Cogan Syndrome in children: Early diagnosis and treatment is critical to prognosis

To present two cases of pediatric Cogan Syndrome and to highlight the differences between the adult and pediatric forms of the disease, as well as the importance of early diagnosis and treatment. Interventional case report. Institutional setting. Corneal lesions were much more diffuse than those obs...

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Bibliographic Details
Published in:American journal of ophthalmology 2004-04, Vol.137 (4), p.757-758
Main Authors: ORSONI, Jelka G, ZAVOTA, Laura, VINCENTI, Vincenzo, PELLISTRI, Isabella, RAMA, Paolo
Format: Article
Language:English
Subjects:
Amblyopia - etiology
Anti-Inflammatory Agents, Non-Steroidal - therapeutic use
Aspirin - therapeutic use
Autoimmune Diseases - complications
Autoimmune Diseases - diagnosis
Autoimmune Diseases - drug therapy
Biological and medical sciences
Child
Child, Preschool
Cyclosporine - therapeutic use
Diseases of cornea, anterior segment and sclera
Drug Therapy, Combination
Fludrocortisone - therapeutic use
Hearing Loss, Sensorineural - complications
Hearing Loss, Sensorineural - diagnosis
Hearing Loss, Sensorineural - drug therapy
Humans
Immunosuppressive Agents - therapeutic use
Keratitis - complications
Keratitis - diagnosis
Keratitis - drug therapy
Male
Medical sciences
Methotrexate - therapeutic use
Ophthalmology
Prognosis
Syndrome
Vasculitis - complications
Vasculitis - diagnosis
Vasculitis - drug therapy
Vision, Low - etiology
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Summary:To present two cases of pediatric Cogan Syndrome and to highlight the differences between the adult and pediatric forms of the disease, as well as the importance of early diagnosis and treatment. Interventional case report. Institutional setting. Corneal lesions were much more diffuse than those observed in adult Cogan syndrome. Immunosuppressive drug combination therapy successfully resolved systemic and ocular inflammation, but the involvement of the pupillary area caused permanent low vision in one case and amblyopia in the other. When chronic ocular inflammation is observed in association with sensory neural hearing loss and any systemic signs of autoimmune inflammation, a diagnosis of Cogan syndrome should be suspected. If immunosuppressive treatment is not initiated as soon as possible, permanent low vision and deafness can result.
ISSN:0002-9394
1879-1891
DOI:10.1016/S0002-9394(03)01122-X