TITLE: Pediatric Rhinosinusitis
SOURCE: Grand Rounds Presentation, UTMB, Dept. of Otolaryngology
DATE: October 26, 2009
RESIDENT PHYSICIAN: Francisco G. Pernas, MD
FACULTY PHYSICIAN: Shraddha Mukerji, MD
SERIES EDITORS: Francis B. Quinn, Jr., MD
ARCHIVIST: Melinda Stoner Qui=
nn,
MS(ICS)
"This material was prepared by resident physicians in
partial fulfillment of educational requirements established for the
Postgraduate Training Program of the UTMB Department of Otolaryngology/Head=
and
Neck Surgery and was not intended for clinical use in its present form. It =
was
prepared for the purpose of stimulating group discussion in a conference
setting. No warranties, either express or implied, are made with respect to=
its
accuracy, completeness, or timeliness. The material does not necessarily
reflect the current or past opinions of members of the UTMB faculty and sho=
uld
not be used for purposes of diagnosis or treatment without consulting
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Introduction
Rhinosinusitis is manifested clinically by an inflammatory response involving the upper respiratory airway tract including the following: the mucous membranes (possibly including the neuroepithelium= ) of the nasal cavity and paranasal sinuses, fluids within these cavities, and/or underlying bone. Broadly speaking, rhinosinusitis is defined as = an inflammation and/or infection involving the nasal mucosa and at least one of the adjacent sinus cavities. Traditionally this condition was called sinusi= tis but the Task Force on Rhinosinusitis believes that for issues of clarity the entity should be referred to as rhinosinusitis to reflect that the condition affects the nasal passages and the sinus mucosa simulataneously. Rhinosinusitis syndromes are discussed in temporal terms and the disease state is categori= zed by how long symptoms have been present.
Acute rhinosinusitis (AS) is defined as the persistence and worsening of upper respiratory symptoms for greater than a 7-day course, which is the typical duration of a viral illness, but lasts l= ess than 4 weeks. Subacute rhinosinusitis (SAS) is defined as nasal symptoms lasting 4 weeks to 12 weeks. The infectious pathogens involved in SAS are similar to those found in AS. 11 Acute Bacterial Rhinosinusitis (ABS) is the fifth most common diagnosis, in the primary care setting, prompting antibio= tic administration and accounts for 0.4% of ambulatory diagnoses. The economic burden of this disease is greater than $1.77 billion per year. Acute rhinosinusitis may lead to chronic rhinosinusitis (CRS).
CRS diagnosis is symptom based and requires persistence of patient complaints of mucosal inflammation for more than 3 consecutive months despite optimal medical therapy or episodes have occurred more than four times a year with persistent radiographic changes. Chronic Recurrent Rhinosinusitis (CRRS) consists of multiple episodes of sudden worsening of CRS with return to baseline between episodes. Typically the ac= ute symptoms are alleviated but the chronic symptoms persist. Rhinosinusitis is rarely life threatening, but the close proximity of the paranasal sinuses to the central nervous system, the multiple fascial plains of the neck, and the associated venous and lymphatic channels can lead to serious complications.=
Background
The incidence of pediatric rhinosinusitis varies amongst various publications from 5-10%, most of these children progressed = from an upper respiratory tract infection. A smaller subset of those patients wi= ll go on to develop chronic rhinosinusitis. True incidence and prevalence is h= ard to determine because many children are treated empirically without necessar= ily obtaining radiographic evidence of sinus disease.
Signs and symptoms of rhinosinusitis include: Da= y and night cough, purulent nasal discharge, nasal airway obstruction, headache, irritability, or facial pain, fever, and postnasal drip. The single most co= mmon symptom in children is nasal discharge followed closely by cough.
Embryology
Classic anatomic treatises attribute initial par= anasal sinus development to lateral nasal wall ridges called ethmoturbinals. A ser= ies of five to six ridges first appear during the eighth week of development; through regression and fusion, however, three to four ridges ultimately per= sist the first ethmoturbinal regresses during development; its ascending portion forms the agger nasi, while its descending portion forms the uncinate proce= ss. The second ethmoturbinal ultimately forms the middle turbinate, the third ethmoturbinal forms the superior turbinate, and the fourth and fifth ethmoturbinals fuse to form the supreme turbinate. These structures are all considered to be ethmoid in their origin. An additional ridge, the maxilloturbinal, arises inferior to these structures. This ridge ultimately forms the inferior turbinate but is not considered ethmoid in its embryolog= ic origin.
