小天使的临床和想象表现:病例报告

Rebeca Santos Pereira Antunes, Juliana Jorge Garcia, Davi Matos de Freitas, Adriano Silva Perez, Miguel Gustavo Setúbal Andrade, A. M. Marchionni
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摘要

简介角弓反张症是一种罕见的遗传性疾病,其特征是儿童双侧颌骨增大,并有在青春期自发缓解的趋势。在某些病例中,它可以在没有家族史的情况下发病。目的:本报告介绍了一例 7 岁患者的非遗传性樱桃小嘴症。病例报告:一名 7 岁的男性患者主诉双侧下颌体和臼齿体积增大,已持续 3 年,无症状,生长缓慢,家族中无类似病例。从口腔内部看,体积的增大从犬齿延伸到臼齿,双侧均有,所涉及的牙齿均有异常。血液化验结果正常。全景X光片和面部CT显示,双侧下颌骨体和臼齿有多处多发病灶。在局部麻醉下进行了切口活检,结果显示存在多核巨细胞。根据临床、影像学和组织病理学检查结果,最终诊断为角膜炎。由于病变具有自限性,因此没有必要进行手术治疗,将对该病例进行随访。讨论:虽然病变往往是对称的,但在本病例中,病变在右侧更为突出。放射学特征是该病的典型特征。病变可因异位糜烂而导致畸形,本病例就是如此。尽管该病具有遗传性,但没有家族史。组织学和实验室分析对于排除其他纤维骨病变非常重要,但临床和影像学检查是诊断的关键。一旦骨骼发育成熟,病变往往会消退。但之后可能仍需进行整容手术。结论该病的诊断应主要依据临床和影像学检查结果,因为组织学分析的价值有限。一旦确诊,应对病例进行随访,必要时可推迟手术治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Clinical and imaginological manifestations of cherubism: A case report
Introduction: Kerubism is a rare hereditary disorder characterized by bilateral enlargement of the gnathic bones in children, with a tendency to spontaneous remission at puberty. In some cases, it can develop without a family history. Aim: This report presents a case of non-hereditary cherubism affecting the dentition in a 7-year-old patient. Case report: A 7-year-old male patient complained of a bilateral increase in volume on the face in the mandibular body and ramus, which had been developing for 3 years, asymptomatically and with slow growth, with no history of similar cases in the family. Intraorally, the increase in volume extended from the canine to the molar, bilaterally, with anomalies in the teeth involved. Hematological tests were within limits. Panoramic radiography and facial CT revealed multiple multiloculated lesions in the mandibular body and ramus bilaterally. An incisional biopsy was performed under local anesthesia, which revealed the presence of multinucleated giant cells. Based on the clinical, imaging and histopathological findings, the final diagnosis was kerubism. Due to the self-limiting behavior of the pathology, surgical interventions were not necessary and the case will be followed up. Discussion: Although the lesions tend to be symmetrical, here they were more prominent on the right side. The radiographic features were typical of the disease. The lesions can cause malocclusions due to ectopic eruptions, as seen in this case. Despite the hereditary characteristic, there was no family history of the disease. Histological and laboratory analyses are important to rule out other fibro-osseous lesions, but clinical and radiographic aspects are paramount in the diagnosis. Once the bone matures, the lesion tends to regress. However, cosmetic surgery may still be necessary afterwards. Conclusion: The diagnosis of the disease should be based mainly on clinical and imaging findings, since histological analysis is of limited value. Once confirmed, the case should be followed up and surgical interventions postponed if necessary at a later date.
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