Medical Management Of Acute Closed-Angle Glaucoma In Patient With Mucopolysaccharidosis In Intensive Care Unit

The purpose of this case is to describe the management of complex patients, such as those with mucopolysaccharidosis type I, and their complications.

In this case report, we observed an angle closure in a patient with mucopolysaccharidosis, who required respiratory support in the context of anaphylaxis, and was admitted to the intensive care unit.

The combination of these factors necessitates strictly medical management of the situation.

Case report at Valle Hebrón hospital. 

The case of a 47-year-old woman with mucopolysaccharidosis type I, subtype Hurler Scheie, is presented, with ocular manifestations in the form of stromal corneal opacities and glaucoma, currently being treated with hypotensive eye drops in both eyes (OU).

She had an acute glaucoma episode in the right eye (OD) previously, and peripheral iridectomies performed in OU.

During an episode of anaphylaxis due to bupivacaine, the patient presented acute loss of visual acuity in OU and intense ocular pain, predominantly in the left eye (OS). In the examination in the intensive care unit, a hand motion visual acuity (VA) was observed in OD and light perception in OS. Corneal generalized opacity were observed, with iris contraction in OD, and unreactive mydriasis in OS. The intraocular pressure (IOP) found was 44 mmHg in OD and 58 mmHg in OS. Since the patient was monitored and required assisted ventilation, medical management was initiated, starting with triple topical hypotensive medication, topical pilocarpine and oral acetazolamide.

One week later we observed an IOP of 20 mmHg in OU, and one month after the VA was 0.1 in OD and hand motion in OS.

When the patient was mobilizable, an anterior segment optical coherence tomography was performed, revealing corneal thickening involving the stroma, partial permeabilization of both iridectomies, and angle closure in OS. An attempt was made to reinforce the iridectomies, unsuccessfully due to corneal opacity, opting for posterior iridoplasty.

This case is a challenge regarding the patient's clinical context. Patients with mucopolysaccharidosis may exhibit various ophthalmological manifestations, like corneal opacities and glaucoma. They can also present with angle closure, which increases the risk of developing an acute closed-angle glaucoma (ACAG). In this case, management was strictly medical and showed a favorable outcome.

Furthermore, the patient had a history of peripheral iridectomies due to a previous ACAG in the OD. However, she experienced a new episode of ACAG, with an image revealing their non-permeability. This could be due to iris thickening over time, leading to iridectomy failure, prompting us to consider iridoplasty as a therapeutic option in such cases.