In addition to the ridge and furrow development,= a cartilaginous capsule surrounds the developing nasal cavity and has an important role in sinonasal development. Bighman et al. highlighted the rol= e of the cartilage capsule through cross-sectional histologic analysis of fetal specimens. At 8 weeks, three soft-tissue elevations or preturbinates are se= en that correlate to the future inferior, middle, and superior turbinates. At = 9 to 10 weeks, two cartilaginous projections invade into the soft tissue preturbinates. An additional soft tissue elevation with an underlying cartilaginous bud emerges at this time, corresponding to the future uncinate process. This structure enlarges, and by 13 to 14 weeks, a space develops lateral to the structure that corresponds to the ethmoidal infundibulum. By= 16 weeks, the future maxillary sinus begins to develop from the inferior aspec= t of the infundibulum. The cartilaginous structures resorb or ossify as developm= ent progresses. The cartilaginous capsule, therefore, plays an important role in sinonasal development
The ethmoid sinus is commonly referred to as “the labyrinth” due to its complexity and intersubject variabil= ity. Fortunately, several rhinologists and surgeons have reduced the complex ethmoidal labyrinth of the adult into a series of lamellae on the basis of embryologic precursors. These lamellae are obliquely oriented and lie paral= lel. With experience, these structures are relatively easy to recognize during s= urgery and are invaluable in maintaining orientation in ethmoid procedures. The fi= rst lamella is the uncinate process; the second lamella corresponds to the ethmoidal bulla; the third is the basal or ground lamella of the middle turbinate; and the fourth is the lamella of the superior turbinate. The bas= al lamella of the middle turbinate is especially important, as it divides the anterior and posterior ethmoids. The frontal, maxillary, and anterior ethmo= ids arise from, and therefore drain into, the middle meatus. The posterior ethmoid cells arise = from, and therefore drain into, the superior and supreme meati, while the sphenoid sinus drains into the sphenoethmoid recess. The lamellae are relatively constant features between human subjects, making intraoperative recognition important.
Anatomy
There are four paired paranasal sinuses: the fro= ntal, sphenoid, ethmoid, and maxillary. The maxillary and ethmoid sinuses begin to develop during gestation; the maxillary sinuses grow rapidly until age 3 years, and then again from the ages of 7 to 18 years, while the ethmoid air cells grow from 3 or 4 cells to 10 to 15 cells per side by the age of 12. The sphenoid sinuses develop at approximately 6 years of age and the frontal sinuses dev= elop around 9 years of age. Up to 5% of adults may not fully develop one or both= of the frontal sinuses. Therefore, absence of well-aerated sinuses on radiolog= ical examination in the young child does not necessarily define a pathologic condition. While the anatomy of children’s sinuses is similar to that= of adults, children’s sinuses are significantly smaller in size, which o= ften makes clinical evaluation more difficult.
Examination of the nasal cavity of a child will = show three outgrowths from the lateral nasal wall called turbinates. The nasolacrimal = duct drains immediately underneath the inferior turbinate, and the nasolacrimal = sac is encased in thin bone immediately superior to the inferior turbinate. The maxillary sinus, frontal sinus and anterior ethmoid sinuses (the anterior g= roup of sinuses) drain in the region of the middle turbinate while the posterior= group of sinuses (posterior ethmoid and sphenoid sinuses) drain at the superior turbinate. The area named the osteomeatal complex is considered the primary site of obstruction leading to stasis of secretions and recurrent sinus disease. Anatomically this area is bounded by the anterior border of the mi= ddle turbinate medially and the lateral nasal wall laterally.
Mucociliary Clearance
Mucociliary clearance is very important for the = well being of the paranasal sinuses. Evidence of this is demonstrated when there= are aberrations of cilia which inevitable result in cilia dysfunction and paran= asal sinus disease. The natural ostia of the paranasal sinuses are not always located in areas that will spontaneously drain, therefore cilia play a big = role in allowing dependant areas to drain adequately. When discussing ciliary function it is important to consider the following factors: number of cilia, their structure a= nd their coordinated activity. If any one of those factors is altered it will = lead to decreased mucociliary clearance.
