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Objective: To summarize the treatment strategies and clinical experiences of 5 cases of giant plexiform neurofibromas (PNF) involving the head, face, and neck., Methods: Between April 2021 and May 2023, 5 patients with giant PNFs involving the head, face, and neck were treated, including 1 male and 4 females, aged 6-54 years (mean, 22.4 years). All tumors showed progressive enlargement, involving multiple regions such as the maxillofacial area, ear, and neck, significantly impacting facial appearance. Among them, 3 cases involved tumor infiltration into deep tissues, affecting development, while 4 cases were accompanied by hearing loss. Imaging studies revealed that all 5 tumors predominantly exhibited an invasive growth pattern, in which 2 and 1 also presenting superficial and displacing pattern, respectively. The surgical procedure followed a step-by-step precision treatment strategy based on aesthetic units, rather than simply aiming for maximal tumor resection in a single operation. Routine preoperative embolization of the tumor-feeding vessels was performed to reduce bleeding risk, followed by tumor resection combined with reconstructive surgery., Results: All 5 patients underwent 1-3 preoperative embolization procedures, with no intraoperative hemorrhagic complications reported. Four patients required intraoperative blood transfusion. A total of 10 surgical procedures were performed across the 5 patients. One patient experienced early postoperative flap margin necrosis due to ligation for hemostasis; however, the incisions in the remaining patients healed without complications. All patients were followed up for a period ranging from 6 to 36 months, with a mean follow-up duration of 21.6 months. No significant tumor recurrence was observed during the follow-up period., Conclusion: For patients with giant PNF involving the head, face, and neck, precision treatment strategy can effectively control surgical risks and improve the standard of aesthetic reconstruction. This approach enhances overall treatment outcomes by minimizing complications and optimizing functional and cosmetic results.

Reconstruction of large craniofacial defect after plexiform neurofibroma excision poses a continuous challenge for plastic surgeons, on account of characteristics of plexiform neurofibroma and patients' aesthetic requirements. Skin graft or free flap is hard to obtain satisfactory results or may pose technical challenges. In an attempt to provide the coverage with 'like tissue', we chose local tissue expansion technique. The expansion period was about an average of 3.4 months. We performed 19 expanded flaps located in the head, face, neck, forearm and superclavical regions to reconstruct the craniofacial defect and achieved satisfactory results. Preoperative endovascular embolism in some cases and several intraoperative hemostatic methods for all cases were undertaken to control the perioperative bleeding. For patients who request aesthetic results and are allowed two-staged operations, our method is viable., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Background: Large temporal plexiform neurofibroma (PNF) is an irritating problem that causes facial disfigurement. Surgical resection of PNF is the only effective way to remove the tumor as well as to improve the patient's facial appearance. However, temporal branch of the facial nerve (TBFN) in the tumor is prone to be destroyed during PNF removal. Thus, TBFN palsy is the inevitable complication after surgery and might induce other malformation and dysfunction. Therefore, the aim of this study is to reconstruct a nearly normal face contour while preserving the facial nerve function., Purpose: Selective PNF removal technique was designed to protect TBFN during PNF lesions resection in our patients., Methods: From May 2011 to June 2015, the authors had 10 patients who suffered from PNF in the temporal region with facial disfigurement and underwent selective PNF removal to correct the facial disfigurement while preserving TBFN as well., Result: All patients obtained the improvement of facial appearance after surgery. The temporal PNF was removed and the TBFN function successfully maintained. Plexiform neurofibroma recurrence has not been relapsed during 6 to 49 months' follow-up., Conclusions: In our initial exploration, TBFN function maintenance and facial appearance improvement can be achieved simultaneously by using PNF-selective removal surgery technique., Competing Interests: The authors report no conflicts of interest.

Purpose: To describe a successful five-level cervical corpectomy and circumferential reconstruction in a patient with a plexiform neurofibroma causing a severe kyphotic deformity., Methods: Case report., Results: 43-year-old man with history of Neurofibromatosis presented with signs and symptoms of myelopathy with spastic lower extremities and gait difficulties. Imaging studies demonstrated a severe kyphotic deformity of the cervical spine with associated cord compression secondary to an anteriorly positioned plexiform neurofibroma. Two-stage surgical procedure was designed to treat this lesion. Stage I consisted of tracheostomy placement, transmandibular, circumglossal approach to the anterior cervical spine, C2-C6 corpectomies, and C1-C7 reconstruction with a custom titanium cage/plate. Stage II consisted of suboccipital craniectomy, C1-C2 laminectomies, and occipital-cervical thoracic instrumented fusion (O-T8). There were no operative complications, but the patient did develop a small pulmonary embolism post-operatively treated with anticoagulation. Patient required two-weeks of inpatient rehabilitation following surgery. Gastrostomy tube and tracheostomy were successfully discontinued with preserved swallowing and respiratory function. Patient-reported outcome measurements revealed significant and sustained improvement post-operatively., Conclusions: Five-level cervical corpectomy including C2 can be safely and successfully performed via a transmandibular, circumglossal approach. Circumferential reconstruction utilizing a custom anterior titanium cage and plate system manufactured from a pre-operative CT scan was utilized in this case. Long segment occipital-cervical-thoracic reconstruction is recommended in such a case. Using such a technique, improvement in myelopathy, correction of deformity, and improved quality of life can be achieved.

A 19-year-old woman with type I neurofibromatosis requested reconstruction of her severe facial disfigurement caused by the plexiform neurofibroma of the right upper eyelid. Previously, she had glaucoma surgery for buphthalmus and enucleation with dermis fat transplantation. She was unable to wear prosthesis in the last year. Transverse palpebral resection of the lesion, lateral canthal reconstruction, and frontal sling ptosis surgery were performed at the same session. Postoperatively, the cosmetic appearance of the patient was markedly improved. No complication or progression was observed during 2 years follow-up. We believe that an individual-based surgical plan may give acceptable results in these challenging cases.

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.

This disease was first described by von Recklinghausen and Festscher and has been known as the von Recklinghausen's disease or neurofibromatosis (NF). Numerous articles have been published on this subject where majority of the authors have stressed their difficulty in achieving favourable surgical results. This disease is pathologically accepted to be of neuroectodermal origin with a positive family history in approximately 50% of the cases, autosomal dominant trait and is known to involve the periorbital regions, orbit (preoperative CT scan: upper left and right photographs), temporal region to a variable extent, mid-facial region to the mandibular region. The indicated treatment for this disease is surgery including cranio-maxillofacial surgery even though re-evaluation of the conventional methods of surgery should be considered. This particular case is an extremely difficult and challenging case for any reconstructive plastic surgeon and required extensive preoperative planning. The systematic multiple stage surgical approach for an extremely severe von Recklinghausen's disease, elephantiasis neurofibromatosa is presented and discussed in detail.

We report a successful application of Oberlin's procedure combined with transfer of trapezius and latissimus dorsi with the teres major for reconstruction of elbow flexion as well as abduction and external rotation of the shoulder to a ten-year-old patient, who had a long defect of the left brachial plexus upper trunk caused by resection of the plexiform neuroma.