Pediatric Ocular Surface Squamous Neoplasia in the Absence of Known Risk Factors and Systemic Conditions: A Case Series.

Introduction: Ocular surface squamous neoplasia (OSSN) includes precancerous and cancerous epithelial lesions of the conjunctiva and cornea, typically affecting older adults with risk factors like immunosuppression, sun exposure, and viral infections. Pediatric OSSN is rare, with few reported cases. We present a series of 16 pediatric and adolescent OSSN cases without known genetic or infectious risk factors. Methods: Descriptive case series at a single tertiary eye care center in Nepal where children with histopathologically proven OSSN were identified, and their disease was characterized. Results: OSSN was observed in 16 eyes of 16 pediatric patients (6-18 years old) with no identifiable risk factors. All patients were seen at Tilganga Institute of Ophthalmology, Kathmandu, Nepal, between May 2018 and June 2022. All lesions were histopathologically proven as OSSN and characterized by type. Nine (56%) were conjunctival intraepithelial neoplasia (CIN) I, 5 (31%) were CIN II, and 2 (13%) were CIN III. There were no cases of squamous cell carcinoma. None of the patients had xeroderma pigmentosum, HIV, or hepatitis B or C. Conclusions: This work reports a case series of pediatric OSSN in the absence of risk factors and systemic conditions. More than half of the patients had mild CIN. Though rare, this case series highlights the importance of considering OSSN in the differential diagnosis of pediatric ocular surface lesions, perhaps especially in this geographic location.