RESUMO
The aim of the study was to report a case of orbital perivascular epithelioid cell tumor (PEComa) in a known diagnosed patient of tuberous sclerosis and retinal astrocytic hamartoma. 43-year-old female presented with rapid progressive painful proptosis in the left eye, also reported new mass growing in her upper back. The patient past medical history is significant for left renal angiomyolipoma and multiple bilateral lung cysts of which she underwent right nephrectomy and lung biopsy, respectively. The lung biopsy turned diagnostic for lymphangiomyomatosis. On external examination, the left eye was grossly proptotic with hypoglobus. A typical butterfly distribution of sebaceous adenoma was noted across the patient cheeks and nose. Visual acuity in the right eye was 20/20 and the left eye, 20/25. Funduscopic examination identified type 1, 2, and 3 retinal astrocytic hamartomas. MRI brain and orbit was significant for a lesion arising from the lateral orbital wall with extensive bone destruction, displacing the left optic nerve medially. CT chest showed left extrathoracic mass had same radiological features as the orbital lesion; thus, an incisional biopsy performed on the former was diagnostic for PEComa with atypical features. This is the first observed case of PEComa in a known diagnosed patent with TS and retinal astrocytic hamartoma. The association of tuberous sclerosis complex and orbital PEComa is rarely and poorly reported in the literature compared to extraocular PEComa.
RESUMO
The ocular counterroll (OCR) reflex generates partially compensatory torsional eye movements during static head roll tilt. We assessed the influence of age, viewing distance and target complexity on the OCR across the age span (13-63 years; n=47), by recording eye movements during head-on-body roll tilt (0+/-40 degrees in 5 degrees steps) while subjects viewed simple vs. complex targets at 0.33 and 1m. We found that subjects > or = 31 years had lower gains than those < or =30 years, but only for far targets. Consistent with prior reports, far targets elicited higher OCR gains than near targets, and target complexity had no effect on gains, suggesting that visual input is primarily used to maintain vergence during OCR.
