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Pediatrics: General Medicine > Pulmonology
Cystic Adenomatoid Malformation
Article Last Updated: Jul 7, 2006
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
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 is a member of the following medical societies: American Academy of Pediatrics and American College of Chest Physicians
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
Editors: Thomas Scanlin, MD, Chief, Division of Pediatric Pulmonary & Cystic Fibrosis, Assistant Professor, Department of Pediatrics, Robert Wood Johnson University Medical Group; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Charles Callahan, DO, Professor, Deputy Chief of Clinical Services, Walter Reed Army Medical Center; Mary E Cataletto, MD, Associate Director, Division of Pediatric Pulmonology, Winthrop University Hospital; Associate Professor, Department of Clinical Pediatrics, State University of New York at Stony Brook; 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
Author and Editor Disclosure
Synonyms and related keywords:
congenital cystic adenomatoid malformation, CCAM, hydrops, microcystic congenital cystic adenomatoid malformation, microcystic CCAM, macrocystic congenital cystic adenomatoid malformation, macrocystic CCAM, pulmonary hypoplasia, hydrops, hamartoma
Background
Congenital cystic adenomatoid malformation (CCAM) is a rare abnormality of lung development. As its name suggests, CCAM is a cystic area within the lung stemming from abnormal embryogenesis during weeks 7-35 of gestation. An adenomatous overgrowth of the terminal bronchioles with a consequent reduction in alveolar growth occurs. CCAM was first described in 1949. The routine use of antenatal ultrasound scanning has led to frequent antenatal diagnosis and has provided great insight into the natural history of CCAM. Improvements in surgical techniques (ie, both antenatal and postnatal) as well as greatly enhanced imaging modalities have altered the surgical approach to this lesion.
Pathophysiology
The pathophysiologic effects of CCAM may be divided into antenatal 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, or premature delivery is attempted in order to salvage the fetus. The other main antenatal event is that of compromised pulmonary growth. Resultant pulmonary hypoplasia may lead to the postnatal development of respiratory distress.
Polyhydramnios has been associated with CCAM as well. This develops as a result of elevated intrathoracic pressure that leads to esophageal compression and the inability to swallow.
CCAM may remain undiagnosed until 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. A risk of malignant transformation in later years is noted.
Antenatal regression and complete antenatal resolution have also been described.
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 antenatally diagnosed. An 8-year review of cases of CCAM in England reported 58 fetuses affected with this condition. As with all anomalies diagnosed prenatally, elective termination of pregnancy may compromise the accuracy of these figures.
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 mortality of antenatally diagnosed CCAMs has been reported with variability ranging from 9-49%.
- Risk factors for a poor outcome include the presence of hydrops fetalis. Risk of mortality in fetuses with hydrops is as high as 80-90%. Other indicators of poor prognosis include the type of lesion, with microcystic CCAM associated with much poorer outcomes. 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 decrease in size or even resolve over time in utero.
- 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 major morbidity is related to pulmonary compromise. A large lesion may be associated with pulmonary hypoplasia. This can cause respiratory distress at birth. Approximately two thirds of patients who are postnatally diagnosed present with respiratory symptoms during the newborn period. Factors that determine the severity of respiratory distress include the degree of lung hypoplasia and the presence or absence of significant mediastinal shift. Iatrogenic morbidity and mortality may also occur. Recurrent infection in later life has commonly been described.
- The potential for malignant transformation exists in all cases of CCAM. 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.
- Eighteen percent of patients with CCAM have another congenital anomaly present. Renal agenesis and cardiac conditions are the most common associated anomalies. The association of CCAM and pulmonary sequestration, both extralobar and intralobar, has also been reported. Case reports of CCAM associated with hypoplasia of intrahepatic bile ducts exist.
- Little data are available regarding functional impairment (if any) in children who had lobectomy or other lung resecting surgery as infants. One small series described 8 children who underwent lobectomy. No impairment was present in these children, and pulmonary function testing showed lung volumes of 90% of predicted values (suggesting that compensatory growth of the remaining lung occurred) and normal gas exchange indices.
Age
CCAM is a congenital condition. Cases are typically identified antenatally by routine ultrasound screening. 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. Cases of patients who are asymptomatic throughout life, with lesions identified during postmortem examination, have been reported.
History
With the increasing use of antenatal ultrasound as well as improvement in technology and skill, most cases of CCAM are diagnosed antenatally. Pregnancy usually is uneventful, other than the occasional complication of polyhydramnios. A phenomenon known as the maternal mirror syndrome has been described in which fetal distress is associated with severe preeclampsia in the mother.
