AUTHOR INFORMATION

Section 1 of 12

Authored by John E McClay, MD, Assistant Professor, Department of Otolaryngology, Division of Pediatric Otolaryngology, Children's Medical Center, University of Texas at Southwestern

John E McClay, MD, is a member of the following medical societies: American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, and American Medical Association

Edited by Orval Brown, MD, Director of Otolaryngology Clinic, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center at Dallas; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Alan D Murray, MD, Consulting Pediatric Otolaryngologist, ENT for Children, PA; Consulting Staff, Department of Otolaryngology, Medical Center of Lewisville, Children's Medical Center at Dallas, Texas Pediatric Surgery Center, The Pediatric Surgery Center; Paul D Petry, DO, FACOP, FAAP, Clinical Assistant Professor of Pediatrics, University of North Dakota, School of Medicine and Health Sciences; Consulting Staff, Altru Health System; and Maureen Strafford, MD, Arnold P Gold Foundation Associate Professor, Departments of Anesthesiology and Pediatrics, Tufts University and Tufts-New England Medical Center

Author's Email:	John E McClay, MD
Editor's Email:	Orval Brown, MD

eMedicine Journal, March 2 2007, VOLUME 8, Number 3

INTRODUCTION

Section 2 of 12

Background: Broadly defined, nasal polyps are abnormal lesions that emanate from any portion of the nasal mucosa or paranasal sinuses. Polyps are an end result of varying disease processes in the nasal cavities. The most commonly discussed polyps are benign semitransparent nasal lesions (see Images 1-3) that arise from the mucosa of the nasal cavity or from 1 or more of the paranasal sinuses, often at the outflow tract of the sinuses.

Multiple polyps can occur in children with chronic sinusitis, allergic rhinitis, cystic fibrosis (CF), or allergic fungal sinusitis (AFS). An individual polyp could be an antral-choanal polyp, a benign massive polyp, or any of a number of benign or malignant tumors (eg, encephaloceles, gliomas, hemangiomas, papillomas, juvenile nasopharyngeal angiofibromas, rhabdomyosarcoma, lymphoma, neuroblastoma, sarcoma, chordoma, nasopharyngeal carcinoma, inverting papilloma). Evaluate all children with benign multiple nasal polyposis for CF and asthma.

Pathophysiology: The pathogenesis of nasal polyposis is unknown. Polyp development has been linked to chronic inflammation, autonomic nervous system dysfunction, and genetic predisposition. Most theories consider polyps to be the ultimate manifestation of chronic inflammation; therefore, conditions leading to chronic inflammation in the nasal cavity can lead to nasal polyps.

The following conditions are associated with multiple benign polyps:

• Bronchial asthma - In 20-50% of patients with polyps

• CF - Polyps in 6-48% of patients with CF

• Allergic rhinitis

• AFS - Polyps in 85% of patients with AFS

• Chronic rhinosinusitis

• Primary ciliary dyskinesia

• Aspirin intolerance - In 8-26% of patients with polyps

• Alcohol intolerance - In 50% of patients with nasal polyps

• Churg-Strauss syndrome - Nasal polyps in 50% of patients with Churg-Strauss syndrome

• Young syndrome (ie, chronic sinusitis, nasal polyposis, azoospermia)

• Nonallergic rhinitis with eosinophilia syndrome (NARES) - Nasal polyps in 20% of patients with NARES

Most studies suggest that polyps are associated more strongly with nonallergic disease than with allergic disease. Statistically, nasal polyps are more common in patients with nonallergic asthma (13%) than with allergic asthma (5%), and only 0.5% of 3000 atopic individuals have nasal polyps.

Several theories have been postulated to explain the pathogenesis of nasal polyps, although none seems to account fully for all the known facts. Some researchers believe that polyps are an exvagination of the normal nasal or sinus mucosa that fills with edematous stroma; others believe polyps are a distinct entity arising from the mucosa. Based on a review of the literature and several intricate studies of the bioelectric properties of polyps, Bernstein derived a convincing theory on the pathogenesis of nasal polyps, building on other theories and information from Tos.

In Bernstein's theory, inflammatory changes first occur in the lateral nasal wall or sinus mucosa as the result of viral-bacterial host interactions or secondary to turbulent airflow. In most cases, polyps originate from contact areas of the middle meatus, especially the narrow clefts in the anterior ethmoid region that create turbulent airflow, and particularly when narrowed by mucosal inflammation. Ulceration or prolapse of the submucosa can occur, with reepithelialization and new gland formation. During this process, a polyp can form from the mucosa because the heightened inflammatory process from epithelial cells, vascular endothelial cells, and fibroblasts affects the bioelectric integrity of the sodium channels at the luminal surface of the respiratory epithelial cell in that section of the nasal mucosa. This response increases sodium absorption, leading to water retention and polyp formation.

Other theories involve vasomotor imbalance or epithelial rupture. The vasomotor imbalance theory postulates that increased vascular permeability and impaired vascular regulation cause detoxification of mast-cell products (eg, histamine). The prolonged effects of these products within the polyp stroma result in marked edema (especially in the polyp pedicle) that is worsened by venous drainage obstruction. This theory is based on the cell-poor stroma of the polyps, which is poorly vascularized and lacks vasoconstrictor innervation.

The epithelial rupture theory suggests that rupture of the epithelium of the nasal mucosa is caused by increased tissue turgor in illness (eg, allergies, infections). This rupture leads to prolapse of the lamina propria mucosa, forming polyps. The defects are possibly enlarged by gravitational effects or venous drainage obstruction, causing the polyps. This theory, although similar to Bernstein's, provides a less convincing explanation for polyp enlargement than the sodium flux theory supported by Bernstein's data. Neither theory completely defines the inflammatory trigger.

Patients with CF have a defective small chloride conductance channel, regulated by cyclic adenosine monophosphate (cAMP), which causes abnormal chloride transport across the apical cell membrane of epithelial cells. The pathogenesis of nasal polyposis in patients with CF could be associated with this defect.

