CONGENITAL CYSTIC ADENOMATOID MALFORMATION OF THE LUNG

PEDIATRICS ◽  
1962 ◽  
Vol 30 (5) ◽  
pp. 759-768
Author(s):  
John Kwittken ◽  
Leopold Reiner
Keyword(s):  
Special Form ◽  
The Other ◽  
Normal Lung ◽  
Congenital Cystic Adenomatoid Malformation ◽  
Elastic Tissue ◽  
Alveolar Cell ◽  
Bronchial Wall ◽  
Cystic Lung ◽  
Cystic Adenomatoid Malformation ◽  
Pulmonary Adenomatosis

Two new cases of congenital cystic adenomatoid malformation of the lung are reported. Both patients died shortly after birth, one at 36 hours and the other on the tenth postnatal day. Cystic adenomatoid malformation is a special form of cystic lung disease characterized by large and small cysts lined variably by a bronchial-type (respiratory) and by cuboidal epithelium. In spite of the bronchial-type epithelium in some of the cysts, bronchial wall elements such as cartilage and mucoserous glands were not demonstrated. There was much elastic tissue in these cysts, of a degree having no counterpart in normal lung. These observations together with the topographic interposition of the cysts with normal appearing alveolar structures suggest a developmental malformation affecting those segments of the lungs normally destined to become the terminal bronchioles, respiratory bronchioles, and alveolar ducts. In one case, groups of alveoli were lined with a tall mucogenic epithelium morphologically resembling that seen in pulmonary adenomatosis of man and jaagsiekte of sheep. The finding is considered the result of alveolar cell metaplasia.

Cystic Adenomatoid Change Is Common to Various Forms of Cystic Lung Diseases of Children

2002 ◽  
Vol 126 (8) ◽  
pp. 934-940
Author(s):  
Yukihiro Imai ◽  
Eugene J. Mark
Keyword(s):  
Lung Disease ◽  
Blood Vessels ◽  
Bronchial Tree ◽  
Congenital Cystic Adenomatoid Malformation ◽  
Cystic Lesions ◽  
Cystic Lung ◽  
Intralobar Sequestration ◽  
Cystic Adenomatoid Malformation ◽  
Lobar Emphysema ◽  
Bronchial Atresia

Abstract Context.—Congenital cystic adenomatoid malformation, intralobar sequestration, extralobar sequestration, bronchopulmonary foregut malformation, bronchial atresia, and lobar emphysema generally are treated as separate entities, but there are commonalities among them. Objective.—To clarify the identity of congenital cystic adenomatoid malformation in relation to other cystic diseases. Design.—We studied 10 consecutive cases of surgically resected cystic lung disease in children. We studied the bronchial and vascular trees by mapping bronchi, pulmonary blood vessels, systemic blood vessels, the size and configuration of cysts, and associated scarring. Results.—The cystic lesions proved to be the following: 1 case of congenital cystic adenomatoid malformation, 2 cases of bronchial atresia with congenital cystic adenomatoid malformation, 1 hybrid case of congenital cystic adenomatoid malformation and intralobar sequestration, 1 case of intralobar sequestration with an aberrant hilum, 3 cases of intralobar sequestration with bronchial obliteration, 1 case of lung abscess, and 1 case of lobar emphysema. Congenital cystic adenomatoid malformation was the sole pathologic diagnosis in 1 case, but 7 other specimens had cystic adenomatoid change to various degrees. There was no bronchial connection between the cystic lesions and the patient's airway in 8 cases. The bronchial tree was absent in the cystic lung in 2 cases. Bronchus tapered into scar near the cystic lesion in 4 cases. Only 1 case had no significant bronchial abnormality. Conclusion.—The high incidence of cystic adenomatoid change in cystic lung disease associated with an abnormality of the bronchial tree suggests that cystic adenomatoid change may develop together with and be related to other congenital or acquired conditions in the lung.

scholarly journals Thoracoscopic Lobectomy for Congenital Cystic Lung Disease in Infants

