Ellen S. Deutsch and Cecille G. Sulman
ANATOMY AND EMBRYOLOGY
The nose is the major portal of air exchange between the internal and external environment. Nasal functions include warming, lubricating, humidifying, filtering, stimulating, and regulating airflow. The roof of the nose also contains olfactory epithelium. In humans, the sense of smell contributes to the perception of taste, warns of impending hazards, and affects social interactions.
The nose is the preferred primary route of breathing. Many infants are obligate nose breathers and cannot compensate by oral breathing if their nose is obstructed. Occlusion of the nose in such an infant may cause serious airway difficulties. This characteristic generally persists from 6 weeks to 6 months of age.
THE NOSE
The first sign of nasal development occurs at about 3 to 4 weeks of fetal life.1 It begins with nasal pits forming on the developing face that then invaginate to form the nasal sacs. The oronasal membrane separates the nasal sacs from the primitive oral cavity. The primitive nasal choanae communicate with the oral cavity when the membrane ruptures during the eighth week of gestation. As the membranous nasal cavities develop, neural crest cells migrate from the anterior skull base and proliferate in the facial processes to form the bony/cartilaginous skull base and nasal vaults, which are completely formed by the end of the 10th week.
The upper third of the nose is supported by paired nasal bones and the frontal process of the maxilla. The middle third of the nose is supported by upper lateral cartilages attached to the undersurface of the nasal bones. The lower third of the nose is supported by lower lateral cartilages. Within the nose, paired inferior, middle, and superior turbinates arise primarily from the lateral nasal walls. The area of drainage below each turbinate is the respective meatus (Fig. 370-1). The turbinates are highly vascular structures that play a primary role in humidifying, warming, and filtering airflow. The nasal valve is the narrowest portion of the nasal passage located inside the anterior aspect of the nose. The valve helps control nasal airflow and affects the subjective sensation of the adequacy of nasal airflow. In neonates, as much as half of the total airway resistance occurs in the nose, and small amounts of additional obstruction can substantially affect airway patency.
The rich and complex vascularity of the nose is supplied by both internal and external carotid systems. In anterior portion of the septum, a confluence of vessels forms Kiesselbach plexus, the most common source of epistaxis. The veins in this region are valveless, which may facilitate spread of infection from the nasal cavity to the orbits and intracranial space.
THE SINUSES
Development of the sinuses begins during fetal life but does not conclude until young adulthood (Fig. 370-1). Eventually, the sinuses are lined by respiratory mucosa composed of ciliated pseudostratified columnar epithelium interspersed by goblet cells. The maxillary sinus drains into the middle meatus under the middle turbinate in the region of the ostiomeatal unit. The ostiomeatal unit is not a discrete anatomic structure but refers collectively to several structures, including the draining routes and ostia of the maxillary, ethmoid, and frontal sinuses. Obstruction in this critical region of confluent drainage can lead to significant disease in the larger frontal and maxillary sinuses. The ethmoid sinuses start to develop during the third fetal month and have generally reached adult size by 12 years of age. The ethmoid bulla is one of the most constant and largest of the anterior ethmoid air cells. Large bullae may narrow sinus drainage outflow and impair mucociliary transport and ventilation. The sphenoid sinus can be identified during the fourth fetal month, and by the late teens, the sphenoid sinus development is nearly complete. There may be significant asymmetry between the two sphenoid sinuses because the intersinus septum tends to be bowed or twisted. The frontal sinus begins developing around 4 years of age and continues into the late teens. The size and symmetry of the adult frontal sinuses varies widely. Normally, the frontal sinus drains into the middle meatus.
The nasal cavity and paranasal sinuses are surrounded by many important structures, and disease in the paranasal sinuses may affect intraorbital and intracranial contents. The roof of the nasal cavity separates the nasal cavity from the brain. Housed within this region are the olfactory bulbs (Fig. 370-1B). Posterior to the sphenoid sinus is the sella turcica. Depending on the extent of aeration, the sphenoid sinus may be intimately related to the carotid arteries, optic nerves, pons, and cavernous sinus. The posterior wall of the frontal sinuses is composed of a thin layer of bone separating the frontal sinus from the anterior cranial fossa. The orbit is surrounded by the frontal, ethmoid, maxillary, and sphenoid sinuses. The posterior aspect of the nasal cavity communicates with the nasopharynx through large openings known as the choanae.
Figure 370-1. Internal nasal anatomy and sinus development. A: Coronal view representing the middle of the nose, demonstrating the developmental stages of the maxillary (lettered in red) and frontal (lettered in blue) sinuses. B: Sagittal view of the lateral nasal wall, demonstrating the ethmoid sinuses, the developmental stages of the sphenoid sinus (lettering in green), and the ostium of the maxillary sinus. Ethmoidal cells are a contiguous honeycomb, located lateral to the lateral wall of the nose with multiple ostium. The roof of the nasal cavity separates the nasal cavity from the brain. Housed within this region are the olfactory bulbs. (Source: Graney DO, Rice DH. Otolaryngology Head and Neck Surgery. 3rd ed. Philadelphia: Elsevier; 1998: 1060-1061.)
