An Interesting Case Of Cogan Reese Syndrome

The purpose of this case report is to elaborate on the clinical features of a patient with Cogan Reese syndrome or Iris Nevus Syndrome and how our management of cataract in these patients would change. 

The Irido-Corneal-Endothelial syndrome consists of three types of presentations - Chandler, Cogan Reese, and Essential Iris Atrophy. Out of the three Chandler syndrome is the most common. Cogan Reese is relatively rare and thus diagnosis may be confusing.

An important differential of Cogan Reese syndrome is iris melanoma and must be ruled out,though it is also quite rare.

A forty two year old female presented to the out patient clinic for blurring of vision in her right eye since ten years,aggravated in the last five years.

On examination her visual acuity was reduced to 6/18 on Snellen's chart, and near vision was impaired to N36 with Jagger's chart for her right eye.

On slit lamp examination, ectropion uvea with corectopia was noted along with multiple discreet nodules on the iris surface, which were iso-pigmented to the colour of the iris. On gonioscopy multiple strands were seen adherent to the cornea and iris in the periphery along with some areas of PAS. Lenticular assessment revealed cataract (NS2) that would have to be operated.

Anterior segment ultrasound biomicroscopy revealed the diagnosis of ICE syndrome, clinically falling under Cogan Reese syndrome. Specular microscopy examination revealed the typical dark light reversal pattern. 

The patient was counseled about her condition, especially regarding her apprehension about her other eye getting involved, which thankfully in these cases is very atypical and rare. 

It is important to note the findings of ICE syndrome, as they present in older individuals and present many challenges to the patient visually.

They further required skilled management during their cataract surgeries as well.