2020 ◽  
Author(s):  
Jinxi Huang ◽  
Chaoming Zhou ◽  
Qiang Chen ◽  
Dianming Wu ◽  
Junjie Hong ◽  
...  
Keyword(s):  
Lung Disease ◽  
Thoracoscopic Surgery ◽  
Pulmonary Sequestration ◽  
Congenital Cystic Adenomatoid Malformation ◽  
Thoracoscopic Lobectomy ◽  
Cystic Lung ◽  
Cystic Adenomatoid Malformation ◽  
Cystic Lung Disease ◽  
Intralobar Pulmonary Sequestration ◽  
Congenital Cystic Lung Disease

Abstract Background: Congenital cystic lung disease is a congenital disease with abnormal development of the trachea, pulmonary parenchyma and pulmonary vessels. Congenital cystic adenomatoid malformation and pulmonary sequestration are the two main types. With the development of endoscopic technology, congenital pulmonary dysplasia has been alleviated in an increasing number of infants through thoracoscopic surgery. However, the timing of the operation is controversial.Methods: Seventy-two infants with congenital cystic lung disease who underwent thoracoscopic lobectomy from March 2017 to April 2020 were selected as subjects. The data were summarized and analysed, including preoperative complications, operative situation, postoperative recovery, postoperative hospital stay time, etc. All children were followed regularly.Results: Seventy-two patients were aged 3 to 7 months, with an average age of 5.40±2.02 months: 44 were male, and 28 were female, with an average weight of 6.95±1.96 kg. Preoperative condition: 48 patients were observed and operated on when they were between 5 and 7 months old. Twenty-four patients were diagnosed with pneumonia, and thoracoscopic surgery was performed after anti-infective therapy. All children were diagnosed with congenital cystic pulmonary disease with a CT scan before the operation. Postoperative pathological diagnosis showed 50 cases of congenital cystic adenomatoid malformation, 16 cases of intralobar pulmonary sequestration, and 6 cases of both congenital cystic adenomatoid malformation and intralobar pulmonary sequestration. Anaesthesia was performed by artificial pneumothorax or bronchial occlusion. The average operative time was 92.17±19.65 min, and the average length of postoperative ventilator assistance was 28.18±11.01 hours. Complications: 3 cases of pneumothorax after the operation and 5 cases of atelectasis, and the incidence of pneumonia was 33%. No bleeding, bronchial pleural fistula or other serious complications were observed. All children were followed for 3 months to 2 years. The growth and development of all the children were not different from those of normal children.Conclusions: In this study, preoperative pneumonia accounted for 23.6% of children. Thoracoscopic lobectomy was effective and feasible for children with congenital cystic lung disease from 3 to 7 months of age.

scholarly journals Congenital cystic adenomatoid malformation of lung: A case report

2015 ◽  
Vol 10 (1) ◽  
pp. 41-42
Author(s):  
Asish Banerjee ◽  
Sumita Pal ◽  
Anish Chatterjee ◽  
A Pan
Keyword(s):  
Case Report ◽  
Respiratory Distress ◽  
Lung Lesion ◽  
Congenital Cystic Adenomatoid Malformation ◽  
Medical Sciences ◽  
Cystic Lung ◽  
Cystic Adenomatoid Malformation ◽  
Cystic Lung Lesion ◽  
The Right ◽  
Tomography Scan

A term neonate developed respiratory distress after 12 hours of birth which was diagnosed as a case of congenital cystic adenomatoid malformation (CCAM) of the right lung by computerized tomography scan. CCAM of the lung is rare congenital cystic lung lesion. DOI: http://dx.doi.org/10.3126/jcmsn.v10i1.12767 Journal of College of Medical Sciences-Nepal, 2014, Vol.10(1); 41-42

scholarly journals It is not pneumothorax: Multicystic lung lesion in a six-year-old child

2016 ◽  
Vol 26 (2) ◽  
pp. 121-124
Author(s):  
Eman Sobh ◽  
Amira Bahour ◽  
Shady Elia Anis
Keyword(s):  
Surgical Resection ◽  
Lung Development ◽  
Lung Lesion ◽  
Congenital Cystic Adenomatoid Malformation ◽  
Congenital Pulmonary Airway Malformation ◽  
Cystic Lung ◽  
Cystic Adenomatoid Malformation ◽  
Pulmonary Airway ◽  
Cystic Lung Lesion

Congenital cystic adenomatoid malformation (CCAM) – recently reported as congenital pulmonary airway malformation – is a rare abnormality of lung development. We present a case of a six-year-old child having a cystic lung lesion that proved to be CCAM after surgical resection. The child is performing well on follow-up.