NASAL EXAMINATION
The infant and young child are best examined while sitting on the parent’s lap, with the parent securely holding the forehead and upper body. In the first portion of the exam, the overall external appearance is noted, including any craniofacial anomalies, masses, and resting mouth position. Incomplete closure of the mouth may be caused by nasal obstruction. Adenoid facies is a term used to describe the appearance of children with an open mouth posture; long, narrow face; high arched palate; dental malocclusion; short upper lip; and a “dull” affect. This appearance is nonspecific for chronic nasal obstruction, and adenoid hypertrophy may be only one of the causes. Nasal airflow can be assessed by holding a mirror under the nostril, which will fog from the humidity of warm airflow. Voice quality may also signal nasal obstruction. Nasal obstruction with too little air escaping through the nose results in hyponasal speech. This is in contrast to hypernasal speech, which results from air inappropriately escaping through the nose when vowels or consonants such as “s,” “b,” and “k” are spoken. Hypernasal speech, or velopharyngeal insufficiency, may occur in patients with cleft palate, velo-cardio-facial syndrome (22q deletion), or other causes of inadequate closure of the soft palate
A nasal speculum or a large ear speculum on an otoscope may be used to examine the inside of the nose. This is generally well tolerated by children and allows assessment of the anterior septum, floor of the nose, inferior turbinates, and mucosa. The septum is visualized for deviation or other abnormalities. The inferior turbinates appear as balls of tissue originating from the lateral wall near the floor of the nose. These may be mistaken for polyps if they are significantly edematous. Visualization more superiorly and posteriorly often provides a view of the middle turbinate and the middle meatus, the “tear-shaped” space (narrow at the superior end) between the middle and inferior turbinate. Hypertrophy of one inferior turbinate may be a result of the normal nasal cycle of alternating engorgement, but simultaneous bilateral hypertrophy may have other causes. Engorgement of the turbinates as a result of inflammation or other causes may result in nasal obstruction. “Cyanotic” or pale, bluish turbinates are classically attributed to allergies, but may occur in association with any cause of decreased nasal airflow. Mucosa quality as well as the presence of secretions may indicate an inflammatory process causing nasal obstruction. If a more thorough evaluation of the nasal cavity and nasopharynx is desired, nasal endoscopy with a flexible nasopharyngoscope can be performed by an otolaryngologist in children of all ages.
CONGENITAL NASAL ANOMALIES
Nasal obstruction in neonates not only causes difficulty breathing but also results in feeding difficulties, aspiration, and sleep problems. Complete nasal obstruction may be life threatening if not addressed in a timely fashion. A large number of congenital and/or genetic anomalies include nasal deformities or malformations, including arhinia, or complete absence of the nose. There may be an associated central nervous system abnormality, such as holoprosencephaly, or these patients may possess normal intelligence.2 Patients with polyrrhinia, or “double nose,” often have bilateral choanal atresia. It is hypothesized that the usual development of the frontonasal process is altered with duplication of the medial nasal processes and septal elements. Patients presenting with these anomalies require a comprehensive approach to both diagnosis and management as discussed in Chapter 177. The more commonly observed congenital nasal anomalies are discussed below.
CHOANAL ATRESIA
Choanal atresia is the most common congenital nasal abnormality, occurring in 1 of every 5000 to 8000 births.3 Choanal atresia may be unilateral or bilateral. Infants born with complete bilateral choanal atresia present with respiratory distress. Acute management includes opening the mouth using an oral airway. Gavage feedings may be necessary due to feeding difficulties since the infant is unable to coordinate breathing, sucking, and swallowing without an adequate nasal airway. Some infants will require intubation until the atresia is surgically repaired. Unilateral choanal atresia is twice as common as bilateral choanal atresia, but symptoms are less obvious; therefore, diagnosis may be delayed. Traditionally, an attempt is made to pass an 8 French catheter through both nasal cavities at birth; passage of the catheter rules out complete choanal atresia but does not guarantee adequate nasal airflow. Nasal obstruction warrants further evaluation, including endoscopy, and in cases of complete obstruction, a computed tomography (CT) scan. Patients with choanal atresia should also be evaluated for concomitant abnormalities. CHARGE association (coloboma, heart disease, atresia choanae, retarded development of the nervous system, genital hypoplasia, and ear anomalies or deafness) occurs in a significant percentage of infants with choanal atresia. Bilateral choanal atresia requires prompt repair, whereas unilateral atresia can generally be delayed until the child is 1 to 2 years of age.
OTHER CAUSES OF CONGENITAL NASAL OBSTRUCTION
Dacrocystoceles
Dacryocystoceles also cause nasal obstruction in the infant. A dacryocystocele occurs when there is occlusion of the lacrimal duct resulting in a triad of a cystic medial canthal mass, dilatation of the nasolacrimal duct, and a submucosal nasal cavity mass in the inferior meatus (Fig. 370-2). Approximately 30% of full-term infants are born with nasolacrimal duct obstruction; this resolves spontaneously in 85% by about 9 months of age.4 If obstruction impairs breathing or feeding, treatment options include endoscopic marsupialization, nasolacrimal duct probing, and stent placement.
Nasal Obstruction without Choanal Atresia (NOWCA)
This term provides a reminder to consider a variety of causes of nasal obstruction in infants in addition to choanal atresia. Symptoms include respiratory distress, rhinorrhea, feeding difficulties, and sleep disturbances. Structural causes such as nasopharyngeal masses, choanal stenosis, or pyriform aperture stenosis (narrowed anterior bony nasal opening), as well as inflammatory causes such as edema or infection, should be sought and treated as indicated. When NOWCA is managed expectantly, weight gain should be monitored because significant obstruction can interfere with growth.
