小儿支气管肺囊肿影像与临床病理学研究进展

小儿支气管肺囊肿临床上较为少见,由于胚胎发育过程中肺芽分支发育畸形,气管和支气管异常芽造成一段或多段支气管完全或不完全闭锁,远段逐渐扩张形成盲囊,囊内细胞分泌的粘液不能排出而积聚膨胀形成囊肿,临床表现无特异性,影像学上变化多样,易引起误诊误治。本文就小儿支气管肺囊肿的影像学表现与临床组织病理学研究进展综述如下。     

小儿先天性肺囊肿的外科治疗

目的 总结小儿先天性肺囊肿的外科治疗经验。方法 本组患儿均在全麻下行外科手术治疗,其中占据一侧胸腔的巨大张力性囊肿６例,２例术前放管引流减压。单纯囊肿摘除９例,肺段及肺楔形切除各２例,肺叶切除３６例,全肺切除１例。结果 本组无手术死亡,术后并发支气管胸膜瘘１例。１１例肺叶切除患儿随访１～１０年,肺功能各项指标均正常。结论先天性肿囊肿应早期诊断、早期治疗。对巨大张力性肺囊尖急诊手术,必要时术前放管囊     

先天性肺囊肿10例

先天性肺囊肿发病率在0．04％～0．16％[1].为提高对本病的认识以及进行及时的手术治疗，现将我科自1992年5月～1998年5月收治先天性肺囊肿10例报告如下。临床资料一、一般情况：本组共10例。男7例，女3例。发病年龄～lmol例，～las例，～3a3例，～sal例。二、临床表现：首次发     

小儿先天性肺囊肿周边肺发育的观察及其临床价值

目的 探讨先天性肺囊肿(CBC)与肺发育不全的关系及其外科治疗。方法 32例CBC患儿和18例正常对照，分别测量肺泡辐射状计数(RAC值)及单位视野内第6级以上肺小动脉数(A值)。行肺小动脉Gomori醛复红弹力纤维染色；随访病例复查胸片，测量TLC、VC。结果 CBC组RAC值及A值低于正常对照，支气管型CBC的RAC值低于肺泡型；肺小动脉内、外弹性膜可见多处断裂，管壁平滑肌变性及肥厚；随访囊肿摘除及肺叶切除术后TCL、VC分别为对照的97％—98％、85％～90％。结论 CBC导致周边肺发育不全，宜尽早手术摘除。     

纵隔支气管囊肿

患儿,男,7岁,因反复咳嗽3年余,加重1月伴胸痛入院。体检:右下肺听诊呼吸音较左侧减低,右侧胸部叩诊呈浊音。VMA:49.06μmol/24 h。正侧位胸片(图1):右后下纵隔紧贴脊柱旁软组织肿块,气管受压不明显;食道受压向左前移位,食道粘膜光滑。CT表现(图2):右后纵隔见密度均匀、边缘光滑的块影,增强后肿块不强化。MRI表现(图3):T2WI呈明显均一高信号,上至主动脉弓平面,下达膈面。影像学诊断:右中后纵隔占位,前肠囊肿可能大。术中见肿块为囊性,呈肠管样,基部肿大,向上一直延伸至颈椎水平。病理诊断:支气管囊肿。     

Arndt支气管内阻塞管在小儿肺囊肿切除术中的应用

小儿肺囊肿是一种常见的支气管、肺发育异常。近年来，随着小儿胸腔镜和开胸手术的增多，对实行单肺通气的麻醉技术提出了更高的要求。Arndt支气管内阻塞管是近年用于单肺通气的新技术，在国内使用报告较少。我科自2006年12月～2007年3月采用支气管内阻塞管行单肺通气和肺叶隔离技术完成3例肺囊肿切除术，均获得满意效果。     

小儿胸膜肺母细胞瘤的临床及病理学特征

目的 探讨小儿胸膜肺母细胞瘤(pleuro-pulnlonary blastoma,PPB)临床及病理学特征和早期诊治的可行性。方法 对1例长期反复呼吸道感染、X线片呈肺野巨大圆形软组织肿块的患儿行肺叶切除术、病理学常规检查和免疫组织化学检查等、明确诊断为PPB,结合文献复习进行分析讨论。结果 2岁女孩肺内病变抗炎治疗无效,无呼吸困难、贫血、消瘦等表现,手术见左上肺尖后段囊实性孤立性、境界清楚肿块,组织结构见分化较差的小圆细胞、梭形细胞,间质成分含横纹肌母细胞、成熟和／或不成熟软骨小岛,腺管分化良好、免痰组化标记检查确诊为PPB。结论 小儿肺部肿瘤病理学检查出现间胚叶组织和颖似上皮细胞且免疫组化标记呈阳性表达时,可诊断为胸膜肺母细胞瘤。     

先天性肺囊肿21例

目的：探讨先天性肺囊肿的诊断、鉴别诊断及手术治疗。方法：先天性肺囊肿21例经X线及CT检查明确有手术指征，分别行肺叶切除或囊肿摘除。结果：术前确诊19例，2例术前误诊为其他肺部疾病患儿术后病理检查证实为本病。均经手术治愈，术后随访1－4年效果良好。结论：先天性肺囊肿易与其他疾病混淆，引起误诊误治。影像学检查发现明确手术指征者应积极采取外科治疗，有感染者应在控制中毒症状后手术。     

支气管肺发育不良

支气管肺发育不良（BPD）是一种慢性肺疾病（CLD），通常发生于罹患肺透明膜病（HMD）、接受氧气、机械通气治疗的早产儿。在早期文献，此病被称为BPD，而近年来被称为CLD。2000年，在美国儿童健康和人类发展研究院（NICHD），心脏、肺和血液研究院（NHLBI）及少见病委员会共同举办的BPD研讨会上，多数人主张采用BPD命名，认为此命名可清楚的区别生命后期多种原因的CLD。     

