RESUMO
IgG4 is a newly known systemic disorder. It can present as orbital inflammation or as an orbital mass. We describe the case of a 5-year-old child with proptosis and globe dystopia who went on to have an orbital biopsy. Initially this was reported to be idiopathic orbital inflammation. After IgG4-RD was considered in the differential, the orbital biopsy was revisited, immunohistochemistry was done and the patient's serum was tested for IgG subclass levels. IgG4-RD was diagnosed and the patient was subsequently treated with prednisone and mycophenolate and made a good recovery. This is the youngest reported case of IgG4-RD in a child.
Assuntos
Hipergamaglobulinemia/diagnóstico , Imunoglobulina G/sangue , Neoplasias Orbitárias/diagnóstico , Pseudotumor Orbitário/diagnóstico , Pré-Escolar , Diagnóstico Diferencial , Quimioterapia Combinada , Feminino , Humanos , Hipergamaglobulinemia/tratamento farmacológico , Hipergamaglobulinemia/imunologia , Imunossupressores/uso terapêutico , Imageamento por Ressonância Magnética , Ácido Micofenólico/análogos & derivados , Ácido Micofenólico/uso terapêutico , Neoplasias Orbitárias/tratamento farmacológico , Neoplasias Orbitárias/imunologia , Pseudotumor Orbitário/tratamento farmacológico , Pseudotumor Orbitário/imunologia , Plasmócitos/imunologia , Prednisona/uso terapêutico , Tomografia Computadorizada por Raios XRESUMO
OBJECTIVE: To ascertain the prevalence of air reflux after external dacryocystorhinostomy, its natural history, and its relation to symptomatic cure. METHODS: More than a year after lacrimal surgery, patients were contacted via a structured telephone interview and were questioned about air reflux and lacrimal symptoms. RESULTS: Interviews were completed by 77 patients (98 operations). Postoperative air reflux had been noted after 46 of 98 operations (47%) and persisted in 36 of 46 eyes (78%), at a mean follow-up of 58 months. Reflux occurred in 36% (8 of 22) of eyes after membranectomy, a rate similar to that without membranectomy (38 of 76 eyes; [50%]); the relative risk of air reflux with membranectomy was 0.7 (95% confidence interval, 0.4-1.3; P = .30). Overall, symptomatic improvement was achieved in 85% of procedures (83 eyes). Reflux was associated with a higher success rate (relative risk, 1.22; P = .02) and was significantly less likely to be associated with unchanged or worse symptoms (relative risk of failure, 0.28; P = .03). CONCLUSIONS: Air reflux is common after external dacryocystorhinostomy, is associated with symptomatic success, and generally persists but is rarely troublesome. Membranectomy does not increase the incidence of reflux, suggesting that the valve of Rosenmüller may not act as a 1-way valve for air flow.