小儿先天性肺囊性腺瘤样畸形四例

先天性肺囊性腺瘤样畸形 (ｃｏｎｇｅｎｉ ｔａｌｃｙｓｔｉｃａｄｅｎｏｍａｔｏｉｄｍａｌｆｏｒｍａｔｉｏｎｏｆｔｈｅｌｕｎｇ ,ＣＣＡＭ)是一种少见的小儿的先天性支气管 -肺发育畸形。由Ｃｈｉｎ等首次报道 (ＡｒｃｈＰａｔｈｏｌ,1949)。 1996～2 0 0 0年我院共收治 4例 ,均经临床病理证实 ,现报道如下。临床资料本组 4例 ,男 2例 ,年龄分别为 5、8个月 ;女 2例 ,年龄分别为 4、8个月。均以长期咳嗽、气喘症状就诊。 2例以肺部感染入呼吸内科 ,经抗感染治疗肺部病变不改善而转入外科。另 2例因胸部透视示支气管不全阻塞误诊为支气管…     

先天性肺囊性腺瘤样畸型1例报告

先天性肺囊性腺瘤样畸型是一种少见的肺发育异常,我院最近发现１例,并经尸检证实,现报告如下：患儿,男,Ｇ１Ｐ１,３７＋６Ｗ,出生体重３２００ｇ,因第２产程延长经低中位产钳娩出,羊水量中等,色清。Ａｐｇａｒ评分,１分钟９分,５分钟１０分,１０分钟８分。患儿生后１５小时出现青紫、呻吟、气促,由我院产婴室转入我科ＮＩＣＵ。患儿母孕期３个月和９个月时各有一次感冒,无异常服药史。体验：Ｔ３６．５℃,Ｐ１６５次／分,Ｒ７０次／分,患儿鼻　,面部、唇周及四肢末端青紫,呼吸浅快,右肺叩诊为浊音,二肺底可闻及中细湿…     

儿童先天性肺囊性腺瘤样畸形四例临床特点分析

目的探讨儿童先天性肺囊性腺瘤样畸形(congenital cystic adenomatoid malformation of lung,CCAM)的临床、影像及组织病理特点,提高临床对该病的认识。方法收集新乡医学院第一附属医院经病理证实为CCAM的4例患儿病例资料,对其临床表现、影像学及病理特点、治疗方法进行分析。结果3例患儿有反复呼吸道感染,1例自发性气胸;2例胸部X线见到囊性改变,2例胸部CT提示存在CCAM,1例提示肺脓肿,1例提示不除外肺隔离症;4例均手术切除病变肺叶,术后病理证实为CCAM,均预后良好。结论反复呼吸道感染者需注意该病可能,需结合临床表现、CT检查、病理结果共同诊断CCAM,手术切除是有效治疗手段。     

先天性肺囊性腺瘤样畸形1例误诊分析

先天性肺囊性腺瘤样畸形（CCAM）是一种罕见的肺发育异常，可发生于任何年龄，1岁以下儿童多见。CCAM的临床表现多种多样。新生儿期发病的CCAM以出生时或出生后不久即出现呼吸窘迫、严重呼吸困难、发绀等重要表现，婴儿及儿童则以周期性或反复发作及局限于一叶的肺感染为主要表现。本文将1例先天性肺囊性腺瘤样畸形（CCAM）在初诊时被误诊为肺炎心衰的病例报告如下。     

先天性肺囊性疾病

先天性肺囊性疾病是较少见的先天性肺部疾病,是肺组织胚胎发育异常所形成的畸形。囊性病变包括囊性腺瘤样畸形、支气管源性肺囊肿、先天性大叶性肺气肿、肺隔离症。此类疾病的临床表现相似,但发生机制及病理改变有各自的特点。囊性腺瘤样畸形是由于细支气管发育停滞,以肺泡不发育为代价引起的肺间质大量增生,目前根据病理类型分成5型。支气管源性肺囊肿是胚胎发育时期支气管树某段的异常出芽形成的。先天性大叶性肺气肿是支气管内阻外压以及支气管壁的病变造成的气体陷闭,肺泡腔扩张。肺隔离症是部分肺组织与正常的支气管肺组织无交通,供血来自体循环,病变肺无正常功能,分叶内型和叶外型。     

