Chandler’s Syndrome: A Rare Diagnosis With Unusual Presentation

We report a case of Chandler’s syndrome with unusual presentation and successful treatment with descemet stripping endothelial keratoplasty (DSEK). A 36-year-old healthy male presented with unilateral corneal edema following trauma with iron nail. On examination, there was microcornea with cornea having increased thickness due to diffuse microcystic and stromal edema, and descemet folds along with a few bullae seen inferiorly without any epithelial defect in the left eye. The anterior chamber was shallow with no inflammatory signs and the optic disc had a cupping of 0.7:1 with thinning of neuroretinal rim with digitally raised intraocular pressure. Endothelial morphology revealed bizarrely shaped endothelial cells with blackout areas, pleomorphism (minimum cell area 689/maximum cell area 1600), polymegathism (CV-19), and reduced cell counts (CD-834) with normal indices in the right eye. With the above findings of unilateral corneal decompensation, glaucoma and abnormal endothelial parameters, a diagnosis of Chandler’s syndrome was made and the patient was taken up for DSEK after failure of medical therapy. With raised intraocular pressure (IOP) and shallow anterior chamber, DSEK was challenging and done under perioperative intravenous mannitol cover. At one-month follow up, the cornea was compact and well adhered to the host cornea, and the IOP was well controlled under anti-glaucoma medications. At 6-month follow up, the patient had a visual acuity of 20/60 with +1 dioptre sphere and -1 dioptre cylinder at 60° and the IOP was well controlled on tapering dose of topical steroid and antiglaucoma medications.