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Objective Meta-analysis was used to evaluate the diagnostic value of a CVR cut-off value of 1.6 for fetal hydrops due to congenital lung malformation (CLM). Methods A systematic search of PubMed, Embase, Web of Science, CNKI, VIP, and Wanfang published before 7/30/2021 for the value of a congenital pulmonary airway malformation volume ratio (CVR) cut-off value of 1.6 for the diagnosis of fetal hydrops. According to the inclusion and exclusion criteria, the literature that met the requirements were obtained. A total of 75 articles were retrieved, and 12 articles were included for further analysis. The quality of these studies was evaluated according to the Quality Assessment for Studies of Diagnostic Accuracy tool (QUADAS-2) criteria. The Q test and heterogeneity I2 were used to evaluate the heterogeneity due to non-threshold effects, and Stata 15.0 was used for statistical analysis to evaluate the diagnostic value of the CVR cutoff value of 1.6 for fetal hydrops due to CLM. Results A total of 12 studies were included. The QUADAS-2 indicated that the risk of bias was relatively low, and the clinical applicability was relatively high. Statistical analysis was performed on included studies using a random effect model. Meta-analysis showed that the pooled sensitivity, specificity, diagnostic ratio and summary receiver operating characteristic (SROC) for the diagnosis of fetal hydrops by CVR were 0.86 (95% CI, 0.72–0.93; I2 = 59.84), 0.90 (95% CI, 0.88–0.93; I2 = 31.94), 58 (95% CI, 22–149; I2 = 100%), 0.93 (95% CI, 0.91–0.95). Conclusions The sensitivity and specificity of CVR cut-off value 1.6 for the diagnosis of CLM-induced fetal hydrops were high, no publication bias was observed, and the CVR cut-off value 1.6 is meaningful for the early diagnosis prediction of CLM-induced fetal hydrops.

Background Congenital pulmonary airway malformation (CPAM) with partial anomalous pulmonary venous connection presenting as episodic retrosternal chest pain on exertion in an adult has not been described. Case presentation A 21-year-old female, non-smoker, presented with a 4-year history of sharp, episodic, retrosternal chest pains brought on with exercise. A contrast-enhanced computed tomography (CT) scan showed a grossly overinflated left lower lobe with partial anomalous pulmonary venous drainage into the left hemi-azygos vein. Lobectomy, the recommended treatment of choice, carried out thoracoscopically, was curative with satisfactory mid-term results. Histology confirmed type-II congenital pulmonary airway malformation. Conclusions CPAM can present in young adults with unusual symptoms of chest pain on exertion. When suspected a contrast-enhanced CT scan is the gold standard for establishing the diagnosis. An anatomical lung resection is curative with satisfactory medium term results.

Purpose The purpose of this study is to evaluate the potential advantages of thoracoscopic versus open resection for symptomatic congenital pulmonary airway malformation (CPAM) in neonates. Methods A retrospective review of the medical records of neonates (age ≤ 28 days) who underwent surgery for symptomatic CPAM from 2010 to 2020. Results Of the 24 patients, 14 patients underwent thoracoscopic resection and 10 patients underwent open resection. 4 patients with CPAM located in the upper or middle lobes underwent lobectomy, and 20 underwent lung-preserving wedge resection in the lower lobe. Between the two groups, there were no statistically significant differences in related preoperative variables, including gestational age at birth, body weight, head circumference, lesion size, cystic adenomatoid malformation volume ratio (CVR), and age at operation (P > .05). The differences in intraoperative variables were statistically significant. The length of the surgical incision was significantly shorter in thoracoscopic resection group than in open resection group (1.4 cm [1.3–1.8] vs. 6.0 cm [5.0–8.0], P = .000), along with significantly less operative blood loss (3 ml [1–6] vs. 5 ml [2–10], P = .030) but significantly longer operation time (159 min [100–220] vs. 110 min [70–170], P = .003). Regarding postoperative variables, ventilator days, duration of chest tube use and length of hospital stay were not statistically significant (P > .05). Conclusion Both thoracoscopic and open resection for symptomatic CPAM achieve good clinical outcomes, even in neonates. Thoracoscopic resection has minimal aesthetic effects and does not increase the risk of surgical or postoperative complications. Lung-preserving resection may be feasible for neonatal CPAM surgery.