Frequency:

• In the US: Overall incidence of nasal polyps in children is 0.1%; incidence in children with CF is 6-48%. Among adults, incidence is 1-4% overall, with a range of 0.2-28%.

• Internationally: Incidence worldwide is the same as incidence in the United States.

Mortality/Morbidity: No significant mortality is associated with nasal polyposis. Morbidity is usually associated with altered quality of life, nasal obstruction, anosmia, chronic sinusitis, headaches, snoring, and postnasal drainage. In certain situations, nasal polyps can alter the craniofacial skeleton because unremoved polyps can extend intracranially and into the orbital vaults.

Race: Nasal polyps occur in all races and social classes.

Sex: Although the male-to-female ratio is 2-4:1 in adults, the ratio in children is unreported. A review of articles reporting on children whose nasal polyposis required surgery showed apparently equal prevalence in boys and girls, although the data are inconclusive. Reported prevalence is equal in patients with asthma.

Age: Benign multiple nasal polyposis usually manifests in patients older than 20 years and is more common in patients older than 40 years. Nasal polyps are rare in children younger than 10 years.

CLINICAL

Section 3 of 12

History:

• The manifestation of nasal polyps depends on the size of the polyp. Small polyps may not produce symptoms and may be identified only during routine examination when they are anterior to the anterior edge of the middle turbinate. Polyps located posterior to the site are not typically seen during routine anterior rhinoscopy examination performed with an otoscope and are missed unless the child is symptomatic. Small polyps in areas where polyps normally arise (ie, the middle meatus) may produce symptoms and block the outflow tract of the sinuses, causing chronic or recurrent acute sinusitis symptoms.



• Symptom producing polyps can cause nasal airway obstruction, postnasal drainage, dull headaches, snoring, and rhinorrhea. Associated hyposmia or anosmia may be a clue that polyps, rather than chronic sinusitis alone, are present. Epistaxis that does not arise from irritation of the anterior nasal septum (ie, Kiesselbach area) usually does not occur with benign multiple polyps and may suggest other, more serious, nasal cavity lesions.



• Massive polyposis or a single large polyp (eg, antral-choanal polyp [Images 4-12] that obstructs the nasal cavities and/or nasopharynx) can cause obstructive sleep symptoms and chronic mouth breathing. Rarely, the massive polyposis seen in CF (Images 1-3) and AFS (Image 41) can alter the craniofacial structure (CF - Images 35-36; AFS - Images 42-44) and cause proptosis, hypertelorism, and diplopia (see Images 45-46). In an article submitted for publication, the author has reported 40% of children with AFS presented with craniofacial abnormalities, compared to 10% of adults with AFS. Massive polyposis rarely causes enough extrinsic compression on the optic nerve to decrease visual acuity. Furthermore, because they grow slowly, massive polyposes usually cause no neurological symptoms, even those that extend into the intracranial cavity.

Physical:

• Begin physical examination for nasal polyps with an anterior rhinoscopy procedure (see Image 13). For small children, a handheld otoscope and otologic speculum are typically used. An otoscope placed in the nasal cavity provides views of the inferior turbinate, anterior septum, and areas in the nasal cavity extending to the anterior edge of the middle turbinate and midportion of the septum. The middle meatus (ie, the area under the middle turbinate laterally [Image 14) can often be seen by anterior rhinoscopy if the child is cooperative and if no significant mucosal edema or secretions are present in the anterior nasal cavity.



• For benign nasal polyps, the middle meatus is the most common location. If adequately visible, views of the middle meatus can reveal whether sufficient pathology is present to warrant ordering a CT scan of the sinuses, rather than preforming a rigid or flexible endoscopic procedure that may distress a young patient and the parents.



• However, rigid or flexible endoscopy is the best method to examine the nasal cavity and nasopharynx to fully assess the nasal anatomy (Images 14, 37-39) and to determine the extent and location of nasal polyps. For small children, a flexible fiberoptic nasopharyngoscope is often used because it is less traumatic for children who may move their heads from anxiety or discomfort. In older cooperative children and adolescents, a rigid endoscopy can be used to assess the middle meatus and the sphenoethmoid recess. Perform adequate decongestion and anesthesia of the nasal cavities before an endoscopic procedure for any child older than 6 months. Video documentation of the procedure decreases the amount of time necessary for the procedure and later enhances patient and parent education.



• For children, evaluating the posterior wall of the oral cavity also can indicate the symptomatology of polyposis (eg, postnasal drainage concomitant with chronic sinusitis). Large polyps or lesions of the nasal cavity also may protrude into the posterior oropharynx from the nasopharynx; these may occur as a lesion behind the palate and uvula, or they may depress the palate inferiorly and anteriorly (see Image 10). Perform otoscopic examinations because extensive polyposis causing eustachian tube dysfunction can cause fluid and infection in the middle ear space. Careful examination of the innervated systems of the cranial nerves and of the craniofacial structure helps define a nasal lesion's potential expansion into surrounding vital structures.

Causes:

• As described in Pathophysiology, chronic inflammation (from whatever source) apparently has an initial role in the pathogenesis of nasal polyps. Multiple polyps occur in children with chronic sinusitis, allergic rhinitis, CF, and AFS. An isolated polyp could be an antral-choanal polyp (Images 4-12), a benign massive polyp, a nasolacrimal duct cyst (Images 23-27), or any of a number of congenital lesions or benign or malignant tumors, including the following:
  • Encephaloceles (see Image 15)

  • Gliomas (see Images 16-17)

  • Dermoid tumors (see Images 28-33)

  • Hemangiomas

  • Papillomas (see Image 18)

  • Juvenile nasopharyngeal angiofibromas

  • Rhabdomyosarcoma (see Images 19-21)

  • Lymphomas

  • Neuroblastomas

  • Sarcomas

  • Chordomas

  • Nasopharyngeal carcinomas

  • Inverting papillomas



• Evaluate all children with benign nasal polyposis for CF and asthma.

