Cystic Adenomatoid Malformation Follow-up

Updated: Dec 08, 2015
  • Author: Anne E Stone, MD; Chief Editor: Michael R Bye, MD  more...
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Follow-up

Further Outpatient Care

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  • 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. [15, 16]
  • 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. [7, 32] Few reports of long-term outcomes are available.
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Further Inpatient Care

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  • 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).
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Inpatient & Outpatient Medications

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

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  • 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. [4]
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Deterrence/Prevention

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  • 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.
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Complications

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  • 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 pneumonia [17]
  • Pneumothorax
  • Hemothorax
  • Malignant change: Rhabdomyosarcoma, pulmonary blastomas, minute squamous cell carcinoma, and bronchioloalveolar carcinoma have all been described in association with CCAM. [15, 16]
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Prognosis

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  • The risk of mortality in fetuses with hydrops is high. [3]
  • Other indicators of poor prognosis include the type of lesion, with microcystic CCAM associated with much poorer outcomes. [3]
  • 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. [14, 33]
  • Polyhydramnios is also associated with a poorer outcome. [3]
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Patient Education

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  • 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.
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