REVIEW URRENT C OPINION
Pediatric chronic rhinosinusitis: when should we operate? Mark D. Rizzi a,b and Ken Kazahaya a,b
Purpose of review The timing and role of surgery in the management of pediatric chronic rhinosinusitis (pCRS) remains unclear. This review attempts to summarize the existing literature regarding this topic. Recent findings pCRS is a complex clinical syndrome that results from multiple potential causes. Multimodal medical therapy is the accepted primary treatment. Adenoidectomy continues to be the appropriate first-line surgical therapy, and the addition of antral lavage or balloon dilation to this procedure may improve outcomes. Functional endoscopic sinus surgery (FESS) in children is safe and effective in relieving symptoms in the majority of patients. Failure to respond to maximal medical therapy continues to be a commonly cited, although poorly defined, requisite for proceeding to surgery. Recent literature has been focused on the outcomes after FESS rather than specifically defining when this intervention should be considered. Nevertheless, the literature seems to continue to support FESS in children with persistent symptoms despite adenoidectomy and appropriate medical treatment for pCRS. As the symptoms of chronic rhinosinusitis (CRS) may result from multiple underlying causes, clinicians must understand that the role and timing of surgery may vary with particular patients’ disease. Summary Surgical management of CRS in children continues to be a frequent topic of study in the otolaryngology literature. As recent research tends to be focused on outcomes after surgery, further prospective studies comparing surgical versus nonsurgical treatment of CRS will likely be required to better define indications for proceeding to surgery at all. Keywords chronic rhinosinusitis, functional endoscopic sinus surgery, pediatric
INTRODUCTION Pediatric chronic rhinosinusitis (pCRS) continues to represent a significant burden for patients, their caregivers and the healthcare system at large. Although its true cost is unknown, billions of dollars are spent on its management annually [1]. Chronic rhinosinusitis (CRS) is specifically defined by the presence of persistent symptoms and signs of sinonasal inflammatory disease for a continuous period of 90 days or more [2]. Recently, the European Position Paper on Sinusitis and Polyposis 2012 defined pCRS as the presence of two or more symptoms, one being either nasal obstruction or nasal discharge coupled with either facial pain or pressure, cough and either endoscopic evidence of disease or changes of rhinosinusitis on computed tomography (CT) [3 ]. The cornerstone of treatment for CRS has been antibiotic therapy coupled with topical or systemic corticosteroid therapy and isotonic saline &&
rinses [4]. In some cases, medical therapy is unable to adequately ameliorate patients’ symptoms. In these patients, surgery may play a viable role in their care. Additionally, there are some patients in whom surgery may play a more primary role, although always as an adjunct to medical therapy. The goal of this review is to summarize recent relevant findings that have addressed the timing and role of surgery in pCRS. a Division of Pediatric Otolaryngology, Children’s Hospital of Philadelphia and bDepartment of Otolaryngology - Head and Neck Surgery, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA
Correspondence to Mark D. Rizzi, MD, Children’s Hospital of Philadelphia, Division of Otolaryngology, 1st Floor, Wood Building, Philadelphia, PA 19104, USA. Tel: +1 215 590 3440; e-mail: [email protected] Curr Opin Otolaryngol Head Neck Surg 2014, 22:27–33 DOI:10.1097/MOO.0000000000000018
1068-9508 ß 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins
www.co-otolaryngology.com
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Nose and paranasal sinuses
KEY POINTS Management of CRS in the pediatric population requires identification and treatment of the multiple potential causes that contribute to this syndrome. The question of when to proceed with surgery in the setting of medical failure has not been addressed by the current literature in an evidence-based manner, and therefore must remain individualized. Adenoidectomy with or without balloon dilation or antral lavage is appropriate first-line surgical therapy for children deemed to have failed medical treatment. Endoscopic sinus surgery is safe and effective in the majority of children who have failed medical therapy and adenoid removal or who meet the criteria listed in the conclusion section.
PATHOGENESIS OF CHRONIC RHINOSINUSITIS The cause of CRS in children has not been fully elucidated. Unlike acute rhinosinusitis, which is primarily an infectious entity, CRS represents a spectrum of pathologic states with varying degrees of contribution from both infection and underlying inflammation. The pathogenesis involves obstruction of sinus ostia with consequent stasis of secretions, reduced oxygen tension, inhibition of ciliary function and bacterial infection. At the local tissue level, changes in both the mucosa and underlying bone have been described [5–7]. Patients with CRS are often divided into those that have associated nasal polyposis (CRSwNP) and those that have CRS without polyps (CRSsNP). The cause of nasal polyposis is unknown despite extensive investigation into their pathogenesis and to the immunologic attributes of patients who suffer from them [3 ]. Although exploration of this topic is beyond the scope of this review, the presence of polyps may portend more recidivistic disease, especially when they are associated with asthma [8]. Staphylococcus aureus and epidermidis, Streptococcus pneumoniae and Hemophilus influenzae have been implicated in CRS [9–13]. Anaerobic bacteria are often present in these infections [9]. In a study by Erkan et al. [14], anaerobes were isolated from over 90% of chronically infected maxillary sinuses. In children, host factors, such as immunodeficiency, comorbid medical conditions and environmental factors all contribute to the development of CRS. Smoking, both active and passive [15–17], industrial pollution [18] and participation in day care [19] are associated with sinonasal disease. Further, local anatomic factors, such as the presence of polyps and chronically colonized or infected &&
28
www.co-otolaryngology.com
adenoids also play a role in the pathogenesis of CRS. Finally, comorbid conditions, such as the presence of reflux [20] or the coexistence of allergic rhinitis [21], cystic fibrosis (CF) [22] and primary ciliary dyskinesia (PCD) [23] are associated with an increased risk of CRS.
