[Idiopathic orbital inflammation syndrome in childhood--case report and literature review].
BACKGROUND
Idiopathic orbital inflammation syndrome (IOIS) is a rare disease in childhood. There are only ca. 70 case reports in the scientific literature.


METHODS
We present a case report and review of literature.


METHODS
A 6-year-old girl developed within one week beginning with a discrete, non-traumatic haemorrhage of the lower palpebra a painful proptosis, periorbital swelling, conjunctival chemosis and injection and motility restriction to lateral gaze of the right eye. MRI showed a retrobulbar and eyelid mass with enhancement and signs of haemorrhage without optic nerve involvement. A rapidly performed biopsy excluded malignancies and confirmed the diagnosis of non-specific inflammation. With high-doses of intravenous and later oral prednisolone the symptoms improved significantly. Because of an impairment under tapering of prednisolone an interim elevation of the dose was necessary, but with very slow tapering over 4.5 months the girl became symptom-free except for a minimal, non-relevant abduction deficit. There has been no recurrence in the last 1.5 years.


CONCLUSIONS
In comparison to the clinical features of adults the rarely reported childhood cases show no relevant differences in orbital signs, frequency of bilaterality and pain, recurrence rate and success of therapy. Only iritis and papilloedema seem to be special features of childhood IOIS, these cases tend to a higher rate of recurrences. Histopathological examination is much more necessary than in adults because of the need for exclusion of rhabdomyosarcoma. Our case shows that haemorrhages can also be a sign for an IOIS.
Klinik und Poliklinik für Augenheilkunde, Universitätsklinikum Carl Gustav Carus, Dresden. viktoria.bau@uniklinikumdresden.de
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Bau
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Röpke
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Marquardt
D000284:Administration, Oral; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D001706:Biopsy; D002648:Child; D003937:Diagnosis, Differential; D004305:Dose-Response Relationship, Drug; D005260:Female; D006801:Humans; D007262:Infusions, Intravenous; D008279:Magnetic Resonance Imaging; D008775:Methylprednisolone / Q000008:administration & dosage; D009915:Orbit / Q000473:pathology; D016727:Orbital Pseudotumor / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D011239:Prednisolone / Q000008:administration & dosage; D012008:Recurrence
D000893:Anti-Inflammatory Agents; D011239:Prednisolone; D008775:Methylprednisolone