Cilia work optimally at a temperature of 37 degr= ees celcius and humidity near 100%. Cilia are located on the respiratory epithe= lium they are responsible for clearance of mucus which may contain bacteria and/= or other noxious micro foreign bodies. To aide in their function the respirato= ry epithelium contains globlet cells, they account for roughly 20% of the epithelium. Cilia account for the other 80%.
The normal structure of cilia is a highly conser= ved 9+2 structure seen even in the simplest unicellular organisms. The outer configuration of the cilia contain 9 doublet microtubules, each one of those microtubules contains an inner and outer dynein arm as well as a radial head and s= poke which interacts with the inner 2 single microtubules in the cilia. Knowledg= e of this is important because there are several diseases in which missing inner= or outer dynein arms lead to dysfunction of the entire ciliary structure. An example of such a disease is Inherited Primary Ciliary Dyskinesia. It is an autosomal recessive trait with extensive heterogeneity. It results in defec= ts of either the inner or outer dynein arms, total or partial defects. The deficiency of these structures leads to decreased beat frequency, outer arms are more detrimental to the beat frequency.
There are other diseases in which the cilia are functionally normal, yet they are overwhelmed by superimposed disease proce= ss. In cystic fibrosis, the cilia are functionally normal, however because of t= he thick mucous produced by the defective sodium channels the cilia are unable= to clear the mucus effectively. To some extent ciliary function is eventually affected in cystic fibrosis. Ciliary function is also affected in chronic r= hinosinusitis, further worsening the disease.
Pathophysiology
Nasal endoscopy has led to a better understandin= g of the etiology of sinus disease in children. The fundamental principle in development of sinus disease is that obstruction in the drainage pathways of the sinuses results in stasis of secretions, leading to sinus disease. The obstruction may be anatomic, physiologic, or a combination of the two. Other conditions such as allergy, gastroesophageal reflux, air pollution, first- = or second-hand smoke, and day care environments may increase the incidence of sinus disease.
Obstruction of the osteomeatal complex may be ca= used by bony anatomic variation, or mucosal inflammation from allergy, infection, irritation, and other intranasal pathology. This obstruction leads to cessa= tion of normal sinus drainage patterns and results in the stasis of secretions w= ith resultant sinusitis. Although the pediatric literature has focused predominantly on the maxillary sinus as the source of sinusitis, anterior ethmoid cells are involved with equal frequency. When the ostium of a sinus becomes obstructed, gas exchange becomes impaired within the normally aerat= ed sinus, favoring growth of anaerobic bacteria and promoting infection. Cilia= ry dysfunction within the sinus mucosal lining worsens, leading to further sta= sis of secretions and thickening of the mucous membranes in the sinuses. This l= eads to repeated or chronic sino-nasal infections.
In general, the pathophysiology of rhinosinusitis relates to impairment of mucociliary clearance, mucosal inflammation and any condition leading to decreased ventilation through a patent sinus ostium. Normal sinus drainage can be affected by GERD, Allergic rhinitis, viral URI= s, immune deficiency and asthma.
Gastroesophageal reflux disease: Recent studies suggest that patients with chronic rhinosinusitis have an increased prevale= nce of gastroesophageal reflux. Many patients, especially children, experience improvement in their chronic sinonasal symptoms after therapeutic trials of antireflux therapy. GER is theorized to have direct effects on nasal mucosa, initiating an inflammatory response associated with edema and impaired mucociliary clearance. Phipps in 2000 reported the results of a prospective trial in which pediatric patients referred for chronic rhinosinusitis were evaluated for gastroesophageal reflux. 19 of 30 patients (63%) were found to have esophageal reflux by pH probe. Six of the 19 patients (32%) demonstrat= ed nasopharyngeal reflux. Fifteen of the nineteen patients had improvement of their sinonasal symptoms after treatment of GERD. Bothwell in 1999 reported that 89% of pediatric candidates for functional endoscopic sinus surgery avoided surgery with treatment for GERD.
Allergy is a known contributing factor to both a= cute and chronic rhinosinusitis. Allergic rhinitis creates edema of the nasal passages, blocking proper drainage of the sinus cavities. This may lead to = an episode of acute sinusitis or contribute to the chronic inflammation of tho= se with chronic rhinosinusitis. Patients with refractory chronic sinusitis or history of atopy should be considered for allergy testing.
