Congenital Pulmonary Airway Malformation Cases Research Articles

The prognosis of type 3 congenital pulmonary airway malformations may not be poor: Antenatal sonographic evaluation and postnatal outcomes of non-cardiac thoracic malformations in a large cohort

Background: To analyze the prenatal sonographic findings, prevalence, and outcomes of non-cardiac thoracic malformations (NCTMs).Method: This study included a retrospective analysis of electronic health records from February 2014 to March 2021. The follow-up sonographic examinations of the fetuses with diagnoses of NCTM and postnatal clinical follow-up were retrieved from electronically stored files and reports. Results: Out of 19,640 fetuses, 25 fetuses were found to have NCTM with a prevalence of 0.12%. The most common NCTM was congenital pulmonary airway malformation (CPAM) (n=8, 32%), followed by pleural effusion (PE), pulmonary hypoplasia (PH), and congenital diaphragmatic hernia (CDH) (4 cases each, 16%), bronchopulmonary sequestration (BPS) (n=3, 12%), congenital lymphangioma (CL), and pectus excavatum (PEx) (1 case each, 4%). All type 3 CPAM cases and BPS cases resolved spontaneously in utero, whereas all type 2 CPAM (n=2) cases required surgery in the neonatal period and one of the type 1 cases resulted in fetal hydrops. All PE, PEx, and PH cases, and 25% of the CDH cases (n=1) were associated with underlying abnormalities. All cases with PH (n=4), all PE cases associated with hydrops (n=2, 50%) resulted in exitus in utero.Conclusion: NCTMs are rare congenital anomalies with a prevalence of 0.12%. The most common type of NCTM was found as CPAM. All cases of type 3 CPAM and BPS showed spontaneous resolution in utero. The prognosis of type 3 CPAM may be favorable.

Can we predict the need for postnatal surgery in patients with prenatal fetal lung masses detected by CVR value?

The purpose of this study is to evaluate whether CPAM-volume ratio (CVR) can predict postnatal management (follow up for resolution and surgical treatment) in fetuses with fetal lung masses in the prenatal period. 44 patients who presented at our center with prenatally diagnosed CPAM (Congenital Pulmonary Airway Malformation) and BPS (Bronchopulmonary Sequestration) were analyzed. Obstetric history and outcomes, karyotype results, CVR, additional sonographic findings, characteristics of masses were recorded. CVR was calculated for all cases. İn the study we sought to identify a CVR threshold and did not use the thresholds classically used in the literature. 20 fetal BPS and 24 CPAM cases were analyzed. After excluding 5 patients, 46% of the patients were diagnosed with BPS and 54% with CPAM. İn this study the cut off < 0,53 for CVR is taken, it predicts the no need for postnatal surgery with a sensitivity of 85% and a specificity of 88%. When we take the > 0,76 cut-off value for patients who will require emergency surgery within the first 10 days, it predicts the need for surgery with 90% sensitivity and 89% specificity. In addition, it was determined that all patients with mediastinal shift were operated. We believe that the CVR value and the presence of mediastinal shift should be evaluated in all cases of CPAM and BPS for prediction of the surgery. Proper counseling about the prognosis could be given to the family in cases with mediastinal shift and CVR value above 0,76.

Congenital pulmonary airway malformation-Two case reports and diagnosis challenges in a resource limited setting

Congenital Pulmonary Airway Malformation (CPAM) is a rare abnormality of pulmonary airway malformation and may remain undiagnosed until it is discovered as an incidental finding later in life. Reported here are two cases of CPAM, a seven-month-old infant and a two-year-old toddler. A seven-month-old ex-premature male infant presented with recurrent pneumonia and failure to thrive. He had an unresolving consolidation on chest radiograph and was eventually treated as pulmonary tuberculosis with no response. Computerised tomography scan (CT-scan) chest revealed bilateral CPAM of lungs. A 2-year-old female toddler presented to the University Teaching Hospital, Department of Paediatrics &amp; Child Health, as a referral from a second level hospital with a long-standing history of recurrent symptoms and signs of pneumonia with failure to thrive. She was commenced on antituberculous treatment with no improvement. A CT-scan of the chest revealed bilateral CPAM of lungs. These two cases highlight clinical, diagnostic and treatment challenges in children with CPAM in a resource limited setting like Zambia.

