Choroidal ganglioneuromas in Francois variant neurofibromatosis-1: A rare retinoblastoma mimic

Neurofibromtosis-1 (NF-1) is the commonest oculo-neuro-cutaneous syndrome with multiple ocular manifestations. Reporting three children who presented with unilateral glaucoma (buphthalmos), ipsilateral facial hemihypertrophy, and eyelid plexiform neurofibroma: completing the triad of François syndrome, a rare NF1 variant. Two presented with leukocoria and were referred to as retinoblastoma suspects. Histopathology showed ganglioneuroma, a benign choroidal tumor, associated with NF-1, which does not need treatment. Knowledge of this rare condition avoids misdiagnosis of retinoblastoma, prevents aggressive management, and the associated psychological impact.

Electron beam radiotherapy for the management of recurrent extensive ocular surface squamous neoplasia with orbital extension.

Recurrent extensive ocular surface squamous neoplasia (OSSN) with orbital invasion can be successfully managed with external radiotherapy using electrons resulting in eye and vision salvage. We report a case of right eye recurrent OSSN in an immunocompetent adult Indian male, with extensive orbital involvement. The patient had two previous surgical excisions with recurrent disease. At this stage, conventionally exenteration is considered the treatment modality. However, he was treated with 5040 cGy radiotherapy (15eV electrons) resulting in complete disease regression. At the end of 3 years follow‑up, the patient was disease free, maintained a vision of 20/25, with mild dry eye, well‑managed with topical lubricants. Extensive OSSN with orbital invasion does not always need exenteration. External beam electron radiotherapy provides a noninvasive cure with organ and vision salvage and should be considered in extensive OSSN not amenable to simple excision biopsies. Long‑term studies to evaluate the effect of radiation on such eyes are suggested.

Novel surgical technique for the management of partial cryptophthalmos.

We report a case of a 2‑month‑old baby with bilateral nonsyndromic partial cryptophthalmos presenting with upper eyelid incomplete development and fusion to the cornea with resultant inability to close the eyes. He was managed successfully with bilateral upper lid reconstruction with composite graft using maternal skin and oral mucous membrane, amniotic membrane, and donor scleral graft. After this one stage surgery, lids were well‑formed, and the patient was able to close both eyes, thus achieving good anatomical, functional, and cosmetic outcome.