Published: 2021-11-23

Parry Romberg Syndrome with contralateral iridocorneal endothelial syndrome: a unique case

Usha K. Raina, Shruti Bhattacharya, Prateeksha Sharma, Varun Saini


Parry-Romberg syndrome (PRS) is a rare disorder which causes progressive hemifacial atrophy, with ocular manifestations like hypotony, enophthalmos and corneal edema on the ipsilateral atrophic side. This is a report of a unique case of PRS with contalateral manifestations like ectropion uvea, correctopia and endothelial deposits, along with polymegathism and pleomorphism seen on specular microscopy suggestive of Iridocorneal Endothelial (ICE) Syndrome. ICE syndrome and PRS have not been reported together in any literature so far. This case highlights the importance of a thorough glaucoma workup and corneal examination on the atrophic facial side as well as on the apparently normal side in all cases of PRS.


Parry Romberg syndrome, ICE syndrome, Hemifacial atrophy, Glaucoma

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