| Identifier |
walsh_2024_s3_c2 |
| Title |
How to Get the Red Out |
| Creator |
Raghu Mudumbai; Courtney Francis; Eugene May |
| Affiliation |
(RM) UW Medicine, Seattle, WA; (CF) (EM) University of Washington Department of Ophthalmology |
| Subject |
Amyloidosis; Carotid Cavernous Fistula; IgG4 Related Disease |
| Description |
A 56 year old male presented for evaluation of chronic redness of the left eye for the past 2 years. It was associated with pain, proptosis, engorged conjunctival vessels, and intraocular pressure elevation. Work up for possible scleritis, infection, uveitis, was negative. The patient also had a course of oral steroids for possible idiopathic orbital inflammation which did not lead to improvement. Exam showed normal vision, 2 mm of proptosis os, boggy, engorged conjunctival and episcleral vessels on the left side. EOMS were full. There was no APD and IOP was 16 mmHg OD and 29 mm OS. Fundus exam did not reveal significant abnormalities aside from left glaucoma cupping. |
| History |
Prior testing included C3, C4 normal, RF neg, ANA neg, MPO/PR3/ANCA neg HLA b27 neg, CCP neg, Hsv1 igG positive, Hga1c 6.3, ACE normal, HIV negative. An angiogram to evaluate for C-C fistula which was negative. MRI of the Orbits indicated Nodular enhancement along the left optic nerve with repeat imaging showing the same as well as the orbital roof. Biopsy of the optic nerve sheath indicated Amyloidosis with fibrosis with diffuse involvement of the orbital fat and optic nerve sheath with Amyloid. There was also amyloid identified in vessel wall. Liquid chromatography tandem mass spectrometry (LC MS/MS) indicated AL-Kappa. Workup for systemic amyloidosis was negative. The patient received orbital radiation with improvement of proptosis and the engorged conjunctival vessels. |
| Disease/Diagnosis |
Orbital and Ocular Amyloidosis |
| Date |
2024-03 |
| References |
1. Taban M, Piva A, See RF, Sadun AA, Quiros PA. Review: orbital amyloidosis. Ophthalmic Plast Reconstr Surg. 20:162-5. 2004 2. Medel Jiménez R, Sánchez España JC, Vasquez LM, Tapia Bahamondes A, Rondón M Orbital and peri-orbital amyloidosis: a report of four cases. Orbit. 3:148-153, 2019 3. Dammacco R, Merlini G, Lisch W, Kivelä TT, Giancipoli E, Vacca A, Dammacco F. Amyloidosis and Ocular Involvement: an Overview. Semin Ophthalmol. 1:7-26, 2019 4. Reynolds MM, Veverka KK, Gertz MA, Dispenzieri A, Zeldenrust SR, Leung N, Pulido JS. Ocular Manifestations of Systemic Amyloidosis Retina. 38:1371-1376, 2018 5. Kanaan MZ, Lorenzi AR, Thampy N, Pandit R, Dayan M. Bilateral Non-arteritic Anterior Ischaemic Optic Neuropathy as the Presentation of Systemic Amyloidosis. Neuroophthalmology. 41: 330-334. 2017. |
| Language |
eng |
| Format |
application/pdf |
| Type |
Text |
| Source |
2024 North American Neuro-Ophthalmology Society Annual Meeting |
| Relation is Part of |
NANOS Annual Meeting 2024 |
| Collection |
Neuro-Ophthalmology Virtual Education Library: Walsh Session Annual Meeting Archives: https://novel.utah.edu/Walsh/ |
| Publisher |
North American Neuro-Ophthalmology Society |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Rights Management |
Copyright 2024. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s629bqbg |
| Setname |
ehsl_novel_fbw |
| ID |
2589444 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s629bqbg |
