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AUTHOR INFORMATION
| Section 1 of 11   |
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| Author: L Nicole Murray, MD, Assistant Professor, Department of Pediatric Otolaryngology, Children's Hospital, Louisiana State University Health Sciences Center Coauthor(s): Karla R Brown, MD, Assistant Professor, Department of Otolaryngology, Tulane University Medical Center |
| L Nicole Murray, MD, is a member of the following medical societies:
Alpha Omega Alpha, and
American Academy of Pediatrics |
| Editor(s): Todd Schneiderman, MD, Consulting Surgeon, Atlanta Ear Nose and Throat Associates; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine;
Gregory C Allen, MD, Assistant Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine;
Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders;
and Arlen D Meyers, MD, MBA, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine |
Disclosure
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INTRODUCTION
| Section 2 of 11   |
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Background: Cystic fibrosis (CF) is a chronic multisystem disorder characterized by recurrent endobronchial infections, progressive obstructive pulmonary disease, and pancreatic insufficiency with intestinal malabsorption.
Most patients with CF present to an otolaryngologist for sinonasal disease. Recent studies suggest that other head and neck problems, such as otitis media and adenotonsillar disease, appear to be similar in prevalence and pathophysiology to those in patients without CF (Cipolli, 1993; Halvorson, 1999). Otitis media actually appears to be less prevalent in patients with CF than in patients without CF, for reasons that remain unclear (Haddad, 1994). This article, therefore, focuses on sinonasal manifestations of CF. Pathophysiology: The CF gene product is a cyclic-AMP mediated chloride channel called the cystic fibrosis transmembrane conductance regulator (CFTR). Malfunction causes abnormal chloride ion transport on the apical surface of epithelial cells in exocrine gland tissues, leading to abnormally viscid secretions.
Patients most commonly have severe chronic lung disease and exocrine pancreatic insufficiency, but they may also have nasal polyposis, pansinusitis, rectal prolapse, chronic diarrhea, pancreatitis, cholelithiasis, cirrhosis, or other forms of hepatic dysfunction.
The exact mechanism by which a malfunctioning CFTR causes sinus disease is not completely understood. Chloride ions cannot be excreted, sodium is excessively absorbed, and water passively follows. This desiccates the mucosal surface and alters the viscosity of the normal mucus blanket, which alone can lead to obstruction of sinus ostia (Rutland, 1981). Additional abnormalities exist in these patients, including ciliary dysfunction, increased inflammatory mediators, and increased colonization with Pseudomonas aeruginosa, that further impair normal sinus clearance and aeration (Hauber, 2003). Chronic sinus infection and inflammation are the net result. Frequency:
- In the US: In whites, CF occurs in 1 per 3500 live births. In blacks, CF occurs in 1 per 17,000 live births.
- Internationally: Worldwide incidence varies from 1 per 377 live births in parts of England to 1 per 90,000 Asian live births in Hawaii.
Mortality/Morbidity: Median survival age varies from country to country and is highest in the United States and Canada, at 28 and 32 years, respectively. Median survival age in Latin America is 6 years. Cause of death is generally respiratory failure and cor pulmonale.
Medical and surgical treatment options are changing these statistics, and an individual with CF born in the United States today is expected to survive longer than 40 years (Elborn, 1991).
Race: Whites, as indicated above, are most often affected, at a rate of about 1 per 3500 births. Incidence is also high for Hispanics, at a rate of 1 per 9500 live births. CF is quite rare in native Asians and Africans (<1 per 15,000 births), but it occurs somewhat more frequently in Asian American or African American populations, reflecting white admixture (National Institutes of Health [NIH] Consensus Statement, 1999).
Sex: No sex predilection has been identified, although for reasons that are unclear, male sex may be associated with a slower rate of pulmonary function decline (Boat, 2000).
Age: Median age at diagnosis is 6-8 months. CF is diagnosed in two thirds of patients by 1 year of age.
