Avengers Assemble! - Video

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Identifier walsh_2016_s4_c1-3
Title Avengers Assemble! - Video
Creator Radha Ram, Carrie A. Mohila, Jeremy Y. Jones, Veeral S. Shah
Subject Papilledema; Increased Intracranial Pressure; Intracranial Tumors; Optic Nerve Tumors; Vision Loss
History A five-year-old otherwise healthy boy presented with a two-week history of behavioral changes, nausea, vomiting, headache, and subacute vision loss bilaterally. Six weeks prior to presentation to our hospital, he had presented to an outside hospital with esotropia and blurred vision in both eyes. At that time, he had bilateral 4+ optic nerve edema. MRI demonstrated a right mesiotemporal lobe arachnoid cyst. Opening pressure on lumbar puncture was elevated, and the patient underwent an arachnoid cyst fenestration. He improved clinically for two weeks but subsequently had a rapid decline in vision and mental status. Repeat MRI showed global enhancement of the leptomeninges, especially of the right mesiotemporal lobe near the arachnoid cyst, and enhancement of both optic nerves and the optic chiasm. Repeat lumbar puncture showed an opening pressure >55cm H2O. He was transferred to our hospital for further care. Initial ophthalmologic examination at our hospital revealed 20/400 visual acuity in the right eye and 20/800 visual acuity in the left eye. Visual fields were constricted to approximately 10-degree nasal islands bilaterally. Motility and alignment were normal. Ophthalmoscopy revealed bilateral 4+ optic disc edema with surrounding peripapillary retinal edema. Given the significant bilateral vision loss, an optic nerve sheath fenestration was attempted, but engorgement of the ophthalmic vessels prevented completion of the procedure. Repeat lumbar puncture showed an opening pressure again of >55cm H2O. An extraventricular drain was placed and dura was biopsied, revealing benign dense connective tissue. An infectious, autoimmune, and paraneoplastic laboratory work-up was unrevealing, and CSF cytology was negative for malignancy and infection. Repeat MRI brain and spine revealed a possible primary tumor involving the right mesiotemporal lobe and hippocampus and multifocal areas of leptomeningeal enhancement outlining the spinal cord conus, raising a question of metastatic disease. Spinal cord arachnoid biopsy was performed, showing histiocytic infiltration with reactive changes. An additional procedure was performed.
Disease/Diagnosis Pediatric malignant spindle cell neoplasm with epithelioid features consistent with malignant peripheral nerve sheath tumor with leptomeningeal dissemination.
Date 2016-02
References 1. Lindsay RA, Gupta D, Keene CD, Bhrany AD, Chang SH. Malignant Peripheral Nerve Sheath Tumor of the Lower Eyelid: Case Presentation and Literature Review. Ophthal Plast Reconstr Surg. 2015 May 20. 2.Waxweiler TV, Rusthoven CG, Proper MS, Cost CR, Cost NG, Donaldson N, Garrington T, Greffe BS, Heare T, Macy ME, Liu AK. Non-Rhabdomyosarcoma Soft Tissue Sarcomas in Children: A Surveillance, Epidemiology, and End Results Analysis Validating COG Risk Stratifications. Int J Radiat Oncol Biol Phys. 2015 Jun 1;92(2):339-48. 3.Patel TD, Shaigany K, Fang CH, Park RC, Baredes S, Eloy JA. Comparative Analysis of Head and Neck and Non- Head and Neck Malignant Peripheral Nerve Sheath Tumors. Otolaryngol Head Neck Surg. 2015 Sep 25. 4.Rajabi MT, Riazi H, Hosseini SS, Rajabi MB, Mazloumi M, Gharehbaghi G, Amoli FA. Malignant peripheral nerve sheath tumor of lacrimal nerve: a case report. Orbit. 2015 Feb;34(1):41-4.
Language eng
Format video/mp4
Type Image/MovingImage
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 84112-6155
Rights Management Copyright 2016. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright
ARK ark:/87278/s6b885rb
Setname ehsl_novel_fbw
ID 179373
Reference URL https://collections.lib.utah.edu/ark:/87278/s6b885rb
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