Perinatal outcomes of prenatal diagnosis of congenital pulmonary airway malformation: an experience

SUMMARY OBJECTIVE: This study aimed to assess the perinatal outcomes of pregnancies with a prenatal diagnosis of congenital cystic adenomatoid malformation. METHODS: We conducted a retrospective cohort study based on information contained in the medical records of pregnant women whose fetuses had been prenatally diagnosed with congenital cystic adenomatoid malformation by ultrasonography. RESULTS: Sample analysis was based on 21 singleton pregnancies with confirmed isolated fetal congenital cystic adenomatoid malformations. The mean maternal±standard deviation age was 28±7.7 years. Types I, II, and III congenital cystic adenomatoid malformation were detected in 19% (4/21), 52.4% (11/21), and 28.6% (6/21), respectively. All fetuses presented with unilateral congenital cystic adenomatoid malformation (21/21) without associated anomalies, and 52.3% (11/21) were in the right lung. In total, 33.3% (7/21) of fetuses presented a "congenital cystic adenomatoid malformation volume ratio" >1.6 and were managed with maternal betamethasone administration. The mean gestational age at the time of steroid administration was 28.5±0.9 weeks, with a reduction in the lesion dimensions of 9.5% (2/21) (Types I and III of congenital cystic adenomatoid malformation). The mean gestational age at delivery was 38.7±2.4 weeks, and a cesarean section was performed in 76.2% (16/21) cases. Postsurgical resection was necessary for 23.8% (5/21) of the patients, and 4.7% (1/21) of them died because of respiratory complications after surgery. Pulmonary hypoplasia occurred in 9.5% (2/21) of the patients, and 4.7% (1/21) of them died because of respiratory insufficiency. The survival rate was 90.5% (19/21), and 57.2% (12/21) remained asymptomatic. CONCLUSION: Despite the isolated prenatal diagnosis of congenital cystic adenomatoid malformation, which showed good survival, congenital cystic adenomatoid malformation is associated with significant perinatal morbidity. Maternal betamethasone administration did not significantly reduce fetal lung lesion dimensions.

Research progress of congenital pulmonary airway malformation-volume ratio in perinatal management of fetal congenital pulmonary airway malformation / 中华围产医学杂志

Congenital pulmonary airway malformation-volume ratio (CVR) greater than 1.6 or 2.0 cm2 was considered to be helpful in predicting fetal hydrops and could serve as an indicator for steroid application, forecasting respiratory symptoms, and the timing of surgery after birth. However, the optimal CVR threshold remains controversial. This review focuses on the value of CVR in fetal prognosis, steroid administration, and the prediction of postnatal respiratory symptoms and surgical timing in fetuses with congenital pulmonary airway malformation, aiming to guide the perinatal management.

[Clinical analysis of 96 cases of congenital cystic lung lesions in children].

Objective: To summarize the diagnosis, clinical manifestations, treatment and prognosis of congenital cystic lung lesions. Methods: A retrospective study described the clinical course of 96 patients (46 female and 50 male) diagnosed with congenital cystic lung lesions treated at the Tianjin Children's Hospital from January 2010 to March 2019. The clinical findings, imaging examinations, pathological findings, treatment and follow-up were analyzed. Results: Totally 96 patients (aged from 4 days to 13 years) with congenital cystic lung lesions were included in this study. Eighty-six patients (90%) were diagnosed when they had cough and fever symptoms. Forty (42%) patients exhibited congenital cystic adenomatoid malformation, 30 underwent surgical excision, two were at emergency operations and one dead. There were 12 (13%) patients with pulmonary sequestration and four were surgical treated. Twelve (13%) patients with bronchogenic cyst were included and 4 were surgically treated. There were 3 (3%) patients with congenital lobar emphysema and one was surgically treated. Another patient with pneumothorax was operated in other hospital 2 months after discharge. Twenty-nine (30%) patients with unclassified congenital cystic lung lesions could not be definitively diagnosed by CT. Some of them were difficult to be distinguished from necrotizing pneumonia. Finally, 2 patients were diagnosed as necrotizing pneumonia after 6, 10 months follow-up. After operation 37 out of 39 patients recovered well. Conclusions: The diagnosis of congenital pulmonary cystic disease depend on imaging and pathological examination. Most patients are diagnosed when they have respiratory tract infection. The main clinical manifestations are cough and fever. The prognosis of operative management is good.

