The Evolution Of Ice Syndrome Over A Decade: From Initial Symptoms To Multisurgical Treatment

To describe the clinical progression and surgical approach in a case of severe iridocorneal endothelial (ICE) syndrome with complications such as treatment-resistant glaucoma, anterior synechiae, and corneal failure. This case report illustrates the long-term course of ICE syndrome, from initial presentation to advanced stages necessitating multiple surgical interventions, including Baerveldt valve implantation and penetrating keratoplasty (PKP).

Cornea Department, Barraquer Ophthalmology Centre, Barcelona, Spain.

A 49-year-old female with a decade-long history of ICE syndrome in her left eye (OS) initially presented with discomfort and asymmetry in her pupil. Her best-corrected visual acuity (BCVA) was 0.65 in OS. Clinical assessment revealed temporal corectopia and iris hypoplasia, impairing the oculomotor reflex in the temporal quadrants, while the remainder of the examination was unremarkable.

As the disease progressed, she developed diffuse iris atrophy, complete loss of oculomotor reflex, extensive (360º) anterior synechiae, and persistent intraocular pressure (IOP) elevation despite medical treatment. A Baerveldt valve was implanted with a fascia lata patch graft after pharmacological management failed to control the IOP.

Further disease progression led to a decline in BCVA to 0.04 due to diffuse stromal and microcystic epithelial edema, corneal opacification, and endothelial deposits. Additionally, polycoria was noted. After IOP stabilization, the patient underwent PKP combined with artificial iris and intraocular lens implantation following lens extraction. The postoperative course was uneventful, with BCVA improving to 0.45.

ICE syndrome results from abnormal endothelial cell proliferation within the anterior chamber. Initial signs are often subtle, such as mild corectopia, but the disease can progress to extensive iris atrophy, corneal decompensation, and refractory glaucoma. In severe cases, PKP combined with artificial iris implantation can significantly improve visual outcomes.

Patients frequently require multiple surgical interventions, particularly glaucoma drainage procedures, as IOP control before keratoplasty is critical for successful outcomes. Early detection of ICE syndrome is essential, especially in younger women presenting with corneal or iris anomalies and/or unilateral glaucoma. Continuous monitoring is key for early diagnosis and management.