Biofilms Biofilms are bacterial aggregates that adhere to an underlying surface while being harbored within a hydrated exopolysaccharide matrix. This matrix is protective against host defenses and antibiotics but allows emission of individual cells, which may repeatedly infect the host [24]. The biofilm concept may serve as an explanation for the unreliable efficacy of antibiotics in some children with CRS. It also provides the rationale for adenoid removal in some patients, as discussed below. Although the presence of biofilms in adults with CRS is well characterized, the degree to which they play a role in children requires further research [3 ]. &&
Diagnosing chronic rhinosinusitis The diagnosis of pCRS can be difficult to make as children suffer from six to eight viral upper respiratory infections annually. The symptoms of these infections overlap considerably with those of bacterial rhinosinusitis. Distinguishing one from the other is challenging. The signs and symptoms and their duration required for the diagnosis of pCRS are outlined in the introduction section. As the diagnosis is primarily clinically made, the acquisition of a proper history is critical. Nasal obstruction, discolored drainage, hyposmia and facial pressure or pain are common complaints [25]; however, many patients do not present with nasal discharge [26]. Headache and facial pain alone rarely represent sinonasal disease but more commonly have a neurological cause [3 ]. Persistent cough is a frequent complaint. Although rhinosinusitis is not often the direct cause of the cough, cough is present because the same inflammatory process is affecting both the upper and lower airways [27]. Numerous studies have supported the idea that the upper and lower airways exist as an interconnected immunologic and reactive network [28]. To appreciate this ‘unified airway’ concept is to be aware that complete treatment of CRS requires recognition and treatment of both upper and lower airways inflammation. Finally, history should also assess for allergic rhinitis, asthma and reflux, all of which have been associated with pediatric CRS. Physical examination and nasal endoscopy are also important. Nasal endoscopy can be considered &&
Volume 22 Number 1 February 2014
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Pediatric chronic rhinosinusitis Rizzi and Kazahaya
to diagnose the specific site of nasal airway obstruction, such as enlarged adenoids or turbinates, nasal polyps, unsuspected masses or foreign bodies [29]. Endoscopy is also superior to lateral neck radiograph in evaluating adenoid size and degree of occlusion of the nasopharynx [30]. Although flexible nasal endoscopy is usually possible in young children, it is often difficult because of fear and intolerance of the procedure. Finally, imaging is of value in selected patients. CT scanning is the gold standard modality because of its clear resolution of bone, soft tissue and air. In interpreting pediatric sinonasal CT scans, it is important to recognize that abnormalities are often present in the absence of disease. Children without rhinosinusitis have an average Lund McKay score of 2.8 [31], and a score of 5 has been proposed to be required for a CT to be considered abnormal [31,32]. It is now clear that CT scans in children increase the risk of leukemia and brain cancer, and efforts should be made to minimize the number of studies performed and the dose of radiation delivered [33 ]. CT imaging should be reserved for patients in whom endoscopic sinus surgery is imminent, when a complication of sinusitis is suspected, or for patients with atypical presentations, such as unilateral obstruction [34]. It is not appropriate for screening or disease surveillance, even in patients known to be at risk for CRS [35 ]. &&
&
Medical treatment
maxillary sinuses may be an effective treatment strategy for patients who fail oral medical therapy [1]. Topical nasal corticosteroids are also considered first-line therapy for pCRS [3 ]. Nasal corticosteroids may also relieve nasal obstruction in patients with moderate-to-severe adenoid hypertrophy [41]. Nasal saline irrigations appear to be effective and well tolerated in the pediatric population [42], although it is likely an underutilized modality [26]. Antihistamines may be used for relief of symptoms of allergic rhinitis in applicable patients, but their efficacy in the treatment of CRS is not proven. &&
Surgical treatment The European Position Paper on Rhinosinusitis and Nasal Polyps 2012 summarized that a logical surgical algorithm for pCRS begins with adenoidectomy with possible antral irrigation or balloon dilation of the maxillary sinuses, with FESS reserved for treatment failures [3 ]. Symptomatic patients with sinonasal polyposis, CF, allergic fungal rhinosinusitis (AFRS), PCD or antrochoanal polyps are more likely to require FESS for disease control. &&
Adenoidectomy First-line surgical therapy for pCRS is adenoid removal. Multiple studies have shown that this treatment modality is effective in the majority of patients [3 ,43,44]. In a meta-analysis that included nine studies, Brietzke and Brigger [45] concluded that 69.3% of patients experienced significant improvement following adenoidectomy. Size of the adenoid does not influence success of adenoidectomy [46,47] perhaps explained by the biofilm concept [24]. In a study examining adenoid tissue, biofilms were identified by scanning electron microscopy in all seven individuals who underwent adenoidectomy for CRS but in zero of nine patients who underwent adenoidectomy for the treatment of obstructive sleep apnea [24]. There is a high correlation between the bacteria present within the adenoids and within the middle meatus in children with CRS [48]. The addition of middle meatal irrigation or balloon dilation may increase the efficacy of adenoid removal in the treatment of pCRS. Balloon catheter dilation of the maxillary ostium has been shown to be safe in children [49]. Ramadan and Terrell [50] reported that balloon therapy when combined with adenoidectomy was more effective than adenoidectomy alone. In this study, some patients also underwent sinus irrigation making the contribution of the balloon less clear. In a separate study, these authors demonstrated successful &&