History: - Elicit a thorough history of sinonasal symptoms from patients with CF. A complete history is critical because most clinical decisions are based on this information.
- While 90-100% of patients have radiologic evidence of sinus disease (Cepero, 1987; Neely, 1976; Schwachman, 1962), the frequency of nasal polyposis is quite variable and may be found in 6-67% of patients (Halvorson, 1999). Clinically symptomatic sinusitis, as evidenced by pain, discharge, fever, or postnasal drip, is found in only about 10% of patients with CF (Cepero, 1987). Most patients with radiologically evident disease, therefore, do not report symptoms. This phenomenon may represent a truly asymptomatic disease state, or it may suggest that patients with this chronic disease have psychologically adapted to their symptoms (Nishioka, 1996).
- Given the above facts, clinical history is often weighed more heavily than radiographic evaluation when treatment decisions are made.
- Symptoms to inquire about include the following (Brihaye, 1994):
- Worsening nasal discharge
- Question patients regarding recent pulmonary status.
- Chronic sinusitis seems closely associated with endobronchial bacterial infections (Ramsey, 1992) and also appears to impact pulmonary reactivity and chronicity of disease within the sinobronchial tract (Nishioka, 1995).
- Decreasing exercise tolerance also correlates with acute sinus exacerbations or worsening chronic disease.
Physical: - Most patients already have a CF diagnosis upon presentation to the otolaryngologist and are being referred for consideration of sinus surgery. Perform a thorough physical examination to evaluate the nose and sinuses as well as to uncover other conditions that may exacerbate sinus disease.
- Facial evaluation may reveal a widened nasal bridge due to chronic polyposis. Sometimes polyps may even protrude from the nares.
- Swollen turbinates, purulent nasal discharge, and nasal polyps may be visible on anterior rhinoscopy.
- Endoscopy may reveal polyps obstructing both the airway and the sinus ostia in the middle meatus. Purulent discharge may also be found on endoscopy. Patients also often have a bulging uncinate process that obstructs the nasal airway.
- The nasopharynx must also be evaluated. Adenoid hypertrophy may be present in younger patients and may play a role in nasal obstruction. Address this problem prior to surgical intervention in the sinuses of these patients, as with patients without CF.
- A CF diagnosis may occasionally be made by the otolaryngologist based on the presence of nasal polyposis in an otherwise healthy child. Patients sometimes tolerate mild forms of CF, thereby escaping early diagnosis. Nasal polyposis is otherwise quite rare in children. Evaluate any child found to have nasal polyps with a sweat chloride test.
Causes: CF is inherited as an autosomal recessive trait. - The gene responsible was identified in 1989 as a transmembrane conductance regulator glycoprotein (CFTR gene) localized to the long arm of chromosome 7 (Rommens, 1989).
- To date, at least 1300 different mutations in the gene have been identified (http://www.genet.sickkids.on.ca/cftr/).
- Half of affected individuals of northern European descent are homozygous for the DF508 mutation, which is the deletion of a single phenylalanine residue at amino acid 508 of the CFTR gene.
- Genotype does not always predict phenotype, which suggests either an environmental component of organ dysfunction or modifying genes that remain unidentified (Boat, 2000).
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DIFFERENTIALS
| Section 4 of 11 |
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Other Problems to be Considered:
As noted, nasal polyposis is rare in children without CF but does sometimes occur. Non–CF-related polyps may be caused by severe allergic rhinitis, inflammation associated with Samter triad (ie, asthma, aspirin insensitivity, nasal polyposis), Kartagener syndrome (ie, situs inversus, ciliary dysmotility), and other immunologic disorders. These conditions can usually be differentiated by a complete history and physical examination, with sweat chloride testing and biopsy as indicated. |
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Patient Education
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Lab Studies:
- Sweat chloride test is as follows:
- Pilocarpine iontophoresis is performed to collect and analyze sweat composition.
