User:Mr. Ibrahem/Congenital pulmonary airway malformation
| Congenital pulmonary airway malformation | |
|---|---|
| Other names | Congenital cystic adenomatoid malformation (CCAM)[1] |
 | |
| Specialty | Medical genetics |
| Symptoms | None, increased work of breathing, bluish skin, swelling[2] |
| Complications | Pneumonia, pneumothorax, cancer[2] |
| Usual onset | Present at birth[2] |
| Types | Type 0 to 4[2] |
| Causes | Unknown[3] |
| Diagnostic method | Ultrasound before birth[2] |
| Differential diagnosis | Pulmonary sequestration, congenital lobar emphysema, pleuropulmonary blastoma[4] |
| Treatment | Conservative, surgery[2] |
| Frequency | 1 in 23,000 newborns[2] |
Congenital pulmonary airway malformation (CPAM) is a birth defect of the lung.[2] Symptoms can vary from none to increased work of breathing, bluish skin, and swelling.[2] Complications may include pneumonia, pneumothorax, or cancer.[2] About 5% of those affected are stillborn.[4]
The cause is generally unknown.[3][4] The underlying mechanism involves the formation of non functional lung tissue during early development.[2] There are five types: with type 1 being the most common and affecting one lobe of the lung; and type 2 being the next most common and occurring with multiple small cysts.[2] Other types are less common.[2] It is classified as a congenital thoracic malformation along with bronchogenic cysts and pulmonary sequestration.[2] Diagnosis is often by ultrasound before birth.[2]
Diagnosis before birth may allow the use of steroids or fetal surgery to improve outcomes.[2][3] If symptoms occur after birth or more than 20% of a lung is involved, surgery is generally recommended.[2] Otherwise either surgery or conservative management may be recommended.[2] Type 1 generally has a good outcome, while type 2 generally has a poor outcome.[2]
CPAM is rare, occurs in 1 in 10,000 to 35,000 newborns.[2] It is the most common birth defect affected the lungs.[2] Males are affected more often than females.[3] CPAM was first described in 1949 and divided into 5 types in 1977.[4][5]
References[edit]
- ^ "Orphanet: Cystic adenomatoid malformation of the lung". www.orpha.net. Archived from the original on 28 August 2021. Retrieved 10 February 2021.
- ^ a b c d e f g h i j k l m n o p q r s t u Mehta, PA; Sharma, G (January 2020). "Congenital Pulmonary Airway Malformation". PMID 31869128.
{{cite journal}}: Cite journal requires|journal=(help) - ^ a b c d "Congenital Pulmonary Airway Malformation (CPAM) | Fetal Care Center". www.cincinnatichildrens.org. Archived from the original on 16 January 2021. Retrieved 11 February 2021.
- ^ a b c d Davenport, Mark; Coppi, Paolo De (2021). Paediatric Surgery. Oxford University Press. p. 144. ISBN 978-0-19-879869-9. Archived from the original on 2021-08-28. Retrieved 2021-02-11.
- ^ Sellke, Frank; Nido, Pedro J. del; Swanson, Scott J. (2015). Sabiston and Spencer Surgery of the Chest E-Book. Elsevier Health Sciences. p. 166. ISBN 978-0-323-39377-5. Archived from the original on 2021-08-28. Retrieved 2021-02-11.