Clinical presentation, diagnosis, and treatment of glaucoma associated with Sturge–Weber syndrome

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Abstract

BACKGROUND: The prevalence of glaucoma in Sturge–Weber syndrome ranges from 30% to 71%.

AIM: The work aimed to study the clinical presentation and surgical outcomes of glaucoma in children with Sturge–Weber syndrome.

METHODS: The study analyzed treatment outcomes of 34 patients (42 eyes) with glaucoma associated with Sturge–Weber syndrome. The obtained data included age, intraocular pressure, anterior-posterior axis, corneal diameter, cupping of optic discs, drug and surgical treatment.

RESULTS: Age of patients at glaucoma onset was 1.8±0.5 years; corneal diameter was 12.4±0.1 mm, which exceeded the normal age range by 22.1%. The eyeball diameter exceeded the normal age range by 17.5%. Glaucoma was stabilized with drug therapy in 13 (31%) eyes. A total of 56 procedures were performed in 29 eyes, with an average of 1.93 per eye. One procedure was sufficient to compensate glaucoma in 14 (50%) eyes. An analysis of the hypotensive effect of the performed procedures showed that trabeculectomy was the most effective. The hypotensive effect was maintained in 76.2% and 50.7% of patients 1 and 5 years postoperatively, respectively.

CONCLUSION: Glaucoma associated with Sturge–Weber syndrome had the same clinical presentation as primary congenital glaucoma and manifested in 69% of children under 1 year of age. Corneal and eyeball diameters were increased by an average of 22.1% and 17.4%, respectively. Surgery was required in 2/3 of cases. The most effective procedure was trabeculectomy.

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About the authors

Natalia N. Sadovnikova

Saint Petersburg State Pediatric Medical University

Author for correspondence.
Email: natasha.sadov@mail.ru
ORCID iD: 0000-0002-8217-4594
SPIN-code: 4537-9231

MD, Cand. Sci. (Medicine)

Russian Federation, Saint Petersburg

Vladimir V. Brzheskiy

Saint Petersburg State Pediatric Medical University

Email: vvbrzh@yandex.ru
ORCID iD: 0000-0001-7361-0270
SPIN-code: 5442-0989

MD, Dr. Sci. (Medicine)

Russian Federation, Saint Petersburg

Marina A. Zertsalova

Saint Petersburg State Pediatric Medical University

Email: mazercalova@mail.ru
ORCID iD: 0000-0003-4559-0051
SPIN-code: 6493-7645
Russian Federation, Saint Petersburg

Andrei Yu. Baranov

Saint Petersburg State Pediatric Medical University

Email: homeandrey@rambler.ru
ORCID iD: 0000-0002-6024-4635
SPIN-code: 2345-3266
Russian Federation, Saint Petersburg

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