For the approximately three quarters of patients with a prenatal diagnosis of congenital pulmonary airway malformation (CPAM) who are asymptomatic at birth, the chance of eventually developing symptoms is unknown. We sought to explore the natural history of asymptomatic CPAM. We searched EMBASE, MEDLINE, and the first 50 results from Google Scholar. Studies describing the natural history of prenatally diagnosed, initially asymptomatic CPAM were included. For asymptomatic patients initially managed non-surgically, we tabulated the proportion who went on to develop symptoms as well as the median age at symptom development. We included data from 19 retrospective studies on 353 patients. Of the 128 patients managed expectantly, 31 (24.2%) developed symptoms requiring surgical intervention. The median age at symptom development was 7.5 months (range 15 days–5 years). The risk for developing respiratory symptoms exists with originally asymptomatic CPAM patients, but the exact risk is difficult to predict. Parents may be given the value of approximately 1 in 4 as an estimate of the proportion of asymptomatic CPAM patients who go on to develop symptoms, which will help them make an informed decision regarding the option of elective surgery.

Variation in management characterizes treatment of infants with a congenital pulmonary airway malformation (CPAM). This review addresses six clinically applicable questions using available evidence to provide recommendations for the treatment of these patients. Questions regarding the management of a pediatric patient with a CPAM were generated. English language articles published between 1960 and 2014 were compiled after searching Medline and OvidSP. The articles were divided by subject area and by the question asked, then reviewed and included if they specifically addressed the proposed question. 1040 articles were identified on initial search. After screening abstracts per eligibility criteria, 130 articles were used to answer the proposed questions. Based on the available literature, resection of an asymptomatic CPAM is controversial, and when performed is usually completed within the first six months of life. Lobectomy remains the standard resection method for CPAM, and can be performed thoracoscopically or via thoracotomy. There is no consensus regarding a monitoring protocol for observing asymptomatic lesions, although at least one chest computerized tomogram (CT) should be performed postnatally for lesion characterization. An antenatally identified CPAM can be evaluated with MRI if fetal intervention is being considered, but is not required for the fetus with a lesion not at risk for hydrops. Prenatal consultation should be offered for infants with CPAM and encouraged for those infants in whom characteristics indicate risk of hydrops. Very few articles provided definitive recommendations for care of the patient with a CPAM and none reported Level I or II evidence. Based on available information, CPAMs are usually resected early in life if at all. A prenatally diagnosed congenital lung lesion should be evaluated postnatally with CT, and prenatal counseling should be undertaken in patients at risk for hydrops.

Video-assisted thoracic surgery (VATS) pulmonary resection in children is a technically demanding procedure that requires a relatively long learning period. This study aimed to evaluate the serial improvement of quality metrics according to case volume experience in pediatric VATS pulmonary resection of congenital lung malformation (CLM). Methods VATS anatomical resection in CLM was attempted in 200 consecutive patients. The learning curve for the operative time was modeled by cumulative sum analysis. Quality metrics were used to measure technical achievement and efficiency outcomes. Results The median operative time was 95 min. The median length of hospital stay and chest tube indwelling time was 4 and 2 days, respectively. The improvement of operation time was observed persistently until 200 cases. However, two cut-off points, the 50th case and 110th case, were identified in the learning curve for operative time, and the 110th case was the turning point for stable outcomes with short operation time. Significant reduction of length of hospital stay and chest tube indwelling time was observed after 50 cases (p = .002 and p = .021, respectively). The complication rate decreased but continued at a low rate for entire study period and the interval decrease was not statistically significant. Conversion rate decreased significantly (p = .001), and technically challenging procedures were performed more frequently in later cases. Conclusions Improvements of quality metrics in operation time, conversion rate, length of hospital stay, and chest tube indwelling time were observed in proportion to case volume. Minimum experience of 50 is necessary for stable outcomes of pediatric VATS pulmonary resection.

