Congenital Cystic Adenomatoid Malformation: A Case Report with Clinical, Radiological, Histological, and Surgical Features

Background: Congenital cystic adenomatoid malformation (CCAM) is a congenital anomaly of lung development, accounting for approximately 25% of congenital lung lesions. Respiratory distress often occurs during the neonatal period, and in 80% to 85% of cases, the diagnosis is made before the age of 2 years following respiratory infections. Case Report: We report a case of MAKC diagnosed in the neonatal period. The diagnosis was based on clinical, radiological and histological elements. Our patient underwent surgical resection. Histological examination confirmed the diagnosis of MAKC without any sign of malignancy. The postoperative evolution was good. Conclusion: Clinicians and pathologists should recognize the early discovery of MAKC in neonatal age. The clinical diagnosis strongly guided by the radiological approach is confirmed by the pathological anatomy insofar as the therapeutic sanction is surgical in the majority of the cases.

Antenatal Diagnosis and Outcome of 12 Congenital Cystic Adenomatoid Malformation of Lung

Objective To investigate the ultrasonic detection probability, type, prenatal diagnosis, and outcome of congenital cystic adenomatoid malformation of lung （CCAM）. Methods In this retrospective study, all 12 cases which were diagnosed with suspected prenatal CC,4M over the period in the hospital were analyzed. Information on diagnosis time, types and progression of the lesions during pregnancy, the additional abnormalities, and the outcome of pregnancies were recorded.Results The positive rate of ultrasonic detection of CCAM was about 1.01‰ （1/11 124） before 28 gestation weeks. There were 1 case of type Ⅰ （8%）, 2 cases of type Ⅱ （17%） and 9 cases of type Ⅲ （75%）. Nine pregnancies were terminated and 2 cases were confirmed by pathology. Three neonates were alive and without any symptom now. Conclusions Type Ⅲ is the major type. Ultrasound examination during 20-28 weeks and following up, prenatal consultation, chromosome examination offetus, delivery with a pediatrician standing by, and all suspected neonates being investigated are recommended.

Usefulness of prenatal magnetic resonance imaging in differential diagnosis of fetal congenital cystic adenomatoid malformation and bronchopulmonary sequestration

BACKGROUND Congenital cystic adenomatoid malformation(CCAM)and bronchopulmonary sequestration(BPS)are the most common lung diseases in fetuses.There are differences in the prognosis and treatment of CCAM and BPS,and the clinical diagnosis and treatment plan is usually prepared prior to birth.Therefore,it is quite necessary to make a clear diagnosis before delivery.CCAM and BPS have similar imaging features,and the differentiation mainly relies on the difference in supply vessels.However,it is hard to distinguish them due to invisible supplying vessels on some images.AIM To explore the application value of magnetic resonance imaging(MRI)in the differential diagnosis of fetal CCAM and BPS.METHODS Data analysis for 32 fetuses with CCAM and 14 with BPS diagnosed by prenatal MRI at Huzhou Maternal and Child Health Care Hospital and Anhui Provincial Children’s Hospital from January 2017 to January 2020 was performed to observe the source blood vessels of lesions and their direction.Pathological confirmation was completed through CT examination and/or operations after birth.RESULTS After birth,31 cases after birth were confirmed to be CCAM,and 15 were confirmed to be BPS.The CCAM group consisted of 21 macrocystic cases and 10 microcystic cases.In 18 cases,blood vessels were visible in lesions.Blood supply of the pulmonary artery could be traced in eight cases,and in 10 cases,only vessels running from the midline to the lateral down direction were observed.No lesions were found in four macrocystic cases and one microcystic case with CCAM through CT after birth;two were misdiagnosed by MRI,and three were misdiagnosed by prenatal ultrasonography.The BPS group consisted of 12 intralobar cases and three extralobar cases.Blood vessels were visible in lesions of nine cases,in four of which,the systemic circulation blood supply could be traced,and in five of which,only vessels running from the midline to the lateral up direction were observed.Three were misdiagnosed by MRI,and four were misdiagnosed by prenatal ultrasonography.CONCLUSION CCAM and BPS can be clearly diagnosed based on the origin of blood vessels,and correct diagnosis can be made according to the difference in the direction of the blood vessels,but it is hard distinguish microcystic CCAM and BPS without supplying vessels.In some CCAM cases,mainly the macrocystic ones,the lesions may disappear after birth.

