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Plastic-reconstructive Correction of Blepharitis Granulomatosis in Melkersson-Rosenthal-syndrome

Ziemssen F., Rohrbach J. M., Bartz-Schmidt K. U., Denk P. O.,
Eberhard-Karls-Universität Tübingen, Universitäts-Augenklinik, Abt. I (Tübingen)

Background: The Melkersson-Rosenthal-syndrome-complex is characterized by asymmetric orofacial oedema, facial palsy, furrowed tongue (lingua plicata) and additional neurological symptoms.
Patient: A 21 year old man presented a bilateral asymmetric oedema of the eyelids increasing over the last 12 years of observation. The lid oedema resulted not only in cosmetic but serious restricted visual fields. Intermittent swelling of the auditory channel took place with resulting hearing problems. Besides the patient complained about unspecific symptoms like headache and tinnitus.
Results: Our patient showed the typical symptoms of the Melkersson-Rosenthal-complex with a lack of the facial palsy often caused by mechanical compression along its course. The differential diagnosis of recurrent swelling of the lids were interdisciplinarily discussed. The biopsy specimen showed granulomatous inflammation. The inflammatory activity could be reduced and the frequency of swelling episodes could be diminished by a treatment with hydroxychloroquine. The initially relapsing, then persistent swelling lead to severe restriction of the visual fields and so to serious social stigmatisation and psychic burden for the patient. The surgical resection of the granulomatous tissue was performed under steroid treatment to prevent a recurrence. Blepharoplasty resulted in an improved cosmetic appearance. The patient remained free of symptoms with an excellent functional result.
Conclusions: After persistence and constancy of the symptoms of a granulomatous blepharitis surgical correction is a good opportunity to minimize the subjective complaints and neurological symptoms as paraesthesia and headache.