Clinical analysis of 96 cases of congenital cystic lung lesions in children / 中华儿科杂志

Objective@#To summarize the diagnosis, clinical manifestations, treatment and prognosis of congenital cystic lung lesions.@*Methods@#A retrospective study described the clinical course of 96 patients (46 female and 50 male) diagnosed with congenital cystic lung lesions treated at the Tianjin Children′s Hospital from January 2010 to March 2019. The clinical findings, imaging examinations, pathological findings, treatment and follow-up were analyzed.@*Results@#Totally 96 patients (aged from 4 days to 13 years) with congenital cystic lung lesions were included in this study. Eighty-six patients (90%) were diagnosed when they had cough and fever symptoms. Forty (42%) patients exhibited congenital cystic adenomatoid malformation, 30 underwent surgical excision, two were at emergency operations and one dead. There were 12 (13%) patients with pulmonary sequestration and four were surgical treated. Twelve (13%) patients with bronchogenic cyst were included and 4 were surgically treated. There were 3 (3%) patients with congenital lobar emphysema and one was surgically treated. Another patient with pneumothorax was operated in other hospital 2 months after discharge. Twenty-nine (30%) patients with unclassified congenital cystic lung lesions could not be definitively diagnosed by CT. Some of them were difficult to be distinguished from necrotizing pneumonia. Finally, 2 patients were diagnosed as necrotizing pneumonia after 6, 10 months follow-up. After operation 37 out of 39 patients recovered well.@*Conclusions@#The diagnosis of congenital pulmonary cystic disease depend on imaging and pathological examination. Most patients are diagnosed when they have respiratory tract infection. The main clinical manifestations are cough and fever. The prognosis of operative management is good.

Evaluation of Newborn Infants with Prenatally Diagnosed Congenital Pulmonary Airway Malformation: A Single-Center Experience

PURPOSE: Congenital pulmonary airway malformation (CPAM)—a rare developmental anomaly—affects the lower respiratory tract in newborns. By comparing the reliability of diagnostic tools and identifying predictive factors for symptoms, we provide comprehensive clinical data for the proper management of CPAM. METHODS: We reviewed the medical records of 66 patients with prenatally diagnosed CPAM delivered at Severance Children's Hospital between January 2005 and July 2017. RESULTS: We enrolled 33 boys and 33 girls. Their mean gestational age and birth weight were 38.8 weeks and 3,050 g, respectively. Prenatal ultrasonography and postnatal radiography, lung ultrasonography, and chest computed tomography (CT) showed inconsistent findings. Chest CT showed superior sensitivity (100%) and positive predictive value (90%). Among the 66 patients, 59 had postnatally confirmed CPAM, three had pulmonary sequestration, one had cystic teratoma, and one had a normal lung. Of the 59 patients with CPAM, 21 (35%; mean age, 23.4 months) underwent surgery, including 15 who underwent video-assisted thoracoscopy. Twenty-five and 12 patients exhibited respiratory symptoms at birth and during infancy, respectively. Apgar scores and mediastinal shift on radiography were significantly associated with respiratory symptoms at birth. However, none of the factors could predict respiratory symptoms during infancy. CONCLUSION: Radiography or ultrasonography combined with chest CT can confirm an unclear or inconsistent lesion. Apgar scores and mediastinal shift on radiography can predict respiratory symptoms at birth. However, symptoms during infancy are not associated with prenatal and postnatal factors. Chest CT combined with periodic symptom monitoring is important for diagnosing and managing patients with prenatally diagnosed CPAM and to guide appropriate timing of surgery.

Congenital pulmonary airway malformation

Congenital pulmonary airway malformation (CPAM) is a developmental malformation of the lower respiratory tract. We report the case of a male newborn diagnosed with cystic lung disease during prenatal ultrasound. A cesarean section was performed at the 32nd gestational week because of premature rupture of the membranes, and soon after the delivery the newborn developed respiratory failure and died. The aim of this study is to report an autopsy case because of its rarity, and to briefly discuss the CPAM subtypes and differential diagnosis of cystic lung diseases of childhood.

Congenital pulmonary airway malformation.

