15例弥漫性食管痉挛病人X线表现及主要症状分析

弥漫性食管痉挛为一种食管运动失调性疾病，其特征为食管中下段同一时间出现非推进性。持续性收缩。临床主要表现为胸痛、吞咽困难等症状。由于症状缺乏特异性以及一些医生对该病认识不足而造成误诊，或在X线检查中未加认真分析而忽视。患者病情反复，多处就医，加重了其心理及经济负担，所以明确该病诊断，以便合理治疗显得尤为重要。现对15例确诊为弥漫性食管痉挛患者的X线表现及主要症状分析如下。     

成人巨食管症诊断分析并文献复习

目的通过探讨巨食管症的影像表现,以提高对巨食管症的认识。方法回顾性分析1例巨食管症病人的影像资料并进行文献复习。结果病人颈椎MRI平扫检查提示气管后方椎体前方食管扩张,T1WI呈低信号,T2WI呈低信号。颈部、肺部CT平扫检查示食管全程显著扩张,扩张最宽处直径约7 cm,内见大量食糜,贲门部未见明显肿块,相邻气管及纵隔内结构受压推移,气管支气管狭窄,胃内见内容物,扩张尚可。服用钡餐后,腹部平扫CT示扩张的食管、胃内均见点状高密度钡剂显影。食管钡餐造影检查提示食管全程显著不均匀迂曲扩张,呈节段性囊样改变,钡剂下行缓慢,食管内见较多食物潴留及多个宽大液平,蠕动尚柔和,黏膜纹增粗,局部显示不清。结论巨食管症的影像表现具有特异性,熟悉巨食管症的影像表现有助于本病的诊断。     

Boonsta-Bosch-Schaff视神经萎缩综合征并婴儿痉挛症1例报告并文献复习

目的报告1例Boonsta-Bosch-Schaff视神经萎缩综合征(BBSOAS)并婴儿痉挛症的临床特点、诊治过程并复习文献。方法回顾性分析解放军总医院第一医学中心住院治疗的1例BBSOAS并婴儿痉挛症患儿的临床资料,检索在线人类孟德尔遗传数据库(OMIM)、PubMed、中国知网、万方数据知识服务平台等数据库,综合文献结果,探讨BBSOAS并婴儿痉挛症的临床特点,探讨其表型-基因型关系及治疗效果。结果本例患儿男,9月龄,因"间断抽搐4个月"入院。患儿全面发育落后,追视差,眼底检查可见视盘苍白(萎缩、小视盘),痉挛发作,脑电图提示高度失律,遗传学检测发现NR2F1基因新发错义突变c.383G>A(p.Cys128Tyr),诊断为"BBSOAS;婴儿痉挛症"。给予促肾上腺皮质激素(ACTH)及多种抗癫■药后痉挛发作未控制,复查脑电图仍有高度失律,院外口服吡仑帕奈后2周,患儿出现发热,热退后痉挛发作完全控制。检索多个数据库并手动筛选后共获得英文文献9篇,共46例BBSOAS,其中9例合并婴儿痉挛症,其基因突变导致的氨基酸改变全部位于DNA结构域内,均可见到视神经萎缩或(和)视神经发...     

内镜下注射肉毒毒素(TBX)治疗食管贲门失弛缓症26例分析

内镜下球囊扩张治疗食管贲门失弛缓症近年来报告较多,但注射TBX治疗该病的报道甚少。自2006-01～2011-08,我科在TBX注射成功治疗痉挛性斜颈、斜视及面肌痉挛、截瘫后痉挛性膀胱尿失禁的经验启示下与消化科共同采用内镜下注射     

Kabuki综合征合并婴儿痉挛症1例并文献复习

目的报道1例Kabuki综合征伴婴儿痉挛症的临床特点、诊治过程并文献复习。方法回顾性分析解放军总医院第一医学中心收治的1例Kabuki综合征合并婴儿痉挛症患儿的临床资料,检索PubMed、中国知网、万方数据知识服务平台及在线人类孟德尔遗传数据库(OMIM),结合文献报道,总结Kabuki综合征合并婴儿痉挛症的临床特点,并探讨其与基因型的关系。结果本例患儿男性,1岁7个月,因"生长发育落后,间断抽搐1年1个月余"入院。患儿生长发育全面落后,小头畸形,身材矮小,痉挛发作,脑电图提示高度失律,遗传学检测发现KDM6A基因新发移码突变(c.2170-c.2171delAT,p.I724Ifs*5),诊断为"婴儿痉挛症;Kabuki综合征"。给予ACTH输注后,患儿痉挛发作完全控制,多次复查脑电图较前明显好转。共获得英文文献16篇、中文文献1篇,共48例Kabuki综合征合并癫■患儿,其中6例为Kabuki综合征合并婴儿痉挛症,这6例中仅有2例基因报告可得,1例为KMT2D基因错义突变(c.96C>G,p.Asp32Glu),1例为KDM6A基因移码突变(c.2515₂     

食管粘膜下血肿(附3例报告并文献复习)

报告3例食管粘膜下血肿,并结合文献复习,对本病的分类、临床及 X线表现特征进行了分析讨论。     

食管憩室胸部X线平片及CT表现1例报告

患者男,68岁。因咳嗽、乏力、胸闷就诊。 胸部正侧位片：后纵隔心后T11椎体水平处见一大小约2．0cm×2．2cm的片块状影,密度不均匀（见图1,2）,考虑占位病变,建议CT检查。     