A history of frequent otitis media, pneumonia and sinusitis may suggest a primary or secondary immunodeficiency state. Serum = IgG, IgA, IgM and IgE should be evaluated as well as ability to respond to polysaccharide capsular antigens of S. pneumoniae and H. influenz= a. Patients identified with immune dysfunction may require IVIG therapy. Genet= ic counseling for the patient and family may be appropriate. Immunization agai= nst S. pneumoniae and H. influenza are suggested.
Sinusitis and asthma are frequently associated; controversy exists over whether they are manifestations in different parts = of the respiratory tract of the same underlying disease process or whether a causal relationship exists wherein sinusitis worsens bronchial asthma. Zimmerman f= ound a 31.2 % incidence of radiographic paranasal sinus abnormalities in asthmat= ic children compared to 0% in controls. Treatment of sinusitis, whether medica= l or surgical, has been shown in multiple studies to decrease use of broncodilat= ors, normalize pulmonary symptoms and improve subjective asthma symptoms.
Cystic fibrosis is inherited as an autosomal rec= essive trait; the mutations associated with CF affect the CFTR protein which is expressed mainly in the epithelial cells of airways and gastrointestinal tr= act. Multiple different CFTR mutations have been characterized in CF patients. Patients with cystic fibrosis develop chronic pulmonary disease in childhoo= d, sinusitis and nasal polyposis, pancreatic insufficiency and focal biliary cirrhosis. Cystic fibrosis patients presenting to the otolaryngologist usua= lly have already been diagnosed with CF; however, some patients may be undiagno= sed and present first to the otolaryngologist with sinonasal symptoms. Not all cystic fibrosis patients with chronic rhinosinusitis have nasal polyps but nasal polyps in the pediatric age group are rare. If cystic fibrosis is suspected, a sweat chloride test or referral for genetic evaluation should = be made. Nasal cultures positive for pseudomonas or S. aureus are suggestive of CF. Recent studies suggest that heterozygous mutations in the CFTR gene are associated with chronic rhinosinusitis as well as isolated chronic pancreatitis, allergic bronchopulmonary aspergillosis and congenital bilateral absence of the vas deferens. Raman found that seven of fifty-eight pediatric patients (12.1%) with chronic rhinosinusitis harbored CFTR mutati= ons; the expected rate is 3-4%. Wang found a 7% incidence of CFTR gene mutations= in 123 chronic rhinosinusitis patients compared to 2% in a control group.
Treatment
Treatment of pediatric rhinosinusitis is differe= nt from treating adults with chronic rhinosinusitis. Initial aims of treatment= are to target the cause of the rhinosinusitis. As discussed above the causes of rhinosinusitis are very diverse and therefore attention should be directed during the history and physical to attempt to elucidate which one of the factors is leading to CRS. Initial therapy is directed at treating the infection with appropriate antibiotics for susceptible microbes. Chronic Rhinosinusitis should be t= reated with a 4 to 6 week course of beta lactam stable antibiotic. Adjuvant therapy with nasal steroids should be employed as well as antihistamines especially= if underlying allergic condition suspected. Mucolytics may be used to help thin secretions and consider reflux treatment if suspicion of GERD is high.
Surgical therapy is a step-wise approach. If med= ical therapy fails first line of treatment is adenoidectomy, nasal endoscopy with/without antral lavage or directed opening of a sinus cavity which is s= een to be blocked on CT scan. Tonsillectomy may be performed also if patient ha= s OSA or recurrent strep throat. Finally a conservative FESS should be considered= if child is not improving after having instituted medical and initial surgical therapy.
Conclusion
Pediatric rhinosinusitis remains a diagnostic di= lemma in many children. Newer technology has helped us diagnosis and allowe= d us to treat patients that were previously inadequately or not treated at all. While the management of pediatric rhinosinusitis is primarily aggressive medical therapy, surgical management is appropriate for certain patients. Further inquiry is required to expand our understanding of = the etiology and natural history of sinus disease in children with additional insight into defining the indications for specific forms of medical and surgical therapy.