Mucoepidermoid carcinoma of the lung arising at the primary site of a bronchogenic cyst: clinical, cytogenetic, and molecular findings

Primary lung tumors are rare in children, and mucoepidermoid carcinoma (MEC) represents less than 10% of them. Additionally, MEC arising from bronchogenic cysts (BC) is particularly unusual. We describe the clinical and genetic findings on a MEC occurring within a previous location of a BC in an adolescent. This particular association has not been previously reported. The lesion revealed normal karyotype without the typical t(11;19)(q21;p13) translocation. Cyclin D1 overexpression (165-fold increase) was demonstrated by real-time PCR although FISH assessment showed normal hybridization at 11q13. Information on these unusual clinical presentations may present relevant insight on tumorigenesis of infrequent pediatric pulmonary tumors.     

Periscapular bronchogenic cyst

Periscapular location of bronchogenic cysts is extremely rare with four cases reported in literature. We report a relatively late presentation in a ten-year-old boy that was clinically mistaken for a typical sebaceous cyst. The definitive treatment is total excision after preoperative imaging to detect the occassional intrathoracic extension. Histologically they are lined by pseudostratified ciliated columnar epithelium, which can rarely undergo malignant transformation.     

Pericardial cyst diagnosed by two-dimensional echocardiography and computed tomography in a newborn

Summary A one-day-old full-term female infant with massive “cardiomegaly” developed marked respiratory distress soon after birth. The two-dimensional (2D) echocardiogram demonstrated a 4×5-cm cystic mass adjacent to the left ventricular free wall. Computed tomography (CT) confirmed the presence and dimension of the cyst and CT number indicated the fluid within the cyst to be serous. A pericardial cyst was successfully removed at thoracotomy. Two-dimensional echocardiography and CT, both noninvasive procedures, are fully adequate tools in the diagnosis of pericardial cysts in the neonate, so that invasive investigations may be avoided.     

Bronchogenic cyst of the right hemidiaphragm mimicking a hydatid cyst of the liver: report of the first pediatric case

Bronchogenic cyst (BC), which develops from abnormal budding of the tracheal diverticulum or ventral foregut, is a congenital bronchopulmonary malformation. Localization of the BC varies depending on the level of the abnormal budding. Thoracic or abdominal-sited diaphragmatic lesions are the rarest presentations of the BC. We present a case of BC that originated from the diaphragm and mimicking hydatid cyst of the liver in a 19-month-old girl. Diagnosis of a diaphragmatic lesion was confirmed during laparotomy and complete resection was successful.     

Paraesophageal bronchogenic cyst: first case reports in pediatric

We described for the first time a paraesophageal intra-abdominal bronchogenic cyst, first considered as hepatic tumor, in a young boy. Resection was successfully realized by laparoscopy. Abdominal bronchogenic cyst and differential diagnosis are discussed.     

基于多层CT技术在儿童先天性胆总管囊肿手术方式制定中的应用

目的 探讨多层螺旋CT在先天性胆总管囊肿手术方式制定中的应用价值.方法 收集我院2006年9月至2009年3月间经手术及病理证实胆总管囊肿病例30例,根据手术中所见腹腔粘连情况,将病例分为粘连组及非粘连组,分别观察两组在多层螺旋CT双期增强检查中横断面图像及多平面重建图像(冠状面及矢状面)上胆总管囊肿分型及囊肿大小、形态、囊壁厚度、囊肿张力及囊壁附着血管数的差异,通过统计学分析筛选出与腹腔粘连有显著性相关的CT征象.结果 胆总管囊肿的囊壁厚度及囊壁附着血管数与腹腔粘连有显著性相关,粘连组壁厚(2.9±1.12)mm,附着血管数(4.9±1.4)个,P<0.05.而胆总管囊肿分型及其大小、囊肿张力则与腹腔粘连无显著性相关.结论 多层螺旋CT增强检查上胆总管囊肿壁厚大于或等于2.9 mm及囊壁附着的血管数大于或等于5支可以术前判断腹腔内感染或粘连较重.可为胆总管囊肿手术方式的选择提供指导.     

Use of computed tomography in evaluation of acute respiratory distress in a 4.5-month-old with bronchogenic cyst

A 4.5-month-old female presented with stridor and acute respiratory distress. Chest radiographs showed hyperinflation of the left lung. Computed tomography of the chest demonstrated a small bronchogenic cyst not clearly identified by other methods.     

A bronchogenic cyst under the scapula with a fistula on the back

A 20-year-old patient with a chronic fistula located at the left scapular region caused by a deep located subscapular bronchogenic cyst is described.     

Congenital midline cervical cleft with an underlying bronchogenic like cyst

Congenital midline cervical cleft (CMCC) is an uncommon malformation. We report a case of a baby girl aged 3 days with a CMCC associated with a cyst reported as a bronchogenic cyst (BC). The pathology is not specific. The association of BC and CMCC is extremely rare and only five cases have been found in the literature. We report our case and review the relevant literature.     

Lingual Bronchogenic Cyst in a Child: An Unusual Site of Presentation

We describe a 4-year-old hispanic male with a bronchogenic cyst (BC) in the base of the tongue. To our knowledge, a BC has not been previously reported in the oral cavity. BC is a developmental anomaly believed to originate from abnormal detachments of accessory lung buds from the ventral foregut. We postulate that the unusual location of the BC in the present case is related to the primitive foregut origin of tongue epithelium posterior to the foramen cecum and the sulcus terminalis. A BC in the base of the tongue appears to represent the most proximal expression of aberrant accessory lung bud detachment from the primitive foregut.