- Chloride concentration above 60 mEq/L is diagnostic. Chloride concentration below 30 mEq/L is within the reference range.
- Values of 30-60 mEq/L may be within the reference range or may represent heterozygous carriers. These carriers, thus, cannot be accurately identified with a sweat chloride test (Farrell, 1996).
- This test can be inaccurate in very young infants or if an inadequate volume is collected (Farrell, 1996). Repeat testing after equivocal results.
- Prenatal testing is as follows:
- Chorionic villous testing for CF can be performed at 10-12 weeks' gestation.
- The only benefit to CF diagnosis at this stage is to offer termination of pregnancy. Prenatal testing is currently being offered less and less frequently because of the ever-increasing predicted lifespan of these patients (Boat, 2000).
- Genetic testing is as follows:
- Genetic testing via blood tests can detect carrier status with up to 95% sensitivity.
- Sensitivity depends on the population and the mutations for which they are screened.
- Testing is recommended for individuals with a positive family history and for couples planning a pregnancy, but it is not necessarily indicated for the general population (NIH Consensus Statement, 1999).
- Newborn screening can be performed by measuring immunoreactive trypsin in the blood spot for the Guthrie test. One recent study showed a positive effect on growth when infants were diagnosed early by screening (Farrell, 2001).
- Requirements for a CF diagnosis include either positive genetic testing or a positive sweat chloride test findings (>60 mEq/L) and 1 of the following:
- Typical chronic obstructive pulmonary disease
- Documented exocrine pancreatic insufficiency
- Positive family history (usually affected sibling)
Imaging Studies:
- CT scanning of sinuses (axial and coronal views without contrast)
- More than 90% of patients with CF exhibit radiographic evidence of chronic sinusitis as evidenced by opacification, medial displacement of the lateral nasal wall in the middle meatus, and uncinate process demineralization (April, 1993).
- Medial bulging of the lateral nasal wall coupled with viscid mucus in the maxillary sinus may be observed in 12% of patients and represents a mucocelelike state, which must be treated surgically (Nishioka, 1996; Brihaye, 1994).
- Chronic sinusitis often causes poor pneumatization and hypoplasia of the maxillary and ethmoid sinuses and poor development of the frontal sinuses (Ledesma-Medina, 1980). Adolescent patients with CF often have no frontal sinus cavity present on CT scan evaluation.
Procedures:
- Sinus aspirates are necessary for culture in patients with CF to detect and appropriately manage infections with Pseudomonas species.
- Cultures from the nasopharynx, throat, or sputum are useless for predicting the identities and sensitivities of organisms infecting the sinuses (Shapiro, 1982).
- Transantral maxillary sinus aspirates of 20 patients with CF showed that organisms most commonly cultured were Pseudomonas species (65%), nontypeable Haemophilus influenzae (50%), alpha-hemolytic streptococci (25%), and anaerobes such as Peptostreptococcus and Bacteroides species (25%). Organisms found in patients with CF were sensitive to the same antibiotics used for patients without CF, except for the Pseudomonas species (Shapiro, 1982).
- Patients with acute sinusitis without CF are most commonly infected with Pneumococcus species, nontypeable H influenzae, and Moraxella catarrhalis. Patients without CF but with chronic sinusitis often have the above organisms in addition to Staphylococcus aureus and anaerobes such as Bacteroides, Veillonella, and Fusobacterium (Wald, 1996).
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TREATMENT
| Section 6 of 11 |
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Medical Care: Attempt aggressive medical management prior to surgical intervention. Patients may report chronic purulent nasal discharge or cough, but initiate therapy whenever they experience a subjective increase in nasal obstruction, cough, or drainage. Oral antibiotics effective against Pseudomonas species and staphylococci, coupled with aggressive nasal toilet, may improve symptoms. - Antibiotic choices: The bacteriology of sinonasal disease in patients with CF differs from that in patients without CF. This difference affects antibiotic choices. The most notable difference is the nearly ubiquitous presence of Pseudomonas species in patients with CF. Culture sinus aspirates to direct treatment against Pseudomonas species.