The mainstay of treatment for pCRS is medically derived. Surgery is often described as being reserved for individuals who fail ‘maximal medical treatment’ although there is no consensus to define the extent of such therapy. Antibiotics are the mainstay of first-line therapy despite there being only weak evidence supporting their efficacy [36]. Penicillin resistance is common [37 ] and is more likely in patients attending daycare, in patients less than 2 years old and in those who underwent recent treatment with antibiotics [38]. Amoxicillin-clavulanate is a commonly cited first-line agent [39,40]. In penicillin allergic patients, a cephalosporin or macrolide can be considered. If methicillin-resistant Staphylococcus aureus is suspected, clindamycin and Bactrim may be effective. Recommended antibiotic duration is commonly described as being between 3 and 6 weeks. In a 2006 review, Wu et al. [32] suggested that such therapy should include extended use of a broad-spectrum antibiotic and nasal steroid spray with or without adjunctive use of an oral antihistamine, an oral mucolytic agent, oral steroids or nasal saline irrigation. Intravenous antibiotics, when combined with irrigation of the &&
1068-9508 ß 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins
www.co-otolaryngology.com
29
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Nose and paranasal sinuses
use of balloon sinuplasty in 81% of patients who previously failed adenoidectomy as measured by change in Sn5 scores [51]. Patients with persistent symptoms despite medical management and adenoid removal as above are candidates for CT scan and potentially FESS. Consideration for work-up of immunodeficiency and other comorbidities should be undertaken in these patients if this has not yet been done.
FUNCTIONAL ENDOSCOPIC SINUS SURGERY The efficacy of FESS in children has been previously reported. In 1998, a meta-analysis by Hebert and Bent [52] revealed favorable outcomes in 88–92% of patients with a mean follow-up of 3.7 years. These results have been replicated consistently since that time. Siedek et al. [53] reported a 77% improvement in symptoms and a 74% improvement in quality of life in children after FESS as measured by patient self-assessment. In a 2007 review, Rudnick and Mitchell [54] showed that the benefits of FESS may extend further than 6 months postsurgery. In 2012, El Sharkawy et al. [55] published a retrospective review of 87 patients who underwent FESS for CRS after failing medical treatment. A detailed description defining medical failure in their individuals was not provided. In this study, an overall success rate, defined as improved endoscopic or CT appearance as well as improved symptoms, was 87.69%. Concerns about impedance of midfacial growth have been allayed by studies of long-term follow-up of patients who underwent FESS at a young age [56]. In 2013, Makary and Ramadan [57 ] performed a review of the literature with the aim of analyzing outcomes from FESS in children with CRS. Their review excluded studies that included patients with CF, polyposis, immunodeficiency and PCD if the original reports did not analyze these patients separately. Eleven studies published between 1993 and 2012 were included. The authors report a success rate from surgery ranging from 82 to 100% . However, the authors noted in their conclusion that the question of when sinus surgery is indicated in children remains unanswered because of lack of adequately powered studies that compare FESS to other surgical options or to medical therapy alone [57 ]. &
&
SURGICAL MANAGEMENT IN SPECIFIC POPULATIONS CF, AFRS, aspirin exacerbated respiratory disease (AERD) and PCD are disease processes which are 30
www.co-otolaryngology.com
associated with refractory CRS and in which surgery is likely to play a more primary adjunctive role to medical therapy than it does in patients with isolated CRS. Polyps are often present in these patients. Even in the absence of these systemic illnesses, polyps are associated with more persistent disease. Lee et al. [58] showed that sinonasal polyps were an independent risk factor for persistent symptoms in patients who underwent FESS. Tsukidate et al. [59] report that 50% of children who underwent FESS for bilateral nasal polyposis were either unchanged or worse at 1-year follow-up but that the proportion of patients improved increases with time beyond that point. In children, sinonasal polyps should be thought to represent underlying CF until proven otherwise. CF is an autosomal recessive disorder that involves mutation of the CFTR gene, which codes for a chloride channel that indirectly affects water transport across cell membranes. The thick, inspissated secretions generated within the airways of these patients leads to a chronic colonized state [60]. Initial colonization and evolution of antibiotic resistance may occur within the paranasal sinuses and transition to the lower airways from there [61]. CF involves the sinuses in 90% of afflicted patients [62] although many do not complain of symptoms [24]. Pseudomonas aeruginosa is a common pathogen of the upper and lower airways in older children, whereas staphylococcal species are often seen in younger patients [63]. There are no established indications for proceeding to surgery in patients with CF. FESS in CF patients significantly relieves symptoms of nasal obstruction and discharge and may improve overall quality of life [64]. A further benefit is improved access for application of antibiotic irrigation [3 ]. Controversy exists as to whether FESS may be beneficial with regard to the pulmonary status of patients with CF although most studies agree that FESS has no effect on pulmonary function tests [65–69]. Nasally inhaled aerosolized DNase postoperatively may improve symptoms, mucociliary clearance and the endoscopic and radiologic appearance of the sinuses in patients with CF [70,71]. Additionally, surgery is beneficial in patients with medically refractory CRS postlung transplantation [66,72 ]. AFRS is a syndrome characterized by type 1 hypersensitivity, nasal polyposis, eosinophilia, characteristic CT findings of unilateral or bilateral soft tissue density with expansile and erosive features and often marked by scattered hyperdensity within the sinuses and the presence of noninvasive fungal elements in pathologic tissue specimens [73]. The underlying cause is unknown although it has &&