CONGENITAL NASAL MASSES
Congenital midline nasal masses are rare and occur in about 1 in 30,000 live births in the United States,1,4 but are important because of their potential for intracranial connections. Congenital nasal masses can be divided into three major groups according to their tissues of origin. Neurogenic tumors are often mid-line, including gliomas, encephaloceles, and neurofibromas. Dermoid cysts are also often midline; they originate from ectodermal and mesodermal tissue. Hemangiomas, vascular lesions arising from mesoderm, are more variable in their location.
Encephaloceles and Gliomas
Encephaloceles and gliomas are thought to arise from the same embryonic defect: faulty closure of the foramen cecum at approximately the third week of fetal development. Herniation of cranial contents through a skull defect is known as an encephalocele. A meningocele includes meninges only; a meningoencephalocele includes both brain and meninges. Patients may have a widened nose, hypertelorism, or other midline central nervous system anomalies. Encephaloceles tend to enlarge with crying (Furstenberg test) and are bluish, soft, compressible masses that transilluminate. The incidence of encephaloceles is three times greater in males. They often occur as a component of syndromes such as Apert syndrome or in association with other anomalies such as ocular or frontonasal dysplasia.2 A glioma is an encephalocele that has lost the intracranial connection, although 15% remain attached to the central nervous system by a fibrous stalk. Gliomas do not transilluminate and are typically reddish, firm, and noncompressible. Diagnosis of encephaloceles and glioma is confirmed with nasal endoscopy and CT scan to delineate bony structures and MRI to identify any intracranial connections. If there is an intracranial connection associated with any of the defects, neurosurgical consultation should be obtained. Otherwise, treatment is by excision.
Figure 370-2. Dacryocystocele in left nares. D, dacryocystocele; MT, middle turbinate; NS, nasal septum.
Dermoids
Dermoids are the most common midline nasal mass.4 Dermoids may present externally as a firm, lobulated, and noncompressible mass with a negative Furstenberg test; they do not transilluminate. The most frequent presentation is a slow-growing cystic mass over the dorsum of the nose, located anywhere along the midline or near-midline from the nasal tip to the glabella. They often originate at a pit, which may have hair, cheesy material, or drainage protruding from the sinus opening. Dermoids may become locally infected and present as an abscess. Slow expansion can result in destruction of the nasal bones or widening of the nasal bridge. Dermoids are believed to be of similar embryologic origin to encephaloceles and gliomas. During development, a portion of the dura develops in close association with the skin of the nose. This normally separates and is retracted up through the foramen cecum, which then closes. Dura that remains in contact with skin as it retracts will result in a sinus or pit. Most dermoid cysts occur sporadically, although rare familial associations have been reported. Possible connection with the central nervous system via a cranial defect should be evaluated by MRI and/or CT scan. Complete excision must be performed to prevent recurrence.2
Teratomas
Teratomas generally originate in the nasopharynx rather than the nose or sinuses, but they can cause nasal obstruction. Teratomas range in complexity from dermoids to epignathi. “Hairy polyps” are a type of dermoid presenting as a fleshy mass comprising disorganized ectoderm and mesoderm. These pedunculated masses originate in the nasopharynx and may protrude beyond the margin of the soft palate; wide excision of the pedicle is usually curative. True epignathi, containing well-developed recognizable fetal parts, are rarely malignant. Depending on their size and location, nasopharyngeal teratomas may interfere with breathing and eating.
NASAL OBSTRUCTION, INFLAMMATION, AND RHINORRHEA
The differential diagnosis for nasal obstruction, inflammation, or rhinorrhea is diverse and challenging. The most common cause of nasal obstruction and rhinorrhea in young children is a viral upper respiratory tract infection. Young children experience an average of six to eight “colds” per year; many are viral and can be treated expectantly. Conservative measures include the use of hypertonic saline to rinse secretions. Various causes of allergic and nonallergic rhinitis, which may also contribute to nasal obstruction, are discussed in Chapter 192.
Infectious Causes
Infections of the nasal skin are uncommon and are often related to skin blemishes or to hair follicles at the anterior choanae. Digital trauma, either “nose picking” or attempts to express the contents of a pustule, may result in folliculitis, vestibulitis, or cellulitis and abscess. Staphylococcus aureus is the most common pathogen. Treatment consists of systemic antibiotics and topical application of antibiotic ointment. Development of increasing erythema, cellulitis, or an abscess of the nose or midface should be treated aggressively because of the potential for the infection to spread to the cavernous sinus via the valveless nasal veins.
Adenoid Hypertrophy and Adenoiditis
Adenoids are located just posterior to the back of the nose. Because of their location, they are difficult to examine. Nevertheless, adenoid hypertrophy or chronic adenoiditis may contribute to sinonasal disease. Adenoids consist of lymphoid tissue and are present early in life, with growth continuing until about 4 to 10 years of age. Adenoid hypertrophy can be evaluated with a lateral neck radiograph or by endoscopy, whereas chronic adenoiditis is diagnosed based on symptoms and may occur without adenoid enlargement. Nasal obstruction, mouth breathing, and persistent nasal congestion or discharge in the child who “always seems to have a cold” may indicate chronic adenoiditis. Adenoidectomy is indicated for chronic adenoiditis, adenoid hypertrophy with upper airway obstruction, and, selectively, for chronic middle ear disease and chronic sinusitis.5
Nasal Foreign Bodies
Children often place foreign bodies in their noses without parental knowledge, resulting in delayed diagnosis associated with characteristic foul odor and unilateral rhinorrhea. Foreign body lodgment should be considered if a child has persistent unilateral rhinorrhea unresponsive to routine management. With appropriate preparation and equipment, many intranasal foreign bodies can be removed in an office setting; occasionally, difficult cases may require removal under general anesthesia.