Congenital pulmonary airway malformation-Two case reports and diagnosis challenges in a resource limited setting

Congenital Pulmonary Airway Malformation (CPAM) is a rare abnormality of pulmonary airway malformation and may remain undiagnosed until it is discovered as an incidental finding later in life. Reported here are two cases of CPAM, a seven-month-old infant and a two-year-old toddler. A seven-month-old ex-premature male infant presented with recurrent pneumonia and failure to thrive. He had an unresolving consolidation on chest radiograph and was eventually treated as pulmonary tuberculosis with no response. Computerised tomography scan (CT-scan) chest revealed bilateral CPAM of lungs. A 2-year-old female toddler presented to the University Teaching Hospital, Department of Paediatrics &amp; Child Health, as a referral from a second level hospital with a long-standing history of recurrent symptoms and signs of pneumonia with failure to thrive. She was commenced on antituberculous treatment with no improvement. A CT-scan of the chest revealed bilateral CPAM of lungs. These two cases highlight clinical, diagnostic and treatment challenges in children with CPAM in a resource limited setting like Zambia.

Congenital pulmonary airway malformation – A histomorphological spectrum of 15 cases: A 5-year study from a tertiary care center

Objective:Congenital pulmonary airway malformation (CPAM) is a rare developmental lung disease. The aim of this study is to analyze the histomorphological spectrum of CPAM in a series of 15 cases.Materials and Methods:A retrospective descriptive study of 15 cases of CPAM was carried out from 2013 to 2018 in our hospital, and cases were classified based on the Stocker's classification.Results:The age ranged from 4 days to 9 years (66.6% were infants). The left lung was most commonly involved (66.6%). The most common lobe was the left upper lobe (60%), followed by right lower lobe (20%). Grossly, cysts measured 0.2–5 cm, filled with mainly serous fluid with few having hemorrhagic and brownish mucoid secretions. On microscopy, single to multiple noncommunicating cysts of size 0.2–5 cm were seen, lined by ciliated columnar epithelium (60%), pseudostratified ciliated columnar epithelium (26.7%), mucin-secreting columnar epithelium (6.7%), and flattened epithelium (6.7%). Few cases showed smooth muscle (20%) and cartilage (13.3%) in the cyst wall. Chronic inflammation (73.3%) with dense histiocytic infiltrate (13.3%) was also seen. Emphysematous changes were also observed (13.3%). Cytomegalovirus inclusions (6.7%), zygomycete fungus (6.7%), and red hepatization (6.7%) were observed. The most common type was type II (60%), followed by type I (33.3%) and type IV (6.7%).Conclusion:Type II was the most common variant in this study. A careful observation should be done to look for fungal hyphae or viral inclusions.

Epithelial cell-adhesion protein cadherin 26 is dysregulated in congenital diaphragmatic hernia and congenital pulmonary airway malformation.

Congenital diaphragmatic hernia (CDH) and congenital pulmonary airway malformation (CPAM) are two inborn pathologies of the lung of unknown origin. Alterations of gene expression in airway epithelial cells are involved in the pathobiology of both diseases. We previously found decreased expression of the epithelial cell adhesion protein cadherin 26 (CDH26) in hypoplastic mice lungs. Here, our objective was to describe the expression and localization of CDH26 in hypoplastic CDH lungs and hyperproliferative CPAM tissues. After ethical approval, we used human lung tissues from CDH and CPAM cases and age-matched control samples from a previously established biobank. Furthermore, lungs from the nitrofen rat model of CDH were included in the study. We performed immunohistochemistry and western blot analysis with antibodies against CDH26 to examine protein localization and abundance. Statistical analysis was performed using Mann-Whitney U test with significance set at p < 0.05. We observed an overexpression of CDH26 within the epithelium of cystic CPAM lesions compared to normal airways within the same lung and compared to control lungs. Western blot demonstrated a downregulation of CDH26 in the nitrofen rat model of CDH compared to healthy controls. Immunohistochemistry could not show consistent differences between CDH and control in human and rat lungs. In the studied human lung samples, CDH26 was localized to the apical part of the airway epithelial cells. CDH26 is differentially expressed in human CPAM lung tissues and may be downregulated in nitrofen-induced hypoplastic rat lungs compared to control lungs. Disruption of CDH26 associated pathways in lung development may be involved in the pathogenesis of lung hypoplasia or cystic lung disease.