DIFFERENTIALS

Section 4 of 12

Asthma
Cystic Fibrosis
Neuroblastoma
Neurofibromatosis
Rhabdomyosarcoma
Sinusitis

WORKUP

Section 5 of 12

Lab Studies:

• Direct laboratory studies at the pathological process believed responsible for the nasal polyps.



• Children with polyposis that is associated with allergic rhinitis should have an evaluation for their allergies; this may include a serological radioallergosorbent test (RAST) or some form of allergic skin testing. Mabry and Marple showed a decrease in the recurrence rate of polyps in children treated with immunotherapy directed at all antigens for which they are allergic, especially molds; therefore, allergy testing and treatment may be important in treating AFS.



• Perform a sweat chloride test or genetic testing for CF in any child with multiple benign nasal polyps.



• A nasal smear for eosinophils may differentiate allergic from nonallergic sinus diseases and indicate whether the child may be responsive to glucocorticoids. The presence of neutrophils may indicate chronic sinusitis.

Imaging Studies:

• The criterion standard to evaluate nasal lesions, especially nasal polyposis or sinusitis, is a thin-cut (1-3 mm) CT scan of the maxillofacial area, the sinuses axially, and the coronal plane. Perform appropriate measures to obtain a compatible CT scan if an intraoperative image-guided system is used. Plain film radiographs have no significant value after polyps are diagnosed.



• Also obtain an MRI scan for patients with possible intracranial involvement or extension of benign nasal polyps.



• CT and MRI scans can help diagnose the polyp or polyps; define the extent of the lesion in the nasal cavities, sinuses, and beyond; and narrow the differential diagnosis of an unusual polyp or clinical presentation.


• • CF has a characteristic symmetrical bulging of the lateral nasal walls medially (see Images 35-36, 40).
  
  
  
  • An antral-choanal polyp may show opacified maxillary sinuses with a protruding lesion heading from the maxillary antrum to the choana (see Images 7-9).
  
  
  
  • A tumor, such as a rhabdomyosarcoma, may show extension of the lesion with invasion of surrounding mucosa (see Images 19-20, 25).
  
  
  
  • A nasolacrimal duct cyst can show dilation of the nasolacrimal duct (see Images 25-26).
  
  
  
  • An encephalocele can show expansion of the nasofrontal region (ie, foramen caecum) with herniation of brain or dura.
  
  
  
  • A glioma can show an isolated nasal lesion that may have a fibrous stalk to the CNS.

  • Patients with AFS exhibit heterogenous areas in the sinuses on CT and MRI scans; these areas consist of both the nasal polyposis and the allergic fungal mucin (see Images 42, 44). This allergic fungal mucin appears black on MRI and can be confused with the absence of disease.
  
  
  

Procedures:

• See rigid and flexible endoscopy procedures described in Physical.

Eosinophil cells are the most commonly identified inflammatory cell, occurring in 80-90% of polyps. Eosinophils, which are found in the polyps of patients with bronchial asthma and allergy, contain granules with toxic products (eg, leukotrienes, eosinophilic cationic protein, major basophilic protein, platelet-activating factor, eosinophilic peroxidases, other vasoactive substances and chemotactic factors). These toxic factors are responsible for epithelial lysis, nerve damage, and ciliostasis. Specific granule protein, leukotriene A4, and platelet-activating factor apparently are responsible for the mucosal swelling and hyperresponsiveness.

Eosinophils in the peripheral blood and in normal nasal mucosa usually last 3 days. In a cell culture of nasal polyps, eosinophils were present at least 12 days. This delayed apoptosis of eosinophils is mediated, in part, by blockage of the Fas receptors, typically with proteases that help begin the process of cell death. Delayed apoptosis is also mediated by an increase in interleukin 5 (IL-5), IL-3, and granulocyte-macrophage colony-stimulating factor (GM-CSF) secreted by T lymphocytes, which help sustain the eosinophil from death. Glucocorticoids seem to help reduce polyps or polypoid reactions in patients with tissue eosinophilia, possibly in part by inhibiting IL-5.

Another inflammatory cell, the neutrophil, occurs in 7% of polyp cases. This type of polyp occurs in association with CF, primary ciliary dyskinesia syndrome, or Young syndrome. These polyps do not respond well to corticosteroids because they lack corticosteroid-sensitive eosinophils. Degranulated mast cells are present. Degranulation presumably occurs in a nonimmunoglobulin E (IgE)-mediated fashion. Increased numbers of plasma cells, lymphocytes, and myofibroblasts also occur.

Chemical mediators

The stroma of nasal polyps have numerous mediators, including cytokines, growth factors, adhesion molecules, and immunoglobulins; polyps also contain vasoactive amines, serotonin, prostaglandins, leukotrienes, norepinephrine, kinins, esterases, heparin, and histamine. The level of histamine in nasal polyps is 100-1000 times the level found in the blood stream.

• Cytokines present in polyps
  • Interleukin 1 (IL-1) - Found regularly

  • IL-3 - Varies, based on study, from absent to intermittent at low levels to regularly present

  • IL-4 - Inconsistently detected

  • IL-5 - Found regularly; IL-5 is essential for proliferation and differentiation of eosinophils. IL-5 is chemotactic to eosinophils, promotes the migration of eosinophils from the systemic circulation to the polyps, and inhibits eosinophil cell death.