Button batteries are a special case; they are very hazardous and should be managed promptly.6 Local damage may occur within the first hour after placement into a body cavity, and nasal septal perforation has been reported within 7 hours of button battery lodgment.6 In the esophagus, burns have occurred as early as 4 hours and perforation as early as 6 hours after button battery ingestion.7 Three mechanisms contribute to extensive damage to surrounding mucosa: (1) moisture in the nasal cavity corrodes the battery casing, releasing alkaline contents; (2) the batteries, bathed in electrolyte-rich nasal secretions, can generate local currents; and (3) local pressure. Septal perforations may contribute to saddle-nose deformity as well as nasal crusting, foul smell, and epistaxis; surgical repair is technically challenging. Unfortunately, these injuries have become more common, as button batteries have become ubiquitous in toys, clothing, and greeting cards; many of which are intended for use by children and are not labeled as to their potential hazard.
NASAL MASSES
In addition to the congenital masses noted previously, benign and malignant neoplasms may present as nasal masses (see also Chapter 373).
Nasal Polyps
Nasal polyps are rare before adolescence, except for in children with cystic fibrosis (CF). Evaluation of a young child with nasal polyps should include consideration of CF or an encephalocele. The role of allergy in causing nasal polyps is controversial and probably limited. Polyps are most commonly visualized emanating from the middle meatus; they often have a gelatinous appearance (eFig. 370.1 ). Antrochoanal polyps are a distinct type of polyp arising from the maxillary sinus and entering the nose via the middle meatus. They expand posteriorly into the nasopharynx and can cause significant nasal airway obstruction.
Inverting papilloma and allergic fungal sinusitis are uncommon causes of nasal polyps but should be considered, especially if the polyps are unilateral and bone expansion or erosion is demonstrated on imaging studies. Inverting papillomas may have the appearance of polyps and similarly appear to arise from the region of the lateral nasal wall but are locally destructive. Surgical excision is the main therapeutic modality; there is a high rate of recurrence, and infrequently, they may become malignant.
Angiofibromas
Juvenile nasopharyngeal angiofibromas (JNA) are aggressive, poorly encapsulated, highly vascular but histologically benign tumors that originate in the nasopharynx in pubertal and prepubertal male adolescents. The classic symptoms include nasal obstruction and epistaxis, facial edema, proptosis, ipsilateral otitis media. Hearing loss and neurologic changes may also be present. Because of their vascularity, biopsy may be associated with significant hemorrhage, and radiologic studies combined with the clinical history are generally adequate to confirm the diagnosis (eFig. 370.2 ). Management options usually include radiotherapy or excision via open and/or endoscopic approaches8; embolization of major feeding vessels is accomplished just prior to resection.
Other Nasal Masses
Uncommon benign sinonasal masses include massive enlargement of a concha bullosa (an aerated cell within the middle turbinate), mucocele, peripheral-nerve-sheath tumors such as schwannomas, squamous cell papillomas, giant cell tumors, pygenic granulomas, and diseases of dental origin. Fibroosseous lesions may present with facial deformity, nasal obstruction, proptosis, epistaxis, and decreased vision. Fibrous dysplasia, ossifying fibroma, and juvenile (aggressive) ossifying fibroma replace normal bone with fibrous tissue.
Malignant sinonasal tumors are rare in children, but lymphomas and sarcomas can occur as early as infancy. Carcinomas and neural malignancies may also occur in children. Rhabdomyosarcomas and fibrosarcomas are the most common soft tissue sarcomas arising within the sinonasal region; osteosarcomas and chondrosarcomas may also occur (eFig. 370.3 ). Non-Hodgkin lymphoma may originate in the sinuses. Primitive neuroectodermal tumor (PNET) is a soft tissue neoplasm that can occur in newborns up to young adults. Many, but not all, sinonasal malignancies have a rapid course and may present with symptoms such as nasal obstruction, rhinorrhea, epistaxis, epiphora, proptosis, photophobia, visual loss, lymphadenopathy, facial paresthesia, or other cranial nerve involvement, as well as acute systemic symptoms such as fatigue, fever, weight loss, bone pain, or abdominal pain.
Diagnosis of olfactory neuroblastomas, also known as esthesioneuroblastomas or olfactory neuroepitheliomas, is often delayed because of the nonspecific symptoms. Olfactory neuroblastomas have a bimodal age distribution, with one peak occurring in the second decade (age 11-20 years) and another in the sixth decade.9
EPISTAXIS: ETIOLOGY AND MANAGEMENT
The most common cause of epistaxis is local trauma (“nose picking”), and several episodes of epistaxis often occur over the course of a few days. Most episodes of epistaxis in children originate in veins along the anterior nasal septum, which includes Kiesselbach plexus, and most acute episodes can be managed with local pressure.