横纹肌肉瘤骨侵蚀X线表现（附4例报告并复习文献）

横纹肌肉瘤骨侵蚀较少见，本文报告４例并复习国内文献报告的８例，对其ｘ线表现进行了分析、总结。横纹肌肉瘤骨侵蚀的Ｘ线表现较为复杂，有骨质破坏，骨膜反应和软组织肿块。为便于认识，作者将骨质破坏分为广泛溶骨型、渗透状、虫蚀状和骨皮质外压吸收四型，这四型可混合存在。通过分析这１２例可见横纹肌肉瘤骨侵蚀多见于四肢骨和颅面骨，病理上以胚胎型、腺泡型为主者多见。     

肿瘤样钙质沉着症影像表现（附1例报告并文献复习）

目的：认识肿瘤样钙质沉着症X线及CT特征表现,提高影像诊断正确率。方法回顾分析1例双下肢肿瘤样钙质沉着症患者的X线、CT影像表现及临床资料,并复习相关文献。结果患者双髋关节、双膝关节周围及大腿肌群软组织内见“卵石样”、“桑葚状”大小不一分叶状、团块状钙化灶,病灶未累及相邻骨质及关节。结论肿瘤样钙质沉着症具有影像学特征表现,结合临床表现可对其作出准确的诊断。     

Imaging of diffuse esophageal leiomyomatosis: two case reports and review of the literature

Diffuse esophageal leiomyomatosis is a rare disorder which may be found in association with leiomyomas in other locations or with other disorders. We report two cases in men, one with associated tracheobronchial involvement, which illustrate the value of imaging in differentiating this entity from other causes of dysphagia and in establishing a diagnosis. Received: 24 December 1998; Revised: 10 May 1999; Accepted: 10 May 1999     

Diffuse esophageal spasm: CT findings in seven patients

OBJECTIVE: The purpose of this study was to determine the frequency and appearance of esophageal wall thickening on CT scans in a series of patients with findings of diffuse esophageal spasm on barium studies. CONCLUSION: CT revealed marked esophageal wall thickening in seven (21%) of 33 patients who had findings of diffuse esophageal spasm on barium studies. CT showed significantly greater esophageal wall thickening in the lower thoracic esophagus 5 cm above the gastroesophageal junction than in the upper thoracic esophagus at the level of the aortic arch or in the midthoracic esophagus at the level of the carina (p < 0.01). This esophageal wall thickening corresponded to the presence of multiple strong nonperistaltic contractions in the lower thoracic esophagus on barium studies. Our findings suggest that diffuse esophageal spasm should be included in the differential diagnosis when CT shows smooth circumferential wall thickening in the lower half of the thoracic esophagus, particularly in elderly patients with dysphagia or chest pain.     

Functional and morphological changes in the pharynx in achalasia and diffuse esophageal spasm

Since the pharynx and the esophagus are a functional unit, functional radiodiagnosis has to be directed at pharyngo-esophageal interaction. Among our collective of 73 patients suffering from achalasia or diffuse esophageal spasm, we were able to recognize a substantially increased incidence of morphological or functional pharyngeal disorders by means of cineradiography. The functional alterations in particular were often not revealed by conventional fluoroscopy. High-speed cineradiography, with its high temporal and spatial resolution, turned out to be a valuable tool in analysis of the origin of pharyngeal dysphagia. Manometry correlated very well with the radiologic findings in tubular esophagus, but proved unreliable in the detection of alterations of the upper esophageal sphincter region, because of problems inherent in the method. Furthermore, membranous stenosis (webs), lateral or dorsal diverticula, and asymmetry of the pharynx were observed strikingly often.     

头皮弥漫性T细胞非霍奇金淋巴瘤1例

患者女,56岁.发现右侧颞顶部无痛性包块5月余入院.查体:右颞顶部有一大小约3 cm×4 cm包块突出于皮肤,质韧,边界清楚,活动度可,无压痛,皮肤表面有破溃,周围3个较小包块,有触痛.实验室检查:周围血象,NEU中性粒细胞数1.99;LYM-R淋巴细胞数率52.9%;肝功能及血电解质、尿常规均未见异常.     

肾上腺神经节细胞瘤CT表现1例报告

病例资料 患者，女，45岁。患者来我院常规体检行CT扫描发现右肾上腺区巨大占位。无任何临床不适症状，无血压增高。体查腹部未及包块。CT表现平扫，右肾上腺区软组织肿块影，分叶状，边界尚清，内密度欠均匀，未见坏死、钙化、囊变，CT值约为28.5—31.0HU，大小约8.8cm×5.6cm×7.0cm。     

部分性葡萄胎MR表现1例

患者女,47岁,已婚,孕2产1,平素月经规律,末次月经时间2012-05-20.主因"阴道淋漓出血1月余"来本院就诊.血hCG 414 967 mIU/mL.妇科B超示:宫腔内98 mm×68 mm回声不均匀影像,内呈蜂窝样改变,考虑滋养细胞疾病,葡萄胎.     

泄殖腔畸形超声表现1例报告

孕妇28岁,孕2,产1,停经22周。于2011年2月13日首次来我院行产前常规超声检查,孕妇一般情况好,无近亲结婚及遗传病史。超声检查示:宫腔内未见羊水回声,胎头及四肢受压,显示欠清,胎儿脊柱前方可见大量无回声区,内未见肢体及脐带