Therapeutic fetal surgical procedures are predicated upon the ability to make an accurate fetal diagnosis. The earliest open fetal surgical procedures were introduced in the 1960s to treat Rh isoimmunisation. They were introduced when it became possible to predict impending fetal demise. Open procedures were abandoned when percutaneous approaches proved superior. The introduction of fetal ultrasound allowed the diagnosis of other congenital anomalies, some being amenable to fetal interventions. Open fetal surgical procedures were initially utilised, with significant maternal morbidity. For some anomalies, percutaneous approaches became favoured. In general, all of these procedures involved significant risks to the mother, to save a baby that was likely to die before or shortly after birth without fetal intervention. Fetal repair for myelomeningocele was a "sea change" in approach. The same maternal risks were taken to improve the quality of life of the affected fetus, not save its life. The completion of the "MOMs Trial" has occasioned a "tsunami" of centres in North America applying this approach. Others are attempting percutaneous repairs, with mixed results. This paper reviews the history of fetal surgery, focusing on the themes of the tension between accurate diagnosis and prognosis and open versus "minimally invasive" approaches.

Thoracoscopic pulmonary lobectomy (TPL) techniques in infants and children are presented practically with concise descriptions and numerous illustrations. TPL is the treatment of choice for congenital pulmonary airway malformation and intralobar pulmonary sequestration, both now commonly diagnosed prenatally. Timing of surgery is somewhat controversial in asymptomatic cases with small isolated lesions. Incomplete fissures and history of chest infections are most problematic. Thorough understanding of anatomic relations preoperatively is vital for successful outcome and thin-slice computed tomography with 3D reconstruction of vessels is valuable. Judicious placement of trocars and switching instruments between trocars improves visualization and safety. Specific techniques for all commonly performed TPL are included.

Congenital pulmonary airway malformation (CPAM) is the most common congenital lesion detected in the neonatal lung, which may lead to respiratory distress, infection, and pneumothorax. CPAM is thought to result from abnormal branching morphogenesis during fetal lung development, arising from different locations within the developing respiratory tract. However, the pathogenic mechanisms are unknown, and previous studies have focused on abnormalities in airway epithelial cells. We have analyzed 13 excised lung specimens from infants (age

Thoracoscopic lobectomy (TL) and open lobectomy (OL) were compared for treating congenital pulmonary airway malformation (CPAM) with preoperative complications, specifically pneumonia/abscess formation (PA). The medical records of 46 CPAM patients treated by lobectomy at our institution from 1990 to 2014 were reviewed retrospectively. Four groups, TL for patients without PA (n = 17; TL−), TL for patients with PA (n = 8; TL+), OL for patients without PA (n = 16; OL−), and OL for patients with PA (n = 5; OL+) were compared for operative time, intra/postoperative complications, blood loss, duration of chest tube insertion, postoperative analgesia, pre: postoperative white blood cell (WBC) ratio, and duration of hospitalization. Operative time for TL+ was longest, but not statistically significant. Incidences of intra/postoperative complications were similar in all groups. Blood loss was significantly less for TL+ versus OL+ (p

Congenital pulmonary airway malformation (CPAM) is classified into pathologically different types. These types are sometimes distinguishable by fetal lung MRI and are usually observed as higher-signal lesions on T2-weighted images than normal lung. We describe a case of unusual CPAM resembling neoplasms, with a lower signal than is found in normal lung. Histopathology showed a large number of mucogenic cells but found no evidence that could explain this feature on fetal MRI. An unusual low-signal mass associated with a pulmonary cyst in fetal lung on MRI may suggest an unusual type 1 CPAM.