There are many superstitions about how to control acute episodes of epistaxis, and families often need instruction in proper techniques. Because the most common site of bleeding in children is along the anterior septum, using the thumb and forefinger to apply gentle but consistent pressure between the ala and the tip of the nose below the nasal bones is often effective. Application of oxymetazoline, if possible, may be helpful. Afterward, antibiotic ointment can be gently applied inside the nasal vestibule nightly for a week to decrease itching and crusting and promote healing.
Persistent epistaxis may require application of topical hemostatic agents, dissolvable hemostatic products, nasal packing, or cautery. Control of epistaxis may require the use of general anesthesia. Less frequently, and rarely before adolescence, procedures such as arterial ligation or embolization, laser ablation, or dermal grafting may be considered.
If epistaxis is chronic or severe, contributory factors should be considered. If symptoms are persistent or there are other factors in the child’s or the family’s history that suggest an underlying coagulopathy, laboratory evaluation is indicated to identify bleeding disorders.
ACQUIRED NASAL ABNORMALITIES
Nasal Trauma
Nasal trauma is a frequent occurrence during an active childhood, especially among males, and fractures, septal hematomas, and septal abscess may occur as a consequence of minor trauma, sport injuries, and, particularly in young children, abuse. If a nasal fracture results in visible deformity or nasal obstruction, the fracture should be reduced, usually under general anesthesia and sometimes with the placement of an internal or external splint. Children with nasal trauma should also be evaluated for the possibility of a septal hematoma or septal abscess if they have persistent nasal obstruction, pain, rhinor-rhea, or fever.
Nasal Septum Deviation
Nasal septum deviation can result from trauma as noted above. It may also be “congenital” presumably being due to trauma sustained from intrauterine molding or during vaginal delivery. Deviated septum is present in about 10% of patients with Marfan syndrome. Nasal septal deviation, especially when located in the region of the nasal valve, can cause significant nasal airway obstruction; posterior deformities are generally less symptomatic. If symptomatic, in most cases a deviated septum can be corrected with a minor surgical procedure, septoplasty.
Other Acquired Disorders
Less commonly, acquired deformities may affect the nasal vestibule, columella, or nasopharynx. For premature infants with pulmonary disease, continuous positive airway pressure (CPAP) applied via nasal prongs is often used to avoid the need for intubation or tracheotomy. In a small percentage of these infants, the nasal CPAP system can result in alar deformity, erosion or transection of the nasal columella, or scarring and subsequent narrowing of the nose within the nostril. Likewise, in children of any age who require nasotracheal intubation or nasogastric tubes, careful attention must be paid to how tape is used to secure the tube to the nose and face to prevent damage to the nasal ala, which may be hidden beneath the tape.
Atrophic rhinitis with ozena, which is characterized by fetid rhinorrhea and nasal crusting, may begin in childhood and progress to atrophy of the nasal mucosa and underlying bone, interfering with nasal airflow. This appearance to the nasal cavity in adolescents may signal the illicit use of cocaine or “crack” glue sniffing, and other chemical inhalants should be considered.
Figure 370-3. Diagram illustrating cycle of factors contributing to chronic sinonasal disease. Items shown outside the cycle are potential interventions that may help alleviate the sinonasal disease. FESS, functional endoscopic sinus surgery; IVIG, intravenous immunoglobulin.
RHINOSINUSITIS
EPIDEMIOLOGY
Rhinosinusitis (RS) is a common disease with a significant adverse effect on health-related quality of life in children. RS is a frequent condition as children average six to eight upper respiratory tract infections (URIs) per year, or more if they attend daycare, and about 5% to 13% of URIs are complicated by RS.10 The prevalence of chronic sinusitis in children is 7.6%, and true sinusitis comprises 4.2% of all visits and 17.3% of visits with cold or cough symptoms in young children seen by primary pediatricians during the winter months.11 Other common conditions predisposed to RS include allergic rhinitis, chronic adenoiditis, adenoid hypertrophy, or anatomic obstructions. Less common predisposing factors include immunodeficiencies and ciliary dysmotility.
PATHOPHYSIOLOGY
Impaired ventilation and drainage contributes to progressive mucosal injury manifested by chronic inflammation, loss of cilia, and goblet cell hyperplasia. The subsequent decrease in pH and oxygen content within the sinus cavity promotes the growth of bacteria, resulting in continuing inflammation (Fig. 370-3). Another contributing factor likely are biofilms, which are collections of microorganisms adhered together on a surface by an extracellular matrix that have been associated with chronic infectious states, including chronic rhinosinusitis (CRS). Biofilms are found more frequently in sinus biopsies or adenoids from patients with CRS compared to controls.12 The exact role and management of biofilms in these chronic mucosal infections is still not completely understood. RS and asthma may be manifestations of an inflammatory process within a continuous airway. Allergic rhinitis has a prevalence of up to 30% in children with CRS, and 80% have a positive family history for atopy,13 compared with a frequency of 15% in the general population. With allergic rhinitis, mast cell degranulation incites mucosal edema and subsequent ostia obstruction impeding mucous clearance resulting in retained secretions. This provides opportunity for bacterial overgrowth (Fig. 370-3). Allergic rhinitis increases postnasal drip and is an irritant burden on the lower airways, which can exacerbate asthma symptoms. Treatment of CRS can aid in normalization of pulmonary function tests and ability to decrease long-term use of bronchodilators.14
Adenoid hypertrophy or nasal polyps may be sufficiently significant to cause stasis of secretions and impair nasal drainage. Septal deviation of a degree significant enough to cause nasal obstruction rarely occurs in children. The most common anatomic variant identified on CT is a concha bullosa, which displaces the uncinate process laterally, obstructing the infundibulum and drainage pathways; this may be found in up to 24% of children with CRS. Many craniofacial abnormalities with midface hypoplasia, such as Down syndrome or Crouzon syndrome, result in narrowing of the nasal airway and nasopharynx with retention of secretions.
