Navigating Surgical Challenges: Managing Juvenile Glaucoma in a Patient with Dorfman–Chanarin Syndrome

Abstract

We report a surgically challenging case, in the context of a diagnosis of juvenile glaucoma refractory to drug therapy, multi-operated, known patient with congenital ichthyosis, part of Dorfman–Chanarin Syndrome (DCS), with a single functional eye. She is a young patient (54) and housewife in an urban environment known to have DCS and BE (both eyes), strong myopia, and congenital nystagmus. She initially underwent cataract surgery in 2015 and again in 2017. As of 2015, she was known to have juvenile glaucoma under maximal therapy. The important increases in pressure started in 2020 when the dermatological condition worsened (exacerbation of skin changes in the context of ichthyosis), the patient is in menopause, and presbyopia has set in. The glaucoma could no longer be controlled with medication and required serial surgery in both eyes (initially in the right eye in 2020 and in the left eye in 2023). The right eye showed a favorable evolution until 2024, when a second trabeculectomy became necessary, with a favorable evolution. Conclusions: To our knowledge, such a case has not been documented in the medical literature. Frequent monitoring of intraocular pressures and prompt treatment are required. It is a rare association, a very complicated case of managing a patient with refractory glaucoma and multiple associated ophthalmic and systemic pathologies. We are also dealing with a single functional eye, difficult to manage due to a thin sclera that has caused intraoperative difficulties, and the association of congenital nystagmus and strabismus.