Idiopathic Orbital Inflammation Leading to Unilateral Blindness Over a 2-Day Presentation in a Child.
Summary of "Idiopathic Orbital Inflammation Leading to Unilateral Blindness Over a 2-Day Presentation in a Child."
An 8-year-old boy developed vision loss to no light perception on the left side over a 2-day period. He initially presented with unilateral eyelid swelling, which progressed to bilateral edema and an eventual left-sided orbital apex syndrome. Orbital imaging revealed enlarged extraocular muscles, and biopsy confirmed idiopathic orbital inflammation. Despite subsequent orbital decompression, high-dose steroids, and additional steroid-sparing therapy, he did not regain vision after 9 months of follow up.
Affiliation
*Scheie Eye Institute, University of Pennsylvania School of Medicine; †Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia; and ‡Department of Ophthalmology, Children's Hospital of Philadelphia, University of Pennsylva
Journal Details
This article was published in the following journal.
Name: Ophthalmic plastic and reconstructive surgery
ISSN: 1537-2677
Pages:
Links
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/20859233
- DOI: http://dx.doi.org/10.1097/IOP.0b013e3181e99cdf
Medical and Biotech [MESH] Definitions
Orbital Pseudotumor
A nonspecific tumor-like inflammatory lesion in the ORBIT of the eye. It is usually composed of mature LYMPHOCYTES; PLASMA CELLS; MACROPHAGES; LEUKOCYTES with varying degrees of FIBROSIS. Orbital pseudotumors are often associated with inflammation of the extraocular muscles (ORBITAL MYOSITIS) or inflammation of the lacrimal glands (DACRYOADENITIS).
Orbital Myositis
Inflammation of the extraocular muscle of the eye. It is characterized by swelling which can lead to ischemia, fibrosis, or ORBITAL PSEUDOTUMOR.
Idiopathic Interstitial Pneumonias
A group of interstitial lung diseases with no known etiology. There are several entities with varying patterns of inflammation and fibrosis. They are classified by their distinct clinical-radiological-pathological features and prognosis. They include IDIOPATHIC PULMONARY FIBROSIS; CRYPTOGENIC ORGANIZING PNEUMONIA; and others.
Idiopathic Pulmonary Fibrosis
A common interstitial lung disease of unknown etiology, usually occurring between 50-70 years of age. Clinically, it is characterized by an insidious onset of breathlessness with exertion and a nonproductive cough, leading to progressive DYSPNEA. Pathological features show scant interstitial inflammation, patchy collagen fibrosis, prominent fibroblast proliferation foci, and microscopic honeycomb change.
Tolosa-hunt Syndrome
An idiopathic syndrome characterized by the formation of granulation tissue in the anterior cavernous sinus or superior orbital fissure, producing a painful ophthalmoplegia. (Adams et al., Principles of Neurology, 6th ed, p271)
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