Craniofacial Neurofibromatosis Type 1: Clinical Features, Challenges Of Management, And A Report Of 2 Nigerian Patients
نویسندگان
چکیده
Objective: The objective of this article is to highlight the various soft tissue and skeletal manifestations of craniofacial neurofibromatosis and the challenges involved in the surgical management of patients with complex cases. Methodology:An extensive literature review was conducted to highlight the clinical features of craniofacial neurofibromatosis. The notable challenges of management from the experience of the authors were highlighted and two cases of Nigerian patients are presented to demonstrate the challenges of management as it affects patients in the developing nations of Africa who often require referral abroad.Result:Patients with craniofacial neurofibromatosis present with varying soft tissue and skeletal features which often necessitate complex, expensive and highly technical surgical management which are often not locally available in some developing countries. In this article, the skeletal features are presented under 3 subheadings; cranial features, orbital features and orofacial features. The 2 cases presented illustrate the clinical features and the technicalities of management with emphasis on the need for a committed craniofacial team. Conclusion:In order to reduce the physical and financial burden in the management of complex craniofacial conditions, the need to improve facilities and expertise and to form effective craniofacial teams in the third world countries is imperative.
منابع مشابه
Unilateral and multiple nodular plexiform Neurofibromas: An uncommon presentation of Neurofibromatosis type 1
Plexiform neurofibroma is a rare variant but pathognomonic of von Recklinghausen disease. We report an uncommon presentation of neurofibromatosis type I in a girl. This 11-year old girl had multiple plexiform neurofibromas on her left hand, and also several Lisch’s nodules. Classification and clinical features are discussed briefly with emphasis on the possibility this condition may not be give...
متن کاملA Large Intrathoracic Mass in a Patient with Neurofibromatosis-1: a case report
Abstracts: Dural ectasia is circumferential expansion or dilatation of the dural sac, and has been frequently reported in association with type 1 neurofibromatosis (NF1). It should be differentiated from posterior mediastinal tumors such as neurofibroma, neuroblastoma, and ganglioneuroma because NF-1 has a high risk of tumor formation. In the spinal deformities of NF-1 patients, despite the sco...
متن کاملHemifacial microsomia: a clinicoradiographic report of a case
Hemifacial microsomia is a rare congenital malformation of craniofacial structures. Its characteristic features are unilateral underdevelopment of the face and ear malformations. This study describes clinical and radiographical features of a rare case of a 4-year-old hemifacial microsomia patient with underdevelopment of the left side of the face and preauricular skin tags on the affected side...
متن کاملSegmental Neurofibromatosis Type 1, a Rare Variant of Neurofibromatosis: Report of Two Cases
Segmental neurofibromatosis type I (SNF-I) is a rare variant of neurofibromatosis (NF). It is classified as NF type V and defined as cafe'-au-lait macules and/or neurofibromas in a single ,unilateral segment of the body .We report two cases with SNF-I with striking similar manifestations.
متن کاملCraniofacial orthodontics and postgraduate orthodontic training in Nigeria.
INTRODUCTION Craniofacial orthodontics has been shown to be a critical component of the care of patients with craniofacial anomalies such as cleft lip and palate. Thus, the purpose of this study was to assess the perceptions and clinical experience in cleft and craniofacial care, of orthodontic residents in Nigeria. METHODOLOGY Questionnaires were sent out to orthodontic residents in the six ...
متن کامل