摘 要
目的：探讨儿童纵隔支气管源性囊肿（Mediastinal BronchogenicCysts）的临床及影像学表现特点，为临床术前诊断、治疗提供可靠的影像学依据。
方法：收集重庆医科大学附属儿童医院 2008 年 7 月?2018 年 5月经手术和病理证实的30例纵隔支气管源性囊肿患儿的临床及影像学资料进行回顾性分析。
结果：
1.临床症状
8 例（26.7%）患儿因健康体检发现，无伴随症状。22 例（73.3%）伴临床症状，其中咳嗽最多见，有 19 例（63.3%）。其他症状包括；吼喘 10 例（33.3%）、发热 7 例（23.3%）、喉间痰响 2 例（6.7%）、胸闷1 例（3.3%）、咳痰 1 例（3.3%）、咳血 1 例（3.3%）、吞咽困难 1 例（3.3%）、头痛 1 例（3.3%）。17 例（56.7 %）患儿有 2 个及 2 个以上临床症状。
症状和诊断之间的间隔从 2 天到 2 年不等，中位数为 20.0 天。这些症状部分以一种渐进的方式出现，4 例（13.3%）患儿后期症状有加重。
2.影像学表现
囊肿位于中纵隔 16 例（53.3%）、后纵隔 6 例（20.0%）、同时跨中后纵隔 8 例（26.7%），无发生于前纵隔的囊肿。位于气管旁 11 例（36.7%）、肺门区 8 例（26.7%）、脊柱旁沟 6 例（20.0%）、气管隆突下区域 4 例（13.3%）、食管旁 1 例（3.3%）。
囊肿轴位最大径范围 19.5mm?59.0mm，平均（34.39±10.85）mm。
有临床症状组囊肿大小与无临床症状组囊肿大小差异分析无统计学意义（P >0.05）。23 例（76.7%）呈类圆形或椭圆形；7 例（23.3%）形态不规则； 3 例（10.0%）可见分隔，为多房。
22 例（73.3%）气管支气管不同程度受压、推移、变窄改变；8 例（26.7%）食管受压、推移。7 例（23.3%）伴少许胸膜病变；2 例（6.7%）邻近血管受压（肺血管及上腔静脉）；1 例（3.3%）伴脊柱侧弯。
CT 扫描 29 例，其中 CT 增强扫描 25 例。囊肿 CT 值范围为 3.3HU?45.5HU。增强扫描 24 例囊内容物未见明显强化，囊壁强化，其中 1例囊壁厚薄不均；1 例囊内容物轻度强化，囊壁不确定。CT 表现呈 1a型 16 例（55.2%）、1b 型 2 例（6.9%）、2a 型 10 例（34.5%），2b 型 1例（3.4%），未见 3 型囊肿。1 型 MBC 的临床症状与 2 型 MBC 的临床症状差异分析均无统计学意义。
MRI 扫描 11 例，其中 MRI 增强扫描 7 例。11 例（100.0%）T2WI均呈均匀高信号，类似脑脊液信号（Cerebrospinal Fluid，CSF）。6 例（54.5%）T1WI 呈均匀低信号，4 例（36.4%）TIWI 信号呈均匀等信号，1 例（9.1%）TIWI 信号不均，可见等低信号。7 例增强扫描均可见囊壁强化，而囊内容物未见明显强化。10 例囊肿同时行 CT 及 MRI扫描，包含 1a 型 7 例、1b 型 1 例、2a 型 1 例、2b 型 1 例。
3.手术观察
手术中见 18 例（60.0%）囊内容物为流动液体，12 例（40.0%）为胶冻状。流动液体组囊肿 CT 值与胶冻组囊肿 CT 值大小差异具有统计学意义，前者较后者小（T 值= -3.581，P<0.05）。
4.组织病理学
囊壁内衬( 假复层) 纤毛柱状上皮 29 例、鳞状上皮 1 例；囊壁上见支气管粘液腺13例(43.3%)、平滑肌10例（33.3%）、软骨9例（30.0%）、囊壁上及周围见炎症细胞浸润 9 例（30.0%）、伴出血坏死 2 例（6.7%）；钙化 1 例（3.3%）。
结论：
1. 支气管源性囊肿是罕见的肺芽异常萌发所导致的先天性疾病，发生于纵隔者多见。
2. 支气管源性囊肿的临床表现变化大，可以无伴随临床症状，部分患儿有临床症状，但均无特异性，其后期部分症状有加重。
3. 大多数纵隔支气管源性囊肿 CT 表现典型。但是，小部分纵隔支气管源性囊肿表现不典型，如囊内容物密度增高、增强扫描囊内容物强化、囊壁增厚、囊壁不确定、囊肿边界模糊，容易和其他疾病混淆，造成误诊。
4. MRI 在确定支气管源性囊肿的囊性本质方面更有优越性，故应优化影像学检查的选择方案，提高影像诊断准确率。
关键词：儿童；支气管源性囊肿；体层摄影术；磁共振成像
Abstract
Objective: To explore the clinical and imaging characteristics ofMediastinal Bronchogenic Cysts in children to provide reliable imagingbasis for preoperative diagnosis and treatment.
