Cystic Adenomatoid Malformation Treatment & Management
- Author: Anne E Stone, MD; Chief Editor: Michael R Bye, MD more...
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 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.
- Thoracocentesis allows drainage of a large cyst with immediate decompression of the CCAM; however, fluid rapidly reaccumulates, thus negating the benefit of the procedure.
- 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.
- 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). 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.[16, 4] Children with asymptomatic CCAM that was diagnosed antenatally can be followed without surgical intervention as some lesions may decrease in size or resolve without intervention. If surgery is recommended, most suggest it be completed before the child is aged 12 months to enhance compensatory lung growth. Studies have not confirmed this hypothetical difference in lung function by age at time of surgical resection.
- Older patients: In one third of cases, the presence of pneumonia indicates the need for more extensive pulmonary resection.
See the list below:
- 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 clear .
- 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.
No specific diet is necessary.
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).
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