Immunodeficiency can predispose an individual to rhinosinusitis. The most common immunodeficiency, isolated IgA deficiency, is not usually problematic; however, when additional immune deficiencies are present, the risk of CRS is increased. An IgG subclass deficiency (IgG3) is most often associated with refractory sinusitis. Recurrent ARS or CRS may be the only manifestation of a humoral immune deficiency. Patients with T-cell or neutrophil dysfunction will usually present with more severe infections, and sinusitis is usually a minor problem. Patients who are immunocompromised or iatrogenically immune suppressed are at risk for invasive fungal, as well as bacterial, sinusitis.
The most common pathogens identified with acute rhinosinusitis include Streptococcus pneumoniae, Haemophilus influenzae, Moraxella catarrhalis, and Streptococcus pyogenes. Anaerobic infection also occurs, and in up to 40% of cases there is a mixed infection of aerobic and anaerobic bacteria. In chronic rhinosinusitis, polymicrobial infection is common, with common pathogens including Streptococcus viridans, S pneumoniae, H influenzae, M catarrhalis, Staphylococcus aureus, Prevotella, and Fusobacterium. Anaerobic bacterial (Peptococcus, Peptostreptococcus, Bacteroides), fungal, and pseudomonal infections also occur and may be unresponsive to usual antibiotic therapies (eTable 370-1 ).
CLINICAL MANIFESTATIONS AND DIAGNOSIS
Sinusitis is usually diagnosed based on clinical presentation. Acute RS (ARS) most commonly presents as a URI that persists beyond 10 days or as a worsening of symptoms at 7 to 10 days. Primary symptoms include nasal discharge, cough, and halitosis. Older verbal patients may complain of facial pain or pressure; headache; fatigue; dental pain; cough; congestion; nasal obstruction; or purulent, discolored nasal discharge. Fever may be present, as well as otitis media, in up to 50% of children. Occasionally, the initial presentation of acute sinusitis is severe with high fevers and prostration, and orbital and intracranial complications may occur (see discussion of complications below).
Chronic RS (CRS) is a more indolent infection that lasts beyond 3 months, with symptoms including nasal congestion, cough, halitosis, behavioral problems, and nasal discharge. Hyposmia and anosmia may also occur. Recurrent ARS (RARS) implies recurrent infections of at least four per year lasting less than 30 days, with relatively asymptomatic periods in between lasting at least 10 days.
Radiographic imaging is generally not required for diagnosis, and should be reserved for children who are refractory to therapy or being considered for surgery, or if there are concerns of potential complications of RS. The American Academy of Pediatrics recommends that imaging studies are not necessary to confirm sinusitis in children younger than 6 years of age and should be used sparingly in children older than 6 years.15 Plain films are not reliable because they both overrepresent and underrepresent the amount of disease located in the ethmoid sinuses. The gold standard for imaging of the sinuses is CT with images in the coronal and axial plane (Fig. 370-4). Plain lateral neck films, however, are helpful in determining adenoid hypertrophy in a child with persistent rhinorrhea and nasal obstruction. Disadvantages of CT scans include cost, radiation exposure, and the potential need for sedation.
TREATMENT
Medical Therapy
Antibiotics continue to be the mainstay of therapy for sinusitis, but antimicrobial resistance is becoming continually more problematic (Table 370-1). Resistance to amoxicillin resulting from beta-lactamase production occurs in approximately 60% of H influenzae and 100% of M catarrhalis, and an alteration in penicillin-binding proteins occurs in about 50% of S pneumonia. Young children with mild to moderate ARS should be treated with high-dose amoxicillin. If a penicillin but not a cephalosporin allergy is present, cefdinir, cefuroxime, or cefpodoxime may be used. Severely penicillin- or cephalosporin-allergic patients should be treated with a macrolide such as clarithromycin or azithromycin. Children that do not respond to first-line therapy, children with more severe initial disease, and children who are considered high risk for resistant S pneumoniae, such as those with recent antibiotic exposure or day care attendance, should be treated with high-dose amoxicillin/clavulanate. The American Academy of Pediatrics recommends antibiotics should be continued for 7 days beyond symptom resolution; and a second-line agent should be used if there is no response in 72 hours.15 For nonallergic children with CRS, amoxicillin/clavulanate or second-generation cephalosporins are recommended. When RS is refractory to antibiotics, cultures are desirable and are best taken from the middle meatus, which can generally be obtained in a clinic setting.
Figure 370-4. Acute maxillary sinusitis. The left maxillary sinus is completely opacified and the ostium is occluded. (Source: Lalwani AK. Current Diagnosis & Treatment in Otolaryngology—Head & Neck Surgery. 2nd ed. New York: McGraw-Hill; 2008.)