产时子宫外处理技术治疗胎儿先天性肺囊性腺瘤样畸形的初步探讨

目的 初步探讨采用产时子宫外处理技术（EXIT）治疗胎儿先天性肺囊腺瘤样畸形的可行性.方法 2010年10月至2012年6月我们对3例胎儿先天性肺囊腺瘤样畸形采用产时子宫外处理技术切除.3例均经2次以上产前彩超诊断为先天性肺囊腺样畸形,且巨大畸形导致胎儿心脏、纵隔部分或全部移位,术前计算CVR值均大于1.6.结果 3例术后均存活,其中男1例,女2例,手术时胎龄36 ～37＋周,平均（36.86±0.79）周,体重2 700～3 000 g,平均（2 866.67±152.75）g;麻醉时间130～134 min,平均（133.67 ±3.51）min;总手术时间92～98 min,平均（94.33 ±3.21） min;手术时间33～49 min,平均（42.67±8.50） min;术中出血量2～20 mL;平均（10.67±9.02） mL.术后病理检查结果均为肺囊性腺瘤样畸形,左肺2例,右肺1例.结论 产时子宫外处理可以作为治疗高风险先天性肺囊腺样畸形的方法之一,但因手术风险大,操作较复杂,需多学科团队进行合作,需严格把握该技术的适应证,结合各方面条件,谨慎选择.     

先天性肺囊腺瘤样畸形40例诊疗分析

目的 分析先天性肺囊腺瘤样畸形胎儿-新生儿的的围产危险因素,探讨其手术时机以及风险管理方法.方法 回顾性分析自2008年10月至2013年1月,来本院就诊40例先天性肺囊腺瘤样畸形病例资料.结合产前诊断以及出生后的胸片、CT检查,分析围产风险因素和手术时机. 结果临床诊断先天性肺囊腺瘤样畸形40例中,顺产17例,剖腹产23例;早产儿3例.出生后有呼吸道症状11例,占27.5％,新生儿期出现呼吸道症状14例,占35.0％.40例中,手术治疗32例;1例放弃治疗,后来死亡;7例家属选择等待.32例手术患儿均治愈出院.平均手术年龄5.6个月.结论 先天性肺囊腺瘤样畸形在新生儿期存在呼吸道风险,应选择在有新生儿外科手术条件的医院生产,出生后需注意预防呼吸道风险,为手术创造条件.     

小儿先天性肺腺瘤样畸形的诊断及外科治疗

目的 总结小儿先天性肺腺瘤样畸形(CPAM)诊治经验.方法 回顾2006年11月至2009年11月手术的13例CPAM临床资料,男6例,女7例,手术年龄50d至13岁.多数患儿有临床症状,经胸部X线和CT检查确诊.3例小于2个月者急诊手术,其余患儿择期手术.术式有单肺叶切除10例,肺叶+不规则肺段切除2例,囊肿剥离术1例.病理示Ⅰ型8例,Ⅱ型和Ⅲ型各2例,Ⅳ型1例.结果 无手术死亡.2例术后早期出现支气管胸膜瘘,1例经保守治疗,1例在胸腔镜下瘘口缝合后治愈.术后平均住院9.7 d,平均随访12.3个月,2例肺叶+不规则肺段切除者活动量轻度受限,其余患儿恢复良好.结论 胎儿超声是CPAM产前诊断的主要手段,胸部CT检查对产后诊断有高度特异性.     

