Ophthalmic findings in pediatric patients with craniofacial scleroderma

Area of Science:

• Ophthalmology
• Pediatric Rheumatology
• Dermatology

Background:

• Craniofacial linear scleroderma is a rare autoimmune condition affecting the skin and underlying tissues of the head and face.
• Ophthalmic involvement in pediatric scleroderma can lead to significant visual impairment if not identified and managed promptly.

Purpose of the Study:

• To determine the incidence and range of eye abnormalities in children diagnosed with craniofacial linear scleroderma.
• To characterize the spectrum of ocular findings in this pediatric population.

Main Methods:

• Retrospective review of medical records for 72 pediatric patients (<18 years) with craniofacial scleroderma treated at a specialized clinic.
• Ophthalmic examinations included visual acuity, ocular motility, slit lamp exams, fundus photography, and other imaging modalities as available.
• Abnormal ophthalmic findings were categorized by ocular segment (anterior, posterior, adnexal).

Main Results:

• Anterior segment abnormalities were present in 74% of patients, with dry eye disease being most common (64%).
• Ocular adnexal abnormalities occurred in 53%, primarily meibomian gland disease (36%).
• Posterior segment abnormalities were documented in 61%, with abnormal retinal vessels (23%) being the most frequent finding; retinal dialysis was rare (1 patient).

Conclusions:

• This study represents the largest cohort investigating ophthalmic findings in pediatric craniofacial scleroderma.
• The results highlight the wide array of ocular manifestations, affecting ocular adnexa, ocular surface, motility, and both anterior and posterior segments.
• Regular and thorough ophthalmic assessments are essential for managing children with this condition.