Accurate division and sealing of lung parenchyma particularly in cases of total or near total incomplete fissure are crucial for preventing air leakage following thoracoscopic pulmonary lobectomy (TPL). However, conventional endoscopic stapling devices cannot be used during TPL in small children because of limited space. Consequently, Ligasure (LS) and Enseal (ES) devices are being used instead. We are the first to compare LS and ES for efficacy and efficiency during TPL. Of 26 TPL (6 upper, 3 middle, and 17 lower) performed for congenital adenomatoid malformation (n = 16) and sequestration (n = 10), incomplete fissure was found in 14. TPL (LS = 11; ES = 15) was performed conventionally in the lateral decubitus position with single lung ventilation using four 5 mm trocars. All cases had a chest tube inserted intraoperatively that was left in situ. Patient demographics, location of pathology, incidence of incomplete fissure, mean age/weight at TPL, mean blood loss, and mean operative time were all similar. However, duration of chest tube insertion was significantly shorter in ES because there was less postoperative air leakage (1.3 vs. 3.9 days; p < 0.05). ES would appear to seal lung parenchyma more effectively during TPL based on the shorter duration of chest tube insertion.

Congenital cystic adenomatoid malformations (CCAMs) are increasingly diagnosed in recent years due to the routine usage of antenatal ultrasound. The aim of this study was to present the natural course and outcome of antenatally diagnosed cystic lung diseases, which were prospectively studied. Between the period June 2004 and June 2010, 25 fetuses with suspected CCAMs were seen in a single tertiary maternal fetal unit. One child was excluded as the fetal MRI suggested congenital diaphragmatic hernia. Data were prospectively entered into a database including antenatal and postnatal findings which were then analyzed. Mean gestational age at the time of diagnosis was 20.8 weeks (range 17–29). Fetal interventions included amnioreduction (n = 2) and fetal thoracocentesis (n = 3) in one child. The mean gestational age at delivery was 38.5 weeks (range 31.6–40.6). None of the mothers underwent termination of pregnancy or spontaneous abortion. All patients underwent postnatal radiological assessment. Of the 24 cases, 5 children were symptomatic in the immediate neonatal period and 19 were asymptomatic. Anatomical surgical resection was undertaken in all symptomatic cases. There was one death in this group due to severe pre-existing pulmonary hypoplasia. Among the 19 asymptomatic cases, 5 children were managed conservatively as the lesions were not significant and the remaining 14 (73%) underwent surgical resection. The mean age at surgery was 11.1 months (range 3.3–34 months). Postnatal pathology confirmed CCAM in all operated cases, except one who had pulmonary sequestration. Antenatally diagnosed CCAMs have an excellent prognosis except in children with a large lesion and associated with lung hypoplasia. Postnatal investigations are required in all cases to confirm the diagnosis. Symptomatic CCAMs require immediate surgery in the neonatal period with a good outcome. Asymptomatic CCAMs can be safely operated between 9 and 12 months of age.

There are still few reports on thoracoscopic lobectomy in neonates. The rate of prenatally diagnosed congenital cystic lung diseases is increasing, and such diseases appear to be a good indication for thoracoscopic lobectomy. We performed a prospective trial of thoracoscopic lobectomy for all congenital cystic lung diseases in children. Complete thoracoscopic lobectomy was performed by employing carbon dioxide insufflation without single lung ventilation. The results were analyzed, comparing patients with pre- and postnatal diagnoses. Seven patients were prenatally diagnosed with congenital pulmonary airway malformations (CPAM) between January 2008 and August 2009, and all but one underwent surgery during the neonatal period (prenatal group). Nine patients underwent surgery after infection subsided due to lesions (7 CPAM, 2 intrapulmonary sequestration) postnatally identified by infection presence (median 2 years; range 15 days to 14 years) (postnatal group). The prenatal group showed a significantly lower rate of adhesions, shorter operation time, and smaller volume of blood loss. There were two conversions in the postnatal group. There were two postoperative complications (persistent air leak and phrenic nerve paralysis) in the prenatal group. The esthetic results were good. Thoracoscopic lobectomy for congenital cystic lung diseases in neonates was practicable, with good esthetic results. Dissection was easier with significantly less blood loss in patients without adhesions, even in neonates. However, this procedure necessitated highly skilled endoscopic maneuvers within a small working space.