- Respiratory distress
- This is the presenting symptom in up to 80% of newborns with a diagnosis of CCAM. It may range in severity from grunting, tachypnea, and a mild oxygen requirement to fulminant respiratory failure requiring aggressive ventilator support or extracorporeal membrane oxygenation (ECMO).
- Multiple mechanisms account for the onset of respiratory difficulty. Pulmonary hypoplasia may arise as a consequence of a large CCAM, mediastinal shift may compromise cardiac and respiratory function, spontaneous pneumothoraces may occur, and air trapping within the cyst leads to compression of functional pulmonary tissue.
- Recurrent infection: Children in whom the CCAM has not been resected are at risk of recurrent pulmonary infections due to bronchial compression, air trapping, and inability to clear secretions.
- Hemoptysis: Hemoptysis has occasionally been described as a manifestation of CCAM in the older child.
- Dyspnea and chest pain: Dyspnea may be a feature of pneumothorax, which has been described as a presenting feature of CCAM.
- Miscellaneous: Cough, fever, and failure to thrive have all been reported in association with the presentation of CCAM.
Physical
Generally, the physical signs observed in children with CCAM are nonspecific.
- Tachypnea: Tachypnea is the most common sign encountered in the newborn period, reflecting respiratory distress.
- Pneumothorax/air trapping: Signs consistent with a pneumothorax or air trapping may be elicited, including tracheal deviation, which indicates mediastinal shift, shifted heart sounds, and decreased air entry on the affected side.
- Cyanosis
- Accessory muscle use
- Grunting
- Failure to thrive
Causes
The cause of CCAM is not understood.
- Based on postmortem studies, some pathologists have suggested that bronchial atresia is the primary event, leading to disordered lung growth beyond the atretic area. This manifests as multiple cystic areas of variable size. The cause of the bronchial atresia is thought to be secondary to an imbalance between cell proliferation and apoptosis.
- In vitro work has focused attention on an imbalance between epithelial and mesenchymal growth on one hand and vascular development in lung tissue of children with CCAM on the other hand. Studies have suggested that an abnormal expression of glial cell–derived neurotrophic factor (GDNF) in epithelial and endothelial cells from CCAMs may mediate this imbalance during embyrogenesis. CCAMs have also been noted to be poorly vascularized compared with normal lung tissue with an increased proliferative index. Studies have also investigated the role of HOXB5 gene and protein expression, as well as other growth factors such as mesenchymal platelet–derived growth factor-BB.
Congenital Diaphragmatic Hernia
Congenital Pneumonia
Hemothorax
Pleural Effusion
Pneumatocele
Pneumothorax
Pulmonary Sequestration
Other Problems to be Considered
Adenomatoid hamartoma
Fibromatosis
Adenomatoid hamartoma
Laryngeal atresia
Airway foreign body
Unilateral pulmonary agenesis
Congenital lobar emphysema
Lab Studies
- Laboratory studies are generally not helpful in the diagnosis of CCAM.
- Perform routine karyotyping on all amniotic fluid obtained or drawn from the child after birth; however, incidence of chromosomal anomalies associated with CCAM is extremely low.
Imaging Studies
- Chest radiography is essential in the workup of the child with suspected CCAM.
- Chest radiography almost invariably identifies CCAM of sufficient size to cause clinical problems.
- The usual appearance is of a mass containing air-filled cysts.
- Other radiological signs that may be evident include mediastinal shift, pleural and pericardial effusions, and pneumothoraces. The diagnosis may not be clear from chest radiography alone. Particularly in the case of type III lesions, chest radiography may demonstrate a mass without any evidence of cysts.
- In cases in which the cystic lesion involutes, chest radiography may not allow sufficient definition to determine whether the CCAM has disappeared completely.
- Computerized chest tomography
- CT scanning of the thorax provides a safe and rapid means of defining the extent of CCAM in all age groups.
- The typical appearance is of multilocular cystic lesions with thin walls surrounded by normal lung parenchyma. The presence of superimposed infection with the lesion may complicate the appearance.
- Air fluid levels may be evident.
- CT scanning of the chest may outline additional coexisting lesions. Series have suggested a small percentage of cases thought to have resolved antenatally that demonstrated persistence of lesions on CT scan.
- The definition of high-resolution chest tomography (HRCT) is sufficient to differentiate between microcystic and macrocystic lesions.
- Antenatal ultrasonography
- With increasing use and technical ability of antenatal ultrasonography and sonographers, most cases of congenital lung anomalies are diagnosed prenatally. No specific diagnostic features of CCAM exist that allow one to distinguish it unequivocally from other lung lesions such as congenital lobar emphysema or pulmonary sequestration.