  • IL-6 - Same as in controls (no increase)

  • IL-8 - Varies, based on study, from undetected to regularly detected; may cause sustained recruitment of leukocytes into nasal polyps and may decrease fibroblastic proliferation

  • IL-10 - Same as in controls; no increase RANTES (ie, regulated on activation, normal T cell expressed and secreted); varies, based on study, from same as controls to regularly detected to increased levels interferon gamma; increases in eosinophils, seromucous glands, and epithelium of nasal polyps

• Growth factors found in nasal polyps
  • Tumor necrosis factor (TNF) alpha and beta - Varies, based on study, from same as controls to regularly detected; believed to be from eosinophils

  • GM-CSF - mRNA and protein amount varies, based on study, from never to intermittent to present

  • Platelet derived growth factor - Present

  • Vascular permeable factors (VPFs) - Present

  • Vascular endothelial growth factors (VEGFs) - Present

  • Insulinlike growth factor I - Present

  • Stem cell factor - Present

• Adhesion molecules
  • Vascular adhesion molecule 1 (VCAM-1) - Present

  • E and P selectin - Present

• Immunoglobulins (Ig)
  • IgG - No increase; same levels as in the middle and inferior turbinate mucosa

  • IgA - More in polyps than in the middle and inferior turbinate mucosa, especially IgA1 over IgA2

  • IgM - No increase, same as in the middle and inferior turbinate mucosa

  • IgD - No increase, same as in the middle and inferior turbinate mucosa

  • IgE - Increased levels compared to the middle and inferior turbinate mucosa; same level in nonallergic patients as in allergic patients

Staging: Polyposis has no uniform staging system.

TREATMENT

Section 6 of 12

Medical Care:

• Oral and topical nasal steroid administration is the primary medical therapy for nasal polyposis. Antihistamines, decongestants, and cromolyn sodium provide little benefit. Immunotherapy may be useful to treat allergic rhinitis but, when used alone, does not usually resolve existing polyps. Administer antibiotics for bacterial superinfections.



• Corticosteroids are the treatment of choice, either topically or systemically. Direct injection into the polyp is not approved by the Food and Drug Administration because of reports of unilateral vision loss in 3 patients after intranasal steroid injection with Kenalog. Safety may depend on specific drug particle size; large molecular weight drugs such as Aristocort are safer and less likely to be transferred to the intracranial area. Avoid direct injection into blood vessels.



• Oral steroids are the most effective medical treatment for nasal polyposis. In adults, most authors use prednisone (30-60 mg) for 4-7 days and taper the medicine for 1-3 weeks. Dosage varies for children, but the maximum dose usually is 1 mg/kg/d for 5-7 days, then taper over 1-3 weeks. Responsiveness to corticosteroids appears to depend on the presence or absence of eosinophilia, so patients with polyps and allergic rhinitis or asthma should respond to this treatment.



• Patients with polyposis not dominated by eosinophilia (eg, patients with CF, primary ciliary dyskinesia syndrome, or Young syndrome) may not respond to steroids. Long-term use of oral steroids is not recommended because of the numerous potential adverse effects (eg, growth retardation, diabetes mellitus, hypertension, psychotropic effects, adverse GI effects, cataracts, glaucoma, osteoporosis, and aseptic necrosis of the femoral head).



• Many authors advocate topical nasal steroid administration for nasal polyps, either as the primary treatment or as a continual secondary treatment immediately following PO steroids or surgery. Most nasal steroids (eg, fluticasone, beclomethasone, budesonide) effectively relieve subjective symptoms and increase the nasal airflow when measured objectively (primarily in double-blind placebo-controlled studies). Some studies indicate fluticasone has a faster onset of action and possible mild superiority to beclomethasone.



• Topical corticosteroid administration generally causes fewer adverse effects than systemic corticosteroid use because of the former's limited bioavailability. Long-term use, especially at high dosages or in combination with inhaled corticosteroids, presents a risk of hypothalamic-pituitary-adrenal axis suppression, cataract formation, growth retardation, nasal bleeding, and, in rare cases, nasal septal perforation.

• As with any long-term therapy, monitor use of topical corticosteroid sprays. However, long-term (>5 y) studies evaluating the use of beclomethasone have shown no degradation of the normal respiratory epithelium to squamous epithelium seen in chronic atrophic rhinitis. Additionally, the newer generation of systemic steroids (eg, fluticasone, Nasonex) appears to have less bioavailability than older nasal steroids, such as beclomethasone.

Surgical Care:

• Surgical intervention is required for children with multiple benign nasal polyposis or chronic rhinosinusitis who fail maximum medical therapy. Simple polypectomy is effective initially to relieve nasal symptoms, especially for isolated polyps or small numbers of polyps. In benign multiple nasal polyposis, polypectomy is fraught with a high recurrence rate.



• Endoscopic sinus surgery (ESS) is a better technique that not only removes the polyps but also opens the clefts in the middle meatus, where they most often form, which helps decrease the recurrence rate. The exact extent of the surgery needed, whether complete extirpation (ie, Nasalide procedure) or simple aeration of the sinuses, is not entirely known, simply because of the dearth of studies. Rare comparisons show that complete extirpation procedures are as effective or superior to aeration of the sinuses; complication rates are low with experienced surgeons. Use of a surgical microdebrider (see Image 34) has made the procedure safer and faster, providing precise tissue cutting and decreased hemostasis with better visualization.



• Direct surgery at diseased tissue that is apparent on the CT scan at the time of surgery. Patients with diseases such as CF, primary ciliary dyskinesia syndrome, or Young syndrome may proceed to surgery without extensive medical treatment because these diseases usually do not respond well to corticosteroid treatment. Once diseased tissue has been removed from the nasal cavity and sinuses, the pulmonary systems usually improve. Consider use of an image-guided system to define the exact location of intranasal, sinus, orbital, and intracranial structures for massive polyposis or revision surgery because surgical landmarks may be absent or altered. For specific techniques in pediatric sinus surgery, with and without polyps, see Pediatric Sinusitis, Surgical Treatment.



• Nasal polyposis occurs in 6-48% of children with CF. Surgery is performed when children become symptomatic. Recurrence of polyps in CF is almost universal, requiring repeated surgeries every few years. In fact, recurrence is typical for many diseases that cause nasal polyps; patients should receive preoperative counseling about this possibility.