剖宫直视下胎儿左侧先天性肺囊腺样畸形切除术后新生儿1例

先天性肺囊腺瘤样畸形(CCAM)是由于末端支气管呈瘤样过度生长,同时损害肺泡,在肺实质内形成有明显界限的一种病变,可在患儿出生后不久导致呼吸循环衰竭而死亡[1]。该症可分为3型。其中Ⅲ型预后不良,Ⅱ型并胎儿水肿及羊水增多,预后亦极差,一般均需外科干预,但往往在出生早期即失去手术机会[2]。从20世纪90年代起美国及欧洲一些国家已逐渐发展形成胎儿手术     

Outcome and treatment in an antenatally diagnosed congenital cystic adenomatoid malformation of the lung

Background  

The natural history of patients with antenatally diagnosed congenital cystic adenomatoid malformation of the lung (CCAM) is still fully unknown. In symptomatic patients with respiratory distress, an operation is performed during the neonatal period. However, in asymptomatic patients, the optimal timing of the operation remains controversial. During the period from 1977 to 2007, we experienced 14 CCAM patients diagnosed antenatally. Therefore, we investigated the outcome of antenatally diagnosed CCAM patients to clarify the optimal treatment for such patients.     

Congenital cystic adenomatoid malformation of the lung

The etiology of CCAM of the lung remains unclear. Presentations vary, with clinical outcomes that may be unpredictable. The neonatal nurse should be equipped to detect the sometimes subtle radiologic images these lesions produce and should be able to distinguish CCAM from similar chest masses. Expediting this diagnosis postnatally benefits the neonate by preventing further expansion of the cysts, which can cause a cascade of complications, including air leaks, cardiorespiratory compromise, and PPHN.     

Management of prenatally diagnosed congenital cystic adenomatoid malformation of the lung.

We have treated four prenatally diagnosed cases of extensive congenital cystic adenomatoid malformation (CCAM) of the lung. The first case in 1982 was associated with severe fetal hydrops. After thoracentesis at 31 weeks of gestation abruptio placentae occurred, and a female baby was delivered by cesarean section. She underwent a right lower lobectomy, but soon died. The second baby without hydrops, diagnosed as having CCAM at 26 weeks of gestation, was followed conservatively until full term. After birth, it was necessary to treat the baby boy with extracorporeal membrane oxygenation (ECMO), but he survived. The third baby with fetal hydrops had an indwelling drainage catheter inserted into the CCAM at 27 weeks of gestation. The hydrops subsided and the baby was delivered at 37 weeks of gestation. He was allowed to breathe spontaneously, but was intubated 16 hours after birth. A right lower lobectomy was successfully performed 24 hours after delivery. The fourth baby without fetal hydrops was followed conservatively until delivery. He underwent left lower lobectomy successfully on the 4th day of life. Although management of prenatally diagnosed CCAM varies among patients, insertion of an indwelling catheter into the cyst appears to be the treatment of choice if indicated; the catheter can be maintained for as long as 10 weeks, as shown in Case 3. Cases of CCAM without fetal hydrops should also be treated carefully, because persistent fetal circulation may occur postnatally.     

Long-term outcome of congenital cystic adenomatoid malformation

To determine the long-term outcome of congenital cystic adenomatoid malformation (CCAM), the records of all patients with CCAM treated in three Hungarian paediatric surgical centers between 1977 and 1996 were reviewed. Patients were followed for up to 20 years following diagnosis and treatment. In 20 patients CCAM was diagnosed postnatally and in 3 prenatally. Biodata including the operative procedures are presented. Follow-up findings between 18 months and 20 years after diagnosis showed better height and weight growth in patients operated upon in later childhood compared with those operated upon in infancy. The older the patient at the time of diagnosis, the better was the long-term outcome. Accepted: 5 January 1999     

Coincidence of congenital cystic adenomatoid malformation and extralobar sequestration of the lung in a newborn

We present a rare case of coincidence of an extralobar sequestration with a congenital cystic adenomatoid malformation of the lung in a newborn. The symptoms, diagnostic features, and therapy are described and the etiology and classification are briefly discussed.