eMedicine Specialties > Pediatrics: General Medicine > Pulmonology

Cystic Adenomatoid Malformation: Follow-up

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

Follow-up

Further Inpatient Care

  • Respiratory support may be required before and after resection of the congenital cystic adenomatoid malformation (CCAM).
  • Complications arising as a result of the surgery must be treated (eg, bleeding, infection, analgesia).

Further Outpatient Care

  • Generally, following resection of the CCAM, anticipate full recovery.
  • Regular follow-up in patients who present after adolescence may be necessary in order to detect the presence of an associated malignancy.11,12
  • In patients who underwent fetal surgery or postnatal resection of CCAM, no clinical evidence of respiratory problems is anticipated; however, a small study has shown that a slight reduction in lung volumes occurred in children who had CCAM postnatally resected.6,23  Few reports of long-term outcomes are available.

Inpatient & Outpatient Medications

  • Antibiotics are necessary in cases with recurrent pneumonia. Otherwise, no medications are specifically indicated for therapy of CCAM.

Transfer

  • Transfer is indicated in cases of CCAM with hydrops to a facility with expertise in fetal surgery.
  • Deliver the baby in a level III NICU.
  • If the infant is delivered in a facility unskilled in the management of critically ill neonates or in cases of CCAM undiagnosed in the antenatal period, transfer these infants to a facility with expertise in neonatology and neonatal surgery.
  • Additionally, the receiving institution should have the capability to offer ECMO.3

Deterrence/Prevention

  • As with all children, particularly those with a history of pulmonary problems, avoid smoking by and around the child.
  • In the absence of any known risk factors for the development of CCAM, no advice regarding prepregnancy preventive measures can be provided.

Complications

  • Fetal death caused by hydrops, fetal surgery, prematurity, or associated malformations
  • Premature delivery due to polyhydramnios.
  • Respiratory distress due to hydrops, pulmonary hypoplasia, pulmonary hypertension, pneumothorax, or prematurity
  • Postnatal death due to respiratory distress, untreated hydrops, or pulmonary hypertension
  • Recurrent pneumonia13
  • Pneumothorax
  • Hemothorax
  • Malignant change: Rhabdomyosarcoma, pulmonary blastomas, minute squamous cell carcinoma, and bronchioloalveolar carcinoma have all been described in association with CCAM.11,12

Prognosis

  • The risk of mortality in fetuses with hydrops is high.2
  • Other indicators of poor prognosis include the type of lesion, with microcystic CCAM associated with much poorer outcomes.2
  • The overall size of the lesion has also been reported as being an important predictor of survival; however, this index may be compromised by the fact that CCAM may undergo involution and even disappear in utero.10,24
  • Polyhydramnios is also associated with a poorer outcome.2

Patient Education

  • Fully inform the parents of an affected fetus and discuss the prognosis and natural history of this condition, including the possibility of fetal demise, postnatal respiratory failure, and need for surgery and its attendant complications.
  • Discourage prenatal and postnatal smoking.
  • In children who have had an uncomplicated resection of CCAM, no residual effects (based on currently available data) are anticipated.
  • Less data are available regarding the longer-term outcomes in children who underwent fetal surgery.

Miscellaneous

Medicolegal Pitfalls

  • Failure to diagnose this condition prenatally
  • Failure to diagnose associated conditions
  • Failure to offer skilled intervention, such as fetal surgery, in high-risk cases
 


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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