Pediatric Nasal Polyps 

Updated: Oct 25, 2019
Author: John E McClay, MD; Chief Editor: Ravindhra G Elluru, MD, PhD 

Overview

Practice Essentials

Broadly defined, nasal polyps are abnormal lesions that originate 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 that arise from the mucosa of the nasal cavity or from one 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 benign or malignant tumor (eg, encephalocele, glioma, hemangioma, papilloma, juvenile nasopharyngeal angiofibroma, rhabdomyosarcoma, lymphoma, neuroblastoma, sarcoma, chordoma, nasopharyngeal carcinoma, inverting papilloma). All children with benign multiple nasal polyposis should be evaluated for CF and asthma. Educating patients about the chronicity of the disease is important to make them aware of the recurrent nature of the problem.

Oral and topical nasal steroid administration is the primary medical therapy for nasal polyposis. Surgical intervention is required for children with multiple benign nasal polyposis or chronic rhinosinusitis in whom maximal medical therapy fails.

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 6-44% of patients with CF [1]
  • Allergic rhinitis
  • AFS - Polyps in 85% of patients with AFS
  • Chronic rhinosinusitis
  • 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, though 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 that polyps are a distinct entity arising from the mucosa. On the basis of a review of the literature and several intricate studies of the bioelectric properties of polyps, Bernstein derived a convincing theory regarding the pathogenesis of nasal polyps, building on other theories and information from Tos et al.[2, 3]

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, though 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.

Etiology

As noted (see Pathophysiology), chronic inflammation (from whatever source) apparently plays 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, a benign massive polyp, a nasolacrimal duct cyst, or any of the following congenital lesions or benign or malignant tumors:

  • Nasolacrimal duct cysts
  • Encephaloceles
  • Gliomas
  • Dermoid tumors
  • Hemangiomas
  • Papillomas
  • Juvenile nasopharyngeal angiofibromas
  • Rhabdomyosarcoma
  • Lymphomas
  • Neuroblastomas
  • Sarcomas
  • Chordomas
  • Nasopharyngeal carcinomas
  • Inverting papillomas

All children with benign nasal polyposis should be evaluated for CF and asthma.

Epidemiology

In the United States, the overall incidence of nasal polyps in children is 0.1%; the incidence in children with CF is 6-48%. Among adults, the incidence is 1-4% overall, with a range of 0.2-28%. Worldwide incidence is the same as the incidence in the United States.

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. 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, though the data are inconclusive.[4] The reported prevalence is equal in patients with asthma. Nasal polyps occur in all races and social classes.

Prognosis

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.

Polyposis recurrence is common following treatment with medical or surgical therapy if multiple benign polyps are present (see Treatment, Surgical Care). Single large polyps (eg, antral-choanal polyps) are less likely to recur. 

Endoscopic sinus surgery appears to improve both olfaction and quality of life in chronic rhinosinusitis patients with nasal polyps.[5, 6]  In a study of 58 pediatric patients (< 18 years) with antrochoanal polyps who were treated with functional endoscopic sinus surgery (FESS), Pagella et al reported a recurrence rate of 20.5%.[7]

The literature contains sparse data comparing treatments. Galluzzi et al performed a systematic review (N = 285) aimed at evaluating recurrence rates after different surgical procedures used to treat antrochoanal polyps in children, including FESS, a combined approach (FESS with a transcanine sinusoscopy or mini Caldwell-Luc procedure), the Caldwell-Luc procedure, and simple polypectomy.[8]  Recurrence rates were as follows:

  • All types of surgery - 15%
  • FESS - 17.7%
  • Combined approach - 0%
  • Caldwell-Luc procedure - 9.1%
  • Simple polypectomy - 50%
 

Presentation

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 [see the images below] that obstructs the nasal cavities, nasopharynx, or both) can cause obstructive sleep symptoms and chronic mouth breathing.

Rarely, patients with cystic fibrosis (CF) and patients with allergic fungal sinusitis (AFS) have massive polyposes. These can alter the craniofacial structure and cause proptosis, hypertelorism, and diplopia. See the images below.

In a retrospective study, McClay et al reported that 42% of children with AFS presented with craniofacial abnormalities, compared with 10% of adults with AFS.[9] 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 neurologic symptoms, even those that extend into the intracranial cavity.

