| Identifier |
walsh_2016_s2_c3-2 |
| Title |
Heart of Darkness - Path PPT |
| Creator |
Shira Simon; Shiraaz I. Rahman; John C. Heymann; Bahram Rahmani; Nicholas J. Volpe |
| Affiliation |
(SS) (SIR) (NJV) Northwestern Memorial Hospital Department of Ophthalmology Chicago, IL; (JCH) Lurie Children's Hospital Department of Neuroradiology Chicago, IL; (BR) Lurie Children's Hospital Department of Ophthalmology Chicago, IL |
| Subject |
Epstein-Barr Virus |
| History |
An 18 year-old woman presented to the emergency department in October 2014 with three days of blurred vision in her left eye. Past medical history included a hypoplastic right heart status-post orthotopic heart transplant at 2 months of age, chronic kidney disease with BK viremia since 12 years of age, and recurrent post-transplant lymphoproliferative disorder (PTLD, diagnosed at 15 years of age, with two subsequent recurrences). Medications included tacrolimus, leflunomide, trimethoprim/sulfamethoxazole, atorvastatin, amlodipine, and 81 mg aspirin. Review of systems was notable only for mild nasal congestion and cough for the past four weeks. Visual acuity was 20/20 in the right eye, 20/40 in the left, with a mild APD. Ishihara plates were full in the right eye, 2/11 in the left. Automated perimetry revealed an inferonasal depression in the left eye. There was no proptosis or ptosis. The anterior segment examination was normal; posterior segment revealed tortuous vasculature in both eyes and mild nasal disc elevation in the left eye, along with a fan-shaped intraretinal hemorrhage nasal to the disc margin. MRI brain and orbits without contrast (contrast withheld in setting of renal dysfunction) demonstrated a 0.7 x 0.5 cm mass along the medial aspect of the left orbital apex, possibly contiguous with the optic nerve. Lumbar puncture on admission demonstrated a normal opening pressure with no malignant cells. Patient was started on dexamethasone and then rituximab for presumed PTLD recurrence. At 1 week, steroids were tapered and the exam was stable. At 2 weeks, visual acuity worsened to 20/200, and there was a 3+ rAPD. Repeat MRI was unchanged. |
| Disease/Diagnosis |
Epstein-Barr Virus-associated smooth muscle tumor (EBV-SMT) of the left optic nerve. |
| Date |
2016-02 |
| References |
1. Josen J, et al. Epstein-Barr virus-associated smooth muscle tumors in children following solid organ transplantation: A review. Pediatr Transplant. 2015 Mar;19(2):235-43. 2. Deyrup AT, et al. Epstein-Barr virus-associated smooth muscle tumors are distinctive mesenchymal tumors reflecting multiple infection events: a clinicopathologic and molecular analysis of 29 tumors from 19 patients. Am J Surg Pathol. 2006 Jan;30(1):75-82. 3. Elawabdeh N et al. 'Epstein-Barr virus associated smooth muscle tumors in post transplant pediatric patients two cases of rare locations, and review of the literature.' Fetal Pediatr Pathol. 2013 Jun;32(3):184-91. |
| Language |
eng |
| Format |
application/pdf |
| Format Creation |
Microsoft PowerPoint |
| 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/ |
| Publisher |
North American Neuro-Ophthalmology Society |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Rights Management |
Copyright 2016. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6cc3x92 |
| Setname |
ehsl_novel_fbw |
| ID |
179334 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6cc3x92 |