References
1.&n= bsp; Immunoglobulins and transcription f= actors in adenoids of children with otitis media with effusion and chronic rhinosinusitis; Young Gyu Eun a,1, Dong Choon Park b,1, Sun Gon Kim a, Myun= g Gu Kim a, Seung Geun Yeo c,*
2.&n= bsp; Functional endoscopic sinus surgery—A retrospective analysis of 115 children and adolescents with chronic rhinosinusitis; Vanessa Siedek *, Klaus Stelter, Christian S. Betz, Alexander Berghaus, Andreas Leunig; International Journal of Pediatric Otorhinolaryngology 73 (2009) 741–745
3.&n= bsp; Failures of Adenoidectomy for Chron= ic Rhinosinusitis in Children: For Whom and When Do They Fail?; Hassan H. Rama= dan, MD, MSc; Jeremy Tiu, MD
4.&n= bsp; Adenoidectomy outcomes in pediatric rhinosinusitis: A meta-analysis; Scott E. Brietzke a,*, Matthew T. Brigger = b
5.&n= bsp; Management of refractory chronic rhinosinusitis in children; Nithin D. Adappa, MDa,4, James M. Coticchia, MD; American Journal of Otolaryngology–Head and Neck Medicine and Surgery= 27 (2006) 384– 389
6.&n= bsp; Immunological investigation in the adenoid tissues from children with chronic rhinosinusitis Seung-Youp Shin, = MD, Gil-Soon Choi, MD, Hae-Sim Park, MD, PhD, Kun-Hee Lee, MD, PhD, Sung-Wan Ki= m, MD, PhD, and Joong-Saeng Cho, MD, PhD, Seoul and Suwon, Korea; Otolaryngology–Head and Neck Surgery (2009) 141, 91-96
7.&n= bsp; Indications for image-guidance in pediatric sinonasal surgery Sanjay R. Parikh *, Hernando Cuellar, Babak Sadoughi, Olga Aroniadis, Marvin P. Fried
8.&n= bsp; The role of adenoids in pediatric rhinosinusitis Kwang Soo Shin, Seok Hyun Cho, Kyung Rae Kim, Kyung Tae, Seu= ng Hwan Lee, Chul Won Park, Jin Hyeok Jeong *
9.&n= bsp; Pediatric chronic rhinosinusitis: a restropective review Michael W. Criddle, MDa,⁎, Amy Stinson, DOb, Mohammedi Savliwala, MDc, James Coticchia, MD, FACS
10.&= nbsp; Sinonasal Mucociliary Clearance in Health and Disease Noam A. Coh= en, MD. PhD; Aimah af Otology, Rhinohsy & Larynnology ll5l9lSuppl l%;2(l= -26
11.&= nbsp; Long-term outcome of facial growth after functionalendoscopic sin= us surgery; MARCELLA R. BOTHWELL, MD, JAY F. PICCIRILLO, MD, RODNEY P. LUSK, M= D, and BROCK D. RIDENOUR, MD
12.&= nbsp; Influence of extensive functional endoscopic sinus surgery (FESS)= on facial growth in children with cystic fibrosis Comparison of 10 cephalometr= ic parameters of the midface for three study groups; A. Van Peteghem *, P.A.R. Clement; In= ternational Journal of Pediatric Otorhinolaryngology (2006) 70, 1407—1413
13.&= nbsp; Twenty-four-hour esophageal pH monitoring in children and adolesc= ents with chronic and/or recurrent rhinosinusitis Disciplinas de 1Otorrinolaringologia Pediátrica, and 2Gastroenterologia Pediátrica, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brasil V.R.S.G. Monteiro1, V.L. Sdepanian2, L. Weckx1, U. Fagundes-Neto2 and M.B. Morais2; BpHra zmilioanni tJooruinrnga iln o cf hMilderdeinca al nadn da dBoioleloscgeicnatls Rweisthe arrhcihn o(2si0n0u5si)t i3s8: 215-220
14.&= nbsp; Gastroesophageal Reflux Contributing to Chronic Sinus Disease in Children A Prospective Analysis C. David Phipps, MD; W. Edward Wood, MD; William S. Gibson, MD; William J. Cochran, MD; Arch Otolaryngol Head Neck Surg. 2000;126:831-836
15.&= nbsp; Pediatric Rhinosinusitis: Diagnosis and Management Gary Josephson, MD; Soham Roy, MD
16.&= nbsp; Pediatric Otolaryngology, select chapters on chronic rhinosinusit= is
17.&= nbsp; Essential otolaryngology, K.J. Lee, Select chapters of Chronic Rhinosinusitis