Physical Examination

The patient's facial appearance may vary, depending on the underlying condition (see the images below).

A 3-month-old infant with hypertelorism and bulgin A 3-month-old infant with hypertelorism and bulging of the nasal dorsum, secondary to encephalocele.
Frontal view of a 2-day-old infant with swelling i 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.
A frontal view of the decompressed nasal lacrimal A frontal view of the decompressed nasal lacrimal ducts following surgical marsupialization. Swelling in the inferior medial canthal areas prior to surgery is no longer seen.
Lateral view of a preteenaged child showing infect Lateral view of a preteenaged child showing infected nasal dermoid. Note the protrusion of the dorsum of the nose.
Preteenaged boy with infected nasal dermoid. A pit Preteenaged boy with infected nasal dermoid. A pith is visible over the superior portion of the swelling between the eyes. Nasal pith is commonly seen with the nasal dermoid.
Frontal view of a 5-month-old infant, showing hype Frontal view of a 5-month-old infant, showing hypertelorism and protrusion in the glabellar region secondary to a small nasal dermoid.
Fifteen year-old adolescent boy with allergic fung Fifteen year-old adolescent boy with allergic fungal sinusitis causing right proptosis, telecanthus, and malar flattening; position of his eyes is asymmetrical, and his nasal ala on the right is pushed inferiorly compared with the left.
Nine-year-old girl with allergic fungal sinusitis Nine-year-old girl with allergic fungal sinusitis displaying telecanthus and asymmetrical positioning of her eyes and globes.

Physical examination for nasal polyps should begin with an anterior rhinoscopy procedure (see the images below). 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) can often be seen via anterior rhinoscopy if the child is cooperative and if no significant mucosal edema or secretions are present in the anterior nasal cavity.

Interior view of the nose and nasal cavities. To t 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.
A close-up view of the right nasal cavity and poly 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 transnasally removed.
An anterior endoscopic view of the nasal cavity in 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. Some congestion in the nasal cavity is usually present. These are often structures that can be seen only by anterior rhinoscopy. If the area is decongested, the area of the middle meatus can occasionally be seen.

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 computed tomography (CT) of the sinuses, rather than performing a rigid or flexible endoscopic procedure that may distress a young patient and the parents. However, rigid or flexible endoscopy is the best method for examining the nasal cavity and nasopharynx to fully assess the nasal anatomy and to determine the extent and location of nasal polyps. (See the images below.)

Rigid endoscopic view of the left nasal cavity, sh 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.
Endoscopic view of the left nasal cavity, showing 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.
Endoscopic view of the left middle meatus. The sep 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.
Rigid endoscopic view of the left nasal cavity, sh 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.
Rigid endoscopic view of the left anterior nasal c 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.
Close-up of the middle meatus, showing the stalk o 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.
Endoscopic view of the left middle meatus, showing 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.
A rigid rhinoscopy photograph of the left anterior A rigid rhinoscopy photograph of the left anterior nasal cavity of a 6-week-old infant. The middle turbinate is superiorly in the midline, and the inferior turbinate is to the right. The septum is to the left.
Anterior nasal papilloma arising from the septum. Anterior nasal papilloma arising from the septum. The skin of the nasal vestibule is seen surrounding the papilloma in the center of the image.
Rigid endoscopic view of left nasal cavity, showin 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.
Endoscopic view of the left nasal cavity posterior 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.
Rigid endoscopic view of the left nasal cavity. Th 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.
A rigid rhinoscopy photograph of the the nasal cav A rigid rhinoscopy photograph of the the nasal cavity of a 6-week-old infant 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.
A rigid rhinoscopy photograph taken in the midport A rigid rhinoscopy photograph taken in the midportion of the left nasal cavity of a 6-week-old infant 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.
A rigid rhinoscopy photograph taken two thirds of A rigid rhinoscopy photograph taken two thirds of the way back along the floor of the nose of the left nasal cavity of a 6-week-old infant. This photograph shows the septum on the left, the choanae straight ahead, and the posterior portion inferior turbinate to the right.
View just inside the nasal vestibule of a fifteen- View just inside the nasal vestibule of a fifteen-year-old adolescent boy with allergic fungal sinusitis showing diffused polyposis extending into the anterior nasal cavity and vestibule; the septum is on the right, and the right lateral vestibular wall (nasal ala) is on the left. The polyps are all in the center. The polyps almost hang out of the nasal vestibule.

