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Parry-Romberg syndrome with iridocorneal endothelial syndrome on contralateral side: a case report

Poster Details

First Author: P. Sharma INDIA

Co Author(s):    A. Kacholia   R. Kumar Sharma   N. Jhamb              

Abstract Details


Parry Rohmberg syndrome (PRS) is one of the rare multidisciplinary disorder which causes progressive hemifacial atrophy . Ocular manifestations although rare include iris atrophy, ciliary body atrophy causing hypotony, uveitis, cataract, lens dislocation while enophthalmos being the most common on atrophic side of face, No ocular findings have been reported on normal side of the face .We report a unique case of PRS with Iridocorneal Endothelial (ICE) Syndrome on normal side of face.


A 25 years old male known case of Parry Rohmberg Syndrome syndrome with left sided hemifacial atrophy presented to eye department of government hospital with soft presenile cataract in right eye on normal side of face .


Atrophic side (left side) had enophthalmos, lagophthalmos,vascularised leucomatous opacity,hazy anterior chamber details,vision HMCF inaccurate projection Right eye was 6/60 not improving with pin hole . Right eye cornea was clear with specular count of 2679/mm 2, Normal reacting, round pupil. Fundus normal. Right eye uneventful phacoemulsification with posterior chamber foldable hydrophobic intraocular lens insertion done with post op vision of 6/6, clear cornea, round pupil, good glow and normal digital tension. Followed up for 6 weeks on topical antibiotics and steroids. Patient presented with blurring of vision in right eye from last 2 weeks after 7 months of surgery.


Right eye BCVA 6/9 . Powdery deposits,pigments on endothelium.Ectropian uvea present nasally distorting the shape of pupil.Intraocular Pressure normal.Fundus ,visual fields normal. Angles open on gonioscopy.Anterior segment Optical coherence tomography hypereflective deposits on endothelium,bunching of iris on nasally. Corneal thickness 570 um. Specular ,confocal revealed polymegathism ,pleomorphism, reduced nerves, endothelial count 1554/mm2 ,coefficient of variation 41,Hexagonality 45.Ultrasound Biomicroscopy showed mild hypotrophy of ciliary body with blunting of ciliary process in right eye, shallow anterior chamber atropic ciliary body ,blunted ciliary process in left eye. Diagnosis of ICE syndrome was made.Regular follow ups adviced for IOP measurement , slit lamp examination.


Patients with Parry Rohmberg Syndrome show hypotony of eye due to atrophy of ciliary process.Corneal edema may also be present along with pigments on endothelium.However,ICE syndrome is not known to occur.To the best of our knowledge,no Parry Rohmberg Syndrome with pesenile cataract and subsequent ICE syndrome on the normal side has been reported .By means of this report we would like to highlight this case of diagnosed Parry rohmberg syndrome with ICE syndrome .We recommend thorough glaucoma workup, corneal examination on normal facial side in all cases of Parry Rohmberg syndrome. Financial support and sponsorship Nil Conflicts of interest NIL

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