Ⅰ型马德龙病1例报告及文献复习

<正>马德龙病(Madelung’s Disease)是一种良性多发性对称性脂肪瘤病,多发生于地中海地区,亚洲地区发病率最低,且好发于中老年肥胖男性,多呈散发^[1]。主要以大量无包膜脂肪团块对称性聚积在皮下筋膜浅层或深层,常累及颌面、颈项、上臂、上背及胸部等部位为典型特征的罕见疾病。该病主要影响颈项面部外观及活动,部分患者可因压迫气管及食管,出现声嘶,呼吸、吞咽困难。可合并有肝功能异常、高尿酸血症、高血脂症、男性乳房发育、神经病变、     

Ⅰ型马德龙病合并神经根型颈椎病1例报告及文献复习

马德龙病(Madelung's disease,MD)是一种罕见的脂肪代谢异常性疾病,其病因尚不明确,临床表现典型,常合并其他疾病,目前以外科治疗为主.本文报道1例Ⅰ型马德龙病合并神经根型颈椎病病例,并结合文献复习,对其病因、临床表现、诊断及治疗等进行讨论,以提高对该病及其合并疾病的认知.     

多发性对称性脂肪瘤病的诊断与治疗

目的：介绍多发性对称性脂肪瘤病的诊断和治疗方法。方法：结合我院收治的典型病例,回顾国内有详细记载的44例病例,详述多发性对称性脂肪瘤病的临床症状、体征、诊断及治疗,并做文献复习。结果：多发性对称性脂肪瘤病患者以中年男性居多,长期的嗜酒史及典型的临床表现对于该病的诊断有一定的帮助,对于影响美观及功能的患者手术治疗效果理想。结论：全面了解多发性对称性脂肪瘤病的发病情况,对其诊断与治疗具有一定的临床价值。     

多发性对称性脂肪瘤病病例报告及文献回顾

多发性对称性脂肪瘤病(multiple symmetrical lipomatosis,MSL)是一种罕见的脂肪代谢异常疾病,多见于嗜酒的中年男性,主要表现为弥漫的、对称的、无包膜的脂肪组织沉积于颈、项、肩、上肢、上背等部位,手术是目前最有效的治疗方法。该文通过回顾1例多发性对称性脂肪瘤病的患者,并对国内外文献做一回顾性分析,对这一类疾病的流行病学、病因学、临床表现、诊断治疗及预后进行介绍。     

遗传性牙龈纤维瘤病1例报告

遗传性牙龈纤维瘤病又名家族性或特发性牙龈纤维瘤病 ,临床较为少见 ,现将我科收治的 1例病例报告如下。　　患者女 ,17岁 ,哈尼族 ,高寒山区农民 ,因上颌前牙未萌出及其余牙牙龈增生影响美观来院就诊。患者 3岁左右即被发现牙龈丰满 ,乳恒牙替换后上颌前牙未萌出 ,其它牙牙龈增生肥大随年龄增长渐重 ,现牙齿大部分被牙龈覆盖 ,质地较硬 ,不易出血 ,基本上不影响咀嚼 ,未曾诊治。家族中无类似疾病患者。检查 :一般情况可 ,发育中等 ,智力一般 ,全身皮肤未见多毛。专科情况 :口腔卫生尚可 ,32 1|12未萌 ,龈下可见牙冠形态突起 ,其它牙牙冠仅…     

多发性对称性脂肪瘤病15例临床分析

目的:探讨多发性对称性脂肪瘤病的临床特点和治疗方法。方法:收集青岛大学医学院附属医院收治的15例多发性对称性脂肪瘤病患者的临床资料,对其发病原因、临床表现、治疗方法和预后进行回顾分析。结果:15例患者均在全麻下行颈部和(或)项部的脂肪瘤分区、分次切除术,2例发生术后并发症,术后随访发现除2例有一定程度复发外,其余患者在颈部、项部脂肪病变切除后,其形态接近正常,无继发功能障碍。结论:全麻下行颈部和(或)项部脂肪瘤分区、分次手术切除术是一种相对安全、有效的治疗多发性对称性脂肪瘤病的方法。     