Antenatal and postnatal management of congenital cystic adenomatoid lung malformation diagnosed by ultrasound and Magnetic Resonance Imaging (MRI)

Purpose: Antenatal diagnosis of congenital cystic adenomatoid lung malformation (CCAM) is vital for disease surveillance and postnatal care. Ultrasonography (US) has been the imaging gold standard for antenatal CCAM assessment. However, one of the limitations of US is the “vanishing phenomenon” caused by isoechogenicity of CCAM tissue and adjacent normal lung parenchyma. Methods: Antenatal serial US were concurrently used with magnetic resonance imaging (MRI) to monitor macro- and microcystic lesions. Results: In both pregnant women, antenatal US and MRI confirmed the presence, in the fetus, of cystic lesions and predicted disease regression/progression as well as the need for postnatal surgical intervention. Several advantages were detected by using both—serial US and MRI (over serial US alone)—including improved signal intensity, exact volume size measurements, precise CCAM location in particular for patients with adverse ultrasound conditions. Both neonates underwent surgical resection and had an uneventful post-operative course. Conclusions: Antenatal use of MRI as well as serial US improved information regarding tissue resolution and delineation of CCAM. The information from two imaging modalities was complementary. Our literature review confirmed the emerging role of prenatal MRI for postnatal monitoring and management of CCAM.

Rare Case of Adult Congenital Cystic Adenomatoid Malformation Diagnosed with CT Perfusion Imaging and Hystological Sample

We describe the case of a 44-year-old man with congenital cystic adenomatoid malformation. Both computed tomographic (CT) scan findings with perfusion protocol and histological biopsy led to diagnosis. CT scan showed multiple terminal bronchiolar-like uniform cysts smaller than 2 cm and CT perfusion allowed to make a differential diagnosis with an usual interstitial pneumonia. In order to make a definite diagnosis, an open lung biopsy confirmed the CT findings.

Congenital Cystic Adenomatoid Malformation of Lung Mimicking Lung Cancer: A Case Report

We report a case of a 66-year-old woman with congenital cystic adenomatoid malformation(CCAM)that presented as a thin-walled cyst on radiological imaging and mimicked lung cancer.The right pulmonary wedge resection was performed through thoracoscopic surgery.Pathologic results proved CCAM which though,uncommon may be misdiagnosed in adults.

胎儿胸腔内异常强回声的声像图特征在产前诊断隔离肺与先天性肺囊性腺瘤样病变中的价值

目的 探讨胎儿胸腔内异常强回声的声像图特征在产前诊断隔离肺与先天性肺囊性腺瘤样病变(CCAM)中的价值,为鉴别诊断和评估预后提供依据。方法 对2017年1月至2019年11月安徽医科大学第一附属医院产前超声检查中发现的31例胎儿胸腔内异常强回声的声像图特征及随访结果进行回顾性分析。结果 31例胎儿胸腔内异常强回声中,其中18例Ⅲ型CCAM产前超声显示胸腔内高回声边界清晰,内部回声尚均匀,未见明显的囊泡样回声且显示为肺动脉供血;12例隔离肺产前超声显示胸腔内高回声界限清晰,呈叶状或三角形,大小不一,内部回声尚均匀,未见明显的囊泡样回声,多位于左侧胸腔中下部为主动脉供血;1例隔离肺合并CCAM表现为胸腔内见稍强回声囊实性包块,形态规则,边界清晰,稍强回声内可见多个小囊性回声,透声良好,伴纵隔移位,可追踪源自胸主动脉的滋养血管。8例CCAM及6例隔离肺晚孕随访肿块缩小甚至消失,3例终止妊娠。剩余28例胎儿产后均无明显呼吸窘迫,生存率达100%。结论 产前超声依据胸腔内异常高回声的边界、形态、内部回声有无囊泡样结构以及异常高回声血供来源在鉴别诊断胎儿隔离肺与CCAM有重要意义、并可用于持续观察随访,对临床处理及判断预后有重要的指导意义。

