeld Rieger anomaly with systemic abnormalities is termed Axenfeld-Rieger syndrome. 30 Clinically it encompasses systemic midline abnormalities (dental anomalies, flattened nasal bridge and midface, and redundant peri-umbilical skin) in addition to the ocular findings.…”

“…A recent series of 32 patients with ARS found that trabeculectomy with anti-fibrotics and Baerveldt GDD showed the greatest success in obtaining IOP control. 33…”