Iridocorneal Endothelial Syndrome: A Different Approach Through Pathological Anatomy.

To describe a clinical case of a female patient with unilateral corneal edema who is diagnosed with Iridocorneal Endothelial Syndrome (ICE) through histological analysis of a specimen of descemet membrane obtained after DMEK (Descemet membrane endothelial keratoplasty) performed for corneal decompensation secondary to this syndrome.

ICE is a rare disorder that frequently affects young adults and middle-aged females. Complications are caused by the increased proliferation and migration of corneal endothelial cells to the iridocorneal angle and the iris. Consequently, they can lead to corneal edema and secondary glaucoma. This is the description of a clinical case of a patient with asymmetric corneal descompensation diagnosed with ICE based on anatomical pathology findings in a tertiary hospital in Barcelon

We describe the case of a 52-year-old female patient who was referred to our centre for asymmetrical corneal edema, predominantly in the right eye. Examination revealed a visual acuity of 0.5 in the right eye and 1 in the left eye and a hammered silver endothelium with an anterior synechia in the right eye. The left eye is unaltered. Intraocular pressure is 14 mmHg in the right eye and 15 mmHg in the left eye. Specular microscopy was performed and ICE cells were not clearly recognised. The patient was followed up by the corneologists and glaucomatologists in our hospital. Corneal edema in the right eye was increasing. No glaucomatous pathology was seen in either eye. At subsequent visits the patient continued to lose visual acuity in the right eye. She underwent surgery DMEK and the descemet membrane and endothelium were sent for a pathological anatomy study. Histological findings support the diagnosis of Iridocorneal Endothelial Syndrome as CKAE1/AE3+ and CD31- cells were found in Descemet's membrane. The patient is currently being followed up at our hospital, the graft remains transparent with a visual acuity of 0.7. The patient has not developed glaucoma during follow-up.

The case described above highlights the importance of multidisciplinary management in complex patients. This case illustrates the coordination, in the first instance, of the cornea and glaucoma specialists, as both had high suspicions of Iridocorneal Endothelial Sydnrome despite the inconclusive results of specular microscopy. For this reason, they contacted the anatomical pathology department, who carried out the immunohistochemistry study of the sample obtained during DMEK in order to reach a definitive diagnosis.