&
Volume 22 Number 1 February 2014
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Pediatric chronic rhinosinusitis Rizzi and Kazahaya
been described as analogous to allergic bronchopulmonary aspergillosis of the lungs [74]. AFRS is commonly unilateral. Medical treatment involves reducing inflammation within the nose and sinuses with corticosteroids. Allergen immunotherapy aimed at typically implicated fungi may also be beneficial [75]. The use of antifungal drugs is not widely accepted. Surgery is often necessary for complete relief of symptoms. The goals of surgery are to decrease the burden of fungal antigen by thorough removal of polyps and eosinophilic mucin and to widely open the sinuses to allow for both ventilation and access for diagnostic endoscopy and topical therapy in the future. PCD is an autosomal recessive disorder that affects approximately 1 : 15 000 patients. Diagnosis is often delayed, and many patients receive suboptimal management. Approximately, 30% of patients have nasal polyps [76 ]. Diagnosis can be difficult because of the heterogeneity of these patients. Many have normal ciliary morphology on electron microscopy. Identification of known genetic abnormalities can be helpful, but the entire genetic spectrum of the disease is not known. Identification of decreased nasal nitric oxide is supportive of the diagnosis but not specific as low nitric oxide levels are seen is some patients with CF or sinusitis [77]. Ultimately, diagnosis must come from astute clinical vigilance. Eighty percent of patients with PCD have a history of neonatal respiratory distress. Daily nasal congestion, year-round wet cough and the presence of situs inversus or situs ambiguous strongly suggest PCD. Patients commonly have chronic ear disease. Evidence-based guidelines for proceeding to sinus surgery in these patients do not exist, and many patients are treated in a similar manner to CF. AERD is characterized by aspirin sensitivity, asthma and CRS. This combination portends severe, protracted sinonasal disease. Many patients have polyps. Treatment involves topical and systemic corticosteroid therapy, and many patients will require sinus surgery although the disease is likely to persist despite such interventions [3 ,8,78]. Aspirin desensitization and maintenance therapy appears to be helpful in some patients, and surgery has been recommended as an adjunct to reduce disease burden prior to this intervention [40]. &&
&&
CONCLUSION The question of when treatment should progress to surgery has not adequately been answered in the literature. Nevertheless, surgery is safe and effective, and it should be considered for children with CRS in the following scenarios:
(1) Patients with CRS with or without nasal polyposis who have failed maximal medical treatment. As no consensus for maximal medical therapy exists in the literature, therapy must remain individualized but should at least include: at least one round of a 3–6 week course of broad spectrum antibiotics coupled with topical or systemic corticosteroid therapy, nasal saline irrigations and concomitant treatment of allergic rhinitis or gastroesophageal reflux should these conditions be present. (2) Patients with nasal polyposis not adequately responsive to medical therapy in which polyps are leading to symptomatic obstruction of the nasal airway or sinus outflow tracts. (3) Patients with CF, in which removal of polyps is indicated for relief of symptoms of nasal obstruction, headache or anosmia that persist despite medical therapy. Sinus surgery is beneficial in patients with CRS postlung transplant. (4) The timing and role of surgical intervention for rhinosinusitis in patients with CRSwNP, AFRS, AERD or PCD is not clear from the literature. Patients with these conditions can be expected to be more likely to require surgery because of the potentially aggressive nature of their disease. CT scan should only be performed in the following scenarios: to evaluate anatomy and disease extent prior to FESS, in patients with persistent symptoms despite adenoidectomy and subsequent medical therapy, if a complication of rhinosinusitis is suspected or for atypical symptoms and signs, such as unilateral obstruction. Acknowledgements None. Conflicts of interest The authors have no conflicts of interest to report relating to this article.
REFERENCES AND RECOMMENDED READING Papers of particular interest, published within the annual period of review, have been highlighted as: & of special interest && of outstanding interest 1. Adappa ND, Coticchia JM. Management of refractory chronic rhinosinusitis in children. Am J Otolaryngol 2006; 27:384–389. 2. Lanza DC, Kennedy DW. Adult rhinosinusitis defined. Otolaryngol Head Neck Surg 1997; 117:S1–S7. 3. Fokkens WJ, Lund VJ, Mulol J, et al. European position paper on rhinosinusitis && and nasal polyps 2012. [in special issue: EPOS2012]. Rhinology 2012; 50 (Supplement 23):1–298. This evidence-based review of rhinosinusitis by a multidisciplinary panel is the most thorough analysis in the literature published in recent years. It covers all important aspects of pediatric and adult sinonasal disease. 4. Sobol S, Samadi S, Kazahaya K, Tom LW. Trends in the management of pediatric chronic sinusitis: survey of the American Society of Pediatric Otolaryngology. Laryngoscope 2005; 115:78–80.
