Purpose

To characterise the spectrum of ophthalmic manifestations in linear scleroderma and Parry-Romberg syndrome, with emphasis on findings relevant to the oculoplastic surgeon.

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Methods

Retrospective case series of 22 consecutive patients with linear scleroderma spectrum disorders, including Parry-Romberg syndrome and en coup de sabre variants, across a tertiary eye care network between 2015 and 2025. Diagnosis was clinical, with histopathology where biopsy was performed. Demographics, laterality, visual acuity, adnexal, orbital, anterior and posterior segment findings, imaging, and interventions were reviewed.

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Results

13 females and 9 males (mean age 14 years, range 1–39; 68% paediatric). Disease was unilateral, with left-sided predominance (59%). Hemifacial atrophy was present in 91% and en coup de sabre in 59%; both coexisted in 50%. Histopathology confirmed diagnosis in 27%. Findings included orbital fat atrophy with enophthalmos/hypoglobus (14%); adnexal abnormalities (ptosis, madarosis, lid coloboma) (18%); medial canthal dystopia (9%); lacrimal involvement (9%); restrictive strabismus and superior oblique palsy (18%); microphthalmia (5%); uveitis with KPs and corneal opacity (14%); and posterior segment findings (Coats-like retinopathy, vascular occlusion, optic disc oedema, atrophy, choroidal folds) (18%). Two eyes progressed to phthisis. Severe visual impairment (≤20/200) occurred in 27%. Reconstructive interventions included autologous fat transfer and medial canthoplasty in selected cases.

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Conclusion

Ophthalmic involvement in linear scleroderma extends beyond hemifacial atrophy, spanning the adnexa, orbit, and globe. Sight-threatening posterior segment vasculopathy and orbital changes warrant systematic screening and multidisciplinary care.

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Additional Authors

First name	Last name	Base Hospital / Institution
Anasua	Ganguly	LV Prasad Eye Hospital

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