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Object: Plexiform neurofibroma is a characteristic lesion of Von Recklinghausen's disease. Conservative surgery is the most widely adopted treatment. However, it is very challenging because of its hemorrhagic nature and the infiltrative aspect of the lesions. The aim of this study was to evaluate our management., Patients and Method: A retrospective study over 16 years was realized and during this period 35 patients with neurofibroma with cervico-facial location were included., Results: There were 18 men and 17 women with an average age of 23 years (3-50 years). The familial form was found in 9% of patients. Aesthetic discomfort was noted in all patients and functional impairment only occurred in 10% of patients. The NFP was localized at the hemiface in 11 cases, periorbital in 6 cases, naso-labial in 5 cases, scalp in 4 cases, jugal in 4 cases and cervico-chin in 5 cases. Size of the lesions averaged 11.6cm (4-45cm). Eighteen patients (51.4%) were operated including 10 by modeling resection, 05 cervico-facial lifting and 3 complete resections. Complication rate was 28% dominated by disunion wound. The average number of procedures was 1.6 (1 to 5). After 3 years average follow-up, aesthetic et functional results was assessed as good over 75 per cent of patients., Conclusion: Cervico-facial plexiform neurofibromas is challenging. Conservative surgery should be the gold standard and long time follow-up is recommended., (Copyright © 2020. Published by Elsevier Masson SAS.)

Plexiform neurofibroma is a rare and benign tumor often associated with type 1 neurofibromatosis (NF1) or Von Recklinghausen's disease. Present in one third of cases of NF1, there are isolated forms where the diagnosis should remain a diagnosis of exclusion. We report four cases of isolated plexiform neurofibromas found in children from topographies and we discuss the pretherapeutic assessment, the surgical management and the long-term follow-up., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Objective: Von Recklinghausen's neurofibromatosis is a dominant autosomic genetic disease characterized by different clinical manifestations. The goal of this work was to study its orthopaedic manifestations and to show the characteristics of their management., Method: A retrospective study was carried out on 15 patients having a Von Recklinghausen's neurofibromatosis. For each patient, different orthopaedic manifestations and their evolution after treatment were analyzed. These manifestations were classified in spinal deformities, pseudarthrosis of long bones and tumours of the peripheral nerves., Results: The spinal deformities were observed in 9 cases. A dystrophic scoliosis was observed in 6 patients with an average angle of 50° and was associated to a kyphosis in 5 patients. The treatment was surgical by posterior arthrodesis in 2 cases and circumferential arthrodesis in 2 cases. The congenital curves and pseudarthroses of leg were observed in 5 cases, localized at the lower third of the leg in all cases. An Ilizarov external fixator with segmental osseous transport was carried out in 2 patients. The duration of the external fixator was 23 months ½ with 5 interventions in each case. Four plexiform neurofibromas and 3 nodular neurofibromas were observed. A transformation into neurofibrosarcoma was found in 2 patients. In one case, a resection without functional sacrifice was carried out and in the other case the patient was dead before the resection., Conclusion: The orthopaedic manifestations of Von Recklinghausen's neurofibromatosis are frequent, varied and have a difficult management. The functional and sometimes vital prognoses are challenging., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Background: Plexiform neurofibroma (NFP) is a benign nervous tumour typically involving the head and neck region due to the rich innervation of the latter. It is considered pathognomonic of neurofibromatosis type 1(NF1). This report describes an unusual case of neurofibroma and discusses its singular presentation, namely an isolated cutaneous tumour of late onset and with myofibroblastic histology., Case Report: A 85-year-old man presented swelling of the cutaneous part of the lower palpebral region which had been present for several months. The lesion was relapsing after repeated incomplete excisions and had grown slowly to become firm and suspect. It was decided to perform a large excision with a frontal rotation flap. Initial histological examinations performed on each excision suggested fibrosis and scarring, leading to diagnosis of fibrocytic change and post-surgical neuroma. The final histological analysis indicated diffuse plexiform neurofibroma with a myofibroblastic component. This was a solitary lesion in a patient without any stigmata or familial history of NF1., Discussion: This case is original in terms of its characteristics: a single cutaneous tumour of late onset in a patient with no stigmata of NF1 (in most cases of plexiform neurofibroma, NF1 is either multiple or else isolated at a mucous site). The histological findings for this tumour with a myofibroblastic component have never previously been described. Plexiform neurofibroma classically involves the head-and-neck region as in our case, with deep invasion of subcutaneous tissues making excision difficult and leading to frequent recurrence., (2010 Elsevier Masson SAS. All rights reserved.)