In small children, a flexible fiberoptic nasopharyngoscope is often used because it is less traumatic for young patients who may move their heads from anxiety or discomfort. In older cooperative children and adolescents, a rigid endoscope can be used to assess the middle meatus and the sphenoethmoid recess. Adequate decongestion and anesthesia of the nasal cavities are necessary before an endoscopic procedure in 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 may also protrude into the posterior oropharynx from the nasopharynx; these may occur as a lesion behind the palate and uvula or may depress the palate inferiorly and anteriorly (see the image below).

Oral cavity and oropharyngeal view of antral-choan 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.

Otoscopic examinations are warranted because extensive polyposis that causes 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.

 

DDx

 

Workup

Laboratory Studies

Laboratory studies should be directed at the pathologic process believed to be responsible for the nasal polyps.

Children with polyposis that is associated with allergic rhinitis should undergo evaluation for their allergies; this may include a serologic radioallergosorbent test (RAST) or some form of allergic skin testing. Mabry et al 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[10] ; therefore, allergy testing and treatment may be important in treating allergic fungal sinusitis (AFS).

Perform a sweat chloride test or genetic testing for cystic fibrosis (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 for evaluating nasal lesions, especially nasal polyposis or sinusitis, is a thin-cut (1-3 mm) computed tomography (CT) scan of the maxillofacial area, the sinuses axially, and the coronal plane. A compatible CT scan should be performed if an intraoperative image-guided system is used. Plain film radiography has no significant value after polyps are diagnosed. Magnetic resonance imaging (MRI) is also warranted in patients with possible intracranial involvement or extension of benign nasal polyps.

CT and MRI findings 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 is associated with a characteristic symmetrical bulging of the lateral nasal walls medially (see the images below).

Coronal section through the ethmoid maxillary sinu 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. Bulging in the medial maxillary walls is observed.
Coronal section showing soft tissue windows rather Coronal section 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 completely obliterated by polyp disease.
A coronal CT scan section through the orbit to max A coronal CT scan section through the orbit to maxillary sinus. The medial maxillary walls bulge medially, which is a typical CT scan view of cystic fibrosis. The ethmoid sinuses have scattered disease.

An antral-choanal polyp may show opacified maxillary sinuses with a protruding lesion heading from the maxillary antrum to the choana (see the images below).

Axial CT scan section through the maxillary sinuse 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 ostiomeatal complex unit. Scale is in centimeters.
Coronal CT scan through the anterior sinuses showi 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.
Coronal CT scan section through the posterior naso 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.

A tumor, such as a rhabdomyosarcoma, may show extension of the lesion with invasion of surrounding mucosa (see the images below).

Axial MRI scan of the orbits, posterior fossa, and 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.
Axial CT scan through the orbits and ethmoid sinus 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.

A nasolacrimal duct cyst can show dilation of the nasolacrimal duct (see the images below).

Axial CT scan section through the orbit, showing t 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.
Axial CT scan through the inferior nasal cavities, 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.

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 central nervous system (CNS).

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

Coronal CT scan showing extensive allergic fungal Coronal CT scan showing extensive allergic fungal sinusitis involving the right side with mucocele above the right orbit and expansion of the sinuses on the right.
Coronal MRI scan showing expansion of the sinuses Coronal MRI scan showing expansion of the sinuses with allergic mucin and polypoid disease; the hypointense black areas in the nasal cavities are the actual fungal elements and debris. The density above the right eye is the mucocele. The fungal elements and allergic mucin in allergic fungal sinusitis always look hypointense on MRI scanning and can be mistaken for absence of disease.

Histologic Findings

Histologically, nasal polyps are characterized by a pseudostratified ciliated columnar epithelium, thickening of the epithelial basement membrane, and few nerve endings. The stroma of nasal polyps is edematous. Vascularization is poor and lacks innervation, except at the base of the polyp. Authors report either hyperplasia of the seromucous glands or almost absent or rare glands when comparing the polyps to the inferior or middle turbinate. Hyperplasia of the gland can cause cystically dilated and degenerated glands containing inspissated mucous.