1068-9508 ß 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins
www.co-otolaryngology.com
31
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Nose and paranasal sinuses 5. Kennedy DW, Senior BA, Gannon FH, et al. Histology and histomorphometry of ethmoid bone in chronic rhinosinusitis. Laryngoscope 1998; 108:502– 507. 6. Perloff JR, Gannon FH, Bolger WE, et al. Bone involvement in sinusitis: an apparent pathway for the spread of disease. Laryngoscope 2000; 110:2095–2099. 7. Park JH, Kim YJ, Sung KJ, et al. Correlation between radiologically identified osteitis and prognosis in chronic rhinosinusitis. Korea J Otolaryngol 2005; 48:998–1003. 8. Mendelsohn D, Jeremic G, Wright ED, Rotenberg BW. Revision rates after endoscopic sinus surgery: a recurrence analysis. Ann Otol Rhinol Laryngol 2011; 120:162–166. 9. Brook I. The role of anaerobic bacteria in sinusitis. Anaerobe 2006; 12:5–12. 10. Orobello PW Jr, Park RI, Belcher L, et al. Microbiology of chronic sinusitis in children. Arch Otolaryngol Head Neck Surg 1991; 117:980–983. 11. Muntz HR, Lusk RP. Bacteriology of the ethmoid bullae in children with chronic sinusitis. Arch Otolaryngol Head Neck Surg 1991; 117:179–181. 12. Goldenhersh MJ, Rachelefsky GS, Dudley J, et al. The microbiology of chronic sinus disease in children with respiratory allergy. J Allergy Clin Immunol 1998; 85:1030–1039. 13. Wald ER, Byers C, Guerra N, et al. Subacute sinusitis in children. J Pediatr 1989; 115:28–32. 14. Erkan M, Ozcan M, Arslan S, et al. Bacteriology of antrum in children with chronic maxillary sinusitis. Scand J Infect Dis 1996; 28:283–285. 15. Baier G, Stopper H, Kopp C, et al. Respiratory diseases and genotoxicity in tobacco smoke exposure. Laryngorhinootologie 2002; 81:217–225. 16. Wang LF, White DR, Andreoli SM. Cigarette smoke inhibits dynamic ciliary beat frequency in pediatric adenoid explants. Otolaryngol Head Neck Surg 2014; 146:659–663. 17. Ramadan HH, Hinerman RA. Smoke exposure and outcome of endoscopic sinus surgery in children. Orolaryngol Head Neck Surg 2002; 127:546– 548. 18. Sih T. Correlation between respiratory alterations and respiratory diseases due to urban pollution. Int J Pediatr Otorhinolaryngol 1999; 49:261–267. 19. Celedon JC, Litonjua AA, Weiss ST, Gold DR. Day care attendance in the first year of life and illness of upper and lower respiratory tract in children with a familial history of atopy. Pediatrics 1999; 104:495–500. 20. Phipps CD, Wood WE, Gibson WS, Cochran WJ. Gastroesphageal reflux contributing to chronic sinus disease in children: a prospective analysis. Arch Otolaryngol Head Neck Surg 2000; 126:831–836. 21. Gutman M, Torres A, Keen KJ, Houser SM. Prevalence of allergy in patients with chronic rhinosinusitis. Otolaryngol Head Neck Surg 2004; 130:545– 552. 22. Roberston JM, Friedman EM, Rubin BK. Nasal and sinus disease in cystic fibrosis. Paediatr Respir Rev 2008; 9:213–219. 23. Knowles MR, Daniels LA, Davis SD, et al. Primary ciliary dyskinesia: recent advances in diagnostics, genetics and characterization of clinical disease. Am J Respir Crit Care Med 2013; 188:913–922. 10.1164/rccm.2013010059CI. 24. Zuliani G, Carron M, Gurrola J, et al. Identification of adenoid biofilms in chronic rhinosinusitis. Int J Pediatr Otorhinolaryngol 2006; 70:1613–1617. 25. Wood AJ, Douglas RG. Pathogenesis and treatment of chronic rhinosinusitis. Postgrad Med J 2010; 86:359–364. 26. Pham V, Sykes K, Wei J. Long-term outcome of once daily nasal irrigation for the treatment of pediatric chronic sinusitis. Laryngoscope 2013; doi: 10.1002/lary.24224. [Epub ahead of print] 27. Chang AB. Chronic cough in children. Paediatr Child Health 2008; 18:333– 339. 28. Krouse JH. The unified airway: conceptual framework. Otolaryngol Clin North Am 2008; 41:257–266. 29. Kubbba H, Phil M, Bingham BJ. Endoscopy in the assessment of children with nasal obstruction. J Laryngol Otol 2001; 115:380–384. 30. Mlynarek A, Tewfick MA, Hagr A, et al. Lateral neck radiography versus direct video rhinoscopy in assessing adenoid size. J Otolaryngol 2004; 33:360– 365. 31. Hill M, Bhattacharyya N, Hall TR, et al. Incidental paranasal sinus imaging abnormalities and the normal Lund score in children. Arch Otolaryngol Head Neck Surg 2004; 130:171–175. 32. Wu AW, Shapiro NL, Bhattacharyya N. Chronic rhinosinusitis in children: what are the treatment options. Immunol Allergy Clin N Am 2009; 29:705– 717. 33. Pearce MS, Salotti JA, Little MP, et al. Radiation exposure from CT scans in && childhood and subsequent risk of leukaemia and brain tumours: a retrospective cohort study. Lancet 2012; 380:499–505. This long-term study retrospectively analyzed a large cohort of patients who underwent CT imaging as children and found a statistically significant increase in the rate of leukemia and brain cancer attributable to CT. 34. Wald ER, Bordley WC, Darrow DH, et al. Clinical practice guidline: management of sinusitis. Pediatrics 2001; 108:798–808. 35. Cavel O, Quintal MC, Marcotte JE, et al. Restricting indications for sinonasal & computed tomography in children with cystic fibrosis. JAMA Otolaryngol Head Neck Surg 2013; 139:54–58. This article focuses on the utility of sinus CT scans in patients with CF. The findings do not report benefit from the use of CT for simple disease surveillance.