• For lesions other than benign nasal polyps that result in a nasal polyp, the polyp should be biopsied or removed, depending on the disease process.

Consultations:

• First notify a pediatric otolaryngologist, especially if medical therapy has failed or if the origin or diagnosis of the underlying pathology of the nasal polyp is unknown.



• Consider consultation with a pulmonary specialist when benign nasal polyps are identified because they could result from asthma, allergy, or CF. Patients with these diseases often have associated pulmonary problems.

Diet: Treatment of nasal polyps involves no special diet.

Activity:

• No activity restrictions are necessary for a child with nasal polyps. The child's activity level may decrease because of diminished ability to breath through the nose, decreasing sport or physical activity performance. After sinus surgery, activities are limited; these limitation recommendations vary from surgeon to surgeon. Most surgeons specifically restrict nose blowing because it may increase intranasal pressure and cause potential problems in areas of already thinned bony dividers in patients with nasal polyposis.

MEDICATION

Section 7 of 12

(See Medical Care.)

Drug Category: Corticosteroids -- Corticosteroids have potent anti-inflammatory action and relieve rhinorrhea, sneezing, itching, and congestion.

Drug Name	Prednisolone (Prelone, Orapred, Pediapred) -- Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reducing capillary permeability.
Adult Dose	Dosage varies: for nasal polyps short course (ie, 5-7 d), 1 mg/kg/d PO up to 40-80 mg/d, depending on weight; for >5-7 d, taper to smaller doses/d over time
Pediatric Dose	1 mg/kg/d PO for 5-7 d in preparation for ESS; occasionally, dose can be used to try to shrink polyps for temporary relief; if used >5-7 d, taper to smaller doses/d over time
Contraindications	Documented hypersensitivity; fungal or tubercular skin lesions
Interactions	Decreases effects of salicylates and toxoids (for immunizations); phenytoin, carbamazepine, barbiturates, and rifampin decrease effects of corticosteroids
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Caution in hyperthyroidism, osteoporosis, cirrhosis, nonspecific ulcerative colitis, peptic ulcer, diabetes, and myasthenia gravis

Drug Name	Prednisone (Deltasone, Orasone, Meticorten, Sterapred) -- May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.
Adult Dose	Dosage varies: for nasal polyps short course (ie, 5-7 d), 1 mg/kg/d PO up to 40-80 mg/d, depending on weight; for >5-7 d, taper to smaller doses/d over time
Pediatric Dose	1 mg/kg/d PO for 5-7 d in preparation for ESS; occasionally, dose can be used to try to shrink polyps for temporary relief; if used >5-7 d, taper to smaller doses/d over time
Contraindications	Documented hypersensitivity; peptic ulcer disease, hepatic dysfunction, connective tissue infections, and fungal or tubercular skin infections; GI disease
Interactions	Coadministration with estrogens may decrease 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
Pregnancy	B - Usually safe but benefits must outweigh the risks.
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

Drug Name	Dexamethasone (Decadron) -- Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reducing capillary permeability.
Adult Dose	6-8 mg IV/IM q6h prn for inflammation for short course of a few d; taper over time if longer than a few d, as in pediatric dose
Pediatric Dose	1-2 mg/kg/d IV divided q6h prn for inflammation; if used > 2 d, taper quickly to lowest possible dose
Contraindications	Documented hypersensitivity; active bacterial or invasive fungal infection
Interactions	Effects decrease with coadministration of barbiturates, phenytoin, and rifampin; decreases effect of salicylates and vaccines used for immunization
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Increases risk of multiple complications, including severe infections; monitor adrenal insufficiency when tapering drug; 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 are possible complications of glucocorticoid use

Drug Category: Nasal Corticosteroids -- These agents induce a nonspecific anti-inflammatory response that should theoretically reduce the size of polyps and prevent regrowth when continuously used. Available nasal steroid sprays appear to be similarly effective and relatively safe for both short- and long-term use.

Drug Name	Mometasone (Nasonex) -- Nasal spray; demonstrated no mineralocorticoid, androgenic, antiandrogenic, or estrogenic activity in preclinical trials. Studies concerning bioavailability are established; should be considered first-line when treating pediatric patients. Not systemically absorbed like other nasal steroids (ie, beclomethasone). Studies concerning bioavailability are established; should be considered first-line when treating pediatric patients. Not systemically absorbed like other nasal steroids (ie, beclomethasone).
Adult Dose	2 sprays (50 mcg/spray) each nostril qd; supplement with saline nasal spray
Pediatric Dose	Not FDA-approved for nasal polyps in children <18 y; dose listed below is FDA-approved dose for allergic rhinitis in children <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
Interactions	None reported
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Use with caution in patients with active or quiescent tuberculosis of the respiratory tract, ocular herpes, or untreated fungal, bacterial, systemic viral infections; rare instances of decreased growth velocity in pediatric patients have been reported; rare instances of nasal septum perforation and increased IOP have also been reported; nasal and inhaled corticosteroids have been associated with development of glaucoma and/or cataracts

Drug Name	Fluticasone propionate (Flonase) -- Topical nasal steroid. Has extremely potent vasoconstrictive and anti-inflammatory activity. Has a weak hypothalamic-pituitary-adrenocortical axis inhibitory potency when applied topically. Studies concerning bioavailability are established; should be considered first line when treating pediatric patients. Not systemically absorbed like other nasal steroids (ie, beclomethasone). Should use nasal steroid spray with fluticasone propionate to help buffer the nose and prevent complications from the spray, such as nasal drying, epistaxis, and, in long-term use, septal perforation.
Adult Dose	2 sprays/nostril qd (50 mcg/spray); supplement with saline nasal spray
Pediatric Dose	4-11 years: 1-2 sprays/nostril qd (50 mcg/spray); supplement with saline nasal spray
Contraindications	Documented hypersensitivity; viral, fungal, and bacterial skin infections
Interactions	None reported
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Do not use to abort asthma attack; may cause hoarseness, burning, epistaxis, pruritus; caution in glaucoma or cataracts

