Congenital Cystic Adenomatoid Malformation (CCAM)

What the Anesthesiologist Should Know before the Operative Procedure

Congenital cystic adenomatoid malformation (CCAM) is a rare bronchopulmonary abnormality (incidence1/20,000-1/30,000 live births) that accounts for 95% of congenital cystic disease. Etiology is unknown but hypothesized to be due to embryologic insult resulting in cessation of bronchial maturation, absence of bronchial cartilage, and suppression of normal alveolar growth. Excessive proliferation of mesenchymal elements of terminal bronchioles due to decreased apoptosis results in the characteristic adenomatoid appearance. The cystic intrapulmonary mass communicates with the airway but does not participate in gas exchange and may cause infection, mediastinal shift and cardiac compression, and result in pulmonary hypoplasia of remaining lung tissue.

Associated malignancies (4-9%) have been described in over 40 case reports, most commonly pleuropulmonary blastomas (infants and young children), bronchiolar and bronchoalveolar carcinoma, adenocarcinoma (older children), and rhabdomyosarcoma. CCAMs usually derive their arterial blood supply and venous drainage from the normal pulmonary circulation. Systemic arterial or anomalous blood supply directly from the aorta has been reported in a single case report and in “hybrid” lesions of CCAM and bronchopulmonary sequestration. Unilobar disease is more common than multilobar disease (85-95%), left-sided disease is more common than right-sided (60%), and 4% are bilateral. Lower lobes are affected more than upper lobes.

Natural history of CCAM ranges from spontaneous regression to neonatal death with severe respiratory difficulty secondary to pulmonary hypoplasia. The clinical course of lesions is unpredictable and variable as some CCAMs will grow and some will shrink. Some lesions will be microcystic and some macrocystic. Implicated genes include Hoxb5, Fgf7, and PDGF-B; overexpression of the latter results in fetal hydrops.

Clinical management of CCAM depends on the timing of diagnosis, severity of respiratory illness, or likelihood of respiratory illness.

Treatment of CCAM may occur early, middle, or late in gestation. Therapies may be minimally invasive or require open fetal surgery.

Other congenital pulmonary cystic lesions include bronchogenic pulmonary cysts and pulmonary sequestrations. Bronchogenic cysts represent abnormal embryologic development of primitive foregut forming cystic structures in numerous anatomic locations such as mediastinum or pulmonary parenchyma but rarely communicate with the tracheobronchial tree. Pulmonary sequestrations are nonfunctioning cystic lung usually localized to lower lobes and that does not contain a bronchial connection with blood supply from bronchial or aortic vessels with azygous venous drainage.

Differential diagnosis: Congenital lobar emphysema, tension pneumothorax, pericardial cyst, hemangioma, mediastinal cystic teratoma, enteric cyst, neurenteric cyst, cystic hygroma.

1. What is the urgency of the surgery?

What is the risk of delay in order to obtain additional preoperative information?

Timing of surgery is controversial and is determined by prenatal behavior, development of mass effects, and postnatal presentation.

CCAM may be potentially life threatening. Most common presentation is a neonate with severe respiratory distress (tachypnea, increased work of breathing, hypoxemia, hypercapnia or overt respiratory failure requiring ventilatory support), hypotension, and mediastinal shift.

Recommended for symptomatic CCAMs in infants and young children diagnosed postnatally. Most common presentation is infants and young children with borderline symptoms who become symptomatic or persist with tachypnea, feeding difficulties, and failure to thrive.

Recommended for asymptomatic lesions in infants to decrease risks of morbidity from infection, pneumothorax, or malignancy, facilitate compensatory lung growth, and accelerate postoperative recovery. Elective surgery before age 10 months is associated with a better outcome than emergency surgery. A recent systematic review and meta-analysis of all published literature supports improved postoperative outcomes in those children undergoing elective surgery when they are an asymptomatic patient, compared to undergoing surgery as part of expectant management (performing surgery only after development of symptoms). Surgery may proceed after reasonable medical therapy has optimized the patient’s status.

2. Preoperative evaluation

The most common manifestation of CCAM in newborns and early infancy is respiratory distress secondary to weak chest wall, and possible tracheal, mediastinal, and airway compression. Forty-six percent of CCAM in the neonatal period initially present with respiratory distress (tachypnea, nasal flaring, and intercostal retractions) or later as recurrent pneumonia. Infants may present with mild respiratory distress, reduced air entry, and an oxygen requirement. More severely affected children may present with undercurrent chest infections, history of pneumothorax due to ball value hyperinflation, and overt respiratory failure. Maximum congenital pulmonary malformation volume ratio (CVR) and prenatal signs of intrathoracic compression have been reported as significant risk factors for respiratory compromise at birth.