32
www.co-otolaryngology.com
36. Mandal R, Patel N, Ferguson BJ. Role of antibiotics in sinusitis. Curr Opin Infect Dis 2012; 25:183–192. 37. Cazzavillan A, Castelnuovo P, Berlucci M. Management of chronic rhinosi&& nusitis. Pediatr All and Immunol 2012; 23 (Suppl 22):32–34. This article provides a complete, thorough review of pediatric sinusitis, focusing both on medical and surgical treatment. 38. Swapna CK, Higgins TS. Pediatric rhinosinusitis: definitions, diagnosis and management: an overview. Am J Rhinol Allergy 2013; 27:S16–S19. 39. Clement P, Bluestone C, Gordts F, et al. Management of rhinosinusitis in children. Int J Pediatr Otorhinolaryngol 1999; 49:S95–S100. 40. Lieser JD, Derkay CS. Pediatric sinusitis: when do we operate? Curr Opin Otolaryngol Head Neck Surg 2005; 13:60–66. 41. Zhang L, Mendoza-Sassi RA, Cesar JA, Chanda NK. Intranasal corticosteroids for nasal airway obstruction in children with moderate to severe adenoidal hypertrophy. Cochrane Database of Systematic Reviews 2008; CD006286. DOI:10.1002/14651858.CD006286.pub2. 42. Wei JL, Sykes KJ, Johnson P, et al. Safety and efficacy of once-daily nasal irrigation for the treatment of pediatric chronic rhinosinusitis. Laryngoscope 2011; 121:1989–2000. 43. Vandenberg SJ, Heatley DG. Efficacy of adenoidectomy in relieving symptoms of chronic sinusitis in children. Arch Otolaryngol Head Neck Surg 1997; 123:675–678. 44. Ramadan HH. Surgical management of chronic sinusitis in children. Laryngoscope 2004; 114:2103–2109. 45. Brietzke SE, Brigger MT. Adenoidectomy outcomes in pediatric rhinosinusitis: a meta-analysis. Int J Pediatr Otorhinolaryngol 2008; 72:1541–1545. 46. Gates GA. Adenoidectomy for otitis media with effusion. Ann Otol Rhinol Laryngol 1994; 163:54–58. 47. Maw AR. Age and adenoid size in relation to adenoidectomy in otitis media with effusion. Am J Otolaryngol 1985; 63:245–248. 48. Elwany S, El-Dine AN, El-Medany A, et al. Relationship between bacteriology of the adenoid core and middle meatus in children with sinusitis. J Laryngol Otol 2011; 125:279–281. 49. Ramadan HH. Safety and feasibility of balloon sinuplasty for treatment of chronic rhinosinusitis in children. Ann Otol Rhinol Laryngol 2009; 118:161–165. 50. Ramadan HH, Terrell AM. Balloon catheter sinuplasty and adenoidectomy in children with chronic rhinosinusitis. Ann Otol Rhinol Laryngol 2010; 119:578–582. 51. Ramadan HH, Bueller J, Hester ST, Terrell AM. Sinus balloon catheter dilation after adenoidectomy failure for children with chronic rhinosinusitis. Arch Otolaryngol Head Neck Surg 2012; 138:635–637. 52. Hebert RL, Bent JP. Meta-analysis of outcomes of pediatric functional endoscopic sinus surgery. Laryngoscope 1998; 108:796–799. 53. Siedek V, Stelter K, Betz CS, et al. Functional endoscopic sinus surgery: a retrospective analysis of 115 children and adolescents with chronic rhinosinusitis. Int J Pediatr Otorhinolaryngol 2009; 73:741–745. 54. Rudnick EF, Mitchell RB. Long-term improvements in quality-of-life after surgical therapy for pediatric sinonasal disease. Otolaryngol Head Neck Surg 2007; 137:873–877. 55. El Sharkawy AA, Elmorsy SM, Eladl HM. Functional endoscopic sinus surgery in children: predictive factors of outcome. Eur Arc Otorhinolaryngol 2012; 269:107–111. 56. Bothwell MR, Piccirillo JF, Lusk RP, Ridenour BD. Long-term outcome of facial growth after functional endoscopic sinus surgery. Otolaryngol Head Neck Surg 2002; 126:628–634. 57. Makary CA, Ramadan HH. The role of sinus surgery in children. Laryngoscope & 2013; 123:1348–1352. This article represents a thorough review of the current literature pertaining to FESS in children. 58. Lee T, Liang C, Chang P, Huang C. Risk factors for protracted sinusitis in pediatrics after endoscopic sinus surgery. Auris Nasus Larynx 2006; 36:655– 660. 59. Tsukidate T, Haruna S, Fukami S, et al. Long-term evaluation after sinus surgery for chronic pediatric sinusitis with polyps. Auris Nasus Larynx 2012; 39:583–587. 60. Gibson RL, Burns JL, Ramsey BW. Pathophysiology and management of pulmonary infections in cystic fibrosis. Am J Respir Crit Care Med 2003; 168:918–951. 61. Hansen SK, Rau MH, Johansen HK, et al. Evolution and diversification of Pseudomonas aeruginosa in the paranasal sinuses of cystic fibrosis children have implications for chronic lung infection. ISME J 2012; 6:31–45. 62. Gentile VG, Isaacson G. Patterns of sinusitis in cystic fibrosis. Laryngoscope 1996; 106:1005–1009. 63. Lavin J, Bhushan B, Schroeder JW. Correlation between respiratory cultures and sinus cultures in children with cystic fibrosis. Int J Pediatr Otorhinolarygol 2013; 77:686–689. 64. Jones JW, Parsons DS, Cuyler JP. The results of functional endoscopic sinus (FES) surgery on the symptoms of patients with cystic fibrosis. Int J Pediatr Otorhinolaryngol 1993; 28:25–32. 65. Rosbe KW, Jones DT, Rahbar R, et al. Endoscopic sinus surgery in cystic fibrosis: do patients benefit from surgery? Int J Pediatr Otorhinolaryngol 2001; 61:113–119. 66. Oomen KP, April MM. Sinonasal manifestations in cystic fibrosis. Int J Pediatr Otorhinolaryngol 2012; 2012:789572. doi: 10.1155/2012/789572. Epub.
