Cystic Adenomatoid Malformation 

  • Author: Anne E Stone, MD; Chief Editor: Michael R Bye, MD   more...
 
Updated: Nov 23, 2011
 

Background

Congenital cystic adenomatoid malformation (CCAM) is a rare abnormality of lung development. CCAM is a cystic area within the lung that stems from abnormal embryogenesis. An adenomatous overgrowth of the terminal bronchioles with a consequent reduction in alveolar growth occurs.[1]

The routine use of prenatal ultrasonography has led to frequent prenatal diagnosis and has provided great insight into the natural history of CCAM. Improvements in surgical techniques (ie, both prenatal and postnatal) as well as greatly enhanced imaging modalities have altered the surgical approach to this lesion.

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Pathophysiology

The pathophysiologic effects of CCAM may be divided into prenatal and postnatal effects. Large lesions may be associated with the development of hydrops fetalis in as many as 40% cases and is a poor prognostic sign. Hydrops is thought to arise from compression of the inferior vena cava, which compromises venous return and leads to a decrease in cardiac output and the development of effusions. Fetal demise may result; premature delivery is attempted in order to salvage the fetus.[2] The other main prenatal event is compromised pulmonary growth. Resultant pulmonary hypoplasia may lead to the postnatal development of respiratory distress.

Polyhydramnios has also been associated with CCAM. This develops as a result of elevated intrathoracic pressure that leads to esophageal compression and the inability to swallow.[3]

CCAM may remain undiagnosed until it is discovered as an incidental finding later in life; however, its usual postnatal presentation is respiratory distress in the newborn period. This may be due to pulmonary hypoplasia, mediastinal shift, spontaneous pneumothorax, and pleural effusions secondary to hydrops. Recurrent chest infections may be a feature later in life.[4] A risk of malignant transformation in later years is noted.[5]

Prenatal regression and complete prenatal resolution have also been described.[5, 6, 7]

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Epidemiology

Frequency

United States

No data are available regarding the frequency of this lesion; however, it is a rare condition.

International

A review of 48 cases from 5 centers in Canada led to an estimated incidence of 1:25,000 to 1:35,000 of patients who were prenatally diagnosed.[6] The use of prenatal ultrasonography has led to an increase in prenatal diagnosis.

Mortality/Morbidity

  • Most series report a mortality rate of 25-30% of all children who present in the newborn period with CCAM; however, these figures do not include asymptomatic children who present later in life. Furthermore, the use of elective abortion may lead to an underestimation of perinatal mortality by preferentially terminating fetuses with a higher risk of mortality. The reported mortality rate of prenatally diagnosed CCAMs ranges from 9-49%.
  • Risk factors for a poor outcome include hydrops fetalis.[2, 6] Other indicators of poor prognosis include the type of lesion; microcystic CCAM is associated with much poorer outcomes.[6, 8]
  • The overall size of the lesion has also been reported as being an important predictor of survival;[9, 10] however, this index may be compromised by the fact that CCAM may decrease in size or even resolve over time in utero.[6] The major morbidity is related to pulmonary compromise. A large lesion may be associated with pulmonary hypoplasia. This can cause respiratory distress at birth.
  • Some authorities have suggested that the presence of bilateral lesions is associated with a worse outcome. More controversially, left-sided lesions may be associated with a greater mortality rate than right-sided lesions.
  • One study suggested that polyhydramnios is also associated with a poorer outcome.
  • The potential for malignant transformation is recognized in all cases of CCAM.[5, 11, 12] Whether or not complete resection of the affected area completely removes this risk is not known.
  • Other complications that have been described include the development of spontaneous pneumothorax, hemopneumothorax, and associated hemoptysis.[6]

Age

CCAM is a congenital condition. Cases are typically identified prenatally by routine ultrasonography screening.[6] Most postnatally identified cases present in the newborn period. CCAM may present in the older child and adult as an incidental finding or secondary to repeated infection.[13, 4, 14]

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Contributor Information and Disclosures
Author

Anne E Stone, MD  Fellow, Division of Pediatric Pulmonary Medicine, Columbia University Medical Center, Children's Hospital of New York-Presbyterian

Anne E Stone, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, and American Thoracic Society

Disclosure: Nothing to disclose.

Coauthor(s)

Michael R Bye, MD  Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons; Attending Physician, Pediatric Pulmonary Medicine, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center

Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, and American Thoracic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Thomas Scanlin, MD  Chief, Division of Pulmonary Medicine and Cystic Fibrosis Center, Department of Pediatrics, University of Medicine and Dentistry of New Jersey, Robert Wood Johnson Medical School

Thomas Scanlin, MD is a member of the following medical societies: American Association for the Advancement of Science, American Society for Biochemistry and Molecular Biology, American Thoracic Society, Society for Pediatric Research, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Charles Callahan, DO  Professor, Deputy Chief of Clinical Services, Walter Reed Army Medical Center

Charles Callahan, DO is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American College of Osteopathic Pediatricians, American Thoracic Society, Association of Military Surgeons of the US, and Christian Medical & Dental Society

Disclosure: Nothing to disclose.

Mary E Cataletto, MD  Director of Children's Sleep Services, Winthrop Sleep Disorders Center, Mineola, NY; Professor of Clinical Pediatrics, State University of New York at Stony Brook, Stony Brook, NY

Mary E Cataletto, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Chest Physicians

Disclosure: Shering Plough Pharmaceuticals Honoraria Consulting

Chief Editor

Michael R Bye, MD  Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons; Attending Physician, Pediatric Pulmonary Medicine, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center

Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, and American Thoracic Society

Disclosure: Nothing to disclose.

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The CT scan of the chest reveals a right lower lobe congenital cystic adenomatoid malformation (CCAM) in a 6-week-old infant who presented with tachypnea. The most striking feature is the solitary enlarged cyst surrounded by numerous microcysts. This lesion was resected without complication.
 
 
 
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