1例肺囊性腺瘤样畸形伴反复气胸患儿的护理

总结1例肺囊性腺瘤样畸形以反复气胸为表现患儿的临床护理经验。护理要点包括组建多学科管理团队,落实早期评估,制订并实施标准化氧疗方案;落实基于证据的胸腔引流置管、阶梯式排气与维护,防范低氧血症;针对多次气胸的青少年患儿,基于患儿需求制订实施各项护理及早期康复锻炼方案。经过多学科团队的合作,患儿顺利出院,门诊复查肺复张良好,未再发生气胸。

先天性肺囊性腺瘤样畸形的影像特征及误诊分析

目的探讨先天性肺囊性腺瘤样畸形(CCAM)的影像特征,并分析误诊原因,总结防范误诊措施。方法回顾性分析2020年5月—2023年11月收治经手术和病理检查证实的CCAM 9例的临床资料。结果本组5例主要表现为间断性咳嗽、咳痰、痰中带血、发热及气短等,3例为住院期间常规查体发现肺部异常,1例为常规体检发现肺部异常。术前首诊确诊2例,误诊7例。误诊为支气管源性肺囊肿3例,肺脓肿、囊性支气管扩张伴感染、肺隔离征和先天性大叶性肺气肿各1例。误诊时间8 d~2周。9例CT检查4例为大囊型,CT表现为占据胸腔的较大含气体或液体的囊腔,囊内见弯曲细线样分隔或气液平面;5例为小囊型,CT表现为局限于一侧肺叶多发薄壁小囊,部分呈蜂窝状改变,部分见短小气液平面。9例皆行单操作孔电视胸腔镜肺叶或肺段切除术,病理检查示4例大囊型为StockerⅠ型,5例小囊型为StockerⅡ型。9例出院后随访0.5~1.0年,均预后良好。结论CCAM因病理分型不同而影像学特征各异,虽易误诊,但仍具有一定影像学特征,确诊需病理学检查。

产前超声诊断胎儿肺囊性病

目的探讨超声诊断胎儿肺囊性病,主要是先天性肺囊腺瘤(CCAM)及隔离肺(PS)的临床应用价值。方法对常规产前筛查怀疑先天性肺囊性病的23胎行进一步超声检查,重点观察胎儿肺内病变的回声特征、血供情况,并定期进行超声复查,观察有无纵隔偏移、羊水量增多或腹腔积液等合并症及其他结构异常,随访妊娠结局。结果 23胎于产前诊断为先天性肺囊性病,其中20胎为CCAM(20/23,86.96%),2胎为PS(2/23,8.70%),1胎为CCAM合并PS(1/23,4.35%)。7胎(7/23,30.43%)发生纵隔移位,3胎(3/23,13.04%)出现羊水过多,另有1胎出现腹腔积液(1/23,4.35%)。随访结果表明,9胎(9/23,39.13%)活产,其中5胎病变在产前出现自发性减小甚至消退;其余14胎引产(14/23,60.87%)。结论产前超声对于诊断胎儿肺囊性病有重要临床价值。

产前超声测量肺头比评估胎儿先天性肺囊腺瘤样畸形预后的研究

目的胎儿先天性肺囊腺瘤样畸形(CCAM)的早期明确诊断及产前评估对产前咨询及临床处理具有重要意义,本文通过产前超声测量CCAM胎儿肺头比(CVR),探讨CVR在CCAM胎儿预后评估中的应用价值。资料与方法回顾性分析产前超声诊断为CCAM的84例胎儿的影像与临床资料,测量胎儿CVR,分为CVR<1.6组及CVR≥1.6组,比较两组胎儿水肿发生率、产后呼吸系统症状发生率及围生儿存活率。结果 84例胎儿中,产前超声诊断为CCAM I型12例(14.3%),CCAM II型48例(57.1%),CCAM III型24例(28.6%);78例(92.9%)活产,4例(4.8%)引产,2例(2.4%)死胎;CVR<1.6组68例,2例(2.9%)胎儿水肿,3例(4.4%)产后出现呼吸困难等呼吸系统症状;CVR≥1.6组16例,12例(75.0%)胎儿水肿,9例(81.8%)产后出现呼吸系统症状。两组胎儿水肿发生率、产后呼吸系统症状发生率及围生儿存活率比较,差异有统计学意义(P<0.001)。结论 CVR≥1.6的CCAM胎儿宫内水肿发生率及产后呼吸系统症状发生率均增高,CVR是评价CCAM胎儿预后的有效指标。