Fetal congenital cystic adenomatoid malformation (CCAM) is a rare lung abnormality with a highly variable prognosis depended on the presence of fetal hydrops and the size of the cysts. In case of fetal hydrops the prognosis is fatal without intervention.Case report and literature review.We report on the ultrasound and pathological findings of a hydropic fetus due to a CCAM Type II at 22 weeks of gestation.Congenital cystic adenomatoid malformation is a rare fetal lung disease with an excellent prognosis in the absence of fetal hydrops. CCAM associated with fetal hydrops carries a grave prognosis but survival rates of 70% can be achieved by thoraco-amniotic drainage in those with macrocystic lesions.

Intralobar sequestration (ILS) associated with congenital cystic adenomatoid malformation (CCAM) is a well-known entity. This hybrid form has many considerations for an appropriate management. This report aims to analyze pitfalls in the diagnosis, treatment, and thoracoscopic procedure pertaining to this association.Between May 2004 and September 2007, 23 fetal lung lesions were diagnosed prenatally and managed at the authors' institution. Seven of these lesions were the hybrid form of ILS-CCAM. The records for these lesions were reviewed retrospectively.In all seven fetuses, prenatal ultrasound diagnosed congenital lung abnormalities. In three cases, fetal magnetic resonance imaging (MRI) demonstrated a macrocystic lesion supplied by an aberrant vessel. In all cases, the intrauterine evolution remained uncomplicated. All the newborns were asymptomatic at birth. Chest computed tomography (CT) performed at the age of 1 month showed the aberrant vessel in five cases. A regression of the lesion was noted in one patient and that of the aberrant vessel in another patient. Elective thoracoscopic lobectomy of the affected lobe was performed for six patients. The average age at the time of intervention was 5 months. At this writing, one patient is under observation due to resolution of the lesion. The operating time ranged from 80 to 160 min. One conversion to thoracotomy was required. All surgically treated patients had an uneventful hospital course. Histopathologic examination confirmed the association of intralobar sequestration with Stocker type 2 CCAM in five cases and with CCAM type 1 in one case.Prenatal diagnosis of the ILS-CCAM association was possible, as was resolution of one or both components of this lesion. Preoperative identification of the aberrant vessel is important for prevention of operative morbidity. This should be controlled before the lobectomy is begun.

The pathogenesis of congenital cystic adenomatoid malformation (CCAM) is unknown and its natural history is unpredictable. Fatty acid binding protein-7 (FABP-7) has been previously described in brain and breast development, but never before in the lung. We investigate gene expression in CCAM, and hypothesize that CCAM results from an aberration in the signaling pathway during lung development. Under IRB approval, tissue specimens of fetal CCAM, fetal control, postnatal CCAM, and postnatal control were examined and microarray analysis was performed. Candidate differentially expressed genes were selected with log-odds ratio (B) >0 and false discovery rate

Congenital cystic lung disease comprises a broad spectrum of rare but clinically significant developmental abnormalities, including congenital pulmonary adenomatoid malformations, bronchopulmonary sequestrations, bronchogenic cysts, and congenital lobar emphysema that result from perturbations in lung and airway embryogenesis. As congenital lung lesions are now more commonly recognized antenatally, mothers require accurate prenatal counseling and appropriate perinatal management. In light of long-term complications of infection and malignancy, there is growing consensus that infants with asymptomatic lesions should undergo elective excision of congenital pulmonary adenomatoid malformation (CPAM) or bronchopulmonary sequestration (BPS). This review will focus on advancements and current practice in the diagnosis and management of CPAM and BPS, identifying aspects of the literature that are confusing or controversial. Although our knowledge and pre- and postnatal management of lung lesions will continue to evolve and improve, there is a compelling need for a unified clinical and pathological classification system that creates a common platform for discussion, clinical management, and research.