In children over 1 year of age, CCAM cysts lose compressive character and may remain asymptomatic until signs of chronic infection, respiratory disease and recurrent pneumonia occur, while producing cough, dyspnea, and thoracic pain, decreased cardiac output, and diminished lung volume and reserve.

Medically unstable conditions warranting further evaluation include the extent of pulmonary hypertension and stabilization prior to surgery.

Delaying surgery may be indicated if there is medical optimization of infections such as pneumonia with antibiotics or chest physiotherapy and bronchospasm with bronchodilators.

3. What are the implications of co-existing disease on perioperative care?

High frequency oscillatory ventilation (HFOV) may be used in perioperative ventilatory management of neonate where spontaneous respirations immediately after birth and conventional ventilation increase risk of hypoxemia in neonate by enlarging and rupturing lung cysts.

b. Cardiovascular system:

Presence of a gallop, murmurs, arrhythmias and inadequate peripheral pulses warrant pediatric cardiology evaluation.

Congenital heart disease (structural and functional cardiac anomalies) is associated with CCAM.

Tamponade physiology may result from cardiac dysfunction due to mass effect or fluid collection. CCAM can cause mediastinal shift, compression of the heart, and esophageal compression resulting in polyhydramnios. The single best predictor of fetal death is hydrops secondary to mass effect and is caused by caval obstruction and cardiac compression.

c. Pulmonary:

Chest needs to be inspected for asymmetric expansion and use of accessory muscles. Auscultation should evaluate wheezes, rales, rhonchi, and absence of breath sounds in supine and sitting positions. Compression of the opposite lung can result in pulmonary hypoplasia.

Associated pulmonary anomalies include bronchopulmonary sequestration, congenital diaphragmatic hernia, and extrapulmonary malformations.

Bilateral CCAM more likely to result in congenital high airway obstruction syndrome (CHAOS).

d. Renal-GI:

Renal anomalies such as renal dysplasia and agenesis have been described. Omphalocele has also been reported.

e. Neurologic:

Neural tube defects have been described.

f. Endocrine:

N/A

g. Additional systems/conditions which may be of concern in a patient undergoing this procedure and are relevant for the anesthetic plan (e.g., musculoskeletal in orthopedic procedures, hematologic in a cancer patient)

Facial clefts and bony abnormalities have been reported. CCAM is associated with prune belly syndrome, pectus excavation, and hydranencephaly.

4. What are the patient's medications and how should they be managed in the perioperative period?

h. Are there medications commonly seen in patients undergoing this procedure and for which should there be greater concern?

N/A

i. What should be recommended with regard to continuation of medications taken chronically?

Pulmonary: Nitric oxide may treat pulmonary hypertension.

j. How to modify care for patients with known allergies

N/A

k. Latex allergy – If the patient has a sensitivity to latex (e.g., rash from gloves, underwear, etc.) versus anaphylactic reaction, prepare the operating room with latex-free products.

N/A

l. Does the patient have any antibiotic allergies – Common antibiotic allergies and alternative antibiotics

N/A

m. Does the patient have a history of allergy to anesthesia?

Malignant hyperthermia (MH)

Documented: Avoid all trigger agents such as succinylcholine and inhalational agents. Follow a proposed general anesthetic plan: total intravenous anesthesia with propofol ± opioid infusion ± nitrous oxide. Ensure an MH cart is available (MH protocol).

5. What laboratory tests should be obtained and has everything been reviewed?

Complete blood count, coagulation studies, type and crossmatch, arterial blood gas.

Hemoglobin levels: Complete blood count.

Electrolytes: Chemistry appropriate depending on clinical condition of patient.

Imaging: Chest x-ray as initial investigation to evaluate airway impingement and mediastinal shift and infection.

CT scan reported as first-line preoperative investigation for asymptomatic babies diagnosed antenatally with CCAM. CT Chest allows confirmation of diagnosis, definition of the cystic nature, and location of pulmonary masses and associated anomalies. Limited value in reliably determining presence of infection or airway communication and thus is of limited value to anesthesiologist.

