Cystic Fibrosis

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What the Anesthesiologist Should Know before the Operative Procedure

Cystic fibrosis (CF) is an autosomal recessive multisystem disease caused by a mutation in the CF transmembrane conductance regulator gene on chromosome 7. Incidence is 1 in 3200 children and pulmonary disease accounts for 85% of mortality. Etiology of lung disease is obstruction of the airway due to inspissated secretions, resultant suppurative endobronchial infection, an exaggerated inflammatory response leading to development of bronchiectasis impaired alveolar ventilation, and progressive obstructive airways disease.

Advancement in the care of patients with CF has been profound in the last 45 years since anesthesia for CF was described. Anesthesia for CF is most often described in retrospective reviews, revealing high overall safety. These include a 5% incidence of perioperative respiratory complications in a retrospective review of 199 anesthetics in 53 patients undergoing elective surgery under general anesthesia.

CF affects exocrine glands in a variable manner and manifests in many organs, especially the upper and lower airways, pancreas, bowels, and reproductive system. Manifestations of the disease include recurrent lower respiratory tract infections (bronchiectasis, cyanosis, clubbing), chronic cough, nasal polyps, sinusitis, and malnutrition and failure to thrive. A 15-year review of children’s hospital patients with CF who underwent surgery identified the most common procedure as nasal polypectomy. Other procedures included vascular access, gastrointestinal (meconium ileus, intussusception, biliary), and pulmonary (bronchoscopy, lavage, pulmonary resections). Patients may present for lung transplantation.

1. What is the urgency of the surgery?

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

Emergent: Emergent surgery for newborn infants with meconium ileus and complete intestinal obstructions and peritonitis. Sepsis and coagulopathy may preclude regional techniques.

Timing of surgical procedure during periods of clinical stability and optimal pulmonary function of the patient is crucial to achievement of improved outcomes. Procedures are better done mid-morning to complete clearance of overnight secretions by chest physiotherapy before induction of anesthesia. Optimization of growth and nutritional status is also indicated, as evidence-based recommendations by CF Foundation patient registry data support the use of increased energy intake for growth and development for children. Anesthetic plan for elective surgery in CF patients should emphasize the need to return the patient to baseline pulmonary function as soon as possible. Perioperative regional anesthesia techniques can be extremely beneficial in achieving this goal.

2. Preoperative evaluation

It is important to quantify the level of disability and extent of cardiopulmonary disease. Cardiorespiratory complications are the most common cause of death in patients. CF is a multifactorial disease involving upper and lower airways, pancreas, bowels, gastrointestinal system, and reproductive system. Manifestations include nasal polyps, sinusitis, recurrent respiratory symptoms, malnutrition and failure to thrive, GERD, liver and gallbladder disease, Crohn’s disease, and celiac disease. Clinical presentation may be variable. Patients with early stage CF can present with chronic coughing, copious phlegm, and decreased exercise tolerance. Patients in later stages may have symptoms of hemoptysis (9.1%), pulmonary hypertension, heart failure, hypoxia, and respiratory failure requiring ventilatory support.

Delaying surgery may be indicated if:

1) Pulmonary function is not optimized through antibiotics, aerosolized bronchodilators, physiotherapy, and postural drainage.

2) If the patient's nutritional status needs to be optimized in order to better control infections.

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

Perioperative evaluation

Communication with pediatric pulmonologist and/or CF team will optimize quality of care. Level of disability and extent of cardiopulmonary disease preoperatively should be quantitated.

Perioperative risk reduction strategies

Chronic medications that need to be continued in perioperative period should be discussed with CF team and guided by results of pediatric pulmonary function test. Optimization of nutritional status and prevention of overhydration and dehydration. Minimization of pulmonary vascular resistance including neuraxial analgesic techniques to avoid respiratory failure and ileus. Humidification of inhaled gases is desirable to minimize inspissation of secretions.

b. Cardiovascular system:

Acute/unstable conditions: Echocardiogram in case of pulmonary hypertension and/or right heart dysfunction.