腮腺脂肪瘤病1例

<正>1临床资料患者,女性,49岁。无明显诱因右耳垂下无痛性肿物四月余,渐进性缓慢增大。于2005年10月24日收治入院。既往病史:7年前患“子宫肌瘤”,行“全子宫及双侧卵巢切除术”。查体:右侧耳垂下范围3.0cm×2.0cm实性包块,质地中等,边界欠清,活动度较差,无明显触压痛,右腮腺涎液分泌量少,质清。     

颈部巨大良性对称性脂肪瘤病4例

良性对称性脂肪瘤病是发生在全身和颌面、头颈部或仅发生在颌面、头颈部的一种少见疾病。本文报道4例良性对称性脂肪瘤病。患者临床主要表现为颈部多发性、无痛性肿块及活动障碍,采用一次或分次彻底切除肿瘤组织,术后1例创口延期愈合,另3例患者采用分次切除肿瘤组织术后创口均一期愈合。术后随访1年余,4例患者的外形及颈部活动功能基本恢复正常,未见肿瘤复发。本文结合相关文献,对颈部良性对称性脂肪瘤病从病因、临床表现、诊断、治疗等进行了讨论。     

Madelung病:病例报告与文献复习

回顾性分析1 例Madelung病患者的病例资料,讨论此病病因,临床表现和治疗方法.该病病因不清,多发男性,肿块对称性地分布在头、颈及上躯干皮下的多个部位,呈现"马颈"或"驼峰"样外形,组织病理学表现为脂肪样组织沉积.目前手术是主要治疗手段,其预后较好.     

良性对称性脂肪瘤病1例报告

良性对称性脂肪瘤病（benign symmertric lipomatosis,BSL）是颌面、头颈部一种少见的疾病,又称Madelung＇s病。常发于中老年男性,以对称性地、缓慢地在肢体头侧堆积脂肪为其主要临床特点,从而影响患者的头颈部运动和美观,部分甚至产生呼吸、吞咽困难等压迫呼吸、消化道的症状。该病于1886年首先由Brode报道,后由Madelung总结并命名为Madelung＇s病。该病罕见,国际上迄今报道约200例,国内为约40例。我科于2006年10月收治一名BSL患者,现报告如下。     

Benign symmetric lipomatosis of the tongue in Madelung''s disease

INTRODUCTION: Madelung's disease is characterized by benign, non-encapsulated accumulations of fat in a symmetrical manner. Although rare, symmetrical lipomatosis involving the tongue exclusively has been previously reported. Here another case is presented of Madelung's disease involving the tongue. CASE REPORT: A 57-year-old woman was referred with the diagnosis of Madelung's disease, with slight difficulty in swallowing, dysarthria and dyspnoea while sleeping due to tongue swelling. Clinical examination revealed multiple, large disfiguring soft masses involving neck, upper arms and hips. There was enlargement of the tongue with bilateral protrusions and buccal displacement of the teeth. The masses were soft and non-tender. Bilateral partial glossectomy was performed under general anaesthesia. Histology revealed adipose tissue, interspersed within the lingual musculature. DISCUSSION: Lipomas in Madelung's disease are characterized by multiplicity, non-encapsulation and invasiveness. Over the years, the fat deposits enlarge significantly, become cosmetically deforming, and cause dyspnoea and dysphagia in advanced cases. The aetiology of benign symmetrical lipomatosis is still unknown. It is often accompanied by liver dysfunction probably due to alcoholism. Treatment is limited to surgical removal of the fatty tissue, in patients with a severe cosmetic deformity causing psychological stress, and in patients with dyspnoea or dysphagia if there is restriction of the aerodigestive tract.     