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 [PAF], 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 PAF 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 (IL)-5, IL-3, and granulocyte-macrophage colony-stimulating factor (GM-CSF) secreted by T cells, 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 non–immunoglobulin (Ig) E–mediated fashion. Increased numbers of plasma cells, lymphocytes, and myofibroblasts also occur.

Chemical mediators

The stromata of nasal polyps have numerous mediators, including cytokines, growth factors, adhesion molecules, and immunoglobulins; polyps also contain vasoactive amines, serotonin, prostaglandins[11] , leukotrienes, norepinephrine, kinins, esterases, heparin, and histamine. The level of histamine in nasal polyps is 100-1000 times the level found in the bloodstream.

Cytokines present in polyps include the following:

  • IL-1 - Found regularly
  • IL-3 - Varies according to 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 regulated on activation, normal T cell expressed and secreted (RANTES); 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 include the following:

  • Tumor necrosis factor (TNF)-α and TNF-β - Varies, depending on the 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 (PDGF) - Present
  • Vascular permeable factors (VPFs) - Present
  • Vascular endothelial growth factors (VEGFs) - Present
  • Insulinlike growth factor (IGF)-1 - Present
  • Stem cell factor - Present

Adhesion molecules include the following:

  • Vascular adhesion molecule (VCAM)-1 - Present
  • E and P selectin - Present

Immunoglobulins include the following:

  • 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 with the middle and inferior turbinate mucosa; same level in patients without allergy as in those with allergy

Staging

In 1993, Lund and Mackay proposed the following three-point system for staging nasal polyps according to endoscopic appearance[12, 13] :

  • 0 - No polyposis
  • 1 - Polyps confined to the middle meatus
  • 2 - Polyps beyond the middle meatus

Subsequently, this system was modified as follows[14, 15] :

  • 0 - No polyps
  • 1 - Polyps restricted to the middle meatus
  • 2 - Polyps extending below the middle turbinate
  • 3 - Massive polyposis, occluding the entire nasal cavity
 

Treatment

Approach Considerations

Historically, children diagnosed with cystic fibrosis (CF) already had digestive and pulmonary disease and were the children with the more severe form of disease. These children were often treated with intravenous (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 endoscopic sinus surgery (ESS) for nasal polyposis without coexisting medical conditions.

Medical Care

Corticosteroids

Oral and topical nasal steroid administration is the primary medical therapy for nasal polyposis.[16, 17, 18] 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 US Food and Drug Administration (FDA) because of reports of unilateral vision loss in three patients after intranasal steroid injection with Kenalog. Safety may depend on specific drug particle size; high-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 dosage is usually 1 mg/kg/day for 5-7 days, which is then tapered over 1-3 weeks. Responsiveness to corticosteroids appears to depend on the presence or absence of eosinophilia; thus, patients with polyps and allergic rhinitis or asthma should respond to this treatment.

Patients with polyposis not dominated by eosinophilia (eg, patients with cystic fibrosis [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 after oral 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).[19]

A systematic review of 19 studies found similar results. The topical steroid preparations fluticasone, mometasone, and budesonide were shown to improve nasal symptoms in patients with nasal polyposis.[20] Some studies indicate fluticasone has a faster onset of action and possible mild superiority to beclomethasone.[21]

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 years) 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 later generation of intranasal steroids (eg, fluticasone and mometasone) appears to have less bioavailability than the earlier nasal steroids, such as beclomethasone.

Leukotriene receptor antagonists

Leukotrienes are cytokines made from arachidonic acid in the presence of 5-lipoxygenase. When released, leukotrienes bind to the cysteinyl-leukotriene receptors CysLT1 and CysLT2, located on the surface of target cells. These receptors are thought to mediate eosinophil recruitment, bronchospasm, vasoconstriction, mucus secretion, and plasma exudation. In addition, it is believed that leukotrienes may play a role in the inflammatory response in nasal polyposis.[22]

Montelukast competitively blocks leukotriene receptors. Klapan et al, in a study analyzing leukotriene levels in the nasal mucosa of patients with sinonasal polyps, found statistically higher levels of leukotriene C4 (LTC4) in patients with recurrent sinonasal polyps after surgery than in healthy control subjects.[23]  Higher LTC4 levels were associated with risk of recurrence of nasal polyps.