Congenital pulmonary airway malformation (CPAM) is a developmental malformation of the lower respiratory tract. We report the case of a male newborn diagnosed with cystic lung disease during prenatal ultrasound. A cesarean section was performed at the 32nd gestational week because of premature rupture of the membranes, and soon after the delivery the newborn developed respiratory failure and died. The aim of this study is to report an autopsy case because of its rarity, and to briefly discuss the CPAM subtypes and differential diagnosis of cystic lung diseases of childhood.

An Atypical Presentation of Congenital Pulmonary Airway Malformation (CPAM): A Rare Case with Antenatal Ultrasound Findings and Review of Literature.

BACKGROUND: Congenital pulmonary airway malformation (CPAM) is a relatively rare congenital anomaly with a wide spectrum of ultrasound features depending on the specific variety of CPAM. Antenatal ultrasound is a valuable, safe, nonionizing, cost-effective, widely available and easily reproducible imaging tool and is indispensable in the diagnosis of CPAM. In this paper, we aimed to report an atypical imaging presentation of CPAM type II in the second trimester, extensively involving all lobes of the left lung. CASE REPORT: A 25-year-old G1P0A0 woman with a gestational age of around 22 weeks was referred for an anomaly scan. The antenatal ultrasound scan showed a single, live, intrauterine foetus corresponding to a gestational age of around 22 weeks and 4 days. There were multiple, anechoic structures noted within the pulmonary tissue in the left hemithorax, each measuring around 3 to 4 mm in diameter. The lesion was extending from the left lower lobe up to the apical (apicoposterior) segment of the left upper lobe. The ultrasound diagnosis of congenital pulmonary airway malformation type II was made. After explaining the condition and the poor prognosis to the patient, an informed consent was obtained after she opted for medical termination of pregnancy. CONCLUSIONS: Congenital pulmonary airway malformation (CPAM) is an uncommon foetal anomaly with a very wide range of ultrasound appearances depending on the specific type of CPAM. CPAM also has a wide spectrum of differential diagnoses and a variable prognosis. Antenatal ultrasound should always be the primary mode of diagnosis in CPAM.

Life-Threatening Congenital Cystic Adenomatoid Malformation in the Premature Neonate.

Congenital cystic adenomatoid malformation is a rare, but well-known disease. It can be managed conservatively in patients without symptoms or require surgical removal when symptomatic. The surgical option of choice is en bloc resection of the affected lesion. We report an experience of life-threatening congenital cystic adenoid malformation in a low-birth-weight (1,590 g) premature neonate who was successfully treated with a lobectomy of the lung.

Life-Threatening Congenital Cystic Adenomatoid Malformation in the Premature Neonate

Congenital cystic adenomatoid malformation is a rare, but well-known disease. It can be managed conservatively in patients without symptoms or require surgical removal when symptomatic. The surgical option of choice is en bloc resection of the affected lesion. We report an experience of life-threatening congenital cystic adenoid malformation in a low-birth-weight (1,590 g) premature neonate who was successfully treated with a lobectomy of the lung.

Congenital cystic adenomatoid malformation type I.

Congenital cystic adenomatoid malformation (CCAM) is an hamartomatous congenital pulmonary airway malformation with incidence ranging between 1:10,000 and 1:35,000 newborns. Currently CCAM is classified into five groups according to clinical and pathological features. The clinical outcome varies depending on the subtype and the extent of involvement. The authors report the case of a premature male newborn with the prenatal diagnosis of CCAM Type 1 associated with cardiac right axis deviation, who died 67 hours after birth due to respiratory failure. In addition to the autopsy report of this rare entity, the authors present its classification and prognosis.

Prenatal ultrasonographic diagnosis and prognosis of congenital cystic adenomatoid malformation / 中华医学超声杂志（电子版）