Drug Name	Budesonide (Rhinocort) -- Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability.
Adult Dose	2 sprays/nostril bid (32 mcg/spray); supplement with saline nasal spray
Pediatric Dose	>6 years: Administer as in adults
Contraindications	Documented hypersensitivity
Interactions	None reported
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Not used to abort acute asthmatic episodes

Drug Name	Triamcinolone (Nasacort, Nasacort AQ) -- Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability.
Adult Dose	2-4 sprays/nostril qd (55 mcg/spray); supplement with saline nasal spray
Pediatric Dose	6-12 years: 1-2 sprays/nostril qd (55 mcg/spray); supplement with saline nasal spray >12 years: Administer as in adults
Contraindications	Documented hypersensitivity
Interactions	None reported
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Do not use at higher than recommended doses

Drug Name	Beclomethasone (Vancenase, Beconase) -- Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability.
Adult Dose	For 42 mcg/spray: 1-2 sprays/nostril bid with saline nasal spray For 84 mcg/spray: 1-2 sprays/nostril qd with saline nasal spray
Pediatric Dose	<6 years: Not established >>/u>6 years: Administer as in adults
Contraindications	Documented hypersensitivity
Interactions	Coadministration with ketoconazole may increase plasma levels, but levels do not appear clinically significant
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Intranasal bioavailability is 44% and reports have shown a slowing of growth curves when beclomethasone inhaled sprays are administered to children Weight gain, increased bruising, cushingoid features, acneiform lesions, mental disturbances, and cataracts may occur (taper medication slowly if these changes occur)

FOLLOW-UP

Section 8 of 12

Further Inpatient Care:

• Historically, children diagnosed with CF already had digestive and pulmonary disease and were the children with the more severe form of disease. These children were often treated with IV antibiotics directed at the most common pathogens found in the lungs and the sinuses (eg, Pseudomonas aeruginosa, Staphylococcus aureus), both preoperatively and postoperatively. Additionally, these children had pulmonary toilet to increase their lung function in the perioperative period, including IV steroids, percussion therapy, and inhaled bronchodilators. Much of this process can now be performed on an outpatient basis, depending on the severity of the associated disease.



• For patients with severe asthma and polyposis requiring surgery, postoperative admission for observation of respiratory compromise or spasm is determined on an individual basis.



• Outpatient surgery is usually performed for older children undergoing ESS for nasal polyposis without coexisting medical conditions.

Further Outpatient Care:

• Closely monitor children with benign multiple nasal polyps, whatever the cause, because recurrence is likely, whether treated medically or surgically. Postoperative follow-up should occur 3-4 times the first month to monitor healing of the sinus cavities; frequency depends on the patient's own geographic location and symptoms.



• A patient with CF can be monitored symptomatically because surgery is not performed until these patients are symptomatic, even if nasal polyposis is seen on CT scan or nasal endoscopy. Certainly, each patient is treated on an individual basis.



• For polyps associated with AFS, close follow-up by an otolaryngologist is recommended until the patient is deemed free of disease, which may be several years or more.



• Any accumulation of fungus may accelerate the antigenic process, which causes symptoms and disease to recur. Recurrence is especially common for polyps, which may be controlled more simply and effectively if recognized early.



• Small nasal polyps are recognized early on a routine follow-up in patients with benign multiple nasal polyps.



• Other diseases may be treated medically or with smaller surgical procedures. For diseases resulting in nasal polyps other than benign multiple nasal polyps, the need for inpatient or outpatient care is determined by the extent of disease, symptoms and situation of the patient, and associated medical conditions.

In/Out Patient Meds:

• See Medical Care and Medication.

Deterrence/Prevention:

• See Medical Care.

Complications:

• Massive polyposis or a single large polyp (eg, an antral-choanal polyp [Images 4-12]) that obstructs the nasal cavities and/or nasopharynx can cause obstructive sleep symptoms and chronic mouth breathing. Rarely, massive polyposis, observed in CF (Images 1-3) and in AFS (Image 41) can alter the craniofacial structure. This can result in proptosis, hypertelorism, and diplopia (see Images 45-46).



• In an article submitted for publication, the author reported that 40% of children (compared to 10% of adults) with AFS presented with craniofacial abnormalities. Massive polyposis rarely causes enough extrinsic compression on the optic nerve to decrease visual acuity. Newcomber reported that 3 of 82 patients with AFS had vision changes from compression of the optic nerve in the sphenoid sinus that resolved over time with removal of disease. However, because these polyps are slow growing, they usually cause no neurological symptoms, even when they extend into the intracranial cavity.

Prognosis:

• Polyposis recurrence is common following treatment with medical or surgical therapy if multiple benign polyps are present (see Surgical Care). Single large polyps (eg, antral-choanal polyps) are less likely to recur. The literature contains sparse data comparing treatments.

Patient Education:

• Educating patients about the chronicity of the disease is important to make them aware of the recurrent nature of the problem.

MISCELLANEOUS

Section 9 of 12

Medical/Legal Pitfalls:

• Unfortunately, many processes that affect the nasal cavity cause the same symptoms. Frequent upper respiratory tract infections, allergic rhinitis, nonallergic rhinitis, chronic sinusitis, recurrent acute sinusitis, adenoid hypertrophy, and chronic adenoiditis, often create symptoms similar to those caused by nasal polyps or tumors. Thus, nasal polyps or tumors may exist for some time before being diagnosed, possibly leading the patient or the patient's parents to assume a delayed diagnosis.



• This is a difficult problem for physicians and must be handled delicately by all caregivers. However, failure to recognize signs and symptoms of advanced disease that affect other areas of the face, head, or neck (eg, paresthesias of the face, alteration of the craniofacial structure, significant persistent epistaxis, neurological symptoms, cranial nerve abnormalities) could expose patients to complications that could have been avoided if those symptoms were discovered earlier.