A retrospective clinical analysis of 20 cases of congenital lung masses

Background Congenital-pulmonary-airway-malformation (CPAM) and bronchopulmonary-sequestration (BPS) are rare conditions. The objective of this study was to describe the sonographic characteristics and prenatal course of fetal lung mass. Methods A retrospective study on 20 pregnancies with CPAM and BPS diagnosed during 2018–2018 was performed. Data evaluated included gestational age (GA) at diagnosis, size of lesion, CPAM-volume-ratio (CVR), associated sonographic findings and pregnancy outcome. Results Fourteen cases of CPAM and six cases of BPS were evaluated. The average GA at diagnosis was 22 weeks’-gestation (range 17–26). 66% were macrocystic–multicystic. Five cases of BPS (71.4%) had other structural anomalies. None of the fetuses with CPAM had additional anomalies. Four pregnancies (20%) underwent termination due to severe hydrops fetalis. Two cases of CPAM (15%) had spontaneous resolution by 30 week’s-gestation; three cases (20%) continued to grow during pregnancy and all the rest reached their largest size at 25–28 week’s-gestation. Most cases delivered at term (average 37.6 range 32.2–41.4). Two children had lobectomy at 11 and 12 months of life. Conclusions Careful follow-up during pregnancy may result in term delivery of adequate-for-gestational-age newborns, with no need for admission to the NICU, with only 10% need for lobectomy during the first year of life.

