Shabbir Syndrome: Case Report of a Rare Disease

  • Rachna Meel Oculoplasty unit, Dr Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi
  • Meenakshi Wadhwani Chacha Nehru Bal Chikitsalya, New Delhi
Keywords: Shabbir Syndrome

Abstract

Purpose: To report a case of Laryngeal Onycho Cutaneous Syndrome in a 10 year old child presenting with an ocular surface mass.

Methods: A 10 year old boy presented in the out-patient department with a painless mass in left eye. There was history of airway obstruction requiring tracheotomy in early childhood and recurrent skin ulcerations. Aslit lamp examination revealed fleshy mass arising from the supero-nasalconjunctiva of left eye. Systemic examination revealed ulcerative lesions on cheeks and ears with dystrophic nails in hands and feet.

Results: The ocular surface lesion was treated successfully with topical steroid drops.

Conclusion: Steroids may be useful in treating early lesion of ocular surface in LOCS. The importance of regular follow must be emphasized for timely management of frequent granulation tissue outgrowth in bid to avoid development of severe symblepharon and blindness.

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Author Biographies

Rachna Meel, Oculoplasty unit, Dr Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi

Assistant Professor

Meenakshi Wadhwani, Chacha Nehru Bal Chikitsalya, New Delhi

Assistant Professor

Published
2019-09-03
How to Cite
Meel, R., & Wadhwani, M. (2019). Shabbir Syndrome: Case Report of a Rare Disease. Nepalese Journal of Ophthalmology, 11(1), 105-107. https://doi.org/10.3126/nepjoph.v11i1.25446
Section
Brief Communications