eMedicine Specialties > Pediatrics: General Medicine > Pulmonology

Cystic Adenomatoid Malformation: Treatment & Medication

Author: Anne E Stone, MD, Fellow, Division of Pediatric Pulmonary Medicine, Columbia University Medical Center, Children's Hospital of New York-Presbyterian
Coauthor(s): Michael R Bye, MD, Attending Physician, Pediatric Pulmonary Medicine, Columbia University Medical Center; Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons; David J Vaughan, MBBCh, Consultant Pediatrician, Department of Pediatrics, Our Lady of Lourdes Hospital, Ireland; Jerry Zimmerman, MD, PhD, Professor, Department of Pediatrics/Anesthesia, University of Washington School of Medicine; Director, Division of Pediatric Critical Care Medicine, Children's Hospital of Seattle
Contributor Information and Disclosures

Updated: Aug 1, 2008

Treatment

Medical Care

No specific medical therapies are described for congenital cystic adenomatoid malformation (CCAM), aside from antibiotics in children with CCAM complicated by pneumonia and supportive care, ranging from oxygen supplementation to mechanical ventilation, in older children with respiratory distress.

Surgical Care

Surgical intervention is the mainstay of therapy for CCAM including fetal surgery and postnatal surgical approaches.

  • Fetal intervention
    • Fetal surgery should be considered in patients with large CCAMs and in cases complicated by hydrops, in which prognosis is poor.2
    • Thoracocentesis allows drainage of a large cyst with immediate decompression of the CCAM; however, fluid rapidly reaccumulates, thus negating the benefit of the procedure.2
    • Another option is to place a thoracoamniotic shunt that continually drains fluid from the CCAM to the amniotic space. This is most beneficial when the CCAM consists of a large fluid-filled cyst. Complications such as obstruction and shunt dislodgement may occur.2
    • Resection of the affected lobe (lobectomy) is an alternative procedure for cases with no dominant cyst available for draining. Fourteen cases treated with maternal-fetal surgery from the Children's Hospital of Philadelphia suggest a 50% survival rate from the time of surgical intervention to infant discharge from the neonatal intensive care unit (NICU).20 Complications included intraoperative bradycardia, the development of preterm labor and maternal mirror syndrome requiring early delivery, and postoperative intrauterine death. Survivors demonstrated residual lung growth and normal development.
  • Postnatal surgery: Resection of CCAM in all children is recommended to remove the risk of direct complications, such as recurrent infection and pneumothorax. Additionally, the malignant potential of CCAM in later life has long been recognized.12,3

Consultations

  • Fetal surgeon: Referral to a facility with expertise in fetal surgery is warranted for all fetuses diagnosed with CCAM and hydrops. The indications for referral of other affected pregnancies without hydrops are less clear3 .
  • Pediatric surgeon: If fetal surgery is not indicated, close collaboration with a pediatric surgeon is essential because postnatal resection of the CCAM is necessary. Rarely, respiratory failure resulting from the CCAM may be severe enough to require treatment with ECMO.
  • Neonatologist: Regardless of whether fetal surgery has been performed, delivery of the affected fetus in a tertiary level facility is essential to optimize outcome and minimize complications arising as a result of CCAM.

Diet

No specific diet is necessary.

Activity

Generally, activity is not limited. Patients with an unresected CCAM (who are at risk of pneumothorax) should not partake in any activity that may put them at risk of developing a pneumothorax (eg, diving, unpressurized air travel).

Medication

Antibiotics are necessary in cases with recurrent pneumonia. Otherwise, no medications are specifically indicated for therapy of congenial cystic adenomatoid malformation (CCAM).

More on Cystic Adenomatoid Malformation

Overview: Cystic Adenomatoid Malformation
Differential Diagnoses & Workup: Cystic Adenomatoid Malformation
Treatment & Medication: Cystic Adenomatoid Malformation
Follow-up: Cystic Adenomatoid Malformation
Multimedia: Cystic Adenomatoid Malformation
References
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References

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Further Reading

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Keywords

congenital cystic adenomatoid malformation, CCAM, hydrops fetalis, microcystic congenital cystic adenomatoid malformation, microcystic CCAM, macrocystic congenital cystic adenomatoid malformation, macrocystic CCAM, pulmonary hypoplasia, hamartoma, premature delivery, polyhydramnios, respiratory distress, mediastinal shift, spontaneous pneumothorax, recurrent chest infection, pulmonary compromise, hemoptysis, failure to thrive

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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, Attending Physician, Pediatric Pulmonary Medicine, Columbia University Medical Center; Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons
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: Merck Honoraria Speaking and teaching

David J Vaughan, MBBCh, Consultant Pediatrician, Department of Pediatrics, Our Lady of Lourdes Hospital, Ireland
David J Vaughan, MBBCh is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

Jerry Zimmerman, MD, PhD, Professor, Department of Pediatrics/Anesthesia, University of Washington School of Medicine; Director, Division of Pediatric Critical Care Medicine, Children's Hospital of Seattle
Jerry Zimmerman, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, Society for Pediatric Research, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

Medical Editor

Thomas Scanlin, MD, Chief, Division of Pediatric Pulmonary & Cystic Fibrosis, Assistant Professor, Department of Pediatrics, Robert Wood Johnson University Medical Group
Thomas Scanlin, MD is a member of the following medical societies: American Thoracic Society and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

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.

CME Editor

Mary E Cataletto, MD, Associate Director, Division of Pediatric Pulmonology, Winthrop University Hospital; Professor of Clinical Pediatrics, State University of New York at Stony Brook; Director of Children's Sleep Services, Winthrop University Hospital
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, Attending Physician, Pediatric Pulmonary Medicine, Columbia University Medical Center; Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons
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: Merck Honoraria Speaking and teaching

 
 
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