Computed tomography (CT) of an 18-year-old man with a history of repeated episodes of pneumonia demonstrated consolidation containing multiple cysts in the right lower lobe. Additional CT angiography and thin-section CT of the lesion revealed neither a supplying systemic artery nor an abnormal bronchus. Lobectomy was performed, and histological examination showed many cysts resembling enlarged bronchioles, diagnosed as congenital cystic adenomatoid malformation.

The allure of fetal surgery is the possibility of interrupting in utero progression of an otherwise treatable condition. In spite of advances in prenatal diagnosis and refinements in surgical techniques, this field has not yet got off the ground because the risks to the mother and fetus, during and after the procedure far outweigh the benefits, and the infrastructure required to support such activity is prohibitively expensive. The various surgical conditions in which fetal surgery has been attempted and the present status of this specialty are discussed.

Bronchopulmonary foregut malformations (BPFM) are a heterogeneous group of pulmonary developmental anomalies that present at varying ages and with overlapping symptoms, signs and radiology. This article discusses the embryology of these lesions with reference to possible common origins and the link between aetiology and radiological appearance. The radiology of each lesion, both antenatally and postnatally, is described and illustrated. A number of quandaries exist in the prediction of prognosis and subsequent treatment of BPFM. We discuss the radiological features that may help to elucidate an individual prognosis and aid in the planning of treatment. The treatment options available for BPFM are briefly discussed. Finally, the link between BPFM, in particular cystic adenomatoid malformations and malignancy, is discussed. We aim to provide a comprehensive overview of the embryology, radiology, prognosis and treatment highlighting contentious issues of BPFM.

Most prenatally diagnosed lung lesions can be managed successfully during the neonatal period. Prenatal imaging and experimental models have provided a comprehensive understanding of the pathophysiology, natural history, and prognosis of intrathoracic and mediastinal lesions. Clinical experience has demonstrated that progression to nonimmune hydrops fetalis and pulmonary hypoplasia is a harbinger of fetal or neonatal demise. Advances in fetal anesthesia, tocolysis, and surgical techniques have made fetal surgery a viable in utero option to ameliorate life-threatening masses. Congenital cystic adenomatoid malformation, bronchopulmonary sequestration, and congenital hydrothorax are the most common abnormalities amenable to surgical intervention. The natural history, evaluation, and treatment of intrathoracic and mediastinal lesions are discussed.

An intraabdominal extrathoracic pulmonary sequestration (IEPS) was detected by prenatal ultrasound in a fetus of 19 weeks' gestation. The well-defined echogenic mass, including multiple cystic areas, was located in the left suprarenal region. Knowledge of the characteristic ultrasound appearance helped to differentiate between neuroblastoma and IEPS before surgical treatment. Histologic examination showed an association between IEPS and features of cystic adenomatoid malformation type 2.

We examined the clinical significance of the lung-thorax transverse-area ratio (L/T) in fetuses with cystic lung disease (CLD). The transverse section of the thorax was analyzed at the level of the 4-chamber view of the heart. L/T was calculated as the bilateral normal lung area divided by the thoracic area. Within a 9-year period, ultrasonography was performed in 15 fetuses with CLD, 13 of which were histologically diagnosed: congenital cystic adenomatoid malformation of the lung (CAM; n = 8), pulmonary sequestration (n = 4), and bronchial atresia (n = 1). Although the initial L/T measurement (29.6 +/- 4.2 weeks) showed significantly lower values than in the controls, the final measurement (36.4 +/- 1. 6 weeks) indicated that the L/T in CLD excluding CAM was significantly higher than that in CAM. Six patients with a final L/T of less than 0.21 required mechanical ventilation immediately after birth. Two of these patients died, and the autopsy findings indicated pulmonary hypoplasia. In these cases, a significant correlation was observed between the maximal value of L/T and preductal alveolar-arterial oxygen difference (A-aDO(2)), although the final L/T was not correlated to A-aDO(2). These results indicate that serial measurements of L/T may be useful in the diagnosis of CLD and may help to predict postnatal respiratory conditions.