Volume 22 Number 1 February 2014
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Pediatric chronic rhinosinusitis Rizzi and Kazahaya 67. Umetsu TU, Moss RB, King VV, Lewiston NJ. Sinus disease in patients with severe cystic fibrosis: relation to pulmonary exacerbation. Lancet 1990; 335:1077–1078. 68. Madonna D, Isaacson G, Rosenfeld RM, Panitch H. Effect of sinus surgery on pulmonary function in patients with cystic fibrosis. Laryngoscope 1997; 107:328–331. 69. Osborn AJ, Leung R, Ratjen F, James AL. Effect of endoscopic sinus surgery on pulmonary function and microbial pathogens in a pediatric population with cystic fibrosis. Arch Otolaryngol Head Neck Surg 2011; 137:542–547. 70. Cimmino M, Nardone M, Cavaliere M, et al. Dornase alfa as postoperative therapy in cystic fibrosis sinonasal disease. Arch Otolaryngol Head Neck Surg 2005; 131:1097–1101. 71. Mainz JG, Schiller I, Ritschel C, et al. Sinonasal inhalation of dornase alfa in CF: a double-blind placebo-controlled crossover pilot trial. Auris Nasus Larynx 2011; 38:220–227. 72. Vital D, Hofer M, Boehler A, Holzmann D. Posttransplant sinus surgery in lung & transplant recipients with cystic fibrosis: a single institutional experience. Eur Arch Otorhinolaryngol 2013; 270:135–139. The authors of this study provide support for attentive and thorough sinonasal medicine and surgery in patients with CF who have undergone lung transplant.
73. Bent JP, Kuhn FA. Diagnosis of allergic fungal sinusitis. Otolaryngol Head Neck Surg 1994; 111:580–588. 74. Manning S, Vuitch F, Weinberg A, et al. Allergic aspergillosis: a newly recognized form of sinusitis in the pediatric population. Laryngoscope 1989; 99:681–685. 75. Marple BF. Allergic fungal rhinosinusitis: current theories and management strategies. Laryngoscope 2001; 111:1006–1019. 76. Campbell R. Managing upper respiratory tract complications of primary && ciliary dyskinesia in children. Curr Opin Allergy Immunol 2012; 12:32– 38. The authors provide a thorough and current review of the medical and surgical treatment of sinonasal and otologic disease in patients with PCD. 77. Leigh MW, O’Callaghan C, Knowles MR. The challenges of diagnosing primary ciliary dyskinesia. Proc Am Thorac Soc 2011; 8:434–437. 78. Awad OG, Lee JH, Fasano MB, Graham SM. Sinonasal outcomes after endoscopic sinus surgery in asthmatic patients with nasal polyps: a difference between aspirin-tolerant and aspirin-induced asthma? Laryngoscope 2008; 118:1282–1286.