A UNIQUE CASE OF CONGENITAL PULMONARY AIRWAY MALFORMATION DIAGNOSED IN AN ADULT

SESSION TITLE: Medical Student/Resident Lung Pathology SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Congenital Pulmonary Airway Malformation (CPAM) is a rare developmental malformation of the lower respiratory tract. Incidence for this rare condition ranges from 1 per 8,300 to 35,000 live births. CPAM is usually detected in the prenatal and neonatal period. Since 1997, there have only been twelve cases of adult CPAM reported in literature. We here present an unusual case of a 22 year old female with cystic lung disease proven to be CPAM. CASE PRESENTATION: An asymptomatic 22 year old female without any medical history presented for an incidental finding on CT abdomen, which showed lobulated cystic lesions throughout the right lower lobe. A CT chest followed showing a cystic structure in the posterior aspect of the right lower lobe measuring 10 cm. No prior imaging was available. Patient had a healthy childhood. Patient did not report any family history of lung disease. After performing the testing to rule out most common cystic lung diseases in the age group, the plan was to assess the patient with a CT aortogram to assess for pulmonary sequestration. However, the patient’s case was complicated by hospitalization for community acquired pneumonia. At that time, CT chest angiogram was done, which was unable to show any aortic arterial vessel feeding to the cystic lesion ruling out pulmonary sequestration. Also the cystic lesions now had air-fluid levels with dense consolidation. After appropriate antibiotic therapy the patient underwent a right lower lobectomy. Upon pathologic evaluation, pulmonary artery/vein supply and bronchial attachment to the right lower lobe were all normal. Histopathology demonstrated variable-sized cysts, lined by pseudostratified ciliated cells interspersed with mucus cells. No cartilage was seen. The gross histopathologic findings along with the radiographic findings support the diagnosis of Type I CPAM. DISCUSSION: CPAM results from imbalance between cell proliferation and apoptosis during organogenesis. CPAM are hamartomatous lesions with cystic and adenomatous components of tracheobronchial tree. CPAM has been differentiated in 4 types based on the origin and distribution within the lung. With an exception of Type 4 malformations, CPAM has no genetic predisposition. More than two-thirds of the cases are diagnosed during the prenatal and neonatal periods due to the advances in imaging. It is exceedingly rare for cases of CPAM to remain undiagnosed until adulthood. A common presentation is recurrent pneumonia. There are reports that CPAM lesions can disguise as malignant lesions. Specifically, Type I is known to be associated with bronchoalveolar carcinoma. CONCLUSIONS: A conservative approach may be reasonable in a patient who is asymptomatic with CPAM. Our patient’s case was complicated by pneumonia; the patient underwent resection of her right lung, which confirmed Type I CPAM. Reference #1: Barreiro, T. J., Henn, L., Ingnam, S., & Sypert, M. (2016). Congenital pulmonary airway malformation in a 36 year-old female. Respiratory Medicine Case Reports, 17, 34–36. doi: 10.1016/j.rmcr.2015.11.005 Reference #2: Kapralik, J., Wayne, C., Chan, E., & Nasr, A. (2016). Surgical versus conservative management of congenital pulmonary airway malformation in children: A systematic review and meta-analysis. Journal of Pediatric Surgery, 51(3), 508–512. doi: 10.1016/j.jpedsurg.2015.11.022 Reference #3: Omar, M. A., Tylski, E., Ghanimeh, M. A., & Gohar, A. (2016). Congenital pulmonary airway malformation (CPAM) with initial presentation in an adult: a rare presentation of a rare disease. BMJ Case Reports. doi: 10.1136/bcr-2016-216957 DISCLOSURES: No relevant relationships by Aadil Ahmed, source=Web Response No relevant relationships by daniel kechker, source=Web Response No relevant relationships by ami Vaidya, source=Web Response

Atypical goblet cell hyperplasia occurs in CPAM 1, 2, and 3, and is a probable precursor lesion for childhood adenocarcinoma.

Congenital pulmonary airway malformation (CPAM) is a developmental disorder. Types 1-2-3 are the more common ones. Atypical goblet cell hyperplasia (AGCH) in CPAM might be a precursor lesion for pulmonary adenocarcinomas. In nine out of 33 CPAM cases, types 1-3 showed foci of goblet cell proliferations. As these cells completely replace normal epithelium, we prefer to name these proliferations AGCH. In 5 cases, adenocarcinomas were seen (AC). All cases were analyzed for proteins possibly being associated with CPAM development: fibroblast growth factor 10 (FGF10) and receptor 2 (FGFR2), forkhead box A1 (FOXA1) and A2 (FOXA2), MUC protein 5AC (MUC5AC), human epidermal growth factor receptor 2 (erbB2, HER2/neu), hepatocyte nuclear factor 4α (HNF4α), SOX2, and Ying Yang protein 1 (YY1). By next generation sequencing, AGCH and adenocarcinomas were evaluated for driver mutations. Expression for FGF10, FGFR2, FOXA1, and FOXA2 was seen in CPAM epithelium and stroma, but not differently in AGCH and AC. SOX2 was positive in CPAM epithelium and AGCH, however weakly in AC. YY1 and MUC5AC showed more intense staining in AGCH and AC than in CPAM epithelium. HER2 was intensely expressed in AC and less intensely in AGCH, but not in CPAM epithelium. KRAS mutation in exon 2 was detected in all AGCH and AC, but was absent in CPAM epithelia. AGCH can arise in CPAM types 1-3. Oncogenic KRAS mutation seems to be the oncogenic driver already in AGCH, proving its role as a precursor lesion for adenocarcinoma. It might upregulate HER2 at the protein level. YY1 seems to be involved in carcinogenesis.