- Because mucociliary clearance is chronically impaired, irrigations are critical and should be a daily routine as patients begin to develop sinonasal symptoms. Nasal saline irrigations serve to decrease bacterial colonization, wash away inspissated secretions that lead to obstruction, and temporarily aid in ameliorating vasoconstriction. Irrigation is also required after any surgical intervention because surgery enlarges sinus ostia but does not address underlying defects in mucociliary clearance.
- Some authors advocate irrigations with antipseudomonal antibiotics such as tobramycin, and these anecdotally appear effective in decreasing bacterial colonization (Lewiston, 1991).
Surgical Care: Initiate surgical therapy if medical therapy is ineffective; however, carefully consider any surgical intervention for patients with CF. These patients may develop severe mucus plugging during general anesthesia, and this risk increases with duration of intubation. - Indications include the following (Nishioka, 1996):
- Persistent nasal obstruction associated with nasal polyposis and/or medial bowing of the lateral nasal wall following intensive medical management
- Medialization of the lateral nasal wall, as demonstrated by endoscopy or CT scanning, even without subjective nasal obstruction, because of the high prevalence of mucocelelike formations
- Pulmonary exacerbations that appear to correlate with sinonasal disease exacerbations, worsening of pulmonary status, or diminished activity level, despite appropriate medical management
- Facial pain or headaches that have no other apparent cause and that adversely affect quality of life
- Desire for improvement in symptom profile beyond what medical management has achieved in a patient with significant nasal cavity and paranasal sinus symptoms
- Contraindications include the following:
- Severe obstructive pulmonary disease may make the risk of anesthesia unacceptable.
- Patients with CF are at risk for vitamin K deficiency because of pancreatic insufficiency, hepatobiliary disease, or both, and if unsupplemented, they may have a bleeding diathesis (Rashid, 1999). A prolonged prothrombin time (PT) preoperatively should prompt correction and performance of surgery after the PT is normalized.
- Chronic sinusitis seems to cause delayed pneumatization and development of the maxillary, ethmoid, and frontal sinuses in these patients. Hypoplastic sinus cavities are often encountered. Preoperative axial and coronal CT scans must be obtained and evaluated. These anatomic abnormalities can make surgery more hazardous.
- Procedural concerns include the following:
- Perform the required surgery, if possible, in less than 1 hour to avoid respiratory compromise. Length of operation certainly varies according to extent of disease, blood loss, number of previous procedures, and experience of the surgeon. Safety and avoidance of complications are the primary concerns.
- If blood obscures the field, repeated irrigations and packing the nose with cottonoids can allow the procedure to progress safely, as in patients without CF.
- Remove polyps as completely as is safe, but remember that recurrence is likely. This procedure is generally performed for improvement, not cure. Leave residual polyps if adequate landmarks are unavailable.
- Powered endoscopic sinus surgery with a microdebrider allows safe rapid removal of tissue under direct visualization and is being practiced by increasing numbers of surgeons. This technique is especially efficacious in patients with CF because it allows rapid and precise removal of diseased tissue with preservation of landmarks. This should allow safer surgery both in terms of anesthetic time and decreased complications in patients who are likely to need multiple revisions (Mendelsohn, 1997).
- Fewer recurrences and longer symptom-free intervals are reported when polypectomy is combined with a sinus drainage procedure (Crockett, 1987).
- In addition to polypectomy, most surgeons advocate endoscopic procedures as follows:
- Wide middle meatal antrostomies
- Anterior and posterior ethmoidectomies
- Removal of polypoid disease in the frontal recess
- The following procedures are generally performed only if findings on examinations or imaging studies indicate necessity:
- Sphenoid sinusotomy
- Septoplasty
- Resection of the anterior edge of the middle turbinate to allow ease of postoperative inspection and irrigation
- Postoperative details include the following:
- Keep patients hospitalized until pulmonary function is clearly adequate for discharge (generally at least 1 night).
