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Surgical Outcomes in Axenfeld-Rieger Syndrome: A Multicenter Retrospective Analysis.
Kasem Seresirikachorn1, Warakorn Thiamthat1, Elena Bitrian2
1From the Department of Ophthalmology, College of Medicine, Rangsit University, Rajavithi Hospital (K.S., W.T.), Bangkok, Thailand; Department of Ophthalmology, Queen Sirikit National Institute of Child Health (K.S., W.T.), Bangkok, Thailand.
Over half of patients with Axenfeld-Rieger syndrome (ARS) develop glaucoma, with early diagnosis and cardiac anomalies being key risk factors. Glaucoma drainage devices (GDDs) show the highest qualified success for primary surgery.
Area of Science:
- Ophthalmology
- Genetics
- Pediatric Medicine
Background:
- Axenfeld-Rieger syndrome (ARS) is a genetic disorder affecting eye development.
- Glaucoma is a common and serious complication of ARS, often requiring early intervention.
- Identifying risk factors and effective surgical treatments for ARS-associated glaucoma is crucial.
Purpose of the Study:
- To determine risk factors for glaucoma development in patients with ARS.
- To evaluate the efficacy of primary glaucoma surgeries in ARS-associated glaucoma.
Main Methods:
- Retrospective comparative interventional case series.
- Included patients diagnosed with ARS (with or without glaucoma) before age 18.
- Surgical success defined by intraocular pressure (IOP) control, medication use, and absence of vision loss or re-operation.
Main Results:
- 57.3% of ARS patients developed glaucoma during a mean 7.5-year follow-up.
- Diagnosis at birth (aOR 6.45) and cardiac anomalies (aOR 10.73) were significant risk factors.
- Glaucoma drainage devices (GDDs) demonstrated higher qualified success (72.9%) compared to goniotomy (37.8%) or trabeculotomy (27.8%) at 3 years.
Conclusions:
- Glaucoma is highly prevalent in ARS, with early diagnosis and cardiac anomalies as major risk factors.
- Complete surgical success rates for primary glaucoma surgery in ARS remain low.
- GDDs may represent a preferred primary surgical option due to higher qualified success rates.