胎儿先天性肺腺瘤样囊肿的超声诊断与临床预后

目的探讨胎儿先天性肺腺瘤样囊肿(CCAM)的超声表现,预测CCAM胎儿的预后与转归情况。方法应用二维超声于胎儿胸腔多切面扫查,观察肺部肿块发生的位置、形态、大小;随访CCAM的大小、声像图变化及是否出现合并症。结果产前诊断19胎CCAM,13胎引产后病理证实,1胎出生后死亡,5胎于36周前肺部肿块消失。肺部病灶声像图变化特点:Ⅰ型囊性肿块逐渐缩小,直至消失;Ⅱ型囊性部分渐渐变小,数目减少,强回声部分逐渐变浅变淡直至与正常肺组织回声相同;Ⅲ型实质性肿块由强回声逐渐变浅变淡直至与正常肺组织回声近似,或由Ⅲ型逐渐演变为Ⅱ型然后随孕周增加恢复为正常肺组织回声。随访中10胎(10/19,52.63%)CCAM随孕周增加而增大,出现心脏、纵隔移位、胸腹腔积液和(或)胎儿水肿等并发症。结论超声检查是诊断胎儿CCAM的可靠方法;可从超声观察角度预测CCAM胎儿的预后与转归;胎儿纵隔、心脏移位、胸腔积液、胎儿水肿或合并其他发育异常是终止妊娠的指征。

胸腔镜辅助下婴儿先天性肺囊腺瘤肺切除术的麻醉管理

目的：探讨胸腔镜辅助下婴儿先天性肺囊腺瘤（CCAM）肺切除术单肺通气（OLV）麻醉的可行性和安全性。方法：43例CCAM患儿，静脉快速诱导后，插入单腔气管导管。注入4～6 mmHg压力的CO2人工气胸，使患侧肺组织塌陷造成健侧OLV。术中监测SpO2、ECG、MAP、PETCO2、T、PaO2、PaCO2、出血量及尿量；记录诱导前后5 min、OLV 10、60 min、术毕5 min时SpO2、PaO2、HR、MAP、PETCO2、PaCO2和T值。结果：与诱导后5 min比较，OLV 10、60 min时患儿PaO2、HR、MAP下降，PETCO2、PaCO2升高（P＜0．05﹚。与OLV 10 min比较，OLV 60 min时患儿PaO2、PETCO2升高（P ＜0．05﹚。结论：采用压力控制通气，合理呼吸管理及麻醉用药，胸腔镜辅助下OLV行婴儿CCAM肺切除术是安全可行的。

胎儿肺发育异常产前超声诊断及产后病理对照研究

目的探讨产前超声诊断胎儿肺发育异常的应用价值。资料与方法对117例胎儿肺发育异常产前超声诊断结果与出生手术后或引产后肺发育异常病理解剖结果进行回顾性分析。结果 117例肺发育异常胎儿中,先天性肺囊腺瘤畸形56例,其中I型9例,II型29例,III型18例;隔离肺48例;先天性肺囊腺瘤畸形合并隔离肺6例;先天性肺叶性肺气肿6例;先天性支气管囊肿1例。产前超声诊断与病理解剖符合106例(90.6%),产前超声诊断与病理解剖结果不一致11例。结论产前超声诊断胎儿肺发育异常具有较高的准确性,但对部分畸形的精确诊断有一定的困难。

小儿先天性肺囊性腺瘤样畸形的影像与病理对照分析

目的评价小儿先天性肺囊性腺瘤样畸形的影像学表现与病理分型的关系。方法回顾10例小儿先天性肺囊性腺瘤样畸形的X线及CT表现,根据CT最大囊直径分为大囊型(囊径>2 cm)、小囊型(囊径≤2 cm)及实性型,并与Stocker分型进行对照。结果小儿先天性肺囊性腺瘤样畸形的X线平片表现为气胸样改变2例、多囊状改变6例、肺炎样改变2例;CT表现为大囊型5例、小囊型4例、实性型1例。所有大囊型均为StockerⅠ型,小囊型中1例为StockerⅠ型,3例为StockerⅡ型,1例实性型为StockerⅡ型。结论 CT检查是诊断先天性囊性肺腺瘤样畸形的可靠方法,根据CT表现可推测其病理分型。