The application of neonate lung ultrasound in congenital pulmonary airway malformation

Objective To analyze the characteristics of lung ultrasound (LUS) in congenital pulmonary airway malformation (CPAM), and to compare the difference of image characteristics between LUS and chest X-ray(CXR) and evaluate the value of neonate LUS in CPAM. Methods From June 2017 to October 2018, the characteristics of LUS and CXR of 32 neonates who were diagnosed with CPAM by prenatal ultrasound and postnatal chest CT were analyzed retrospectively, and the difference of their direct signs and indirect signs were compared. Results Among 32 CPAM cases, 10 cases (31.25%) showed a single large cystic lesions and 3 cases with consolidation, 4 cases (12.5%) characterized by multiple hypoechoic lesions and 3 cases with consolidation, 16 cases (50%) showed consolidation with or without intensiveaerated bronchus sign or dendritic air-filled bronchi, 2 cases (6.25%) only characterized by dense B-line. The non-characteristic sonographic findings including pleural line was not smooth and not clear; A-line decreased or disappeared, dense B-lines were observed. The ability of LUS in measuring the size of CPAM was limited.Among 32 CPAM cases, 10 cases (31.25%) showed large cystic hypoechoic lesions and 22 cases (68.75%) showed small cystic hypoechoic lesions by CT scan. In this group, there were 11 cases(34.4%) with normal CXR. There was no significant differences of the direct signs (including single large cystic lesions and multiple hypoechoic lesions) between LUS and CXR (P=0.80), however the indirect signs of LUS were more obvious than CXR, with significant difference(P=0.001). Conclusions The neonatal LUS findings of CPAM is multiple, it can be used as a preliminary qualitative screening method.The diagnosis value of indirect sign of LUS is superior to CXR. Key words: Ultrasonography; Lungs; Congenital pulmonary airway malformation; Computed tomography; Chest X-ray

Establishment of a biobank for human lung tissues of congenital diaphragmatic hernia and congenital pulmonary airway malformation

BackgroundHuman tissue samples are an invaluable and little available source of information for translational studies of congenital lung diseases such as Congenital Diaphragmatic Hernia (CDH) or Congenital Pulmonary Airway Malformation (CPAM). PurposeWe aimed to establish a human lung tissue biobank of CDH and CPAM patients together with age-matched controls, coupled with a clinical database. MethodsPathology records from autopsies or surgical specimens for CDH and CPAM cases between 1980 and 2017 were reviewed. For surviving individuals, clinical patient data was obtained from corresponding pediatric surgery reports. Formalin-fixed, paraffin-embedded tissues of patients and age-matched controls were systematically stored for further translational studies. RNA integrity was determined on selected CDH blocks. ResultsA total of 16 CDH and 18 CPAM and age-matched control lung tissue blocks were included in our biobank. Ages ranged from 22 to 41 weeks of gestation (GA) in CDH (33.9 ± 6.35 weeks) and 26 weeks (GA) and 12 years in CPAM (2.3 ± 3.7 y). RNA isolation from CDH and control blocks yielded good RNA quality (OD 260/280 ratio: 2.01–2.09, OD 260/230 ratio: 2.04–2.09). ConclusionWe established a unique human biobank for CDH and CPAM tissues. The combination with clinical patient data will allow us to design future translational studies to improve our understanding of the disease pathogenesis of these congenital malformations.

Fetal Congenital Pulmonary Airway Malformation: The Role of an Objective Measurement of Cardiomediastinal Shift.