Objective To investigate the ultrasound characteristics of congenital cystic adenomatoid malformation (CCAM). Methods The ultrasonographic features of 21 cases of fetal CCAM confirmed by prenatal ultrasound were analyzed. And ultrasound ifndings were compared with the postnatal thoracic computed tomography (CT), surgical result and follow-up data. Results (1) Prenatal ultrasonographic ifndings:2 cases of CCAM typeⅠ(10%, 2/21), 13 cases of typeⅡ(62%, 13/21), and 6 cases of typeⅢ(28%, 6/21). Of them, 95%(20/21) lesions were located in unilateral thorax, while 5%(1/21) in bilateral thorax. CCAM was ifrst detected at 18th-25th gestational week with a median of 22nd week. The CVR was1.6 in 16 (76%, 16/21) and 5 (24%, 5/21) cases respectively. After reaching the maximum volume, the size of mass remained stable in 8 cases and regressed in 13 cases with even complete remission in 5 cases. There were mediastinal shift in 19 fetuses (90%, 19/21), polyhydramnios in 6, and concomitant structural abnormalities in 3 cases such as pulmonary sequestration, diaphragmatic hernia and fetal hydrops. (2) Clinical outcomes:15 newborns were conifrmed as CCAM by CT, in which 9 cases underwent surgical resection. One case was conifrmed by autopsy. Nineteen cases showed good prognosis. Whereas 1 pregnancy with fetal hydrops was terminated, and 1 fetus with diaphragmatic hernia died after birth. Conclusions Dynamic prenatal ultrasound assessment is the key factor in prognosis prediction of fetal CCAM. CCAM without other structural abnormalities has a good outcome.

A Case of Malignant Transformation in a Congenital Cystic Adenomatoid Malformation / 대한내과학회지

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare condition with the potential for malignant transformation. Several cases that presented with malignant changes have been reported worldwide. However, no report about a malignant transformation case in a CCAM in Korea has been published. We report the first patient who had a malignant change in a CCAM. A 32-year-old female underwent a bilobectomy for a cystic lung lesion that was a type 1 CCAM associated with malignant transformation. A biopsy specimen from the resected cystic lesion showed foci of adenocarcinoma.

Congenital pulmonary airway malformation.

BACKGROUND: Congenital cystic adenomatoid malformations (CCAMs) are considered rare developmental anomalies of the lower respiratory tract. These are hamartomatous abnormalities of the lung with adenomatoid proliferation of cysts resembling bronchioles and ususally occur sporadically occur and unilaterally with single lobe involvement. METHOD: A 6-year-old girl was admitted to our center because of prolonged fever and non-productive cough lasting3 months before admission. RESULTS: The only other complaint was night sweating. She did not have dyspnea and did not mention any respiratory symptoms. On examination, coarse crackle and decreased lung sounds in the left side were detected. White blood cell count was 9.100 /µL, hemoglobin was 11.2 g/dL, erythrocyte sedimentation rate was 50 and C-reactive protein was 1+. IgA and IgM for hydatid cyst were tested and both were raised (14 and 1.4, respectively). CONCLUSION: The patient underwent surgery, with the probable diagnosis of hydatid cyst but in operating room diagnosis was changed and it was adenomatoid cystic malformation. In follow-up, she was in good general condition without any post-surgical complaints (Fig. 3, Ref. 11).

A Case of Congenital Cystic Adenomatoid Malformation Infected with Mycobacterium avium-intracellulare Complex.

We present a case of congenital cystic adenomatoid malformation (CCAM) in a 25-year-old male who was presented with chronic cough. Chest radiography revealed an abnormal mass-like shadow in the right lower pulmonary zone. A contrast enhanced computed tomography showed an 11 cm solid, cystic mixed mass on the right lower lobe. A right lower lobectomy was performed by video-assisted thoracoscopic surgery without complications. The gross specimen showed a massive cavitation with multiloculated cysts of varying size, consistent with CCAM, along with noticeable granulomatous inflammation. Non-tuberculosis mycobacteria were isolated from a bronchial wash specimen, and the resected tissue homogenates were positive for Mycobacterium avium-intracellulare complex by polymerase chain reaction.

A Case of Congenital Cystic Adenomatoid Malformation Infected with Mycobacterium avium-intracellulare Complex / 결핵및호흡기질환

We present a case of congenital cystic adenomatoid malformation (CCAM) in a 25-year-old male who was presented with chronic cough. Chest radiography revealed an abnormal mass-like shadow in the right lower pulmonary zone. A contrast enhanced computed tomography showed an 11 cm solid, cystic mixed mass on the right lower lobe. A right lower lobectomy was performed by video-assisted thoracoscopic surgery without complications. The gross specimen showed a massive cavitation with multiloculated cysts of varying size, consistent with CCAM, along with noticeable granulomatous inflammation. Non-tuberculosis mycobacteria were isolated from a bronchial wash specimen, and the resected tissue homogenates were positive for Mycobacterium avium-intracellulare complex by polymerase chain reaction.

Congenital cystic adenomatoid malformation with bronchial atresia in elderly patients.

