congenital cystic adenomatoid malformation


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congenital cystic adenomatoid malformation

A rare developmental defect of the lungs seen primarily in infants, which displays both immaturity and malformation of the small airways and parenchyma distal to it. Pulmonary sequestration can be difficult to distinguish from congenital cystic adenomatoid malformation (CCAM), but the presence of a feeding artery from the systemic circulation is helpful.

Types
Type 0: Very rare, incompatible with life; presents as a solid mass containing bronchial-like structures and abundant cartilage.
Type 1: Most common type; contains large, multilocular cysts with broad fibrous septa, mucinous cells and focal cartilage.
Type 2: Contains small, cystically dilated bronchiolar structures (usually <2.0 cm) lined by respiratory epithelium; wall may contain striated muscle.
Type 3: Presents as a solid lesion with minute cysts (usually <0.2 cm) lined by cuboidal epithelium, surrounded by immature, fetal-type parenchyma.
Type 4: Has large cysts that are peripheral and are lined by type-1 pneumocytes and low columnar epithelium and have hypercellular stroma.

Congenital cystic adenomatoid malformation (CCAM)

A condition in which one or more lobes of the fetal lungs develop into fluid-filled sacs called cysts.
Mentioned in: Prenatal Surgery
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References in periodicals archive ?
Early in the pregnancy, doctors diagnosed baby boy Cabellotrejo with a very large lung mass, called congenital cystic adenomatoid malformation, or CCAM for short.
Congenital cystic diseases of the lung and mediastinum include congenital cystic adenomatoid malformation (CCAM), bronchopulmonary "sequestration", congenital lobar emphysema, broncogenic cyst, esophageal duplication cysts and neurogenic cysts (1).
Congenital cystic adenomatoid malformation is an embrionic developmental disorder characterized by cystic enlargement and overgrowth of terminal bronchioles which are surrounded by various epithelial cells.
Congenital cystic adenomatoid malformation (CCAM) of the lungs is a developmental abnormality (hamartoma) (1).
Jennings discussed a variety of fetal anomalies that have been successfully treated at the center, including hypoplastic left heart syndrome, congenital cystic adenomatoid malformation, cervical teratoma, bronchogenic cyst, and congenital diaphragmatic hernia.
The EXIT to ECMO procedure has also been used in cases where a congenital cystic adenomatoid malformation threatened to compromise lung development and function through mechanical occupation of the thoracic space.
Congenital cystic adenomatoid malformation of the lung (CCAM) is an uncommon condition characterized by cysts of various sizes lined by bronchiolar- or alveolar-like epithelium.
Differential diagnostic considerations vary based on the time of imaging and include pneumothorax, pneumatocele, atelectasis, or hypoplasia of the lung with hyperinflation of the contralateral lung, diaphragmatic hernia, and congenital cystic adenomatoid malformation.
The radiologic diagnosis of congenital cystic adenomatoid malformation (CCAM) was confirmed pathologically and subclassified as type 3.
One of the most common conditions treated at the center is congenital cystic adenomatoid malformation (CCAM), a multi-cystic mass of pulmonary tissue with proliferation to the bronchial structures that is usually restricted to one lobe of the lung.
Congenital cystic adenomatoid malformations (CCAM) are the most commonly diagnosed prenatal lung malformations and account for approximately 30% to 40% of all congenital anomalies.

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