IRIS Registry data mined for understanding of pediatric NK

Neurotrophic keratopathy (NK) has an estimated prevalence in the US of approximately 21 per 100,000.¹ Its prevalence in the pediatric population, however, is unknown. To address the latter gap in knowledge, Asmas A. Zidan, MD, and colleagues in the Department of Ophthalmology at Harvard Medical School, Boston, Massachusetts, undertook a retrospective study leveraging Intelligent Research in Sight (IRIS) Registry data to examine the prevalence and clinical characteristics of pediatric NK. Their research was presented in an ePoster at the American Academy of Ophthalmology 2025 annual meeting in Orlando, Florida.²

Based on the findings, Zidan et al. concluded, “NK is not uncommon in the pediatric population, requires early recognition and proactive management to preserve vision.”

Unique considerations in children

Differences between adult and pediatric eyes in both corneal structure and thickness suggest the potential for differences in NK between the 2 populations. As another consideration for acquiring better understanding of pediatric NK, the investigators observed, “Children are at higher risk of scarring and amblyopia, which requires long-term monitoring and adapted protocols.”

Searching the IRIS Registry for the years 2016 to 2022, they found data entered for 67,855,466 patients. Applying the ICD code for NK led to the identification of 47,700 cases of NK of which 429 occurred in the pediatric population, defined by age of less than 18 years. Two-thirds of the pediatric patients had unilateral NK.

The calculated NK prevalence in 2022 was 9.1 per 100,000 pediatric IRIS Registry patients, and the calculated average annual incidence of NK for the years 2016-2022 was 3.98 per 100,000 patients.

Demographics and clinical outcomes

Analyses of the demographic characteristics of the pediatric NK patients that three-fourths of the affected individuals were aged 6 to 17 years; 11% of patients were aged less than 3 years and 13% were in the 3- to-5-year age bracket. Median age at NK diagnosis was 10 years. Sex distribution showed a slight predominance of males (52%).

Visual acuity (logMAR) measured up to 1 year before NK diagnosis and 2 years postNK diagnosis was investigated as the primary outcome of the analysis. Visual acuity data were available for 392 cases. Compared with the year prior to diagnosis, mean VA was significantly decreased at the time of NK diagnosis, declining from 0.50 logMAR (Snellen equivalent 20/63) to 0.68 logMAR (Snellen equivalent 20/100) (P < .001). Vision gradually improved over time and among eyes seen at 19 to 24 months postdiagnosis, mean VA was 0.52 logMAR (Snellen equivalent 20/63) (P < .012).

Comorbidities and interventions

The review also extracted data on corneal comorbidities and therapeutic interventions. It found that the most common associated ophthalmic diagnoses were corneal ulcer (29.5%), corneal opacity (29%) and herpetic keratitis (4.9%). Burn injury and corneal disorder due to contact lens wear were each present in 2.3% of cases.

The most common interventions consisted of punctal plugs (6.5%), therapeutic contact lenses, including scleral lens (5.6%), and amniotic membrane transplantation (5.2%). Tarsorrhaphy was performed in 2.9% of cases and 4 eyes (0.7%) underwent penetrating keratoplasty.

References

1. Bian Y, Ma KK, Hall NE, et al. Neurotrophic keratopathy in the United States: an Intelligent Research in Sight Registry analysis. Ophthalmology. 2022;129(11):1255-1262. doi: 10.1016/j.ophtha.2022.06.019.

2. Zidan A, Gilbert, JB, Goldberg E, et al. Pediatric neurotrophic keratopathy in the United States: an IRIS Registry study. Poster presented at: American Academy of Ophthalmology 2025 annual meeting; October 18-20, 2025; Orlando, Florida.