Using selective laser trabeculoplasty to manage glaucoma in a child with phakomatosis pigmentovascularis.

PURPOSE: To describe the management and complications associated with using selective laser trabeculoplasty (SLT) to treat elevated intraocular pressure (IOP) in a child with phakomatosis pigmentovascularis (PPV).

OBSERVATIONS: A 13-year-old female with bilateral cutaneous hemangiomas of the face and lower limb length discrepancy presented with elevated IOP in the right eye despite maximal medical glaucoma management. There was no evidence of buphthalmos, Haab's striae, posterior embryotoxon, or anterior segment dysgenesis. Initial nerve fiber layer and visual field testing confirmed structural and functional damage, respectively. Genetic testing confirmed a variant in the guanine nucleotide-binding protein G(q) subunit alpha (GNAQ) gene, confirming a diagnosis of PPV. The right eye pre-treatment IOP was 33 mmHg and the patient was treated with 360-degree SLT. Two days later, she developed a shallow peripheral choroidal effusion in the right eye that resolved with medical management. Three months after SLT, the right eye IOP was successfully reduced to 19 mmHg after the discontinuation of acetazolamide. She maintained an IOP of 18 mmHg in the right eye 11 months after SLT on latanoprost and dorzolamide-timolol only.

CONCLUSIONS AND

IMPORTANCE

Glaucoma filtering procedures in cases of PPV pose a high risk of complications and SLT offers a less-invasive, potentially clinic-based alternative. While the patient did develop a choroidal effusion post-SLT, suspected to be secondary to decreased IOP, this resolved with medical management alone without the need for incisional surgery thus illustrating both the risks and benefits of SLT in treating patients with phakomatoses-associated glaucoma.