Currently, congenital cystic adenomatoid malformation of the lung (CCAM) is often diagnosed antenatally by ultrasound, allowing prompt and appropriate medical and surgical management after birth. The authors report 21 cases of CCAM admitted from 1988 to 1997 to a neonatal intensive care unit and treated by high-frequency oscillation (HFO) and early surgery. Six infants developed respiratory distress, of whom 4 required ventilation by HFO. HFO was also the mode of ventilation used in all cases except 1 during the perioperative period. There was no death from respiratory failure. The authors emphasize the usefulness of antenatal diagnosis, the efficiency of HFO in cases with severe respiratory failure, and well-tolerated early surgery.

Background. The purpose of this study was to attempt to improve upon conventional coronal computed tomographic (CT) images of neonatal chest which to date have been made by reformatting thinly spaced axial images. Materials and methods. Nine neonates were studied by direct coronal CT scans with the patients' long axis 90 degrees to the scanning table. They were studied to further define their thoracic abnormalities detected on plain film. Spiral CT and cine scan (Imatron) were utilized. Results. Congenital lung lesions such as congenital cystic adenomatoid malformation could not be diagnosed but their anatomical location could be accurately depicted, enabling easier surgical planning. The arterial supply to bronchopulmonary sequestrations was also identifiable. Tracheobronchial abnormalities such as tracheobronchus and bronchial atresia were also identifiable. Causing of air trapping, both intrinsic such as an atretic bronchus and extrinsic such as vascular compression were readily demonstrated. Conclusion. With neither special devices nor paraphernalia, the described method of direct coronal CT scans were both feasible and provided significant information. This technique allows for improved assessment of the tracheobronchial tree and more accurate detection, localization, and characteristics of lesions adjacent to the diaphragm.

Bronchopulmonary malformations associated with esophageal atresia (EA) and tracheoesophageal fistula (TEF) are extremely rare. The authors describe a case of type II congenital cystic adenomatoid malformation (CCAM) of the right lower lobe associated with EA and TEF (Vogt-Gross type C) in a full-term female infant. The CCAM presented as an incidental radiologic finding, and a contralateral tension pneumothorax developed shortly after surgical repair of the EA. Early recognition of this rare association is essential for correct operative management.

We report the first case in the literature of thoracoscopic bronchial reimplantation in 5 month-old boy. The child was born with congenital cystic adenomatoid malformation of the right lower lobe associated with lower intralobar pulmonary sequestration diagnosed prenatally. An iatrogenic middle lobar bronchus injury was detected per-operatively during thoracoscopic lobectomy. Management and follow-up were exposed.

Congenital malformations of the lung are rare and vary widely in their presentation and severity. The most common manifestation of the congenital cystic disease of the lung at newborn and early infancy is respiratory distress. Later on in life, cysts usually lose this compressive character and may remain asymptomatic until infection occurs, while producing cough, dyspnea, and thoracic pain. The purpose of this study is to review authors institutional experience of congenital cystic lung disease, with specific reference to diagnosis, treatment, as well as outcome, furthermore, to present some cases with unusual clinical manifestations.

Fetal therapy is a logical extension of fetal diagnosis. Fetal surgery has been performed in humans for the past 10 years. During this time, technical obstacles have been overcome and the natural history of many fetal disorders has been defined. A select group of disorders amenable to potential improvement by fetal treatment has been identified including fetal urinary tract obstruction, fetal diaphragmatic hernia, fetal congenital cystic adenomatoid malformation and fetal sacrococcygeal teratoma. The fetal surgical experience with each of these lesions is reviewed and the maternal risk of fetal surgery is discussed.

A two-month-old girl was operated for a left-sided eventration of the diaphragm. At surgery, a mass representing an extralobar pulmonary sequestration (EPS) was found inferior to the left lobe of the lung and was excised. Histopathological examination of the resected specimen showed presence of a type II congenital cystic adenomatoid malformation (CCAM) within it. A review of the relevant literature is presented.

