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Vitreoretinopathy-Associated Pediatric Retinal Detachment Treatment Outcomes: IRIS® Registry (Intelligent Research in
Peter J Belin1, Ameay V Naravane2, Siying Lu3
1VitreoRetinal Surgery, PA, Edina, Minnesota.
Insights
Pediatric retinal detachments (RDs) linked to hereditary vitreoretinopathies show high reoperation rates and fellow eye involvement. Treatment outcomes indicate limited visual acuity improvement, highlighting the complexity of these rare conditions.
Area of Science:
- Ophthalmology
- Genetics
- Pediatrics
Background:
- Hereditary vitreoretinopathies are a group of genetic disorders affecting the vitreous and retina.
- Pediatric retinal detachment (RD) in this context presents unique challenges compared to adult RDs.
- Understanding treatment patterns and outcomes is crucial for managing these rare conditions in children.
Purpose of the Study:
- To determine the treatment patterns and outcomes of pediatric retinal detachments (RDs) associated with hereditary vitreoretinopathies.
- To analyze surgical interventions and their effectiveness in this specific pediatric population.
Main Methods:
- Retrospective cohort analysis of the IRIS® Registry (Intelligent Research in Sight) database from 2013-2019.
- Inclusion of patients under 18 years old with rhegmatogenous RD and associated hereditary vitreoretinopathies.
- Identification of cases using ICD codes and text search, with exclusion of traumatic and exudative RDs. Surgical procedures identified via CPT codes.
Main Results:
- 2115 eyes of 1722 patients were analyzed (mean age 10.4 years).
- Median time to first surgery was 7 days; 36% developed fellow eye RD, often within 32 days.
- Initial surgeries included complex RD repair, scleral buckle, and pars plana vitrectomy. Visual acuity showed no significant improvement (20/54 to 20/62).
- High rates of reoperation (mean 1.68 surgeries/eye/year) and new diagnoses of glaucoma and aphakia were observed.
Conclusions:
- The IRIS Registry provides valuable insights into pediatric vitreoretinopathy-associated RDs.
- These RDs are characterized by a high incidence of reoperation and fellow eye involvement.
- Current treatment strategies may not significantly improve visual acuity in this complex pediatric population.
Purpose:
To determine the treatment patterns and outcomes of pediatric retinal detachments (RDs) associated with hereditary vitreoretinopathies.
Design:
Retrospective cohort analysis using IRIS® Registry (Intelligent Research in Sight) database.
Participants:
Patients < 18 years old with a rhegmatogenous RD and a systemic disorder associated with vitreoretinal degeneration (e.g., Stickler syndrome) or other malformation of the vitreous from 2013-2019.
Methods:
Cases were identified using International Classification of Diseases, Ninth and Tenth Revisions (ICD-9, ICD-10) diagnostic codes from the IRIS® Registry cohort. Other hereditary vitreoretinopathies that are not encoded by specific ICD code(s) were captured by text search. Nonspecific vitreous abnormality ICD codes were also included. Exclusion criteria included traumatic retinal detachments using ICD codes for ocular trauma and serous or exudative retinal detachment. Surgical procedures were identified using Current Procedural Terminology (CPT) codes for repair of retinal detachment. Baseline demographic information collected included age, gender, race/ethnicity, geographic region of the provider location, and health insurance status.
Main Outcome Measures:
Main outcomes measured in this study were average time to first surgery, number of eyes presenting with bilateral detachments, and choice of initial surgical procedure.
Results:
A total of 2115 eyes of 1722 patients were identified (mean age, 10.4 years; 58% male). The median time to first surgery was 7 days (interquartile range, 40 days). One thousand four hundred seven eyes of 1134 patients had ≥ 1 year of follow-up, with 506 eyes (36%) developing a fellow eye RD. Thirty-three percent of patients presenting with bilateral detachments, and 349 eyes had initial RD surgery within 1 year of the index date documented by CPT code. Fellow eye detachment occurred a mean of 32 days after initial presentation. The mean number of surgeries per eye within 1 year was 1.68. Best-corrected visual acuity did not improve from a baseline 20/54 to 20/62. The initial procedure was most commonly complex RD repair (n = 176), followed by scleral buckle (n = 102), pars plana vitrectomy (n = 89), laser (n = 59), cryotherapy (n = 5), and pneumatic retinopexy (n = 5). There were 51 new diagnoses of glaucoma and 37 new diagnoses of aphakia within 1 year after the surgical procedure.
Conclusions:
IRIS Registry data provide insight into rare pediatric vitreoretinopathy-associated RDs, which have a high rate of reoperation and fellow eye involvement.
Financial Disclosures:
Proprietary or commercial disclosure may be found after the references.