Baseline coronary artery disease or cardiac dysfunction - Goals of management: Cor pulmonale in end stage CF disease occurs secondary to pulmonary hypertension

c. Pulmonary:

Pulmonary disease is the major cause of morbidity and mortality. Attacks of wheezing respiration may be mistaken for asthma. Chronic infection of the airways with Staphylcoccus aureus , Haemophilus influenzae , and Pseudomonas aeruginosa is typical. Lower airway inflammation and chronic infections may progress to end-stage lung disease characterized by extensive airway damage (bronchiectasis, cysts, and abscesses) and fibrosis of lung. Nasal polyps occur due to chronic sinus infections.

d. Renal-GI:

Electrolyte disturbances, nephrotic syndrome. and diabetic nephropathy have been described and may be encountered in the surgical setting. Nephrotoxic effects from aminoglycoside antibiotics may affect duration of neuromuscular blockade.

Meconium ileus in 5-10% of newborns, rectal prolapse in 10% of children with CF. GERD (26%), Crohn's disease, and celiac disease may be present. Liver disease is a spectrum from neonatal obstructive jaundice, elevation of transaminases and gamma-glutamyl transferase, direct hyperbilirubinemia, malabsorption/pancreatic insufficiency, clubbing of fingers to biliary cirrhosis, or portal hypertension or liver failure that might benefit from liver transplantation.

e. Neurologic:

Hypoxia, hypercarbia, and encephalopathy due to liver involvement may alter neurologic status.

f. Endocrine:

Glucose intolerance (75%) and diabetes mellitus (7-10%) are seen.

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)

Rheumatoid arthritis, hypoxic polyarthritis, amyloidosis, and drug-induced arthritis have been described in patients with CF.

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

Continue premedications of bronchodilators, antibiotics, and cardiotonic drugs.

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

Nine Cochrane systematic reviews have addressed the effectiveness of aerosolized antibiotics, dornase alfa, hypertonic saline, oral corticosteroids, inhaled corticosteroids, oral NSAIDs, macrolide antibiotics, inhaled bronchodilators, and oral antistaphyloccal antibiotics. Recommendations for children over 6 years of age include:

1) Inhaled tobramycin reduces exacerbations when used chronically2) Dornase alfa results in improved lung function compared to placebo3) Inhaled hypertonic saline improves lung function and reduces exacerbations4) Oral ibuprofen to slow the loss of lung function5) Chronic macrolide therapy (azithromycin)6) Inhaled beta 2 adrenergic agonists7) Oral antistaphyloccocal antibiotics

Negative recommendations on chronic use of inhaled anticholinergic agents (ipratropium), oral corticosteroids in children, inhaled corticosteroids, and N-acetylcysteine. Inhaled corticosteroids, cromolyn, and leukotriene modifiers have not been found to improve lung function or reduce exacerbations in patients over age 6.

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

Routine medications such as bronchodilators, corticosteroids and cardiotonic drugs should be continued into perioperative period.

j. 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 that the MH cart is available [MH protocol].

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

Hemoglobin levels: Complete blood count.

Electrolytes: Electrolytes including glucose and liver function tests (LFTs).

Coagulation panel: Prolonged prothrombin time, partial thromboplastin time, and international normalized ratio might indicate decreased synthesis of clotting factors and vitamin K deficiency–related coagulopathy. Coagulation studies are necessary when considering regional anesthesia techniques.

Imaging:Include stress tests, renal imaging tests, etc. Chest radiography may reveal overinflation, increased markings and detect abnormalities such as atelectasis or infiltrates which should be treated or evaluated further. Include ECG and echocardiogram in case of pulmonary hypertension and/or right heart dysfunction.

Other tests: Liver function tests are necessary when considering regional anesthesia techniques. Arterial blood gas (ABG) and pulmonary function tests (PFTs) to assess disease progression. On ABG, arterial carbon dioxide tension is usually low/normal; elevated values indicate advanced disease, reduction of arterial oxygen tension and elevations of alveolar-arterial oxygen differences in pulmonary fibrosis, bronchospasm, or viscous secretions. PFTs usually reveal obstructive pattern with increased FRC, decreased FEV1, decreased peak expiratory flow, and decreased vital capacity.