- Aggressively administer irrigations using normal saline or hypertonic sodium chloride solution.
- Postoperative cleaning prevents synechia formation. Young patients who cannot tolerate this procedure can briefly return to the operating room 2-3 weeks later for this purpose.
Consultations: Patients with CF should be cared for by a team of physicians familiar with their needs. Surgery on these patients must be undertaken in a cautious manner with attention to their pulmonary status. Preoperative communication with the pediatric, pulmonary, and anesthesia teams is therefore essential. - Severe obstructive pulmonary disease may make the risk of anesthesia unacceptable. The decision and timing of surgery must be carefully planned with the pulmonary team.
- Surgery is most commonly performed at the end of hospitalization for a pulmonary tune-up with intensive chest physiotherapy, bronchodilators, and intravenous antibiotics.
- Anesthetic concerns (Davidson, 1995)
- The procedure may be performed under local anesthesia for appropriately selected patients. Consider arterial hypotension to a systolic pressure of 85-90 mm Hg to allow faster and safer surgery in a relatively bloodless field. Prepare the nose with a topical vasoconstrictive agent, such as cocaine or oxymetazoline, and inject it with lidocaine or bupivacaine with epinephrine.
- In this manner, systemic narcotics, with their deleterious effects on postoperative respiration, are used minimally or avoided entirely. Deeply extubate patients, if possible, to avoid airway irritation, hypertension, and coughing on emergence.
Diet: Postoperatively, patients may resume their normal diet as tolerated. Activity: Patients must avoid strenuous activity for about 10 days postoperatively because such activity may precipitate bleeding.
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MEDICATION
| Section 7 of 11 |
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Nasal steroids are generally helpful (both for acute exacerbations and for long-term therapy) to decrease mucosal edema and promote mucus clearance. Short-term bursts of systemic steroids may improve acute exacerbations. Systemic steroids may also help if administered preoperatively to decrease intraoperative bleeding in patients with nasal polyposis. Decongestants are of variable benefit. Antihistamines are generally not beneficial and may be detrimental because of their drying effect on secretions. Mucolytics are generally ineffective in patients with CF except for recombinant human deoxyribonuclease, which is useful for bronchopulmonary disease.
Drug Category: Nasal corticosteroids -- These agents decrease mucosal edema and promote mucus clearance. Drug Name
| Mometasone furoate monohydrate (Nasonex) -- Has potent glucocorticoid activity, weak mineralocorticoid activity, and the least systemic absorption of nasal steroid preparations. Decreases rhinovirus-induced up-regulation in respiratory epithelial cells and modulates pretranscriptional mechanisms. Reduces intraepithelial eosinophilia and inflammatory cell infiltration (eg, eosinophils, lymphocytes, monocytes, neutrophils, plasma cells). |
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| Adult Dose | 2 sprays (50 mcg/spray) each nostril qd |
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| Pediatric Dose | <2 years: Not established
2-11 years: 1 spray (50 mcg/spray) each nostril qd
>12 years: Administer as in adults| Contraindications | Documented hypersensitivity; nasal septal perforation; nasal surgery; nasal trauma |
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| Interactions | None reported |
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| Pregnancy |
C - Safety for use during pregnancy has not been established.