To examine the relationship between cardiomediastinal shift angle (CMSA) and adverse perinatal outcomes and hydrops in cases of congenital pulmonary airway malformation (CPAM). This retrospective study evaluated CPAM cases referred to our institution from 2008 to 2015. The primary outcome was a composite score for adverse perinatal outcome. CMSA was measured for each case and evaluated for its association with the primary outcome. The prediction accuracy of CMSA for adverse perinatal outcome was assessed using receiver operator characteristic (ROC) curves. Eighteen (21.2%) of the 85 cases experienced an adverse perinatal outcome. Increases in CMSA were associated with adverse perinatal outcomes and hydrops in bivariate analyses. Adjusted analyses found each 10-degree increase in CMSA to be associated with increased odds of an adverse perinatal outcome (adjusted odds ratio [aOR] 2.2, 95% confidence interval [CI]: 1.4-3.3) and hydrops (aOR 3.0, 95% CI: 1.5-6.1). CMSA performed well and was comparable to CPAM volume ratio in predicting adverse perinatal outcomes (area under the curve 0.81 and 0.84, respectively). We describe a novel measurement of mediastinal shift in cases of CPAM and its relationship with adverse perinatal outcomes and hydrops. These findings may shape the evaluation and management of CPAMs, improve our understanding of their prognosis, and influence patient counseling.

Lung Ultrasound Findings in Congenital Pulmonary Airway Malformation.

Congenital pulmonary airway malformation (CPAM) is a group of rare congenital malformations of the lung and airways. Lung ultrasound (LU) is increasingly used to diagnose neonatal respiratory diseases since it is quick, easy to learn, and radiation-free, but no formal data exist for congenital lung malformations. We aimed to describe LU findings in CPAM neonates needing neonatal intensive care unit (NICU) admission and to compare them with a control population. A retrospective review of CPAM cases from three tertiary academic NICUs over 3 years (2014-2016) identified five patients with CPAM who had undergone LU examination. LU was compared with chest radiograms and computed tomography (CT) scans that were used as references. CPAM lesions were easily identified and corresponded well with CT scans; they varied from a single large cystic lesion, multiple hypoechoic lesions, and/or consolidation. The first two LU findings have not been described in other respiratory conditions and were not found in controls. We provide the first description of LU findings in neonates with CPAM. LU may be used to confirm antenatally diagnosed CPAM and to suspect CPAM in infants with respiratory distress if cystic lung lesions are revealed. Further studies are necessary to define the place of LU in the management of CPAM.

Is congenital pulmonary airway malformation really a rare disease? Result of a prospective registry with universal antenatal screening program

Congenital pulmonary airway malformation (CPAM) is an increasingly recognized disease with potential mortality. Owing to limited published studies, the true incidence is yet to be determined. We carried out this prospective study with the aim to estimate its true incidence on a population basis. An antenatal ultrasonography program was implemented since 2009. Fetuses with suspected intra-thoracic lesions were monitored by regular follow-ups. Antenatal course, postnatal outcomes, and other demographics were compared to those of patients with CPAM in the previous decades (1989-2008). The incidence of CPAM was calculated in different periods. 66 CPAM patients were identified between 2009 and 2014 with 62 patients being detected by antenatal scan. In contrast, 45 patients were identified between 1989 and 2008 with 27 patients being detected antenatally. The incidence rate during the past and recent period was estimated as~1 in 27,400 and~1 in 7200 live births, respectively (p=0.024). With increasing awareness of clinicians and the universal use of latest ultrasound technology, it is likely that more CPAM cases will be detected in the future. Here, we presented our best estimated incidence rate of CPAM, yet only a larger scale study can reveal its true incidence.

Complicated pneumothorax and congenital lung cystic malformation

Congenital cystic adenomatoid malformation, also named congenital pulmonary airway malformation (CPAM), is a congenital lung abnormality which is uncommon in adults. The usual radiological appearance of CPAM is a cystic space-occupying lesion. We present one case of CPAM with unusual clinical and radiological findings, a complicated spontaneous pneumothorax with intracystic haemorrhage with successful conservative initial treatment, despite acute haemodynamic instability.