- Ultrasonography may demonstrate evidence of hydrops, such as fetal ascites or pleural effusions.
- Type I lesions (see Histologic Findings) appear as multiple large cystic areas in the lung. In type II lesions, multiple small cysts are evident on ultrasonography. Because of the extremely small size of the cysts in type III lesions, the antenatal ultrasonographic appearance is often one of a homogenous mass.
- Magnetic resonance imaging
- The number of reports that describe the use of antenatal MRI is increasing. MRI permits increased definition of a particular lesion, thereby enhancing the clinician's ability to accurately diagnose and offer an informed prognosis.
- Additionally, maternal problems that prevent the optimal use of fetal ultrasonography, such as obesity, poor fetal lie, and oligohydramnios, pose no obstacle to MRI. MRI may be useful particularly in distinguishing CCAM from congenital diaphragmatic hernia.
- Lastly, no maternal or fetal exposure to ionizing radiation occurs in contrast to the use of CT scanning.
- The appearance of CCAM on MRI is determined by the size of the lesion as well as the number and size of the cysts. A greater number and volume of cysts leads to a greater T2-weighted signal intensity. In contrast to pulmonary sequestrations, which typically have a homogeneous appearance, CCAM usually demonstrates some degree of inhomogeneity because of the multiple cysts. In the postnatal period, the use of MRI may reveal evidence of CCAM that, by ultrasonography and chest radiography, appeared to involute completely; however, whether or not any benefit exists in using MRI in the postnatal period when compared to chest CT scanning is unclear.
- Perform renal and cerebral ultrasonography in all newborns with CCAM in order to exclude coexisting renal and CNS anomalies.
- Perform echocardiography in all newborns with CCAM to rule out any coexisting cardiac lesions. Furthermore, in infants with respiratory distress, echocardiography may provide evidence of persistent pulmonary hypertension (eg, right-to-left shunting, increased pulmonary artery pressures).
Procedures
- Amniocentesis may be indicated to obtain amniotic fluid for karyotyping.
Histologic Findings
CCAM has been described as a hamartoma, namely abnormal tissue with an excess of 1 or more tissue components. CCAMs generally communicate with the bronchial tree and derive their blood supply from the pulmonary circulation, in contrast to pulmonary sequestration, which derives its blood supply from the aorta.
In 1977, Stocker grossly classified CCAM into 3 types based on an analysis of 38 infants with CCAM. Type 1 includes multiple large cysts (>2 cm in diameter) or a single large cyst surrounded by numerous smaller cysts. Histologically, the predominant cell type is ciliated columnar epithelium. The walls consist of thick smooth muscle and elastic tissue. Type I is the most common type of CCAM and is associated with an excellent prognosis.
Type II CCAM is defined as a lesion with multiple small cysts, usually less than 1 cm in diameter. The cell type usually is columnar or cuboidal epithelium. The walls are predominantly composed of a thin muscular coat. Type II CCAM accounts for over 40% of cases of CCAM. In Stocker's series, the type II lesion was particularly associated with other lesions, specifically renal agenesis. Up to 60% of type 2 lesions are associated with other congenital anomalies that may affect prognosis.
Type III CCAMs are large and account for less than 5% of all cases. They consist of multiple microcysts, measuring less than 0.5 cm in diameter.
In 1999, Stocker reclassified CCAMs into 5 categories, adding types 0 and 4 (both rare). Type 0 includes very small cysts lined with ciliated pseudostratified epithelium from a tracheal origin. Type 4 consists of cysts with a maximum diameter of 0.7 cm with nonciliated, flattened, alveolar lining cells.
In 1993, Adzick reported his group system of classification. Microcystic lesions (cysts measuring <5 mm) were usually associated with fetal hydrops and, hence, a poor prognosis. Macrocystic lesions (ie, cysts > 5 mm) were not usually associated with hydrops and had a good prognosis.
Medical Care
No specific medical therapies are described for CCAM. Antibiotics are indicated for children with CCAM complicated by pneumonia. Support of the respiratory system, ranging from oxygen supplementation to mechanical ventilation, may be necessary in neonates with respiratory distress. High-frequency oscillatory ventilation has been reported as a therapeutic option in CCAM. Medical treatment of persistent pulmonary hypertension of the newborn (PPHN) that complicates CCAM may also be necessary. This can include chemical paralysis and sedation, nitric oxide, hyperventilation, and alkalization.
Surgical Care
Surgical intervention is the mainstay of therapy for CCAM. A number of options are available to the surgeon. These options include fetal surgery, termination of pregnancy, and postnatal surgical approaches. Fetal surgery is currently performed in a few select facilities, reflecting its extremely specialized nature.