Table 370-1. Therapy for Rhinosinusitis*
Many adjuvant medical therapies have been used, including topical nasal steroids, saline rinses, topical decongestants, and allergy medications. Nasal steroids are beneficial in reducing nasal edema by decreasing inflammation. Nasal steroids do not change baseline cortisol levels or suppress the hypothalamic-pituitary-adrenal axis, and there is little systemic absorption. Systemic steroids may be beneficial if used judiciously, and the risks are carefully discussed with the parents. Short bursts of systemic steroids can be helpful for patients with allergies. Nasal saline has been found to be beneficial, even as the sole modality of treatment in CRS; however, saline is not as effective as an intranasal steroid.16 Saline works by increasing ciliary beat frequency and mucociliary clearance.
Other medical therapies have not been proven to be effective by randomized controlled trials but may provide symptomatic relief. Decongestants cause vasoconstriction and allow less fluid to leave the blood vessels and enter the nose and sinus linings, which results in decreased inflammation of nasal membranes and decreased mucous production. Topical decongestants such as oxymetazoline are helpful but become less effective after a few doses. If overused, these can cause a “rebound congestion” (rhinitis medicamentosa), where the mucosal surface of the sinuses is damaged due to ischemia. Mucolytics are sometimes used to thin secretions, but they have variable efficacy.
The US Food and Drug Administration has determined that over-the-counter cold medicines, such as decongestants, expectorants, anti-histamines, and antitussives, are not effective in children younger than 6 years and should not be used for infants or children younger than 2 years because of reports of infant deaths.17
In patients for whom allergic rhinitis predisposes to RS, treatment reduces the incidence of infection. Nasal steroids help with the local inflammatory response. Antihistamines are useful in controlling allergy symptoms in children older than 2 years. However, the use of H1 antihista-mines in nonallergic children with acute bacterial sinusitis is not recommended. Leukotriene modifiers may be helpful in downregulating the eosinophilic response, thereby reducing mucosal edema and improving ciliary function. Immunotherapy has been demonstrated to decrease the incidence of RS and improve health-related quality of life in children with known allergy. Aggressive treatment of sinusitis can significantly improve asthma symptoms and quality of life in children.18 The evolving development of health-related quality of life survey instruments, such as the “SN(sino-nasal)-5 survey,” may be useful in both outcomes studies and individual patient care.11 Vaccination against S pneumococcus may help reduce repeated sinus infections and is indicated for those at high risk, including children with immunocompromised states.
Surgical Therapy
When medical therapy for sinusitis has failed, surgical interventions are entertained, but the initial approach to sinus disease may be directed at the adenoids. Adenoidal hypertrophy can potentially contribute to rhinosinusitis via mechanical obstruction, or, in patients with chronic adenoiditis, adenoid tissue may serve as a repository for bacteria that might subsequently involve the middle ear and sinuses. There is a significant correlation between quantitative bacteriology of adenoid core samples and sinonasal symptoms, which are independent of the absolute size of the adenoid pad. Adenoidectomy is generally considered the first surgical option for young children who experience CRS symptoms despite appropriate medical management.19 Maxillary sinus irrigation and the utilization of intravenous antibiotics have also shown efficacy.
Functional endoscopic sinus surgery (FESS) is considered when there are continued symptoms despite maximal medical therapy supported by physical findings and radiographic evidence of disease. FESS is performed under general anesthesia using endoscopes that both illuminate and magnify intranasal and intrasinus structures (Fig. 370-5 and eFig. 370.4 ). A limited, conservative approach is used, generally including uncinectomy, maxillary antrostomy, and anterior ethmoidectomy. Complications of FESS may be very serious but are uncommon and occur less than 1% of the time. Overall, FESS is considered to be a safe and effective treatment of refractory chronic sinusitis in children with a 75% to 90% positive outcome.20 Children with CF and immunodeficiencies for whom the underlying disease persists often require multiple surgical procedures.
Mucoceles occur when inspissated secretions accumulate because of ostial obstruction, expanding the bony walls of the sinus, and these generally require surgical removal. They may be caused by inflammatory scarring or cystic fibrosis; infrequently, they may be postsurgical. CT typically demonstrates a nonenhancing, low-attenuation mass in the sinus; expansion thins and erodes the bony margins of the sinus. Contrast-enhanced MRI may be useful in differentiating mucocele from sinonasal neoplasms. Mucoceles should not be confused with mucous retention cysts of the maxillary sinus. They have a smooth, dome-shaped appearance and often originate along the floor of the maxillary sinus. Unless the mucocele is obstructive of the outflow of mucous from the maxillary sinus, resulting in chronic infection, these may be managed expectantly.
Figure 370-5. Endoscopic surgical enlargement of the left maxillary sinus ostium and view of pus within the sinus lumen. (Source: Brunicardi FC, Andersen DK, Billiar TR, Dunn DL, et al (eds). Schwartz’s Principles of Surgery. 9th ed. New York: McGraw-Hill; 2010.)
ORBITAL AND INTRACRANIAL COMPLICATIONS OF ACUTE SINUSITIS
Most episodes of rhinosinusitis (RS), although unpleasant, resolve without long-term sequelae. Occasionally, however, fulminant acute bacterial sinusitis progresses to orbital and intracranial complications severe enough to cause permanent blindness and death. RS is the predominant cause of orbital infections in children, and orbital subperiosteal abscess is the most common complication of sinusitis. The classic conceptualization of progression from least to most severe orbital complications was articulated by Chandler21:
1. Inflammatory edema, affecting the eyelids with or without edema of the orbital contents.
2. Orbital [preseptal] cellulitis. Diffuse edema of the orbital contents without discrete abscess formation. Impairment of visual acuity may occur.
