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04-03-2023, 07:35 PM
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Some Examples of Von Recklinghausen Disease or Neurofibromatosis Type I
Source is a study: “Type I Neurofibromatosis With Cutaneous Localization: A Study Of 63 Cases”: This is a retrospective study over a period of 16 years (January 2001 to December 2017) on 63 patients with neurofibromatosis type 1 skin manifestations, managed in the Plastic and Reconstructive Surgery Department of the Aristide Le Dantec Hospital, in Dakar, Senegal. We studied epidemiological, clinical and therapeutic parameters. There were 29 men and 34 women for a 0.85 sex ratio. The average age was 24 years. The sporadic isolated cases accounted for 90.5% of cases. The esthetic discomfort constituted the main reason for consultation, followed by the functional discomfort in 58.3% of the cases. From a clinical point of view, all patients had multiple localized skin neurofibromas and "café au lait” spots (figure1). Plexiform neurofibromas were almost always present (96.7%). The major tumor was located in 60 % of the cases in the cervical-cephalic region (Figures 2 and 3), followed by the upper limb (14%). The average size of the neurofibromas was 10.49 cm [1.5-45 cm]. The indication of excision was considered based on the size and number of tumors as well as the extent of aesthetic and functional discomfort. Thirty-nine patients were operated representing 60.9 % of the cases. Extra-tumoral resection was the procedure employed in 43.6% of cases or 17 patients, followed by skin closure by direct suture with or without an associated local cutaneous plasty. Intra-tumoral reshaping resections accounted for 56.4 % of procedures or 23 patients. Supra-infection of the operative site and hypertrophic scars were the two complications encountered in 2 patients. The average follow-up was 5 months [ 10 days - 6 years]. The different procedures gave satisfactory results both at aesthetic (69% of cases) and functional (100% of cases) levels (Figures 4 and 5). |