We report a case of a 29 year old male who at age 25, developed a pulmonary abscess in the left lower lobe. A left lower lobectomy was performed following two recurrences on antibiotic treatment. The result of histopathological examination confirmed congenital cystic adenomatoid malformation (CCAM), a congenital disease characterized by multiple cysts resulting from adenomatous hyperplasia of the bronchial epithelia. In many cases, respiratory distress occurs during the neonatal period, and in about 80-85% of patients, CCAM is diagnosed before the age of two years due to respiratory infection. CCAM which was diagnosed in adulthood is very rare.

Prenatal detection and serial sonographic study of fetuses with congenital cystic adenomatoid malformation (CCAM) now makes it possible to define the natural history of this lesion, determine the pathophysiologic features that affect clinical outcome, and formulate management based on prognosis. In a series of 22 prenatally diagnosed cases, the overall prognosis depended on the effects of the lung mass and the consequent physiologic derangement: A large mass may cause mediastinal shift, hypoplasia of normal lung tissue, polyhydramnios, and cardiovascular compromise leading to fetal hydrops. Two types of CCAM were distinguished by gross anatomy, ultrasonographic findings, and prognosis. Microcystic lesions (cysts5 mm; solid appearance) are often associated with fetal hydrops and have a poor prognosis. Macrocystic lesions (single or multiple cysts5 mm) are not usually associated with hydrops and have a more favorable prognosis. Four initially large lesions partially disappeared on serial prenatal sonography, suggesting that improvement can occasionally occur during fetal life. The finding that fetuses with hydrops are at high risk for fetal or neonatal demise led to fetal surgical resection of the massively enlarged pulmonary lobe (fetal lobectomy) in two cases. Fetuses with CCAM but without hydrops have a good chance for survival with maternal transport, planned delivery, and immediate neonatal resuscitation and surgery.

A 3-month-old Chinese girl with type I cystic adenomatoid malformation involving both lobes of her left lung was successfully operated upon using the video-assisted thoracoscopic surgical (VATS) approach. The conventional endoscopic stapling device was used for resection without complications.

The differential diagnosis of echogenic areas in the fetal chest include congenital diaphragmatic hernia (CDH), cystic adenomatoid malformation (CAM), sequestrated lung and tracheal or bronchial atresia. The purpose of this study was to evaluate the accuracy of prenatal diagnosis and document outcome in fetuses with echogenic chest lesions. Seventeen fetuses with echogenic chest masses were seen in our unit between 17 and 36 weeks' gestation over a 5-year period. We reviewed these cases retrospectively for prenatal diagnosis, postnatal diagnosis and outcome. Prenatal diagnosis was correct in 13 fetuses, with CDH in 8, sequestrated lung in 4 and tracheal atresia in 1. Four fetuses had incorrect or uncertain prenatal diagnoses. In three fetuses CDH and CAM could not be differentiated. After delivery two of these had CDH and one had sequestrated lung. One fetus with bilateral lesions had prenatal diagnosis of bilateral CAM. Post-mortem examination revealed tracheal atresia as part of Fraser syndrome. All five babies with sequestrated lung are well and none required surgery. Ten fetuses had CDH, two pregnancies were terminated, one died in utero, five died as neonates and two babies survived following surgery. The study reveals that in a minority of fetuses CDH and CAM could not be differentiated prenatally. We agree with recent reports of fetal sequestrated lung describing sonographic improvement in utero. A large lesion on initial scan does not necessarily predict a poor neonatal outcome in this condition. This, together with the poor outcome in fetuses with echogenic CDH and tracheal atresia, has important implications for prenatal counselling.

This report describes a case of a pre-term infant with congenital cystic adenomatoid malformation involving the whole lung who received a life-saving pneumonectomy. The challenges in decision-making, surgery, and subsequent management are discussed. The principles are applicable in general to a number of babies in a paediatric intensive care unit.

Congenital cystic adenomatoid malformation of the lung (CCAM) is an uncommon developmental abnormality presenting most often in the neonatal period. We report the clinical, radiological and pathological findings in a 9-year-old boy who presented with acute chest pain.