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| Precautions | Systemic absorption with effects on growth and the hypothalamic-pituitary-adrenal axis theoretically possible but has not been demonstrated in multiple trials; rare reports exist of nasopharyngeal candidiasis and septal perforation with prolonged use; wound healing after nasal surgery possibly impaired by nasal steroids, initiate after healing in these circumstances; glaucoma and cataracts have been rarely associated with nasal steroids; patients should seek ophthalmologic consultation for changes in vision while using these drugs; use with caution in patients with active or quiescent tuberculosis of the respiratory tract; untreated fungal, bacterial, systemic viral infections; or ocular herpes |
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Drug Name
| Fluticasone propionate (Flonase) -- Has potent glucocorticoid activity, weak mineralocorticoid activity, and the least systemic absorption of nasal steroid preparations. |
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| Adult Dose | Two sprays (100 mcg) each nostril qd |
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| Pediatric Dose | <4 years: Not established
>4 years: One spray (50-mcg) each nostril qd| Contraindications | Documented hypersensitivity |
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| Interactions | None reported |
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| Pregnancy |
C - Safety for use during pregnancy has not been established.
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| Precautions | Systemic absorption with effects on growth and the hypothalamic-pituitary-adrenal axis theoretically possible but has not been demonstrated in multiple trials; rare reports exist of nasopharyngeal candidiasis and septal perforation with prolonged use; wound healing after nasal surgery possibly impaired by nasal steroids, initiate after healing in these circumstances; glaucoma and cataracts have been rarely associated with nasal steroids; patients should seek ophthalmologic consultation for changes in vision while using these drugs |
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Drug Category: Systemic corticosteroids -- Short-term bursts may be beneficial for acute exacerbations and may prevent increased intraoperative bleeding in patients with severe polyposis.Drug Name
| Prednisolone (Delta-Cortef, Econopred) -- Suppresses key components of immune system. Widely available in syrup form; easily dosed for children. |
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| Adult Dose | 40-60 mg PO qd with gradual taper |
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| Pediatric Dose | 0.5-2 mg/kg PO qd with gradual taper |
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| Contraindications | Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease |
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| Interactions | Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics |
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| Pregnancy |
C - Safety for use during pregnancy has not been established.
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| Precautions | Suppresses adrenocortical axis with use >2 wk; therefore, taper doses gradually if used longer; relative insufficiency may persist for months after therapy; administer stress doses of corticosteroids and sometimes mineralocorticoids in these patients, if necessary, during times of medical stress; may suppress growth and development if used long-term in children; psychic derangements may be caused or exacerbated by high-dose steroids |
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Drug Name
| Prednisone (Deltasone, Meticorten, Orasone) -- Suppresses key components of immune system. Widely available in syrup form; easily dosed for children. |
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| Adult Dose | 40-60 mg PO qd with gradual taper |
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| Pediatric Dose | 0.5-2 mg/kg PO qd with gradual taper |
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| Contraindications | Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease |
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| Interactions | Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics |
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| Pregnancy |
B - Usually safe but benefits must outweigh the risks.
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| Precautions | Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use |
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Drug Name
| Dexamethasone (Decadron, Dexasone, Solurex) -- Can be administered IV at induction of anesthesia; may help immediate postoperative inflammation. |
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| Adult Dose | 24 mg IV qd as one dose or 24 mg IV qd in divided doses |
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| Pediatric Dose | 1 mg/kg IV qd up to 24 mg single or divided doses |
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| Contraindications | Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics |
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| Interactions | None reported |
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| Pregnancy |
C - Safety for use during pregnancy has not been established.
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| Precautions | Suppresses adrenocortical axis with use >2 wk; therefore, taper doses gradually if used longer; relative insufficiency may persist for months after therapy; administer stress doses of corticosteroids and sometimes mineralocorticoids in these patients, if necessary, during times of medical stress; may suppress growth and development if used long-term in children; psychic derangements may be caused or exacerbated by high-dose steroids |
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Drug Category: Decongestants -- These are helpful in improving the nasal airway and shrinking down swollen tissues in some patients.Drug Name
| Pseudoephedrine hydrochloride (Sudafed, Actifed) -- Sympathomimetic agent that shrinks swollen nasal mucosa. |
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| Adult Dose | 60 mg PO q4-6h prn or 120 mg PO q12h in long-acting formulation |
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| Pediatric Dose | 1 mg/kg PO q4-6h prn |
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| Contraindications | Documented hypersensitivity; severe anemia; postural hypertension or hypotension; closed angle glaucoma; head trauma; cerebral hemorrhage |
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| Interactions | Propranolol, MAOIs, and sympathomimetic agents may increase toxicity of pseudoephedrine; methyldopa and reserpine may reduce effects of pseudoephedrine |
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| Pregnancy |
C - Safety for use during pregnancy has not been established.
