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Unilateral Congenital Buphthalmia and Megahemicrania caused by a Wagner-Meissner-neurinoma based on Neurofibromatosis Type I

1Schröder A. C., 1Käsmann-Kellner B., 2Shamdeen M. G., 1Ruprecht K. W.,
1Universitätskliniken des Saarlandes, Augenklinik und Poliklinik (Homburg/Saar)
2Universitätsklinik für Kinder- und Jugendmedizin Homburg (Homburg/Saar)

Background: Neurofibromatosis type I (von Recklinghausen-Syndrome) is one of the most frequent types of phakomatoses. While young children show a lot of café-au-lait-spots elderly children develop neurinomas and fibromas along the peripheral nerves.
Patient Presentation: The little patient was found by sonography to have intrauterinely an exophthalmia with right megalocephaly. Postpartally the right cornea was cloudy. An intraocular pressure of 30 mmHg made a trabeculectomy necessary at an age of 10 days. Nevertheless exophthalmia and buphthalmia increased. By local therapy the intraocular pressure stayed within normal limits and the cornea cleared. In MRI an intracranial tumor was found reaching into the apex of the orbita. Neurologically a pathological EEG and frequent epileptic attacks occurred. Histologically a Wagner-Meissner-Neurinoma was found by exploring excision walling the optic nerve as well as the carotis interna. Although the tumor is benign it can not be excised for its localisation and spreading growth. The carotis interna is occluded by now due to the increase in tumor size. MRI-pictures show atrophic areas within the right cranial hemisphere and extended hydrocephalus. Gene-analysis was positive for the NF1-gene. Currently the one-year-old girl is free of fits being treated with Lamictal. She turns towards the light and fixates moving objects.
Results: The poster presents the young patient and her syndromatic disease. It demonstrates a rare cause of congenital exophthalmia and buphthalmia. The life-expectance is doubtful due to the severeness of the illness.