Alberto Distefano, Julie Cho, Adeniyi Fisayo, Mahsa Sohrab
Subject
Erdheim-Chester disease, Orbital tumors, Proptosis
Presenting Symptom
A 60 year-old female with history of hypertension, hyperlipidemia, and Lyme disease 12 years ago treated with doxycycline, developed extreme thirst and polyuria four years ago and was diagnosed with central diabetes insipidus. Magnetic resonance imaging (MRI) at that time showed enhancement of the pituitary gland and stalk. She was treated with DDAVP with clinical improvement. Repeat MRI six months later showed some improvement. She underwent extensive testing including Positron Emission Tomograpy/Computed Tomography (PET/CT) and lumbar puncture, which were inconclusive or indeterminate. Her disease was categorized as idiopathic hypophysitis and infundibulitis. One year ago, the patient underwent right ear surgery, and afterward developed dizziness. MRI was performed and showed bilateral intraconal enhancing masses with proptosis. The previously noted hypophyseal enhancement had resolved. She was started on oral prednisone 60 milligrams (mg) without significant improvement, and was then referred to our ophthalmology clinic for further evaluation. On exam, best corrected visual acuity was 20/30 in the right eye and 20/25 in the left eye. Pupils were briskly reactive without relative afferent pupillary defect. Intraocular pressure was within normal limits. Motility was severely reduced in supraduction. Color plates were full bilaterally. Exam revealed mild proptosis greater on the left than the right. Otherwise, ophthalmic exam, including dilated fundus exam, was normal. Imaging was reviewed and an orbital biopsy was performed.
Date
2018-03
Language
eng
Format
video/mp4
Source
2018 North American Neuro-Ophthalmology Society Annual Meeting
Relation is Part of
NANOS Annual Meeting 2018: Frank B. Walsh Session 2