Early-Onset Terrien Marginal Degeneration With Documented Topographic Progression In A 14-Year-Old Pediatric Patient: Multimodal Imaging And Management Considerations.

To report a case of early-onset Terrien marginal degeneration (TMD) in a child with documented tomographic progression over a 3-year follow-up period. This report will address the challenges of diagnosing pediatric TMD, differential diagnosis, and treatment strategies based on current ESCRS clinical standards. 

The case was followed longitudinally at two tertiary referral ophthalmology centers with dedicated corneal units in Spain, providing specialized pediatric and anterior segment care. 

Observational case report of a pediatric patient with bilateral superior and inferior peripheral corneal opacification. A complete ophthalmologic evaluation was performed, including UDVA/CDVA, slit-lamp biomicroscopy, corneal tomography (Pentacam, Oculus), pachymetry mapping, and AS-OCT follow-up. Systemic and serologic studies were negative for vasculitic or autoimmune disease.

At presentation, UDVA was 20/25 OD and 20/22 OS. Slit-lamp exam showed bilateral superior and inferior opacities with superficial neovascularization, involving the visual axis in OD. Ferrous lines were noted at both margins. Peripheral stromal thinning was observed in OD (superior and inferior) and OS (superior). Bilateral limbal racial melanosis was present.

A pediatric male presented with asymptomatic superior peripheral corneal opacification. CDVA improved to 20/20 with refraction. Slit-lamp exam showed intact epithelium, superficial neovascularization, and superior crescent-shaped stromal thinning with lipid at the leading edge.

Tomography revealed localized superior steepening with asymmetric astigmatism and normal central thickness; pachymetry confirmed isolated peripheral thinning. AS-OCT demonstrated anterior stromal hyperreflectivity and furrow formation, consistent with TMD.

Over 3 years, serial tomography showed sustained superior keratometric progression. Inflammatory markers, RF, ANA, and ANCA were negative. No clinical signs of FSMK, PUK, or Mooren ulcer were found.

Terrien marginal degeneration (TMD) in children may show early tomographic progression before visual acuity declines, often as a quiescent, non-inflammatory condition. Multimodal imaging is crucial for diagnosis and follow-up, particularly corneal tomography and AS-OCT, which detects subtle structural changes not clinically evident and supports early monitoring.

Given the risk of progressive peripheral thinning and secondary ectasia, close longitudinal surveillance is required. This case highlights the need to recognize atypical early-onset TMD and implement structured imaging-based follow-up in pediatric corneal practice. TMD typically affects middle-aged men and is rare in children, warranting heightened clinical suspicion.