The aim of the study was to evaluate the outcome of treatment of facial deformities in patients with Recklinghausen’s disease. Twenty-nine patients suffering from Recklinghausen’s disease were treated at the Hospital of Plastic Surgery in Polanica Zdrój in the years 1990–2005. The age of patients when they were first seen was from 1 to 23 years (mean age 11 years). Most of the patients were followed up for 1 to 31 years and were submitted to surgical treatment only periodically. All the patients had facial lesions, including 14 with tumours within the orbits and eyelids. Altogether, 198 partial or complete resections of neurofibromas and corrective procedures were performed (average 6.8 operations per one patient). Nodules on the head, eyelids, cheeks and nose were most commonly resected. Reduction in the tumour mass resulted in improvement of facial symmetry. The resections were often repeated several times in the same area with additional resections in other body areas. Histopathological examinations revealed plexiform neurofibroma in seven cases, while malignant carcinoma was not diagnosed in any of the cases. Three patients developed mild complications (1.5%). Facial neurofibromas occurring in the course of von Recklinghausen’s disease require surgical treatment, which is often multi-stage because of benign but progressive proliferation of tumours. Systematic reduction in tumour mass may decrease the risk of malignant transformation.
European Journal of Plastic Surgery – Springer Journals
Published: Nov 1, 2007
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