Korean J Ophthalmol.  2014 Jun;28(3):272-274. 10.3341/kjo.2014.28.3.272.

Proteus Syndrome: Report of a Case with Developmental Glaucoma

• Sarman ZS
• Yuksel N
• Sarman H
• Bayramgurler D

• KMID: 2133328
• DOI: http://doi.org/10.3341/kjo.2014.28.3.272

Abstract

The purpose of this study was to report developmental glaucoma and pseudopapilledema in a patient with Proteus syndrome. We defined the presence of developmental glaucoma, right pseudopapilledema and myopia in a 4.5-year-old patient with Proteus syndrome. Marked right hemihypertrophy, lipoma, macrodactyly, and asymmetry of the limbs were observed on systemic examination. A cavernoma was also detected in magnetic resonance imaging of the brain. The patient underwent bilateral goniotomy surgery due to glaucoma. The surgical outcomes were satisfactory in both eyes. In conclusions developmental glaucoma and pseudopapilledema might be associated with Proteus syndrome.

Keyword

Developmental glaucoma; Proteus syndrome; Pseudopapilledema

MeSH Terms

Child, Preschool
Disease Progression
Glaucoma/diagnosis/*etiology/physiopathology
Humans
Intraocular Pressure/*physiology
Magnetic Resonance Imaging/*methods
Male
Proteus Syndrome/*complications

Figure

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  Fig. 1 (A) Right hemihypertrophy, (B) subcutaneous tumors and lipoma, (C) macrodactyly of the third and fourth digits of the right hand, and (D) macrodactyly of nearly all the toes.

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  Fig. 2 Magnetic resonance imaging of the case, showing temporal lobe cavernoma.

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  Fig. 3 (A) Buphthalmia in the left eye, (B) pseudopapilledema in the right eye, and (C) appearance of left optic disc.

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