Methods: Clinical and imaging data of 30 children with mediastinalbronchiogenic cyst who were confirmed by surgery and pathology from July2008 to May 2018 in Children's Hospital Affiliated to Chongqing MedicalUniversity were retrospectively analyzed.
Results:
1. Clinical manifestation.
No accompanying symptoms were found in 8 cases (26.7%) due tophysical examination.22 cases (73.3%) were associated with clinicalsymptoms, of which cough was the most common, with 19 cases (63.3%).
Other symptoms include：10 cases (33.3%) of roar asthma, 7 cases of fever(23.3%), 2 cases (6.7%) of interlaryngeal sputum noise, 1 case of chesttightness (3.3%), 1 case of expectoration (3.3%), 1 case of hemoptysis(3.3%), 1 case of dysphagia (3.3%), and 1 case of headache (3.3%).17cases (56.7%) had two or more clinical symptoms. The interval betweensymptoms and diagnosis ranged from two days to two years, with a median of 20.0 days.Some of these symptoms appear in a gradual way, 4 cases(13.3%) of these symptoms exacerbated later.
2. Imaging findings.
16 cases (53.3%) were located in middle mediastinum, posteriormediastinum in 6 cases (20.0%) and 8 cases (26.7%) involved in middleand posterior mediastinum. None occurred in the anterior mediastinum.
The cysts were located in paratracheal region in 11 cases (36.7%), hiluspulmonis region in 8 cases (26.7%), Paravertebral space in 6 cases (20.0%),subcarinal angle in 4 cases (13.3%) and paraesophageal in 1 case (3.3%).
The maximum cyst axial diameter was 19.5mm ~ 59.0mm, average（34.39±10.85）mm. There was no statistically significant differencebetween the size of the cyst in the symptomatic group and thenon-symptomatic group (P >0.05). 23 cases (76.7%) were round or oval. 7cases (23.3%) were irregular. 3 cases (10.0%) were visible separated formultiple rooms.
In 22 cases (73.3%), bronchial trees were compressed, displaced andnarrowed. The esophagus was compressed and displaced in 8 cases(26.7%).7 cases (23.3%) had pleural lesions.Compression of adjacentvessels (pulmonary vessels and superior vena cava) in 2 cases (6.7%);1case (3.3%) was associated with scoliosis.
CT scan was performed in 29 cases, including CT enhanced scan in 25cases. The cyst CT value ranged from 3.3~45.5HU. In contrast-enhanced scan, 24 cases had no enhancement of cystic contents and cystic wallenhanced, 1 case had uneven thickness of cyst wall. 1 cases of cysticcontents were slightly strengthened and the wall was not definite. CTmanifestations were 1a type in 16 cases (55.2%), 1b type in 2 cases (6.9%),2a type in 10 cases (34.5%), 2b type in 1 case (3.4%) and no type 3 cystwas found. There was no statistically significant difference between theclinical symptoms of type 1 MBC and type 2 MBC.