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

Anesthetic management is best achieved through regional or monitored anesthesia care techniques, but many surgeries such as nasal polypectomy require general anesthesia. In that case, it is paramount to minimize pulmonary vascular resistance.

a. Regional anesthesia


Benefits: Epidural or spinal anesthesia can be successfully used with or without general anesthesia for upper abdominal surgery. Epidural anesthesia described as method of choice in the older child provided no underlying coagulopathy. Continuation of analgesia through the epidural catheter into the postoperative period will ensure good analgesia with minimal pulmonary dysfunction. Intraoperative systematic opioid use and respiratory depression will be minimized to provide good analgesia and allow for early extubation.

Drawbacks: Regional techniques require normal coagulation.

Issues: Continuous epidural anesthesia without intubation was used in case report and may be considered in patients with severe pulmonary disease. Local anesthetic toxicity is possible in patients with severe liver dysfunction

Peripheral Nerve Block

Intercostal nerve block may be single shot or continuous catheter. Blockade requires two dermatomes above and below the level of surgical incision. Any peripheral nerve block can be used as appropriate for the surgical procedure.

Benefits: Intercostal nerve blocks useful for thoracic procedures and preservation of pulmonary function.

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: General anesthesia in CF patients has been largely limited to procedures that require only a short period of tracheal intubation, such as nasal polypectomy. This permits the administration of a high inspired concentration of oxygen. Volatile agents offer advantages of bronchodilatation, decreased muscle relaxant dosage, and reduced airway hyperactivity. This is useful in uncooperative patients.

Drawbacks: Inhalation induction may cause excessive coughing. Hypoxia may develop rapidly during anesthesia because of V/Q disturbances. Ventilation/perfusion abnormalities from obstruction of small and medium-sized airways by thick mucus with subsequent bronchitis and bronchiectasis may result. In those with advanced pulmonary disease, inhalation induction will be prolonged because of V/Q mismatch, high functional residual capacity, and small tidal volumes exacerbated by less soluble volatile agents such as sevoflurane and copious secretions that line and plug the airways. Intravenous induction is usually smoother. Lung compliance is reduced and very high airway pressures may be required to adequately ventilate the lungs and prevent hypoxemia. Tracheal intubation and general anesthesia impair mucociliary clearance.

Other issues: Pneumothorax is a risk and intermittent positive pressure ventilation should be avoided where possible in all children with CF and s recent history of hemoptysis. Spontaneous ventilation with a laryngeal mask airway may avoid the use of intermittent positive pressure ventilation and has been described. Dehydration (mucus plugging) and overhydration (excess secretions) should be avoided.

Airway concerns: Airway suctioning and gas humidification are helpful.

c. Monitored anesthesia care (MAC)

Benefits: MAC averts the use of intermittent positive pressure ventilation under general anesthesia. Intravenous sedation and local anesthesia are described for many procedures such as vascular access and bronchial artery embolization.

Drawbacks: MAC requires cooperative patient and effective local anesthesia by surgeon.

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

Avoid premedication drugs if possible, if essential, a short acting agent such as oral midzaolam 0.3-0.5 mg/kg. Adequate preoxygenation prior to standard Inhalation induction followed by intravenous access, or intravenous induction with propofol and muscle relaxant acceptable. Anticholingergic agents to reduce airway secretions may be given at the time of induction; however, the use of atropine is controversial with some clinicians maintaining drying of respiratory mucosa and more tenacious resultant secretions, and others advocating preoperative use of atropine as acceptable and challenging thickening of secretions by its use. The vocal cords should be topicalized with 4% lidocaine to facilitate smoother extubation at end of procedure. Intubation with cuffed endotracheal tube in a deep plane of anesthesia to avoid coughing, bronchospasm and inspissated secretions.

