Bilateral noncalcified astrocytic hamartomas in retinitis pigmentosa: Multimodal imaging evaluation over 8 years of follow-up

Abstract

Purpose: To report the evolution of optic disk noncalcified astrocytic hamartomas in a patient with retinitis pigmentosa throughout 8 years of follow-up. Methods: Case report. Results: A 42-year-old white man with a diagnosis of retinitis pigmentosa was referred to our medical retinal center for the first time in 2010, for the development of a new optic nerve head lesion in the right eye. Fundus examination, fundus autofluorescence, fluorescein and green indocyanine angiography, spectral-domain optical coherence tomography were performed and confirmed the presence of a papillary noncalcified astrocytic hamartoma in the right eye. Four years later, multimodal imaging evaluation revealed in the left eye the presence of a first optic disk benign tumor, and in 2018, a second in the nasal retina, while in the right eye the sole papillary lesion increased slightly in size. Conclusions: Noncalcified astrocytic hamartomas can occur in patients with retinitis pigmentosa and can increase in size and number in time. A complete multimodal imaging evaluation is necessary to identify and classify any kind of new lesion that, as known, are associated with these group of hereditary retinal degenerations.

Optic Disc Edema and Elevated Intracranial Pressure (ICP): A Comprehensive Review of Papilledema

“…The nerve fiber layer, which is normally translucent, may be partially myelinated from anomalous migration of oligodendrocytes beyond the lamina cribosa, blurring the margins of the optic disc [ 69 ]. Other causes of elevated optic disc head include peripapillary astrocytic hamartomas and other peripapillary tumors [ 70 , 71 ]. One of the most common causes of pseudopapilledema is optic disc drusen: conglomerates of mucopolysaccharides and proteinaceous material that develop within nerve tissue and may lead to an elevated disc head.…”

Papilledema: A Problem of Modern Ophthalmology, Neurology, and Neurosurgery

“…Новообразования в области головки диска ЗН (например перипапиллярные астроцитарные гамартомы) также придают краям диска приподнятый и размытый вид, при этом не вызывая истинного отека [24,25].…”

“…Состояние может протекать асимптомно либо с изменениями в поле зрения в виде расширения границы слепого пятна, скотом. Лечение обычно не требуется [24,27].…”