High-resolution US with MRI can readily differentiate the majority of lesions. MRI is a useful tool for distinguishing CCAM from other intrathoracic lesions and indicating lobe location and visualizing and determining the presence of compressed normal lung.

Ultrasound is diagnostic on routine screening in pregnancy. Assessment in the second trimester of lung size and degrees of mediastinal shift, presence of polyhydramnios (secondary to compression of fetal esophagus preventing swallowing of amniotic fluid), and fetal hydrops (secondary to vena caval obstruction, cardiac compression). Color Doppler sonography allows identification of feeding vessels noninvasively.

For patients undergoing EXIT—fetal ultrasonography, fetal MRI, fetal echocardiography. Fetal MRI may be used to image suspected lesions and the surrounding lung and can confirm presence of mass, delineate blood supply, and assess residual tissue as well as distinguish CCAM from other intrathoracic lesions. Fetal echocardiogram provides a baseline assessment of cardiac function as well as evaluation for coincident cardiac anomalies.

Other tests: Echocardiogram for neonates diagnosed in the postnatal period with congenital malformations of respiratory system suspected of associated cardiac anomalies that will impact perioperative care. For those prenatally diagnosed, fetal echocardiography is useful because of high incidence of structural and functional cardiac anomalies associated with CCAM. A baseline assessment of cardiac function is useful in monitoring changes in pregnancy.

Arterial blood gas on room air.

Type and cross (proximity to great vessels and risk of inadvertent damage)

Intraoperative Management: What are the options for anesthetic management and how to determine the best technique?

Open fetal surgery for CCAM is recommended for fetuses that develop hydrops before 32 weeks of gestation. Fetal hydrops may present as ascites, pleural effusion, and scalp edema. Solitary lesions with a large single cyst may be treated minimally invasively with one time or serial needle aspiration or ultrasound guided minimally invasive placement of a thoracoamniotic shunt.

Open fetal surgery and fetal CCAM resection are recommended if thoracocentesis or thoracoamniotic shunt for large cysts is not possible. In utero fetal pulmonary lobectomy useful in a midgestation fetus with immature lungs with significant physiologic compromise. After the lobectomy is completed, the fetus is returned to the uterus for further growth and development.

EXIT procedure can be undertaken If the fetus is late in gestation and the lesion is causing distress or may interfere with neonatal resuscitation. This is performed with fetal thoracotomy and pulmonary lobectomy before umbilical cord clamp. A fetus with a huge malformation severely compromising the airway or hydropic fetuses greater than 32 weeks gestation will require EXIT procedure, intubating the baby before delivery is complete and transferring to ECMO.

Postnatal pulmonary lobectomy if the mass is asymptomatic and the child can be delivered normally. Surgery may be done by thoracotomy or thoracoscopy.

a. Regional anesthesia

Neuraxial

For patients undergoing CCAM resection by thoracotomy, thoracic epidural anesthesia for postoperative pain management usually in concert with general anesthesia. Low thoracic epidural anesthesia usually by insertion of catheter by caudal canal after induction of general anesthesia and intubation and before commencement of surgical procedure.

Benefits

Placement of thoracic caudal epidural catheter provides excellent postoperative pain management. Intraoperative use of epidural may reduce intraoperative anesthetic requirements. Fetal transthoracic aspiration of the cysts under ultrasound guidance described under continuous epidural anesthesia.

Drawbacks

Requires normal coagulation profile, experienced level of provider, and assistant for positioning of patient. Catheter placement may require confirmation by fluoroscopy, epidurogram, electrical nerve stimulation, ECG guidance, or ultrasound imaging. Not useful for patients having thoracoscopy.

Issues

Local anesthetic solutions such as 0.1% bupivacaine or 0.1% bupivacaine with fentanyl 2-5 mcg/mL (or levobupivacaine) 0.1-0.3 mL/kg/hr for 48 hours.

Peripheral nerve block

Iintercostal nerve block may be single shot or continuous catheter. Blockade requires two dermatomes above and below the level of surgical incision.

Benefits

Provides excellent postoperative analgesia, inadequate for intraoperative analgesia.

Drawbacks

Contraindicated with local infection, lack of expertise of provider.