TEST QUESTIONS

Section 10 of 12

CME Question 1: What are the most common cells found in nasal polyps?

A: Neutrophil cells
B: Eosinophil cells
C: Basophil cells
D: Lymphocyte cells
E: Macrophage cells

The correct answer is B: Eosinophil cells are the most common cells found in most polyps and occur in 80-90% of cases. Neutrophils are found in 7% of polyps.

CME Question 2: What is the most common comorbidity associated with nasal polyps?

A: Asthma
B: Chronic rhinosinusitis
C: Allergic rhinitis
D: Allergic fungal sinusitis
E: Cystic fibrosis

The correct answer is A: Bronchial asthma occurs in 20-50% of patients with polyps.

Pearl Question 1 (T/F): Most patients with nasal polyposis have concurrent allergic rhinitis.

The correct answer is False: Most studies suggest than polyps are associated more strongly with nonallergic diseases than allergic diseases. Nasal polyps statistically are more common in nonallergic asthma than allergic asthma (13% vs 5%, probable error <0.01), and only 0.5% of 3000 atopic individuals have nasal polyps.

Pearl Question 2 (T/F): The presence of eosinophilia indicates the responsiveness of nasal polyps to steroids.

The correct answer is True: The presence of eosinophilia indicates the responsiveness of nasal polyps to steroids.

Pearl Question 3 (T/F): Polyps are more common in children than adults.

The correct answer is False: Overall incidence in children is 0.1%, compared to 1-4% in adults. Incidence in children with cystic fibrosis ranges from 6-48%. Benign multiple nasal polyposis usually presents after age 20 years and more commonly presents after age 40 years; it is rare in children younger than 10 years.

Pearl Question 4 (T/F): Plain film x-rays help diagnose and treat nasal polyps.

The correct answer is False: Plain film x-rays have no value in the diagnosis or management of nasal polyps. A CT scan usually is adequate to diagnosis and treat benign multiple nasal polyps. An MRI scan helps when disease is extensive and diagnosis is uncertain.