- Fetal surgery
- Fetal surgery has evolved alongside the development of refinements in fetal imaging techniques. Its use is reserved for fetuses with conditions associated with an extremely poor prognosis.
- Two patients are involved in fetal surgery, the fetus and the pregnant mother. A multidisciplinary team with expertise in fetal surgery should evaluate each case.
- Preoperative workup includes detailed fetal ultrasonography to diagnose and define the extent of the lesion and to determine the presence of other anomalies. Also, an ultrafast CT scan is indicated. Fetal echocardiography is necessary to rule out a congenital heart lesion. Amniocentesis should be performed to rule out a major chromosomal anomaly.
- The major indication for fetal surgery of CCAM is the presence of hydrops and gestation less than 32 weeks. Hydrops is associated with an extremely high perinatal mortality rate. A number of options are available to the pediatric surgeon when considering fetal therapy.
- 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 in the situation in which 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.
- Absolute contraindications to fetal surgery include the presence of a significant chromosomal or structural anomaly, multiple pregnancies, maternal drug or nicotine abuse, and the presence of the maternal mirror syndrome.
- Chorioamniotic separation leading to the formation of amniotic bands, which may result in fetal trauma or loss, has also been described in the literature. In 1998, Adzick et al reported the results of their fetal surgery program in treating CCAM. One hundred and thirty-four fetuses with CCAM were identified. Fetal lobectomy was performed in 13 fetuses, all of whom had hydrops at the time. Eight fetuses (62%) survived to the neonatal period, with resolution of hydrops and appropriate lung growth. Of the 5 deaths, 4 occurred intraoperatively or postoperatively and 1 resulted from uncontrolled premature labor. Six fetuses underwent shunt placement, with 5 survivors. In a group (all of whom had severe hydrops) managed conservatively, the mortality rate was 100%. Indications for intervention remain poorly defined. Near unanimity exists regarding the benefit of fetal surgery in fetuses with marked hydrops.
- After 32 weeks' gestation, delivery with or without ex utero intrapartum treatment is performed.
- Postnatal surgery
- Resection of CCAM in all children is recommended. A number of reasons exist for this. The first reason is to remove the possibility that direct complications, such as recurrent infection and pneumothorax, will arise. Additionally, the malignant potential of CCAM in later life has long been recognized.
- Tumors that have been described in association with CCAM include rhabdomyosarcoma, pulmonary blastomas, minute squamous cell carcinoma, and bronchioloalveolar carcinoma. A number of surgical approaches have been described. Video-assisted thoracic surgery (VATS) has been successfully used to perform lobectomies in children with CCAM. The authors concluded that VATS was a safe and effective technique in children. Regardless of the approach or technique used, a guiding principle should be that the minimal possible amount of viable lung tissue is resected in order to allow the maximum potential for growth.
- Cases of pulmonary sequestration that occur in conjunction with CCAM exist. The consensus surrounding the management of these lesions is that surgical removal is most beneficial.
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 clear. As outlined previously, prognostic markers other than the presence of hydrops poorly predict fetal outcome. Hence, the expertise available in a facility skilled in fetal surgery may prove invaluable in allowing parents and health care providers to make an informed decision.
- 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).
Antibiotics are necessary in cases with recurrent pneumonia. Otherwise, no medications are specifically indicated for therapy of CCAM.
Further Inpatient Care
- Respiratory support may be required before and after resection of the 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.
- 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 resected postnatally. Unfortunately, reports describing the long-term outcome in these children are scarce.
In/Out Patient Meds
- 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 neonatal intensive care unit. 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. Cases have been reported of CCAM requiring ECMO support postoperatively for intractable pulmonary hypertension.
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 pneumonia
- Pneumothorax
- Hemothorax
- Malignant change: Rhabdomyosarcoma, pulmonary blastomas, minute squamous cell carcinoma, and bronchioloalveolar carcinoma have all been described in association with CCAM.
Prognosis
- The risk of mortality in fetuses with hydrops is as high as 80-90%.
- Other indicators of poor prognosis include the type of lesion, with microcystic CCAM associated with much poorer outcomes.
- 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.
- 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.
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 antenatal 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.
Medical/Legal Pitfalls
- Failure to diagnose this condition antenatally
- Failure to diagnose associated conditions
- Failure to offer skilled intervention, such as fetal surgery in high-risk cases
| Media file 1:
The computerized tomographic scan of the chest demonstrates 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 a number of microcysts. This lesion was resected without complication. |
 |  View Full Size Image | |
Media type: CT
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Cystic Adenomatoid Malformation excerpt Article Last Updated: Jul 7, 2006
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