MRI scan was performed in 11 cases, including MRI enhanced scan in7 cases. 11 cases (100.0%) of T2WI showed homogeneous hyperintensity,similar to CSF. 6 cases (54.5%) showed homogeneous hypointense on T1WI,4 cases (36.4%) showed homogeneous isointensity on TIWI, and 1 case(9.1%) showed heterogeneous signal on TIWI. The enhancement of thecystic wall was seen in all 7 cases, but no significant enhancement wasobserved in the contents of the cyst. CT and MRI scan were performed in 10cases, including 7 cases of type 1a, 1 case of type 1b, 1 case of type 2a and 1case of type 2b.
3.Intraoperative observation.
18 cases (60.0%) of the cyst contents were fluid and 12 cases (40.0%)were gelatinous. The CT value of the cyst of the fluid group wassignificantly different from that of the gelatinous contents group (T =-3.581, P<0.05).
4. Histopathology.
There were 29 cases of ciliated columnar epithelium and 1 cases ofsquamous epithelium. There were 13 cases (43.3%) of bronchial glands, 10cases (33.3%) of smooth muscle, 9 cases (30.0%) of cartilage, 9 cases(30.0%) of inflammatory cell infiltration, 2 cases (6.7%) with hemorrhageand necrosis and 1 case of calcification (3.3%).
Conclusions:
1.Bronchogenic cyst is a rare congenital disease caused by abnormalgermination of lung buds. It occurs mostly in mediastinum.
2.The clinical manifestations of bronchogenic cysts vary greatly andmay not be accompanied by clinical symptoms. Some children haveclinical symptoms, but none of them are specific, Some of the them areaggravated in the later.
3.CT findings of most mediastinal bronchogenic cysts are typical.however, a small number of mediastinal bronchogenic cysts are not typical,such as increased cyst content density, cyst content enhancement,thickening of the cyst wal, uncertain cyst wall, blurred cyst boundary,easily confused with other diseases, resulting in misdiagnosis.
4.MRI is more advantageous in determining the cystic nature ofbronchogenic cysts, so the selection scheme of imaging examination shouldbe optimized to improve the accuracy of image diagnosis.
Keywords: children; Bronchogenic cyst; Tomography; Magnetic resonance imaging.
目录
1 材料与方法
1.1 研究对象
收集我院 2008 年 7 月至 2018 年 5 月期间经手术及病理确诊的纵隔支气管源性囊肿 30 例，男女比例为 14：16，年龄范围从 1 月至 11 岁 3 月，平均年龄为 3.55±3.06 岁，中位数为 3.0 岁。
1.2 影像学检查
1.2.1 X 线
采用 GE Deffinium8000 DR 机，参数：管电压 60kV，管电流 1～2.5mAs。
1.2.2 CT（Computed Tomography，CT）扫描：
采用 GE LightSpeed 64 排 VCT64 排螺旋 CT 机或 Phlips Brilliance iCT256 排螺旋 CT 机。主要扫描参数为: 管电压 80～120kV，管电流 35～85mAs，螺距 0.984：1，层厚、层距同为 5.00mm，必要时以 MSCT 薄层重建图像辅助观察，图像重建层厚 0.625mm，视野 160~240mm，矩阵为 512×512 或 1024×1024。增强扫描采300mgI/ml 或 270mgI/ml 非离子型对比剂，剂量为 2ml/kg，用高压注射器经静脉团注，注射速率为 0.6~3.0ml/s。
1.2.3 MRI（Magnetic Resonance Imaging）扫描：
利用 GE 1.5T HD 扫描仪，常规行 T1WI 及 T2WI 加脂肪抑制序列扫描，增强扫描采用钆喷酸葡胺(Gd-DTPA)对比剂，剂量为 0.2ml/kg，用高压注射器经静脉注射后行轴位、矢状位扫描。
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1.2 影像学检查
1.3 影像学分析
1.4 分型及分类方法
1.5 统计学方法
2 结果
2.1 临床症状
2.2 影像学表现
2.3 手术观察
2.4 组织病理学
3 讨论
3.1 支气管源性囊肿概述
3.2 发病部位
3.3 临床表现分析
3.4 影像学特点分析
3.5 鉴别诊断
3.6 结语
全文总结
参考文献