UNLABELLED: Congenital cystic adenomatoid malformation (CCAM) is an uncommon, nonhereditary anomaly caused by arrest of lung. Patients with CCAM may present with respiratory distress as newborns, or may remain asymptomatic until later in life. CCAM type I is rarely found in association with bronchial atresia (BA) in adults; we present such a case. CASE: A 54-year-old female presented with chronic cough and blood-tinged sputum. Physical examination and laboratory tests were unremarkable. Chest radiographs and a CT scan of the chest showed multiple large air-filled cysts consistent with a CCAM in the right lower lobe, and an oval-shaped opacity in the distal right middle lobal bronchus. Based on the radiologic findings, right middle lobectomy and a medial basal segmentectomy of the right lower lobe were performed via a thoracotomy. These lesions were consistent with Stocker's Type I CCAM and BA in the different lobes.

Space occupying lesions in the fetal chest evaluated by MRI.

BACKGROUND: The most common space occupying lesions of the fetal thorax are congenital diaphragmatic hernia (CDH), congenital cystic adenomatoid malformation (CCAM), and bronchopulmonary sequestration (BPS). Although applications of prenatal MRI have been vastly improved in the recent years, its use in the assessment of space occupying lesions of the fetal chest differs among centers. OBJECTIVES: To evaluate MRI findings in the diagnosis and follow-up of space-occupying lesions in the fetal chest with the review of relevant literature. PATIENTS AND METHODS: The fetuses with space-occupying lesions of the chest were retrieved from our 1.5T fetal MRI database of 347 patients. MRI features including the shape, signal characteristics, feeding artery, margin, mass effect, affected organ parts and anatomic location were reviewed. The results were correlated with the pathology results, follow-up and surgical findings. RESULTS: Nineteen MR images of 17 fetuses (mean gestational age, 23.8 weeks) with spaceoccupying lesions (5 CCAMs including one involuted case), 2 BPSs, 2 hybrid lesions, 8 CDH) were evaluated. One case of CCAM completely involuted in utero, four newborns were operated, and the resulting 12 fetuses were terminated. The surgical and pathological findings were in accordance with MRI findings. CONCLUSION: MRI can reliably differentiate CDH from CCAM and BPS in utero. Follow-up is of utmost importance as lesions may involute or progress in utero. Prenatal MRI findings help postnatal decision-making, surgical planning and parental counseling.

Congenital Cystic Adenomatoid Malformation with Bronchial Atresia in Elderly Patients / 결핵및호흡기질환

Congenital cystic adenomatoid malformation (CCAM) is an uncommon, nonhereditary anomaly caused by arrest of lung. Patients with CCAM may present with respiratory distress as newborns, or may remain asymptomatic until later in life. CCAM type I is rarely found in association with bronchial atresia (BA) in adults; we present such a case. Case: A 54-year-old female presented with chronic cough and blood-tinged sputum. Physical examination and laboratory tests were unremarkable. Chest radiographs and a CT scan of the chest showed multiple large air-filled cysts consistent with a CCAM in the right lower lobe, and an oval-shaped opacity in the distal right middle lobal bronchus. Based on the radiologic findings, right middle lobectomy and a medial basal segmentectomy of the right lower lobe were performed via a thoracotomy. These lesions were consistent with Stocker's Type I CCAM and BA in the different lobes.

Coexistence of Intrapulmonary Bronchogenic Cyst and Congenital Cystic Adenomatoid Malformation: A Case Report

Congenital cystic lesions of the lung are uncommon and a conjunction of two or more lesions is very rare. We report here on a case of coexisting intrapulmonary bronchogenic cyst and congenital cystic adenomatoid malformation in a 13-year-old female with a cystic mass in the right upper lobe of the lung. Computed tomography showed a cystic lesion measuring 2.5 cm with an air fluid level and surrounding multicystic lesions in the right upper lobe. On gross examination, the cut surface showed a cystic mass containing inspissated mucinous material, and the cystic mass was surrounded by multiple small cysts. Microscopically, the larger cystic cavity was lined with pseudostratified ciliated columnar epithelium. The submucosal tissue contained mucinous glands and plates of cartilage. The surrounding smaller cysts or irregular spaces were lined with bronchiolar-type respiratory epithelium. We propose that this hybrid lung lesion may represent the missing link in a common embryologic pathway determined by the timing of mesenchymal and epithelial interactions.