| Identifier |
walsh_2022_s2_c4 |
| Title |
Thinking Outside of the Lines |
| Creator |
Carleigh Bruce, Thomas Clark, Gregory Griepentrog, Andrea Stahulak, Mariam Ratiani |
| Subject |
vasculitides, optic neuropathy, autoimmune diseases, orbital inflammation, IgG4 related disease |
| History |
A 61-year-old male with past medical history of long-standing cocaine abuse presented with ten days of painless acute; vision loss in his right eye (OD). Initial exam revealed a visual acuity (VA) 20/100 OD, relative afferent pupillary defect; (rAPD) OD, dyschromatopsia OD, hypertropia with impaired infraduction and adduction OD, and 2 mm of OD proptosis.; Dilated fundus examination revealed 360 degrees of right optic disc edema, obscuration of surface vessels, and a; superotemporal flame hemorrhage. MRI/MRV demonstrated chronic sinus destruction, a diffuse, infiltrative, enhancing,; soft-tissue process of the bilateral pterygopalatine fossae, orbits, left more than right, and pachymeningeal enhancement of; the lateral cavernous sinuses. Otolaryngology performed bilateral maxillary antrostomies with biopsy that revealed; polypoid chronic sinusitis with focal squamous metaplasia. Extensive lab workup, including lumbar puncture, p-ANCA,; serine protease antibody, and myeloperoxidase antibody, was negative except for positive C-ANCA (1:640) and elevated; serum IgG4 (233). The patient was lost to follow up for two years and then presented with two days of left eye (OS) pain,; lower lid swelling, and binocular diplopia. He endorsed continued intranasal cocaine usage. Exam included a VA of 20/125; OD, 20/32 OS, rAPD OD, -2 abduction, adduction, supraduction, and -1 infraduction of OS, and 3 mm OS proptosis. Notably; there was a firm, nontender mass in lower left eyelid extending from below the lateral canthus to medial lid, and OS; chemosis and injection. Dilated exam was negative for acute optic nerve edema. MRI brain revealed an extensive,; enhancing, and infiltrative mass-like signal abnormality involving the left greater than right orbits and skull base, progressed; from previous imaging, with similar, mild smooth pachymeningeal enhancement along the anterior cranial fossa floor, and; scattered areas of failed CSF suppression within the cerebral sulci, as well as the frontoparietal regions near the vertex; suggesting meningeal extension. |
| Disease/Diagnosis |
CIMDL and IgG4 related disease |
| Date |
2022-02 |
| References |
1. Trimarchi, Bertazzoni, Bussi, Cocaine induced midline destructive lesions, Rhinology, 52, 104-111, 2014.; 2. Ortiz-Seller, Hernandez-Pons, Pascual, Comin Perez, Dolz Gaiton, et al., Severe Cocaine-Induced Midline; Destructive Lesions (CIMDL) Leading to Orbital Apex Syndrome and Peripheral Ulcerative Keratitis, Ocul Immunol; Inflamm 1-4, 2021.; 3. Muniz Castro, Do, Bhattacharjee, Adesina, Richiani, A Unique Presentation of Levamisole-Induced Antineutrophil-; Cytoplasmic-Antibody-Positive Vasculitis Presenting as Inflammatory Orbitopathy, Ophthalmic Plast Reconstr; Sur,. 36, e96-e100, 2020.; 4. Umehara, Okazaki, Kawa, Takahashi, Goto, et al., The 2020 revised comprehensive diagnostic (RCD) criteria for; IgG4-RD, Mod Rheumatol, 3, 529-533, 2021.; 5. Goto, Takahira, Azumi, Diagnostic criteria for IgG4-related ophthalmic disease, Jpn J Ophthalmol, 59, 1-7, 2015. |
| Format |
application/pdf |
| Type |
Text |
| Source |
54th Annual Frank Walsh Society Meeting |
| Relation is Part of |
NANOS Annual Meeting 2022: Walsh Session II |
| Publisher |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Rights Management |
Copyright 2022. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s66cg1f5 |
| Contributor Primary |
Carleigh N. Bruce, MD |
| Contributor Secondary |
Thomas Clark, Gregory Griepentrog, Andrea Stahulak, Mariam Ratiani |
| Setname |
ehsl_novel_fbw |
| ID |
2100237 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s66cg1f5 |