Issues

Local anesthetic toxicity can be an issue as absorption from intercostal space is rapid.

b. General anesthesia

Benefits

GA can be administered rapidly to secure the airway and facilitate complete control of airway, breathing, and circulation. It is titratable and easily reversible, and it provides immobile patient, good muscle relaxation, and operating conditions for long periods of operative time, with excellent amnesia and analgesia. It is useful in patients with known allergy to local anesthetics. High frequency oscillatory ventilation (HFOV) may lower peak airway pressure and reduce possibility of air entrapment and barotraumas; ventilate the lung of a neonate with CCAM; and achieve hyperventilation with lower peak airway pressure, which is helpful for decreasing the pulmonary vascular resistance.

Drawbacks

Conventional mechanical ventilation (CMV) may cause expansion of the affected lobe due to emphysematous enlargement of cysts accompanied by ball-valve air entrapment. High peak airway pressures during positive pressure ventilation may cause barotrauma. Precipitous hypotension is a potential problem with volatile agents in patients with low cardiac reserve

Other issues

The majority of CCAM are of solid variety so positive pressure ventilation can be accomplished without cardiopulmonary compromise.

Airway concerns

Lung isolation may be desirable as resection of CCAM not uncommonly results in the release of copious mucoid, purulent and/or bloody secretions from the mass into the ipsilateral mainstem bronchus. CCAM is usually brought out of the chest for resection and OLV is not mandatory. Awake intubation is alternative to inhalation induction.

6. What is the author's preferred method of anesthesia technique and why?

Premedication of oral or intravenous midazolam if over 1-year-old and patient has minimal respiratory compromise. Albuterol and atropine to dry secretions, blunt cholinergic mediated airway reactivity, and prevent bradycardia during laryngoscopy and intubation. Standard ASA monitors, precordial stethoscope opposite side being operated, arterial catheter if extensive blood loss or resection is expected. Femoral central venous line may be considered.

Rapid sequence induction to secure the airway and prevent a crying, struggling neonate trapping air during inspiratory efforts and enlarging the cystic malformation secondary to ball valve effects due to abnormal or deficient bronchial cartilage. Lidocaine 1-2 mg/kg, propofol 1 mg/kg, or ketamine or dexmedetomidine prior to intubation to facilitate placement of the tube without hemodynamic or respiratory responses. Until chest is open, maintain spontaneous ventilation with sevoflurane in an air/oxygen mix with occasional manual assistance with minimal airway pressure.

The necessity of one lung ventilation should be discussed with the pediatric surgical team. If necessary, endobronchial intubation with a standard cuffed endotracheal tube (ETT) in the contralateral mainstem bronchus may be placed to achieve lung isolation. A 5 French pediatric endobronchial blocker in the ipsilateral mainstem bronchus provides the better option than endotracheal tube placement over a Fogarty catheter which may increase risk for damage or rupture of the airway secondary to high-pressure low-volume balloon. While not available in the United States, the Marraro® bilumen tube for infants, consisting of two separate uncuffed tracheal tubes of different lengths attached longitudinally, has been described. After confirming air entry and proper fixation of ETT, position in lateral position with operative side up.

Continuous epidural anesthesia for postoperative pain management for thoractomy with placement of epidural catheter via sacral hiatus and advancement to thoracic levels after induction and before surgical procedure commences using bupivacaine 0.1% without fentanyl. Remifentanil may be used as alternative to morphine infusion.

After chest and pleura are opened, muscle relaxation may be provided with rocuronium and controlled ventilation, avoiding high peak airway pressures. Maintenance air/oxygen/isoflurane or desflurane and rocuronium. Fluid management 10 mL/kg/hr plasmalyte or lactated Ringer’s. Extubation in the operating room or neonatal intensive care unit.

Prophylactic antibiotic received within 1 hour prior to surgical incision. First- or second-generation cephalosporins satisfy the criteria for most operations. If a patient is allergic to beta-lactams, fluoroquinolones and clindamycin in selected situations. Prophylactic antibiotics discontinued within 24 hours after surgery end time (from SCIP, August 13, 2011, “CMS Core Measure SCIP Surgical Care Improvement Project,” from http://www.sjhlex.org/documents/Physicians/SCIP_Poster_Full_Size.pdf)

What do I need to know about the surgical technique to optimize my anesthetic care?

You need to know the surgical approach to management of CCAM at the individual’s institution (operative resection via open thoracotomy or video assisted thoracoscopy). Choice of approach may be dictated by complexity of operative case or surgical preference, however, utilization of thoracoscopic resection for congenital cystic lung disease has been reported to be increasing in the United States and has been validated to be a safe alternative to open surgery.

