Elsevier

Early Human Development

Volume 90, Issue 12, December 2014, Pages 935-939
Early Human Development

Best practice guidelines
Congenital cystic lesions of the lung

https://doi.org/10.1016/j.earlhumdev.2014.09.014Get rights and content

Abstract

Congenital cystic lesions of the lung are present in 1 in 10,000–35,000 births and present as a spectrum of anomalies. Majority of these cystic lesions comprise congenital cystic adenomatoid malformations, pulmonary sequestrations, congenital lobar emphysema, and bronchogenic cysts. Most of these lesions are nowadays detected antenatally, however some will present either in the newborn or during later childhood. A review of the aetiology, classification, natural history, investigations, and treatment of congenital cystic lung lesions is discussed.

Section snippets

Congenital cystic adenomatoid malformations

Congenital cystic adenomatoid malformations (CCAM) are characterised by multicystic areas of over-proliferation and dilatation of terminal respiratory bronchioles with lack of normal alveoli. CCAM are intrapulmonary lesions that contain various types of epithelial linings and maintain communication with the normal trachea–bronchial tree and retain a normal blood supply. They are usually unilobar and unilateral. The reported incidence is between 1 in 10,000 to 1 in 35,000 with no predilection

Pulmonary sequestration

Pulmonary sequestrations are portions of the lung that are in isolation from its neighbouring lung tissues — it has no communication with the bronchial tree and receives its blood supply from a systemic artery, and venous drainage may occur through either a pulmonary or systemic vein. [Fig. 2] It is estimated to represent up to 6% of congenital lung anomalies. Pulmonary sequestrations can be divided into two categories: intralobar (15%) and extralobar sequestrations (85%).

The aetiology of these

Congenital lobar emphysema

Congenital lobar emphysema is identified as hyperinflation of one or more pulmonary lobes and leads compression of surrounding structures. [Fig. 3] This compression of may lead to mediastinal shift. Most commonly the left upper or right middle lobes are affected. The aetiology of congenital lobar emphysema is idiopathic in half of cases, whereas the other 50% have several mechanisms proposed to explain the air-trapping, which can be divided into intrinsic and extrinsic subtypes. Intrinsic

Bronchogenic cyst

Bronchogenic cysts are usually single, unilocular cysts filled with fluid or mucous, however when located in the lung parenchyma they may be multiple. They are lined by pseudostratified ciliated columnar respiratory epithelium and contain hyaline cartilage plates. Bronchogenic cysts most commonly occur in the mediastinum, although they may occur anywhere throughout the thoracic cavity or in such places as the retroperitoneum, neck, tongue, and subcutaneous tissue. When located in lung

Conclusion

Pulmonary sequestrations, bronchogenic cysts, congenital lobar emphysema, and congenital cystic adenomatoid malformations represent a spectrum of congenital cystic lung lesions that may present with symptoms ranging from foetal demise to chronic cough/infections to incidental detection in the asymptomatic child. Although the detection of these lesions has dramatically improved with the use of antenatal ultrasound and postnatal imaging, there remains controversy over the pathophysiology of these

Key guidelines

  • Congenital cystic lesions of the lung comprise a spectrum of anomalies that include pulmonary sequestrations, congenital lobar emphysema, bronchogenic cysts, and congenital cystic adenomatoid malformations.

  • Understanding of the antenatal and postnatal presentation of these lesions is important for investigations, diagnosis, and treatment

  • Congenital cystic lesions of the lung are most commonly identified on antenatal ultrasound and treatment is controversial.

Conflict of interest statement

The authors declare that there are no financial or personal relationships with other people or organisations that could inappropriately influence this work.

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    Citation Excerpt :

    Those infants who remain asymptomatic at birth are not recommended to undergo resection and may be observed clinically for progression of symptoms.1,4,8,33 For infants with BC, asymptomatic lesions are still recommended to undergo surgical resection due to risk of further infections, persistent exponential growth rate as children age, and the potential for malignant transformation.4,28 Malignant transformation of CPAMs is a concern and has been used as an argument for resection.

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