Given the role of leukotrienes in the recruitment of eosinophils and association with recurrence of nasal polyposis, it is reasonable to hypothesize a beneficial effect of the addition of montelukast in the management of nasal polyps.

Surgical Care

Surgical intervention is required for children with multiple benign nasal polyposis or chronic rhinosinusitis in whom maximal medical therapy fails. Simple polypectomy is effective initially to relieve nasal symptoms, especially for isolated polyps or small numbers of polyps. (See the image below.) In benign multiple nasal polyposis, polypectomy is fraught with a high recurrence rate.

Scale is in inches. The left side of the lesion wa 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.

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 as or superior to aeration of the sinuses; complication rates are low with experienced surgeons. The use of a surgical microdebrider has made the procedure safer and faster, providing precise tissue cutting and decreased hemostasis with better visualization. (See the images below.)

A surgical microdebrider entering the middle meatu 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. Some blood overlying the ethmoid cavity is noted where polyps were present in the center of the picture.
Endoscopic view of the left middle meatus, showing 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.

Surgery is directed at diseased tissue that is apparent on computed tomography (CT) 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.

Use of an image-guided system should be considered for defining 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, necessitating repeat surgery 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.

Complications

Massive polyposis or a single large polyp (eg, an antral-choanal polyp) that obstructs the nasal cavities and/or nasopharynx can cause obstructive sleep symptoms and chronic mouth breathing. Rarely, massive polyposis, observed in CF and in allergic fungal sinusitis (AFS) can alter the craniofacial structure. This can result in proptosis, hypertelorism, and diplopia.

In a retrospective study, McClay et al reported that 42% of children with AFS (compared with 10% of adults) presented with craniofacial abnormalities.[9] Massive polyposis rarely causes enough extrinsic compression on the optic nerve to decrease visual acuity. One study reported that three 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 neurologic symptoms, even when they extend into the intracranial cavity.

Activity

No activity restrictions are necessary for a child with nasal polyps. The child's activity level may decrease because of diminished ability to breathe 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.

Consultations

A pediatric otolaryngologist should be notified first, 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.

Long-Term Monitoring

Children with benign multiple nasal polyps, whatever the cause, should be monitored closely because recurrence is likely, regardless of whether the polyps were treated medically or surgically. Postoperative follow-up should occur three or four times in 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 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.

 

Medication

Corticosteroids

Class Summary

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

Prednisolone (Prelone, Orapred ODT, Pediapred)

Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reducing capillary permeability.

Prednisone (Prednisone Intensol)

May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

Dexamethasone (Decadron)

Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reducing capillary permeability.

Corticosteroids, Intranasal

Class Summary

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-term and long-term use.

Mometasone, intranasal (Nasonex)

Nasal spray; demonstrated no mineralocorticoid, androgenic, antiandrogenic, or estrogenic activity in preclinical trials. Studies concerning bioavailability are established; should be considered first-line therapy for pediatric patients. Not systemically absorbed like other nasal steroids (eg, beclomethasone). 

Fluticasone intranasal (Children's Flonase Allergy Relief, ClariSpray, Flonase Allergy Relief)

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).

Nasal drying, epistaxis, and, in long-term use, septal perforation has been reported. Advise patients to administer spray toward lateral nasal wall, avoiding irritation to septum or having drug run down back of pharynx.

Budesonide intranasal (Rhinocort Allergy)

Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability.

Triamcinolone, intranasal (Nasacort Allergy 24HR)

Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability.

Beclomethasone, intranasal (Beconase AQ, QNASL)

Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability. Delivers 42 mcg/actuation.

Leukotriene Receptor Antagonists

Class Summary

These agents prevent or reverse some of the pathologic features associated with the inflammatory process mediated by leukotrienes.

Montelukast (Singulair)

Potent and selective antagonist of leukotriene D4 (LTD4) at the cysteinyl leukotriene receptor, CysLT1. Prevents or reverses some of the pathologic features associated with the inflammatory process mediated by leukotrienes C4, D4, and E4. Used off-label for nasal polyps.