Extralobar Sequestration with Frequently Associated Congenital Cystic Adenomatoid Malformation, Type 2: Report of 50 Cases

1999 ◽  
Vol 2 (5) ◽  
pp. 454-463 ◽  
Author(s):  
Richard M. Conran ◽  
J. Thomas Stocker
Keyword(s):  
Pulmonary Sequestration ◽  
Posterior Mediastinum ◽  
Anterior Mediastinum ◽  
Normal Lung ◽  
Congenital Cystic Adenomatoid Malformation ◽  
Left Hemithorax ◽  
Cystic Adenomatoid Malformation ◽  
Pulmonary Parenchyma ◽  
The Right

Extralobar pulmonary sequestration (ELS) represents a mass of pulmonary parenchyma separate from the normal lung. The coexistence of congenital cystic adenomatoid malformation (CCAM) in ELS has been reported. To define this association, the clinical, gross, and histologic features of 50 ELS cases were analyzed. The age at diagnosis varied from birth to 65 years with 24% of cases diagnosed prenatally and 61% (23/38) diagnosed within the first 3 months of life. Fifty-two percent of cases were in females and 48% in males. Forty-eight percent of ELS(s) were located in the left hemithorax, 20% in the right hemithorax, 8% in the anterior mediastinum, 6% in the posterior mediastinum, and 18% beneath the diaphragm. The blood supply to the sequestration in 77% of cases was directly from the aorta. Grossly, the lung, though hypoplastic in some cases, was otherwise unremarkable. Fifty percent (23/46) of ELS cases were associated with a coexistent CCAM. In contrast to the series as a whole, 92% (11/12) of the ELS/CCAM cases, excluding those prenatally diagnosed, were diagnosed within the first 3 months and 57% occurred in females. ELS/CCAM lesions, while randomly distributed, were more frequently seen on the left side. Gross features of the ELS/CCAM cases were similar to those cases with ELS alone. All CCAM cases had a type 2 pattern on histologic examination with 48% of those cases also displaying rhabdomyomatous dysgenesis. Our findings indicate that the occurrence of CCAM in ELS is more frequent than reported in the literature and differs in presentation from ELS cases not associated with CCAM.

Ultrastructure and Staining of Developing Elastic Tissue

1971 ◽  
Vol 29 ◽  
pp. 244-245
Author(s):  
E. N. Albert
Keyword(s):  
Fine Structure ◽  
Phosphotungstic Acid ◽  
Staining Method ◽  
Rat Aorta ◽  
Uranyl Acetate ◽  
The Other ◽  
Elastic Fibers ◽  
Elastic Tissue ◽  
Lead Citrate ◽  
New Born

Silver tetraphenylporphine sulfonate (Ag-TPPS) was synthesized in this laboratory and used as an electron dense stain for elastic tissue (Fig 1). The procedures for the synthesis of tetraphenylporphine sulfonate and the staining method for mature elastic tissue have been described previously.The fine structure of developing elastic tissue was observed in fetal and new born rat aorta using tetraphenylporphine sulfonate, phosphotungstic acid, uranyl acetate and lead citrate. The newly forming elastica consisted of two morphologically distinct components. These were a central amorphous and a peripheral fibrous. The ratio of the central amorphous and the peripheral fibrillar portion changed in favor of the former with increasing age.It was also observed that the staining properties of the two components were entirely different. The peripheral fibrous component stained with uranyl acetate and/or lead citrate while the central amorphous portion demonstrated no affinity for these stains. On the other hand, the central amorphous portion of developing elastic fibers stained vigorously with silver tetraphenylporphine sulfonate, while the fibrillar part did not (compare figs 2, 3, 4). Based upon the above observations it is proposed that developing elastica consists of two components that are morphologically and chemically different.

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