Positioning issues should be discussed with surgical team. Surgery in the prone or lateral position can cause ventilatory changes in children accentuating the decrease of FRC under general anesthesia. Further, the endotracheal tube can dislodge with movement. Appropriate padding in lateral positions should be emphasized.

What can I do intraoperatively to assist the surgeon and optimize patient care?

Formulate a team approach with the surgical team ahead of time. A range of options for antenatal intervention are available included drains or thoracoamniotic shunts to reduce the size of cystic lesions or fluid collections causing fetal compromise, open fetal surgery by fetoscopy or open fetal surgery to perform pulmonary lobectomy to resect large masses causing fetal compromise, EXIT procedure for pulmonary lobectomy to resect masses that will interfere with neonatal resuscitation. Postnatal resection may include complete resection by lobectomy, pneumonectomy in cases of extensive multilobar involvement or segmental resection. Discuss the need for lung isolation with the surgeon. Frequently, surgeons retract and/or pack the operative lung as needed for operative exposure.

Cysts can enlarge during surgery. On induction, positive pressure ventilation and positive end-expiratory pressure should be minimized to prevent rapid expansion of the involved lobe with sudden mediastinal shift and cardiac arrest. Avoidance of nitrous oxide and controlled mechanical ventilation.

For patients undergoing EXIT, continuous fetal echocardiography to assure adequate fetal volume loading to decrease risk of fetal cardiovascular collapse post thoracotomy. Have ECMO on standby.

Discuss alternative ventilation modalities such as HFOV if available which might assist the patient and provide a quiet surgical field during thoracotomy.

Adequate muscle relaxation with controlled ventilation employing humidified gases. Manual ventilation may provide information on changes in compliance or airway resistance especially infants or procedures where there is recurrent obstruction of airway.

What are the most common intraoperative complications and how can they be avoided/treated?

Complications may occur during surgical excision of cystic pulmonary masses, including hemorrhage, tracheal wall tear, pneumothorax and sinus bradycardia.

Risk of spillage of infected cystic contents into the tracheobronchial tree may be minimized by avoidance of positive pressure ventilation, careful rib retraction, and lung separation using selective bronchial intubation or using bronchial blockers (Cook) or Fogarty catheters.

Fetal complications can occur during EXIT related to failure to preserve uteroplacental gas exchange due to cord compression, placental abruption or loss of uterine relaxation. Preservation of uteroplacental blood flow by maintaining uterine relaxation with deep inhalational anesthesia and maintaining uterine volume using a strategy of partial delivery of fetus and amnioinfusion may result in improved outcomes.

Complications

Cardiac

Precipitious hypotension with volatile agents can occur in patients with low cardiac reserve.

Pulmonary

Nitrous oxide can accumulate in cysts with air fluid levels resulting in emphysematous enlargement and should be avoided. The rate of rise of inhalational anesthetics may be slowed in the presence of intrapulmonary shunting.

a. Neurologic:

N/A

b. If the patient is intubated, are there any special criteria for extubation?

N/A

c. Postoperative management

What analgesic modalities can I implement?

Continuous epidural, intravenous opioids or NSAIDs such as ketorolac, intercostal blocks, local anesthetic infiltration by surgeon, acetaminophen. Addition of fentanyl to epidural infusions of bupivacaine in infants undergoing thoracotomy for resection of CCAM may prolong recovery and increase the incidence of adverse respiratory events without providing significant analgesic benefit.

What level bed acuity is appropriate?

PICU bed for postoperative ventilation and monitoring.

What are common postoperative complications, and ways to prevent and treat them?

Postoperative course depends on the surgical procedure and underlying diseases.

A meta-analysis of nine studies showed a greater than 2-fold increase in risk of complications of developing a postoperative complication when surgery was carried when the patient was symptomatic versus asymptomatic. (Risk ratio 2.8, P<0.005) The rate of complications is greater in neonate and infants for emergency surgery (28%) than for elective surgery.

Early complications include hypoxemia, hypoventilation, air leak, effusion, bleeding, and infection. Late complications of elective surgery include residual disease. Residual CCAM reported after fetal lobectomy and neonate is followed with serial chest radiographs and/or CT scans during the first 6 to 12 months of life, depending on the presence or absence of chronic respiratory symptoms.

A recent retrospective review found better post operative outcomes and similar postoperative complication rate from a minimally-invasive surgical approach of congenital cystic lung lesions compared to conventional thoracotomy.

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