Chiasm-compressing rhabdomyosarcoma in a patient presumed to have juvenile-onset open-angle glaucoma.

摘要

Juvenile-onset open-angle glaucoma (JOAG) is an early-onset form of primary open-angle glaucoma (POAG) beginning between the ages of 4 and 35 years and is characterized by autosomal dominant inheritance, more rapid progression and poor response to medical treatment. The typical visual field (VF) defects in glaucoma are arcuate scotomas and nasal steps, whereas those associated with compressive lesions of the optic chiasm are central scotomas and temporal or incongruous hemianopsia respecting the vertical midline. However, glaucomatous VF defects may also be observed with intracranial compressive lesions. Optic chiasm-compressing lesions have been reported to be found later in some patients initially diagnosed with normal tension glaucoma. To our knowledge, this is the first report of a case of chiasm-compressing primary suprasellar rhabdomyosarcoma in a patient who presented with the characteristics typical of JOAG.