Kopczyński Przemysław, Flieger Rafał, Matthews-Brzozowska Teresa
Poznan University of Medical Sciences, Poznan, Poland.
Contemp Oncol (Pozn). 2012;16(5):453-5. doi: 10.5114/wo.2012.31780. Epub 2012 Nov 20.
In the report the authors present changes in the masticatory organ in the case of a 9-year-old girl affected with Recklinghausen's disease. The characteristics included neurofibromatosis of the face, salivary gland and external ear in the form of an extensive tumour. The disease also resulted in acute right hemifacial hypertrophy. The computer-assisted tomography examination identified acute atrophy of the zygomatic bone, maxilla, mandible alveolar ridge and right base of the skull. The clinical intraoral examination of the patient revealed right buccal occlusion and lingual occlusion on the opposite side. The patient underwent functional orthodontic treatment of the co-existing malocclusion with an orthodontic appliance for the upper and lower jaw which was to prevent further mandible movement towards the right. According to researchers, neoplasia is an inherent characteristic of a phenotype seen in patients suffering from Recklinghausen's disease. Therefore, it is the main factor influencing the selection of a therapy. Although there are certain single attempts of pharmacological treatment of ganglioneurofibromas at their early development stage, it is agreed that, in fact, no preventive actions are possible. Hence, in the case of neurofibromatosis type 1, reconstruction and aesthetic procedures are widely applied in treating the disease.
在该报告中，作者介绍了一名患雷克林豪森氏病的9岁女孩咀嚼器官的变化。其特征包括面部、唾液腺和外耳出现广泛肿瘤形式的神经纤维瘤病。该疾病还导致急性右侧面部肥大。计算机断层扫描检查发现颧骨、上颌骨、下颌牙槽嵴和右侧颅底急性萎缩。对患者进行的临床口腔检查发现右侧颊侧咬合及对侧舌侧咬合。该患者使用上下颌正畸矫治器对并存的错牙合畸形进行了功能性正畸治疗，以防止下颌进一步向右移动。据研究人员称，肿瘤形成是雷克林豪森氏病患者所表现出的一种表型的固有特征。因此，它是影响治疗选择的主要因素。尽管在神经节神经纤维瘤早期发展阶段有某些药物治疗的单一尝试，但事实上人们一致认为无法采取预防措施。因此，对于1型神经纤维瘤病，重建和美容手术在治疗该疾病中被广泛应用。
