Comprehensive and Global Approach of Soft-Tissue Deformities in Craniofacial Neurofibromatosis Type 1

Conclusions: According to the experience and surgical outcomes presented in this study, the soft-tissue surgical approach of the craniofacial NF-1 should be global, comprehensive, and individualized.
Source: Annals of Plastic Surgery - Category: Cosmetic Surgery Tags: Head and Neck Surgery Source Type: research

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We present a case of successful correction of upper eyelid entropion and severe eyebrow ptosis in a 48-year-old orbito-temporal plexiform neurofibromas patient with combined forehead lift and blepharoplasty techniques including double fold formation and mass excision.
Source: Journal of Craniofacial Surgery - Category: Surgery Tags: Brief Clinical Studies Source Type: research
Conclusion: Segmental box osteotomy with internal orbital reconstruction redistributes orbital volume safely and accurately addresses globe malposition from neurofibromatosis.
Source: Journal of Craniofacial Surgery - Category: Surgery Tags: Original Articles Source Type: research
Gingival enlargement, although frequently encountered in clinical settings, is rarely associated with systemic diseases or syndromes. Among the diverse pathological conditions of neurofibromatosis type 1 (NF-1), minor manifestations in the orofacial region are occasionally overlooked. Herein, the authors present an unusual case of gingival neurofibroma in a patient with NF-1 associated with characteristic osseous defects in the alveolus in the long-term course of 17 years from the first examination. A 5-year-old boy with NF-1 was referred for the evaluation of gingival enlargement in the posterior left maxilla. An incisal...
Source: Journal of Craniofacial Surgery - Category: Surgery Tags: Brief Clinical Studies Source Type: research
Conclusion: The novel morphometry is convenient and reproducible, which optimizes its application in pathologic cases, especially those involving deformed orbits.
Source: Journal of Craniofacial Surgery - Category: Surgery Tags: Brief Clinical Studies Source Type: research
Conclusion: The surgical technique could achieve good surgical outcomes in both functional and cosmetic terms. Additionally, the relatively objective evaluation technique based on Mimics software could be a more convincing method for evaluating the surgical outcomes of craniofacial patients with PNF.
Source: Journal of Craniofacial Surgery - Category: Surgery Tags: Brief Clinical Studies Source Type: research
Background: Sphenoid wing dysplasia in patients with neurofibromatosis type 1 may result in challenging and significant changes, including ultimately vision loss. The authors describe the radiographic patterns of sphenoid dysmorphology with time and age, and the impact of surgical intervention on preservation of vision. Methods: A retrospective study was performed at a single pediatric hospital, identifying subjects with neurofibromatosis type 1. Records were reviewed in their entirety for each subject, with attention to ophthalmologic evaluation. Radiographic images were digitally analyzed and scored for sphenoid tra...
Source: Plastic and Reconstructive Surgery - Category: Cosmetic Surgery Tags: Pediatric/Craniofacial: Original Articles Source Type: research
Surgical rehabilitation of orbital dystopia can be challenging. The authors demonstrate the effective use of spectacle lenses to visually correct misalignments of the globe and the orbit. Presented is a retrospective review of 4 patients undergoing aesthetic rehabilitation through use of spectacle lenses and in a number patients a cosmetic shell. Two patients with neurofibromatosis presented with inferior dystopia of the globe and orbit. A base-down prismatic lens applied to the spectacles in conjunction with a prosthetic shell successfully visually corrected the facial asymmetry and improved patients’ aesthetic app...
Source: Journal of Craniofacial Surgery - Category: Surgery Tags: Clinical Studies Source Type: research
ConclusionThe radiological sign of the unilateral deformed mandible should be included in the diagnostic criteria for NF1. In addition to the diagnostic value in this syndrome, these findings are important for avoiding surgical complications in planned interventions in this region.
Source: Journal of Cranio Maxillofacial Surgery - Category: ENT & OMF Source Type: research
Conclusion Lateral cephalograms revealed no significant alteration of the facial skeleton in NF1 patients as compared to controls. Indeed, the stigma of a so-called ‘NF1 facies’ cannot be derived from the cephalometric findings presented. Notably, a wide range of deviating readings were recorded for individuals with facial PNF. Clinicians who treat patients with NF1 should be aware of deviations from cephalometric standards on lateral cephalograms in NF1 patients, especially when craniofacial surgical procedures are planned. Some of these findings, particularly asymmetries of the facial skeleton, could be indic...
Source: Journal of Cranio Maxillofacial Surgery - Category: ENT & OMF Source Type: research
Abstract: Type-1 neurofibromatosis, a common autosomal dominant disease, is also known as von Recklinghausen disease. Surgical procedures to treat this condition are challenging because of the brittleness of the surrounding blood vessels and soft tissues that bring the risk of causing fatal bleeding. With improvements in neurovascular embolization procedures, some literatures have been published about the application of preoperative embolization for neurofibromatosis. This case report describes a 60-year-old female with Type-1 neurofibromatosis, who presented giant facial neurofibromas with intratumoral hemorrhage on both ...
Source: Journal of Craniofacial Surgery - Category: Surgery Tags: Clinical Studies Source Type: research
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