Introduction: We report a case of total oral rehabilitation with basal implants (cortically anchored disk-design implants) on a patient who received a facial allograft 1 year earlier., Observation: A 31-year-old patient was suffering from a plexiforme neurofibroma spread into the soft tissues of the oral cavity with huge deformations of the jaws. The operation consisted in pulling out numerous supernumerary impacted teeth, removing unnecessary soft tissues, settling six basal implants in the maxilla and seven in the mandible. The following day, two resin bridges were adjusted and cemented onto the implant abutments. The permanent bridges were settled 2 months and half later. The outcome was still unremarkable 2 years after the implant procedure., Discussion: This case report raises important issues, notably the relevance of an oral rehabilitation with implant-supported prostheses on an immunosuppressed patient. In this specific case, this was impossible to achieve using removable prostheses. A facial reconstruction with its plastic, functional, and social goals seemed inconsistent without an oral rehabilitation. Even if they have not been published, cases of grafted patients with dental implants are unexceptional. Lastly, this rehabilitation has been planned for a particularly monitored patient. The second important question regards the choice of the basal implantology. This option has been motivated by the possibility to complete the case in one sole operation with provisional prostheses cemented the day after that act like orthopaedic external fixators providing an exceptional primary stability. The cortical anchorage, which was the only reliable on this patient, allowed to avoid bone grafting. Finally, the particular thinness of the implant emergence limits to the minimum the communications between bone and oral cavity. We would like to stress that the only objective of this observation was to document a specific case and possibly to open the way to a scientific study in accordance with the regulations of clinical research.

Introduction: Orbito-temporal plexiform neurofibroma is characteristic of Von Recklinghausen neurofibromatosis. We report 6 cases of orbito-temporal plexiform neurofibromas and review different aspects of diagnosis, course and treatment., Patients and Methods: Six patients, 4 males and 2 females, with orbito-temporal plexiform neurofibromas were treated between 1986 and 2003. Patient age varied between 10 and 29 years (age average=19). Computed tomography and magnetic resonance imaging were performed in all 6 patients. Surgical resection of the neurofibroma was performed in all patients., Results: The tumor was located on the left in 5 cases and on the right in one. Spheno-orbital dysplasia was found in all patients and was associated with fronto-temporal meningoencephalocele and pulsate exophthalmia. Surgical resection enabled reduction of the neurofibroma. Exenteration was performed in all patients because of a nonfunctional eye. Two patients underwent cure of fronto-temporal meningoencephalocelus with reconstruction of the large wing of the sphenoid using an iliac bone graft and a titanium plate., Discussion: Surgery is essential for orbito-temporal plexiform neurofibroma but is usually difficult, particularly when the tumor has developed increasing the risk of hemorrhage. Bony reconstruction is difficult considering the risk of progressive osseous dysplasia. The cosmetic results achieved in this patient were considered good.

We report a case of predominantly facial neurofibromatosis type I in a 7-year-old girl, exposing the difficulties encountered in surgical management. Infiltration of facial soft tissues by plexiform neurofibroma is difficult to control. Resection of large areas of facial skin is unconceivable, facial functions must be preserved. Orbitocranial surgery is reserved for orbital manifestations of neurofibromatosis with osseous sphenoid dysplasia and pulsatile exophthalmos. Despite progress in tumor imaging and surgery, facial neurofibromatosis remains a challenge for the surgeon. We discuss surgical technique and indications for surgery in these cases.

After recalling the frequency and pathophysiology of type 1 neurofibromatosis, or Von Recklinghausen disease, the authors report the various cutaneous manifestations: café-au-lait spots, lentigines, cutaneous neurofibromas, plexiform neurofibromas. Treatment must be multidisciplinary, as surgery is difficult and often gives disappointing results. The various surgical treatments are considered according to the type and site of the lesions to be treated, with particular emphasis on the treatment plan, which must be discussed with the patient.