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| Precautions | Caution in patients with hypertension, hyperthyroidism, diabetes mellitus, heart disease, peripheral vascular disease, glaucoma, and prostrate hypertrophy |
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FOLLOW-UP
| Section 8 of 11 |
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Further Inpatient Care:
- Keep patients with CF who undergo postoperative functional endoscopic sinus surgery (FESS) hospitalized until pulmonary function is clearly adequate for discharge (generally at least 1 night).
Further Outpatient Care:
- Postoperatively, regularly monitor patients with office endoscopy. If symptoms return, treat patients as outlined above. Attempt medical therapy prior to returning to the operating room. Clearly, however, many patients require revision sinus surgery for recurrent disease.
In/Out Patient Meds:
Deterrence/Prevention:
- As noted above, medical management with aggressive nasal toilet and appropriate use of antibiotics may be beneficial both symptomatically and in lengthening the interval between surgeries.
Complications:
- Complications of FESS in patients with CF do not occur more frequently than in patients without CF (Albritton, 2000; Duplechain, 1991; Crockett, 1987; Cuyler, 1992; Nishioka, 1995).
- As in patients without CF, revision cases with decreased landmarks can be more hazardous. This fact must be considered in patients with CF who may require multiple procedures over time, and surgical landmarks should be left in place if at all possible.
Prognosis:
- In his review article in 1999, Halvorson states, "Sinusitis is not a curable aspect of CF." Studies show that polypectomy alone in these patients leads to a recurrence rate of 89%, but when polypectomy is combined with another sinus procedure (Caldwell-Luc or ethmoidectomy), this rate drops to 35% (Crockett, 1987).
- Many patients need a subsequent procedure for polyp regrowth after polypectomy and FESS; therefore, these procedures should be viewed as treatments and not necessarily cures (Cuyler, 1992).
- Although all 7 patients in Cuyler's 1992 study showed radiologic evidence of recurrent disease after 1 year, all patients felt subjective improvement.
- Symptoms of nasal airway obstruction, olfactory dysfunction, purulent nasal discharge, and decreased activity level are all significantly improved after FESS (Nishioka, 1995).
- Effect on pulmonary function
- Many surgeons and physicians believe a positive effect on pulmonary function testing (PFT) results occurs after sinus surgery, but the data are limited to retrospective reviews and are somewhat conflicting.
- Halvorson's 1998 retrospective review of adult patients with CF showed a statistically significant improvement in forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1) in 8 of 8 patients after FESS.
- Madonna's 1997 retrospective review of 15 pediatric and adult patients with CF showed improvements in FVC and FEV1; the improvements, however, were not statistically significant.
- In 1990, Umetsu treated 4 adult patients using the Caldwell-Luc operation and nasoantral windows. These patients showed fewer postsurgical hospital admissions for pulmonary exacerbations.
- These are all retrospective reviews of a very small number of patients. Prospective studies are underway to attempt to answer this important question (Halvorson, 1999).
Patient Education:
- Educate patients and their caregivers about the importance of aggressive nasal toilet.
- These chronically ill patients and their families generally become very well-educated about their disease and are generally very compliant.
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MISCELLANEOUS
| Section 9 of 11 |
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Medical/Legal Pitfalls:
- Evaluate by sweat chloride test any previously healthy child presenting with nasal polyps. Mild forms of CF may go undiagnosed until children develop sinonasal symptoms. Nasal polyps are unusual in children without CF.