胎儿先天性肺囊腺瘤样畸形的MRI诊断

目的:探讨MRI在胎儿先天性肺囊腺瘤样畸形(CCAM)中的诊断价值。方法:收集我院2013年3月-2015年12月53例产前MRI诊断为CCAM的胎儿,所有胎儿出生后均行胸部低剂量CT增强检查及手术治疗,以病理结果为金标准,对胎儿CCAM的MRI特点进行回顾性分析;其中8例胎儿产前行两次MRI检查,分析病灶变化的MRI特征。结果:53例胎儿中,病理证实CCAM 52例,先天性肺气肿(CLE)1例。52例CCAM中,大囊型28例,MRI表现为边界清晰的多囊性长T_2病灶,囊内分隔呈短T_2信号,囊泡直径≥5 mm;微囊型24例,MRI表现为边界清晰的实性或微囊状长T_2病灶,囊泡直径<5 mm。8例CCAM行2次MRI检查,其中5例病灶大小及信号无明显变化;3例病灶变小,信号降低。52例CCAM中,心脏右移17例,心脏左移23例。52例胎儿均未合并胎儿水肿,合并羊水过多者3例。结论:MRI对胎儿CCAM的诊断价值较高,不仅能够清晰显示病灶部位、类型及病灶变化情况,还能够观察病灶周围器官受压程度,为胎儿临床预后提供准确信息。

儿童先天性肺囊性腺瘤样畸形Ⅲ型的CT表现及鉴别诊断

目的探讨CT在儿童先天性肺囊性腺瘤样畸形(CCAM)Ⅲ型诊断与鉴别诊断中的应用价值。方法回顾性分析经手术切除、病理证实为CCAMⅢ型5例患儿的胸部CT影像学特点,并与病理结果进行对照,术前5例均行胸部CT平扫及增强扫描。结果 5例先天性肺囊性腺瘤样畸形的CT表现为囊实型3例,实质型2例,均为单发;右肺受累4例,左肺受累1例;病理检查为异常增生的管腔或腺瘤样结构。结论 CT检查能够清晰的显示CCAMⅢ型的病变、范围及周围关系,对其临床治疗及鉴别诊断起到积极作用,是诊断CCAMⅢ型的重要手段。

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.

胎儿肺囊性腺瘤样病变的超声诊断(附33例报告并文献复习)

目的探讨胎儿肺囊性腺瘤样病变（CCAM）的超声诊断价值及其预后情况。方法对我院2013年1月-2014年12月在产前超声检查中发现的33例胎儿先天性肺囊性腺瘤样病变的声像图征象及其随访结果进行回顾性分析。结果本组33例胎儿CCAM,左侧18例,右侧15例;Ⅰ型3例,Ⅱ型18例,Ⅲ型12例。随访过程中18例引产,1例出生后CT证实为肺隔离症,12例肿块逐渐减小直至消失,另2例失访。结论超声检查是诊断胎儿CCAM的重要手段,并可协助临床判断CCAM胎儿的预后与转归。

MRI诊断胎儿先天性肺囊腺瘤样畸形及肺隔离症的应用价值

目的探讨MRI在诊断胎儿先天性肺囊腺瘤样畸形及肺隔离症的应用价值。方法收集我院2010年2月~2017年9月产前MRI诊断为先天性肺囊腺瘤样畸形(CCAM)的27例胎儿及诊断为支气管肺隔离症(BPS)的11例胎儿,出生后均行胸部低剂量CT增强复查及手术治疗,以病理结果为金标准,并与产前MRI检查结果进行对照,对胎儿CCAM及BPS的MRI特点进行回顾性分析。结果38例胎儿中,术后病理诊断为BPS 12例(肺内型9例、肺外型3例),CCAM 26例(大囊型16、微囊型10例),其中BPS产前MRI诊断正确10例,CCAM产前MRI诊断正确25例;其中1例微囊型的CCAM,产前MRI诊断为BPS,其中2例BPS产前MRI诊断为微囊型CCAM,所有病例均未合并胎儿水肿,7例出现心脏左偏(CCAM5例、BPS2例),4例心脏右偏(CCAM2例、BPS2例)。结论MRI对大囊型CCAM及显示供血动脉的BPS能够明确诊断,根据供血血管走行对于BPS和CCAM鉴别诊断有一定提示性,但是对于未显示供血血管的微囊型CCAM和BPS的鉴别诊断比较困难。