| Identifier |
walsh_2016_s1_c3-2 |
| Title |
Is It or Isn't It? - Path PPT |
| Creator |
Peter W. MacIntosh, Scott Jones, Milena Stocic, Amy Lin, Heather Moss |
| Subject |
Orbit; IgG4; Xanthogranuloma |
| History |
A 58-year-old woman with recurrent left idiopathic orbital inflammation (IOI) presented with one day of rapidly progressive bilateral proptosis, edema, right eye redness, photophobia, and blurry vision. Her right eye had previously been asymptomatic. Her left eye had macular scarring with reduced vision from presumed IOIassociated posterior uveitis. Past medical history was remarkable for lymph node and orbital biopsy during a previous episode of IOI that were negative for malignancy. Recently she had been diagnosed with asthma. Vision was 20/50 in the right eye and 20/200 in the left. Both eyes were proptotic, left 1mm more than right. She had non-tender bilateral upper eyelid edema with left medial canthal xanthelasma-like changes. The right eye had 3+ conjunctival injection, and 3+ anterior cell. Left eye slit lamp exam was normal except for iris synechiae. The dilated exam was normal in the right eye, but the left eye had macular pigment and a fibrotic scar without active inflammation. The nerves appeared normal, without pallor or edema. She was treated with IV solumedrol, topical steroids and cycloplegics to the right eye. Six hours later, right vision had deteriorated to 20/200. There was new hypopyon and corneal haze in the right eye. Orbital CT scan showed bilateral enlargement of the extraocular muscles and lacrimal glands, ethmoid sinus disease, but no abscess, mass or bony erosion. T1 MRI orbits with contrast showed bilateral enhancement of the retro-orbital fat and extraocular muscles including the tendons, but no optic nerve enhancement. The T2 MRI orbits showed straightening of the optic nerve, but no abnormal signal. Parotid and submandibular glands were enlarged. Thyroid function, TB, syphilis, ANA, RF, ANCA, ACE, Lysozyme, Chest X-ray were normal. Serum IgG4 levels were elevated. A diagnostic procedure was performed. |
| Disease/Diagnosis |
Adult onset asthma with periocular xanthogranuloma. |
| Date |
2016-02 |
| References |
1. Mudhar HS, Bhatt R, Sandramouli S. Xanthogranulomatous variant of immunoglobulin G4 scerosing disease presenting as ptosis, proptosis and eyelid skin plaques. Int Ophthalmol 2011. 31:245-48. 2. Jakobiec FA, Mills MD, Hidayat AA, et al. Periocular xanthogranulomas associated with severe adult-onset asthma. Trans Am Ophthalmol Soc 1993. 91:99-125. 3. Kerstetter J, Wang J. Adult orbital xanthogranulomatous disease. A review with emphasis on etiology, systemic associations, diagnostic tools, and treatment. Dermatol Clin 2015. 33:457-463. 4. Mottow-Lippa L, Jakobiec FA, Smith M. Idiopathic inflammatory orbital pseudotumor in childhood. Results of diagnostic tests and biopsies. Ophthalmology 1981. 88(6):565-74. 5. Xu X, Du Y, Ding Y, Ye H, Yang H. Idiopathic orbital inflammation associated with panuveitis in an adult and a review of the literature. Eye Sci 2013. 28(1):40-3. |
| Language |
eng |
| Format |
application/pdf |
| Type |
Text |
| Source |
48th Annual Frank Walsh Society Meeting |
| Relation is Part of |
NANOS Annual Meeting Frank B. Walsh Sessions; 2016 |
| Collection |
Neuro-Ophthalmology Virtual Education Library - Walsh Session Annual Meeting Archives https://novel.utah.edu/Walsh/index3.html |
| Publisher |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E SLC, UT 84112-6144 |
| Rights Management |
Copyright 2016. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6w986tz |
| Setname |
ehsl_novel_fbw |
| ID |
179330 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6w986tz |