PICTURES

Section 11 of 12

Caption: Picture 1. Rigid endoscopic view of the left nasal cavity, showing the septum on the left. Polyps with some blood and hemorrhage are on top of them in the center portion. The rim of white from 1 o'clock to 4 o'clock indicates the lateral nasal wall vestibule. The polyps cover the inferior turbinate, which is partially visible at 4 and 5 o'clock.
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Caption: Picture 2. Endoscopic view of the left nasal cavity, showing a polyp protruding from the uncinate process. The middle turbinate is to the left. A suction is visible on top of the inferior portion of the uncinate process and inferior portion of the polyp. The lateral nasal wall is on the far right. The polyp is directly in the center and is pale, glistening, and white.
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Caption: Picture 3. Endoscopic view of the left middle meatus. The septum is on the far left. The middle turbinate is next to the septum on the left. A large, glistening, translucent polyp is visible in the center of the screen next to the middle turbinate. The lateral nasal wall is on the right side of the screen. The inferior turbinate nub posteriorly is in the bottom right hand corner.
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Caption: Picture 4. Rigid endoscopic view of the left nasal cavity, showing the septum on the left, inferior turbinate on the right, middle turbinate superiorly, and antral-choanal polyp among the floor of the nose.
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Caption: Picture 5. Rigid endoscopic view of the left anterior nasal cavity, showing the septum on the left, a suction pushing the inferior turbinate on the right, and the clear antral-choanal polyp at the center of the endoscopic view.
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Caption: Picture 6. Close-up of the middle meatus, showing the stalk of the antral-choanal polyp emanating from the maxillary sinus behind the uncinate process on the bottom right-hand side of the picture. The left side of the picture shows the septum and the middle turbinate being pushed over via suction.
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Caption: Picture 7. Axial CT scan section through the maxillary sinuses showing opacification of the left maxillary sinus with antral-choanal polyp in the posterior nasal cavity and choana exiting from beneath the middle turbinate in the area of the osteomeatal complex unit. Scale is in centimeters.
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Caption: Picture 8. Coronal CT scan through the anterior sinuses showing opacification of the left maxillary sinus with opacification of the inferior half of the nasal cavity on the left, filled by the antral-choanal polyp. The rest of the sinuses are clear.
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Caption: Picture 9. Coronal CT scan section through the posterior nasopharynx showing the sphenoid sinus superiorly and the antral-choanal polyp filling the nasopharynx in the center of the scan.
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Caption: Picture 10. Oral cavity and oropharyngeal view of antral-choanal polyp filling the posterior oral pharynx and pushing the soft palate anterior and inferiorly. The polyp is visible behind the uvula and the soft palate.
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Caption: Picture 11. Scale is in inches. The left side of the lesion was the portion of the polyp in the nasal cavity. The right was a stalk attached to the medial maxillary wall.
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Caption: Picture 12. Endoscopic view of the left middle meatus, showing the septum on the left, the middle turbinate in the center superiorly, and a large maxillary antrostomy with a curved suction on the right. This is following antral-choanal polyp removal.
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Caption: Picture 13. An anterior endoscopic view of the nasal cavity in a 5-month-old infant. The vestibule is seen in the periphery of the picture. In the center of the picture, the septum is visible to the left, and the inferior turbinate is to the right. These structures are reddish in hue. There is some congestion in the nasal cavity. These often are structures that can be seen only by anterior rhinoscopy. If the area is decongested, the area of the middle meatus occasionally can be seen.
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Caption: Picture 14. A rigid rhinoscopy photograph of the left anterior nasal cavity. The middle turbinate is superiorly in the midline, and the inferior turbinate is to the right. The septum is to the left. This photograph is the first of 4 images of the rigid rhinoscopy pathway down the nasal cavity of a 6-week-old infant.
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Caption: Picture 15. A 3-month-old infant with hypertelorism and bulging of the nasal dorsum, secondary to encephalocele.
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Caption: Picture 16. Interior view of the nose and nasal cavities. To the right of the patient's left nostril, the right nasal cavity has no obstruction. On the left of the picture, a reddish polyp is visible. The reddish mass is a nasal glioma.
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Caption: Picture 17. A close-up view of the right nasal cavity and polyp #5 in a 5-month-old infant. The obstructing reddish polyp is visible. This is an intranasal glioma that was arising from the attachment of the inferior turbinate anteriorly; it was removed transnasally.
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Caption: Picture 18. Anterior nasal papilloma arising from the septum. The skin of the nasal vestibule is seen surrounding the papilloma in the center of the image.
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Caption: Picture 19. Axial MRI scan of the orbits, posterior fossa, and nasal cavity. The solid tumor is seen filling the posterior ethmoid complex, brain stem, cavernous sinuses, and left anterior cranial fossa.
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Caption: Picture 20. Axial CT scan through the orbits and ethmoid sinuses, showing the rhabdomyosarcoma in the same areas, including the posterior ethmoid complex, left middle fossa, and skull base of cavernous sinuses.
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Caption: Picture 21. Rigid endoscopic view of left nasal cavity, showing a polyp in the center of the picture, with extension of the rhabdomyosarcoma. The septum is on the left and the middle turbinate is on the right.
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Caption: Picture 22. Endoscopic view of the left nasal cavity posteriorly, showing a polyp emanating from the sphenoid sinus in the center of the picture and purulence above and below the polyp. On the left is the septum. On the right is the lateral aspect of the middle turbinate.
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Caption: Picture 23. Frontal view of a 2-day-old infant with swelling in the inferior medial canthal area on both sides. The right side appears more prominent on this picture. CT scan showed infected nasal lacrimal duct cysts.
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Caption: Picture 24. Rigid endoscopic view of the left nasal cavity. The septum is on the left, and the lateral nasal wall is on the right. The inferior turbinate is in the center of the picture, and the middle turbinates are visible in the superior midsection of the picture. The nasal lacrimal duct cyst is the yellow dilated lesion underneath the inferior turbinate.
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Caption: Picture 25. Axial CT scan section through the orbit, showing the dilated nasal lacrimal ducts in the medial anterior area compared to the orbits. Scale on the bottom right is in centimeters.
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Caption: Picture 26. Axial CT scan through the inferior nasal cavities, showing the dilated nasal lacrimal duct cysts at the inferior location. Scale on the bottom right is in centimeters. The dilated cysts are in the center of the image.
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Caption: Picture 27. A frontal view of the decompressed nasal lacrimal ducts following surgical marsupialization. The swelling in the inferior medial canthal areas, portrayed in Image 23, is no longer seen.
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Caption: Picture 28. Lateral view of a preteenaged child showing infected nasal dermoid. Note the protrusion of the dorsum of the nose. This is the same child as in Image 29.
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Caption: Picture 29. Preteenaged African American boy with infected nasal dermoid. There is a pith visible over the superior portion of the swelling between the eyes. Nasal pith commonly is seen with the nasal dermoid.
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Caption: Picture 30. Frontal view of a 5-month-old infant, showing hypertelorism and protrusion in the glabellar region secondary to a small nasal dermoid.
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Caption: Picture 31. Axial CT scan (bony windows) showing a 5-month-old infant with nasal dermoid anterior to the nasal and maxillary bones. No bony dehiscence or bony abnormalities are visible.
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Caption: Picture 32. A coronal MRI scan through the nasal dermoid. Scale on the left is 2 mm per small bar and 1 cm per tall bar. The arrow points to the lesion. The lesion appears to be approximately 6-7 mm in this dimension. This is the same patient as in Images 30, 31, and 33.
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Caption: Picture 33. An interoperative view of dermoid removal. This is the same patient as in Images 30, 31, and 32.
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Caption: Picture 34. A surgical microdebrider entering the middle meatus. The septum is on the far left. The middle turbinate is in the left center. The surgical microdebrider is on the inferior center. Inferior turbinate is seen on the bottom right. There is some blood overlying the ethmoid cavity where polyps were present in the center of the picture.
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Caption: Picture 35. Coronal section through the ethmoid maxillary sinuses and orbits. This is a 2-year-old child with cystic fibrosis, showing complete opacification of the maxillary and ethmoid sinuses. There is bulging in the medial maxillary walls.
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Caption: Picture 36. Coronal section of the same patient as in Image 35, showing soft tissue windows rather than bony windows. It indicates the infection by the thick mucus in the maxillary and ethmoid cavities by the heterogeneity of the opacification in the sinuses. Note that the nasal cavity is obliterated completely by polyp disease.
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Caption: Picture 37. A rigid rhinoscopy photograph taken all the way back into the choanae of the left nasal cavity. The photograph shows the septum on the left, the small adenoids on the posterior superior wall of the nasopharynx in the center, and the eustachian tube orifice on the right. This photograph is the fourth of 4 images of the rigid rhinoscopy pathway down the nasal cavity of a 6-week-old infant.
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Caption: Picture 38. A rigid rhinoscopy photograph taken in the midportion of the left nasal cavity showing the septum on the left, the inferior turbinate on the right, and the middle turbinate superiorly. The choanae is seen in the dark area in the center. This photograph is the second of 4 images of the rigid rhinoscopy pathway down the nasal cavity of a 6-week-old infant. The first one is Image 14.
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Caption: Picture 39. A rigid rhinoscopy photograph taken two thirds of the way back along the floor of the nose of the left nasal cavity. This photograph shows the septum on the left, the choanae straight ahead, and the posterior portion inferior turbinate to the right. This photograph is the third of 4 images of the rigid rhinoscopy pathway down the nasal cavity of a 6-week-old infant.
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Caption: Picture 40. A coronal CT scan section through the orbit to maxillary sinus. The