McCune-Albright综合征1例报告并文献复习

目的:探讨并提高对 McCune-Albright综合征的认识.方法:通过1例McCune-Albright综合征患者的临床资料和文献复习,详细分析McCune-Albright综合征的病因、临床表现、诊断、治疗及预后.结果:目前遵循的MAS 诊断标准为: 具有多发性骨纤维发育不良、加上至少一种典型的内分泌功能亢进, 和(或) 特异性皮肤色素沉着.基因诊断,可通过从超声引导下穿刺卵巢滤泡得到的囊内液、异常骨组织等病灶中取材行基因分析,发现Gs α基因的突变而确定.结论:McCune-Albright综合征在临床上十分少见且尚未被国内多数临床医生所认识,易被误诊或漏诊.治疗主要是对症治疗, 尚无有效根治方法.对于性早熟的患者以及皮肤的咖啡色色素沉着的患者应考虑到该病, 早发现有助于治疗.     

Adenomatoid odontogenic tumour: A case report

Adenomatoid odontogenic tumour (AOT) is a rare benign odontogenic tumour characterized by a progressively slow growing pattern and symptomless behavior. The differential diagnosis between AOT and other odontogenic tumours, such as ameloblastoma, should be well conducted in order to avoid extensive ablative surgery. This report presents an unusual case of an 11-year-old male patient who referred to the oral surgeon due to a significant painless gingival swelling in the anterior mandible. A panoramic X-ray revealed a round radiolucid image of an intraosseous lesion with well defined boards and related to the left lateral incisor and left canine. The Computerized Tomography was performed and the sagittal sections revealed a tooth image in contact with the inferior board of the tumour. Additionally, the coronal sections showed the presence of a tooth inside the lesion. Several calcifying nodules could be distinguished within the cystic area. The clinical diagnostic hypothesis was of calcifying epithelium odontogenic tumour but the histological sections were consistent with AOT. The tumour was enucleated under local anesthesia. After one year follow-up there were no signs of reoccurrence. With respect to the distinguishing tumour enlargement and localization in the lower jaw, the reported case is an uncommon example of AOT.     

良性对称性脂肪瘤病

<正>良性对称性脂肪瘤病(benign symmetric lipomatosis,BSL)又称Madelung,’s病、Launois-Bensaude病、多发对称性脂肪瘤病、肥颈综合征等,最早由Brodie于1846年报道,1888年Madelung作了详尽的描述,1898年Launo和Bensaude进一步描述了该     

面部先天性浸润性脂肪增殖症伴发癫痫1例

面部先天性浸润性脂肪增殖症是一种罕见的先天性脂肪组织过度增生性疾病,临床表现主要为面部渐进性膨隆、邻近骨增生、牙齿发育畸形等。本文报道面部先天性浸润性脂肪增殖症伴发癫痫1例,并结合文献对其发病特点、病因、诊断及治疗进行讨论。     

Aneurysmal bone cyst of the mandible: A case report

Aneurysmal bone cyst (ABC) is a relatively rare, benign osteolytic lesion that typically involves long bones. Approximately 2% of this tumour occurs in the jaws, with the posterior portion of the mandible being the predominant site. This lesion usually presents in the first two decades of life without sex predilection. Clinically and radiographically, ABCs are known for their diverse features, therefore often posing a diagnostic dilemma.This is the case report of a 2 year old boy with hereditary Thrombocytopenia, who was referred to us for management of his symptomatic, non healing traumatic ulcer on the right buccal mucosa and swelling of his right cheek. The lesion was initially diagnosed as haemangioendothelioma by the referring Hospital. Based on the MRI, panaromic radiograph and CT images, the lesion is suggestive of ameloblastoma of the right mandibular. Histopathological examination revealed that the lesion is consistent with ABC. The lesion was treated by surgical curettage under general anaesthesia. After one year follow up, clinical signs and symptoms have resolved well. As ABCs are known for its various clinical and radiological features and severity, histopathological examination is essential for definitive diagnosis and management.     

Uremic mixed bone disease: A case report

Renal osteodystrophy (ROD) is a complex disease of bone caused by renal failure. A case of uremic mixed bone disease with severe maxillofacial involvement is reported. The pathogenesis and various pathologic features of ROD are discussed.