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NASAL POLYP Autor: John E McClay, MD Associate Professor of Pediatric Otolaryngology, Department of Otolaryngology-Head and Neck Surgery, Children's

Hospital of Dallas, University of Texas Southwestern Medical School http://emedicine.medscape.com/article/994274-overview Updated: Apr 18, 2012
McClay JE. Nasal polyps. eMed J 2001;2(12). www.emedecine.com/ped/topic1550.htm

BACKGROUND

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 (see the images below) 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.

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 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 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.

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, 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.[1, 2] 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.

Epidemiology
Frequency
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%. International Worldwide incidence is the same as the 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. [3] The 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 PRESENTATION

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 causeobstructive sleep symptoms and chronic mouth breathing.

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 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 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. 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 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 nasopharynx showing the sphenoid sinus superiorly and the antral-choanal polyp filling the nasopharynx in the center of the scan.

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. 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.

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.

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.

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 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 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.

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. 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 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. 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 CT scan showing typical unilateral appearance of allergic sinusitis with hyperintense areas and inhomogeneity of the sinus opacification; the

hyperintense areas appear whitish in the center of the allergic mucin. 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. 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 displaying telecanthus and asymmetrical positioning of her eyes and globes.

In an article submitted for publication, the author has reported 40% of children with AFS presented with craniofacial abnormalities, compared with 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

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 causeobstructive sleep symptoms and chronic mouth breathing.

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 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 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. 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 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 nasopharynx showing the sphenoid sinus superiorly and the antral-choanal polyp filling the nasopharynx in the center of the scan.

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. 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.

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.

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.

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 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 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.

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. 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 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. 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 CT scan showing typical unilateral appearance of allergic sinusitis with hyperintense areas and inhomogeneity of the sinus opacification; the

hyperintense areas appear whitish in the center of the allergic mucin. 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. 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 displaying telecanthus and asymmetrical positioning of her eyes and globes.

In an article submitted for publication, the author has reported 40% of children with AFS presented with craniofacial abnormalities, compared with 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
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, a benign massive polyp, a nasolacrimal duct cyst (as shown below), or any congenital lesion or benign or malignant tumor listed below.

Nasolacrimal duct cysts

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. 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. 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, 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. 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.

Encephaloceles (see the image below)


with hypertelorism and bulging of the nasal dorsum, secondary to encephalocele.

A 3-month-old infant

Gliomas (see the images below)

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 polyp #5 in a 5-monthold 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.

Dermoid tumors (see the images below)

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 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 hypertelorism

and protrusion in the glabellar region secondary to a small nasal dermoid. 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.

A coronal MRI scan through the nasal dermoid of a 5-month-old infant. The 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. interoperative view of dermoid removal from a 5-month-old infant.

An

Hemangiomas

Papillomas (see the image below)

Anterior nasal papilloma arising from the septum. The skin of the nasal vestibule is seen surrounding the papilloma in the center of the image.

Juvenile nasopharyngeal angiofibromas

Rhabdomyosarcoma (see the images below)

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 sinuses, showing the rhabdomyosarcoma in the same areas, including the posterior ethmoid complex, left middle fossa, and skull base of cavernous sinuses.

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.

Lymphomas Neuroblastomas Sarcomas Chordomas Nasopharyngeal carcinomas Inverting papillomas Evaluate all children with benign nasal polyposis for CF and asthma.

Differential Diagnoses

Asthma Cystic Fibrosis Neuroblastoma Neurofibromatosis

Rhabdomyosarcoma Sinusitis
WORKUP

Laboratory 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 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;[4] 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.
IMANGING 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 a compatible CT scan if an intraoperative image-guided system is used. Plain film radiography has no significant value after polyps are diagnosed. Also perform MRI in patients with possible intracranial involvement or extension of benign nasal polyps. CT scan findings 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 has a characteristic symmetrical bulging of the lateral nasal walls medially (see the

images below).

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 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 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 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 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 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 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 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 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, 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 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 the images below). This allergic fungal mucin appears black on MRI and can be confused

with the absence of disease.

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.

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 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.

Procedures

See rigid and flexible endoscopy procedures described in Physical.

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, 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 corticosteroidsensitive eosinophils. Degranulated mast cells are present. Degranulation presumably occurs in a nonimmunoglobulin Emediated 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[5] , 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.
o

Cytokines present in polyps IL-1 - Found regularly

IL-3 - Varies, based on study, from absent to intermittent at low levels to regularly present o IL-4 - Inconsistently detected o 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. o IL-6 - Same as in controls (no increase) o 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 o 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 o Tumor necrosis factor (TNF) alpha and beta - Varies, based on study, from same as controls to regularly detected; believed to be from eosinophils o GM-CSF - mRNA and protein amount varies, based on study, from never to intermittent to present o Platelet derived growth factor - Present o Vascular permeable factors (VPFs) - Present o Vascular endothelial growth factors (VEGFs) - Present o Insulinlike growth factor I - Present o Stem cell factor - Present Adhesion molecules o Vascular adhesion molecule 1 (VCAM-1) - Present o E and P selectin - Present Immunoglobulins (Ig) o IgG - No increase; same levels as in the middle and inferior turbinate mucosa o IgA - More in polyps than in the middle and inferior turbinate mucosa, especially IgA1 over IgA2 o IgM - No increase, same as in the middle and inferior turbinate mucosa o IgD - No increase, same as in the middle and inferior turbinate mucosa o IgE - Increased levels compared with the middle and inferior turbinate mucosa; same level in patients without allergy as in those with allergy

Staging
Polyposis has no uniform staging system

TREATMENT

Medical Care
Oral and topical nasal steroid administration is the primary medical therapy for nasal polyposis.[6] 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 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 is usually 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; 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 following 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).[7] 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.[8] Some studies indicate fluticasone has a faster onset of action and possible mild superiority to beclomethasone.[9] 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. The use of a surgical microdebrider (see the image below) has made the procedure safer and faster, providing precise tissue cutting and decreased hemostasis with better visualization.

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.

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

Medication Summary
See Medical Care.

Corticosteroids
Class Summary
Corticosteroids have potent anti-inflammatory action and relieve rhinorrhea, sneezing, itching, and congestion.
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Prednisolone (Prelone, Orapred, Pediapred)


Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reducing capillary permeability.
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Prednisone (Deltasone, Orasone, Meticorten, Sterapred)


May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.
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Dexamethasone (Decadron)
Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reducing capillary permeability.

Nasal corticosteroids
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.
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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).
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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.
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Budesonide inhaled (Rhinocort Aqua)


Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability.
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Triamcinolone inhaled (Nasacort AQ)


Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability.
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Beclomethasone (Beconase AQ)


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

FOLLOW UP

Further Inpatient Care

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 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 intravenous 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.

Further Outpatient Care

Closely monitor children with benign multiple nasal polyps, whatever the cause, because recurrence is likely, whether medically or surgically treated. 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 allergic fungal sinusitis (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.

Inpatient & Outpatient Medications


See Medical Care and Medication.

Deterrence/Prevention
See Medical Care.

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 AFS can alter the craniofacial structure. This can result in proptosis, hypertelorism, and diplopia. In an article submitted for publication, the author reported that 40% of children (compared with 10% of adults) with AFS presented with craniofacial abnormalities. Massive polyposis rarely causes enough extrinsic compression on the optic nerve to decrease visual acuity. One study 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, antralchoanal 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.

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