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Respiratory disorders
Published in Rachel U Sidwell, Mike A Thomson, Concise Paediatrics, 2020
Rachel U Sidwell, Mike A Thomson
Cystic adenomatoid malformation (CAM Lung). This is the second most common congenital Lung malformation after congenital Lobar emphysema. A single Lobe of one Lung is enlarged and cystic, and usually causes mediastinal shift with compression of the other Lung. It presents with neonatal respiratory distress, a pneumothorax or recurrent respiratory infections. It may be seen on CXR and CT scan of the thorax. Management is surgical
Respiratory disorders
Published in Angus Clarke, Alex Murray, Julian Sampson, Harper's Practical Genetic Counselling, 2019
This malformation used to be known as congenital cystic adenomatoid malformation of the lung. It is rare but more common than used to be appreciated as it is sometimes recognised on fetal ultrasound scan but then resolves before birth or, at least, never causes symptoms. It may be best for the lesion to be removed as it can occasionally contain malignant elements.
Thoracic Abnormalities
Published in Asim Kurjak, CRC Handbook of Ultrasound in Obstetrics and Gynecology, 2019
Congenital cystic adenomatoid malformation of the lung is the most frequent congenital lung abnormality. The disease is caused by an excessive growth of terminal bronchioles and is usually unilateral. According to the morphological appearance of the lesions, the disease is classified in three types.2 Type I is characterized by the presence of large pulmonary cysts. In type II, the cysts are much smaller, being less than 2 cm in size, while in type III, the cystic dilatation of the bronchioles is too small to be visualized by ultrasound. Helpful signs to detect type III disease are mediastinal shift and increased echogenicity of the lungs.3,4
Invasive mucinous adenocarcinoma of the lung arising in a type 1 congenital pulmonary airway malformation in a 68-year-old patient: a case report
Published in Acta Chirurgica Belgica, 2021
A. E. Frick, H. Decaluwé, B. Weynand, M. Proesmans, D. Van Raemdonck
The development of a congenital parenchymatous lung disorder, known as congenital pulmonary airway malformation (CPAM), has previously been described as congenital cystic adenomatoid malformation (CCAM) with an estimated incidence at 1:25,000–1:35,000 births. The classification by Stocker et al. in 1977 originally described 3 different subtypes and was later expanded and renamed into 5 subtypes on the basis of clinical and pathological features [1–3]. Most congenital CPAMs are uncommon and primarily identified in infants and in adults. Depending on the type of CPAM, clinical presentation and prognosis are different. Typical symptoms are recurrent pulmonary infection, productive cough, and hemoptysis. Some patients remain asymptomatic [4]. A number of reports have been published describing the association of adenocarcinoma with type 1 CPAM. The occurrence of rhabdomyosarcoma or pleuropulmonary blastoma arising in a CPAM has also been rarely documented [5,6].
Effect of maternal betamethasone on hydrops fetalis caused by extralobar pulmonary sequestration: a case report
Published in Journal of Obstetrics and Gynaecology, 2019
Cécile Guenot, Karine Dubrit, Karine Lepigeon, Eric Giannoni, David Baud, Yvan Vial
The patient presented with spontaneous labour at 35 + 5 WG and delivered a boy weighing 2570 g, 50 cm, with an Apgar score 8/10/10 at 1, 5 and 10 minutes, pH 7.33/7.41. The chest radiography and ultrasound of the newborn on day 1 confirmed a left pulmonary mass. He rapidly developed a pleural effusion treated with a pleural catheter. Dexamethasone was initiated for one week, with a transient regression of effusion, but it reappeared one week after the end of the treatment. A thoracotomy and segmentar pulmonary resection was realised at one month of life. The post-operative follow-up was uneventful and the patient was discharged home at two months of life. The anatomo-pathological examination confirmed the prenatal diagnosis of BPS. There was no histologic or vascular argument for a congenital cystic adenomatoid malformation (CCAM).
Pleuropulmonary Blastoma Developing in a Case of Misinterpreted Congenital Pulmonary Airway Malformation: a Case Report
Published in Fetal and Pediatric Pathology, 2018
Moupali Ghosh, Nelofar Islam, Arindam Ghosh, Priyanka Maity Chaudhuri, Koushik Saha, Uttara Chatterjee
After the establishment of International Pleuropulmonary Blastoma Registry (IPPBR), review of several cases showed that many cases which had been earlier diagnosed as CPAM were actually PPB type I [22–24]. This diagnostic problem is highlighted in the articles by Hill et al and Dehner et al titled “A cautionary note about congenital cystic adenomatoid malformation (CCAM) type 4” and “Beware of ‘degenerating’ congenital pulmonary cysts” [22,25].