Congenital pulmonary airway malformation of lung in fetus: a clinicopathological analysis

To study the etiology, pathogenesis, clinicopathologic characteristics, prognosis and treatment of congenital pulmonary airway malformation (CPAM). Eighteen cases of CPAM were enrolled into the study. The clinical history, autopsy findings and immunohistochemical results were evaluated, with review of literature. The pathogenetic mechanism, pathologic features and differential diagnosis of CPAM were studied. Histologic examination showed that 2 cases were classified as Stocker type I, 12 cases as type II, and 4 cases as type III. The lesion was unilateral and involved single lobe in 13 cases. The remaining 5 cases had bilateral diseases. Of the 18 cases studied, 12 cases showed single organ involvement and 6 cases had malformations affecting multiple organs. The associated malformations included cardiac anomalies (4 cases), polycystic kidney with gastrointestinal atresia (1 case) and nuchal cystic hygroma with hydrothorax (1 case). CPAM is a rare pulmonary disorder. The etiology of this non-neoplastic condition is unknown. Imaging analysis is a valuable tool to suggest CPAM, while definite diagnosis requires pathologic examination. The overall prognosis is determined by the presence of associated malformations, fetal hydrops and pulmonary hypoplasia.

Congenital pulmonary airway malformation: A report of two cases

Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation is a congenital disorder of the lung similar to bronchopulmonary sequestration. In CPAM, usually an entire lobe of lung is replaced by a non-working cystic piece of abnormal lung tissue. This abnormal tissue will never function as normal lung tissue. The underlying cause for CPAM is not known. It occurs in approximately 1 in every 30000 pregnancies. The association between CPAM and malignancy has been well documented. There is a small risk (0.7%) of malignant transformation within the cyst. So early diagnosis and surgical resection is important to prevent the grave complications. Herein, we are reporting two interesting cases of CPAM and one belonged to Type II and other belonged to Type III of Stocker's classification.

Revisiting the Histopathologic Spectrum of Congenital Pulmonary Developmental Disorders

A retrospective analysis of lobectomy, pneumonectomy, cystectomy specimens (10 years) and stillbirth/neonatal autopsies (5 years) was carried out to analyze the histologic spectrum of congenital pulmonary developmental disorders/malformations. The autopsy data was analyzed to identify the lesions that are more diffuse and not amenable to surgical management. A total of 166 cases of pulmonary developmental disorders/ malformation were found. Out of 2,155 stillbirth/neonatal autopsies, there were 105 cases of pulmonary hypoplasia, 2 cases of congenital pulmonary lymphangiectasis, 2 cases of extralobar sequestration, and 3 cases of congenital pulmonary airway malformation (CPAM). Among the surgical specimens, there were 21 cases of CPAM, 11 cases of intralobar sequestration, 5 cases of congenital lobar emphysema and 17 cases of bronchogenic cyst. All these malformations have been described in reference to the latest updated classification of developmental disorders/malformations.

Congenital Pulmonary Airway Malformation

Congenital pulmonary airway malformation (CPAM) is classified into 5 subtypes (types 0 to 4). We attempted to correlate computed tomography (CT) findings with those of pathologic examination and evaluated the predictability of the CPAM subtype. We retrospectively reviewed CT findings in 13 pathologically proven cases of CPAM seen between 1981 and 2005. Patient's age ranged from 4 days to 5 years and 10 months. Six were boys and 7 were girls. According to CT findings, lesions with a cyst larger than 2.5 cm, lesions with cysts 2.5 cm or less and solid lesions were classified into groups A, B, and C, respectively. We assumed that Stocker's types 1 and 4, type 2, and types 0 and 3 would correspond to CT groups A, B, and C, respectively. Then, we assessed whether this assumption is correct or not. Eight, 3, and 2 cases were diagnosed as groups A, B, and C, respectively. All of the 8 cases diagnosed as group A were Stocker's type 1. One of the 3 cases diagnosed as group B was type 2, but the remaining 2 were type 1 and type 4, respectively. One of the 2 cases diagnosed as group C was type 3 but the other was type 2. Lesions with the largest cyst being larger than 2.5 cm was type 1. It seemed, however, difficult to distinguish among types 1, 2, and 4 when they consisted of small cystic components and between types 2 and 3 when they appeared as a solid lesion.