3. [Orbital] subperiosteal abscess comprises a purulent collection between the periorbita and the bony wall of the orbit.
4. Orbital abscess comprises a discrete abscess within the orbital tissues, with increased exopthalmos and chemosis, ophthalmoplegia, and severe visual impairment.
5. Cavernous sinus thrombosis may include progression of signs involving the contralateral eye, marked prostration, meningismus, and meningitis.
Disease limited to inflammatory edema may be treated solely with antibiotics, but patients with more advanced stages of disease require hospitalization, intravenous antibiotics, and possibly surgical intervention. Consultation by otolaryngology and ophthalmology, and frequent evaluation for the possibility of impaired extraocular mobility or deterioration of vision are mandatory. CT scans should be obtained with radiographic contrast.
Intracranial spread of sinusitis from the frontal, ethmoid, or sphenoid sinuses may cause meningeal irritation and infection, cerebritis, subdural or epidural abscess or brain abscess, bradycardia, hypotension, and death; recognition of sinus disease as the source of local findings, neurologic symptoms, and mental status changes is not always obvious to the clinician or the radiologist. The sinus most frequently responsible for intracranial spread of infection is the frontal sinus, primarily by retrograde thrombophlebitis via the valveless diploic veins in the posterior table of the frontal sinus.22 Management includes intravenous antibiotics and urgent consultation to otolaryngology and neurosurgery. Surgical drainage of the sinuses and extracranial abscesses may be external, endoscopic, or both, and craniotomy may also be indicated (eFig. 370.5 ).
FUNGAL SINUSITIS
Fungal sinusitis may be noninvasive or invasive. Children who are immune suppressed or immunocompromised are at risk of invasive fungal rhinosinusitis, which may be fulminant and fatal. The most common fungi causing infection of the nose and paranasal sinuses are species of Aspergillus because they are ubiquitous in soil, dust, and decaying matter. The most common presenting symptom is fever; other symptoms include facial pain, swelling, erythema, and tenderness over the involved sinuses. Necrosis of the mucosa manifesting as black crusts, or skin necrosis, are late findings and suggest rapidly advancing disease. Diagnosis is confirmed by histologic evidence of fungal invasion into sinus tissues. In contrast to bacterial sinusitis, for which drainage is therapeutic, treatment of invasive fungal sinusitis includes extensive surgical debridement of necrotic tissue in addition to systemic antifungal therapy.23
FIGURE 370-6. Sinus fungal ball. (Source: Brunicardi FC, Andersen DK, Billiar TR, Dunn DL, et al (eds). Schwartz’s Principles of Surgery. 9th ed. New York: McGraw-Hill; 2010.)
Noninvasive fungal sinusitis may include isolated intrasinus fungal balls (mycetoma) (Fig. 370-6) or allergic fungal sinusitis, which is typically more diffuse; either can occur in immune-competent patients. Treatment of sinus fungal balls includes removing the mass and establishing sinus ostium patency. The most commonly involved organisms are Alternaria, Bipolaris, and Curvularia.24 Computed tomography demonstrates marked soft tissue attenuation with heterogeneity of sinus contents and expansion, thinning, or remodeling of the sinus wall. Treatment includes surgical removal of the polyps and osteitic bone in the affected areas, marsupialization of sinus recesses, broad-spectrum antibiotics for intercurrent bacterial infections, and corticosteroids.
SINONASAL MANIFESTATIONS OF SYSTEMIC DISEASE
Cystic fibrosis (CF) occurs in 1/3200 newborns and predominantly in Caucasians (see Chapter 514). A family history of CF, pulmonary findings, nasal polyps, pancreatic insufficiency, and failure to thrive should raise suspicion for CF. Rhinosinusitis (RS) in the CF population is often severe and refractory to therapy. Patients with CF have a higher likelihood of requiring sinus surgery because the RS can worsen pulmonary status or cause complications such as nasal polyps, chronic nasal obstruction, headache, or mucoceles.
Primary ciliary dyskinesia (PCD) is a rare disorder of ciliary structure or function. The disorder can occur alone, but 50% of the time it is associated with Kartagener syndrome (KS), which manifests with a classic triad of chronic sinusitis, situs inversus, and bronchiectasis. Males are sterile because of immotile spermatozoa. The inability to clear nasal secretions results in thick obstructive mucous discharge, which leads to chronic RS. Approximately 30% of patients with KS have nasal polyps. Electron microscopy of mucosal biopsies from the nose or trachea shows abnormalities of the 9 + 2 microtubule structure or a decrease in dynein arm count.
Various granulomatous and vasculitic systemic diseases may affect the nose and sinuses, including Wegener granulomatosis, polymorphic reticulosis, midline lethal granuloma, and Churg-Strauss (allergic granulomatosis) syndrome; presentation may mimic fungal disease. Biopsy, culture, special stains, and overall organ system evaluation are required to differentiate these entities.
ACKNOWLEDGMENT
The authors thank Dr. Sally R. Shott for generously allowing the use of portions of her material from the previous edition of this text.