[ @entity2694 ( XXXX ): diagnosis and therapy].

@entity1 @entity227 @entity5 @entity6437 @entity2694 @entity134 @entity6436 @entity4903
BACKGROUND: Pseudotumors of the orbit comprise a group of idiopathic inflammatory processes and are, except for @entity4903 , the most common reason for exophthalmos in adults. @entity6436 , also called @entity2694 ( @entity2694 ), can be determined from orbital involvement in systemic fibrosing diseases. Finding the correct diagnosis can be challenging. Due to the topographic relations of the orbit to neighbouring structures, a multidisciplinary cooperation is highly recommended. CASE REPORT: We report a case of a 42-year-old @entity1 with unilateral exophthalmos. Additionally we found impaired motility of the affected bulbus, @entity227 . Orbital MR imaging demonstrated dense fibrotic masses filling the whole orbita including the extraocular muscles as well as the optic nerve. Tissue specimens were extracted while performing orbital decompression via a lateral orbitotomy. Histological examination revealed a lymphatic infiltration and fibrotically destroyed tissue containing the lacrimal gland. After surgical decompression, oral @entity134 therapy and immunotherapy, a recovery of the @entity227 could be seen. CONCLUSIONS: Intraorbital fibrosclerosing @entity6437 often require a difficult long-term treatment. Therapeutic options are @entity134 therapy, immunotherapy, radiotherapy and surgery. The diagnostic steps include blood tests, ultrasound, CT and/or MRI as well as histological differentiation. @entity5 and orbital involvement in diseases of the hematopoetic system have to be excluded. Since intraorbital fibrosis can be accompanied by manifestations in various other organs, a complete investigation of the body and thorough follow up are crucial.