- Informed consent for sinus surgery is generally similar to that for sinus surgery in patients without CF. Exceptions are as follows:
- Anesthetic risks may be higher for patients with CF, depending on their pulmonary status.
- These patients may require several sinus procedures throughout their lives, and as in patients without CF, surgical risks are higher with revision surgery. Inform patients and their families of this increased risk. Surgeons should keep this in mind and attempt to preserve landmarks to facilitate subsequent surgery.
Special Concerns:
- The only definitive treatment for patients with CF accompanied by advanced lung disease is heart-lung or double-lung transplant. Survival is similar to that for lung transplant recipients without CF and is most adversely affected by rejection or complications of immunosuppression. Performance of this procedure in patients with advanced CF is only limited by the availability of donor organs (Rubin, 1999).
- Gene therapy for CF involves the insertion of DNA encoding a normal CFTR gene into respiratory cells. Vectors are generally viruses or liposomes. Multiple phase I clinical trials are underway. In preliminary trials, half of patients exhibit successful gene transfer, and a third exhibit at least partial correction of the chloride defect (Jaffe, 1999).
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PICTURES
| Section 10 of 11 |
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| Caption: Picture 1. Unilateral nasal polyposis with medial bulging of the lateral nasal wall and viscid secretions in the maxillary sinus is shown. This is often observed bilaterally in patients with cystic fibrosis (CF) and represents a mucocelelike state.
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Picture Type: CT |
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BIBLIOGRAPHY
| Section 11 of 11 |
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Albritton FD, Kingdom TT: Endoscopic sinus surgery in patients with cystic fibrosis: an analysis of complications. American Journal of Rhinology 2000; 14: 379-385.
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April MM, Zinreich SJ, Baroody FM, Naclerio RM: Coronal CT scan abnormalities in children with chronic sinusitis. Laryngoscope 1993 Sep; 103(9): 985-90[Medline].
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Boat TF: Cystic fibrosis. In: Nelson Textbook of Pediatrics. Philadelphia, Pa: WB Saunders Co; 2000: 1315-1327.
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Brihaye P, Clement PA, Dab I, Desprechin B: Pathological changes of the lateral nasal wall in patients with cystic fibrosis (mucoviscidosis). Int J Pediatr Otorhinolaryngol 1994 Jan; 28(2-3): 141-7[Medline].
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Cepero R, Smith RJ, Catlin FI, et al: Cystic fibrosis--an otolaryngologic perspective. Otolaryngol Head Neck Surg 1987 Oct; 97(4): 356-60[Medline].
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Cipolli M, Canciani M, Cavazzani M, et al: Ear disease is not a common complication in cystic fibrosis. Eur J Pediatr 1993 Mar; 152(3): 265-6[Medline].
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Crockett DM, McGill TJ, Healy GB, et al: Nasal and paranasal sinus surgery in children with cystic fibrosis. Ann Otol Rhinol Laryngol 1987 Jul-Aug; 96(4): 367-72[Medline].
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Cuyler JP: Follow-up of endoscopic sinus surgery on children with cystic fibrosis. Arch Otolaryngol Head Neck Surg 1992 May; 118(5): 505-6[Medline].
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Davidson TM, Murphy C, Mitchell M, et al: Management of chronic sinusitis in cystic fibrosis. Laryngoscope 1995 Apr; 105(4 Pt 1): 354-8[Medline].
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Duplechain JK, White JA, Miller RH: Pediatric sinusitis. The role of endoscopic sinus surgery in cystic fibrosis and other forms of sinonasal disease. Arch Otolaryngol Head Neck Surg 1991 Apr; 117(4): 422-6[Medline].
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Elborn JS, Shale DJ, Britton JR: Cystic fibrosis: current survival and population estimates to the year 2000. Thorax 1991 Dec; 46(12): 881-5[Medline].
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