LMA is an airway alternative if the patient is at low risk of aspiration. Humidification of inspired gases essential. Nitrous oxide should be avoided. Use maintenance, oxygen/air/volatile agent, or relaxant and narcotic technique. Careful titration of use of short acting neuromuscular blockers in view of concomitant aminoglycosides is essential. There is no place for extubation during deep anesthesia in these patients. Frequent suction during the procedure and at the end of surgery is mandatory. After extubation at end of surgery, patients should be placed in 30-40-degree sitting position.

What prophylactic antibiotics should be administered?

Prophylactic antibiotic received within 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 are used in selected situations. Prophylactic antibiotics are discontinued within 24 hours after surgery end time [downloaded from SCIP. (August 13, 2011). "CMS Core Measure SCIP Surgical Care Improvement Project." from].

Complications are mainly found in the pulmonary resection and meconium ileus groups with operative morbidity of 3% and mortality of 0.5% and may include pneumothorax, sepsis, respiratory depression, hematoma, and death.

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

Awake extubation after ETT suction, lung inflation, and chest physiotherapy.

b. Postoperative management

What analgesic modalities can I implement?

Neuraxial techniques reduce the amount of systemic opioids for postoperative analgesia and the risk for ileus. Thoracic epidural infusions of 0.1% ropivacaine with 2 mcg/mL of fentanyl, 0.0625%-0.125% bupivacaine with or without 5 mcg/ml of fentanyl, or dexmedetomidine infusions can eliminate the need for supplemental intravenous narcotics in patients receiving regional anesthesia for thoracic procedures such as lung transplantation.

Multimodal analgesic with acetaminophen, NSAIDs (nonsteroidal anti-inflammatory agents) and local wound infiltration can also be used. Postoperative epidural can be placed if adequate pain control compromises CF patient’s respiratory function. Intrathecal opioids should be avoided as they have been associated with severe respiratory depression.

Placement of an ultrasound-guided continuous thoracic paravertebral catheter using 0.2% ropivacaine via elastomeric infusion pump has been described for inpatient and outpatient pain management of acute rib pain secondary to cystic fibrosis pulmonary exacerbation.

What level bed acuity is appropriate?

Step-down or PICU for close perioperative monitoring, intravenous hydration, airway management, and chest physiotherapy of patients with mild to severe respiratory failure.

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

Respiratory depression, pneumothorax, pneumonia, atelectasis and airway obstruction, and secretions are complications. A recent cohort study at a tertiary pediatric institution suggests that children with mild to moderate cystic fibrosis lung disease who undergo general anesthesia for PICC line insertion do not experience significant deterioration in central or peripheral airway function 24-48 hours post-GA.

Aggressive respiratory therapy, oxygen supplementation, and chest physiotherapy to clear secretions are critical to minimize the risk of reintubation. This may be achieved through bronchodilators and mucus-controlling agents; aerosol therapy, chest physiotherapy, antibiotics, and corticosteroids are respiratory therapy interventions. Encourage coughing, deep breathing, and early activity. Nutritional status during the perioperative period is critical to facilitate wound healing and wound function.

What's the Evidence?

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(Authors investigated the efficacy of thoracic epidural analgesia for adequate pain control after lung transplantation in adult patients, 26.1% of which had indication of cystic fibrosis.)

Cutshall, C, Hutchins, J. "Ultrasound-guided continuous thoracic paravertebral catheter management of acute rib pain secondary to cystic fibrosis exacerbation in a pediatric patient. Anesth Analg Case Rep". Feb. vol. 1. 2015. pp. 29-30.

(Author discuss ultrasound-guided continuous thoracic paravertebral catheter for inpatient and outpatient management of acute pain from rib avulsion in a pediatric patient.)

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(Authors discuss pathophysiology, surgical procedures, anesthetic management, and perioperative complications.)

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(These authors discuss evidence-based recommendations for chronic medications in cystic fibrosis.)

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(Author discusses clinical presentation and historical anesthetic management.)

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