1068-9508 ß 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins
www.co-otolaryngology.com
33
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Pediatric chronic rhinosinusitis: when should we operate? Mark D. Rizzi a,b and Ken Kazahaya a,b
Purpose of review The timing and role of surgery in the management of pediatric chronic rhinosinusitis (pCRS) remains unclear. This review attempts to summarize the existing literature regarding this topic. Recent findings pCRS is a complex clinical syndrome that results from multiple potential causes. Multimodal medical therapy is the accepted primary treatment. Adenoidectomy continues to be the appropriate first-line surgical therapy, and the addition of antral lavage or balloon dilation to this procedure may improve outcomes. Functional endoscopic sinus surgery (FESS) in children is safe and effective in relieving symptoms in the majority of patients. Failure to respond to maximal medical therapy continues to be a commonly cited, although poorly defined, requisite for proceeding to surgery. Recent literature has been focused on the outcomes after FESS rather than specifically defining when this intervention should be considered. Nevertheless, the literature seems to continue to support FESS in children with persistent symptoms despite adenoidectomy and appropriate medical treatment for pCRS. As the symptoms of chronic rhinosinusitis (CRS) may result from multiple underlying causes, clinicians must understand that the role and timing of surgery may vary with particular patients’ disease. Summary Surgical management of CRS in children continues to be a frequent topic of study in the otolaryngology literature. As recent research tends to be focused on outcomes after surgery, further prospective studies comparing surgical versus nonsurgical treatment of CRS will likely be required to better define indications for proceeding to surgery at all. Keywords chronic rhinosinusitis, functional endoscopic sinus surgery, pediatric
INTRODUCTION Pediatric chronic rhinosinusitis (pCRS) continues to represent a significant burden for patients, their caregivers and the healthcare system at large. Although its true cost is unknown, billions of dollars are spent on its management annually [1]. Chronic rhinosinusitis (CRS) is specifically defined by the presence of persistent symptoms and signs of sinonasal inflammatory disease for a continuous period of 90 days or more [2]. Recently, the European Position Paper on Sinusitis and Polyposis 2012 defined pCRS as the presence of two or more symptoms, one being either nasal obstruction or nasal discharge coupled with either facial pain or pressure, cough and either endoscopic evidence of disease or changes of rhinosinusitis on computed tomography (CT) [3 ]. The cornerstone of treatment for CRS has been antibiotic therapy coupled with topical or systemic corticosteroid therapy and isotonic saline &&
rinses [4]. In some cases, medical therapy is unable to adequately ameliorate patients’ symptoms. In these patients, surgery may play a viable role in their care. Additionally, there are some patients in whom surgery may play a more primary role, although always as an adjunct to medical therapy. The goal of this review is to summarize recent relevant findings that have addressed the timing and role of surgery in pCRS. a Division of Pediatric Otolaryngology, Children’s Hospital of Philadelphia and bDepartment of Otolaryngology - Head and Neck Surgery, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA
Correspondence to Mark D. Rizzi, MD, Children’s Hospital of Philadelphia, Division of Otolaryngology, 1st Floor, Wood Building, Philadelphia, PA 19104, USA. Tel: +1 215 590 3440; e-mail: [email protected] Curr Opin Otolaryngol Head Neck Surg 2014, 22:27–33 DOI:10.1097/MOO.0000000000000018
1068-9508 ß 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins
www.co-otolaryngology.com
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Nose and paranasal sinuses
KEY POINTS Management of CRS in the pediatric population requires identification and treatment of the multiple potential causes that contribute to this syndrome. The question of when to proceed with surgery in the setting of medical failure has not been addressed by the current literature in an evidence-based manner, and therefore must remain individualized. Adenoidectomy with or without balloon dilation or antral lavage is appropriate first-line surgical therapy for children deemed to have failed medical treatment. Endoscopic sinus surgery is safe and effective in the majority of children who have failed medical therapy and adenoid removal or who meet the criteria listed in the conclusion section.
PATHOGENESIS OF CHRONIC RHINOSINUSITIS The cause of CRS in children has not been fully elucidated. Unlike acute rhinosinusitis, which is primarily an infectious entity, CRS represents a spectrum of pathologic states with varying degrees of contribution from both infection and underlying inflammation. The pathogenesis involves obstruction of sinus ostia with consequent stasis of secretions, reduced oxygen tension, inhibition of ciliary function and bacterial infection. At the local tissue level, changes in both the mucosa and underlying bone have been described [5–7]. Patients with CRS are often divided into those that have associated nasal polyposis (CRSwNP) and those that have CRS without polyps (CRSsNP). The cause of nasal polyposis is unknown despite extensive investigation into their pathogenesis and to the immunologic attributes of patients who suffer from them [3 ]. Although exploration of this topic is beyond the scope of this review, the presence of polyps may portend more recidivistic disease, especially when they are associated with asthma [8]. Staphylococcus aureus and epidermidis, Streptococcus pneumoniae and Hemophilus influenzae have been implicated in CRS [9–13]. Anaerobic bacteria are often present in these infections [9]. In a study by Erkan et al. [14], anaerobes were isolated from over 90% of chronically infected maxillary sinuses. In children, host factors, such as immunodeficiency, comorbid medical conditions and environmental factors all contribute to the development of CRS. Smoking, both active and passive [15–17], industrial pollution [18] and participation in day care [19] are associated with sinonasal disease. Further, local anatomic factors, such as the presence of polyps and chronically colonized or infected &&
28
www.co-otolaryngology.com
adenoids also play a role in the pathogenesis of CRS. Finally, comorbid conditions, such as the presence of reflux [20] or the coexistence of allergic rhinitis [21], cystic fibrosis (CF) [22] and primary ciliary dyskinesia (PCD) [23] are associated with an increased risk of CRS.
Biofilms Biofilms are bacterial aggregates that adhere to an underlying surface while being harbored within a hydrated exopolysaccharide matrix. This matrix is protective against host defenses and antibiotics but allows emission of individual cells, which may repeatedly infect the host [24]. The biofilm concept may serve as an explanation for the unreliable efficacy of antibiotics in some children with CRS. It also provides the rationale for adenoid removal in some patients, as discussed below. Although the presence of biofilms in adults with CRS is well characterized, the degree to which they play a role in children requires further research [3 ]. &&
Diagnosing chronic rhinosinusitis The diagnosis of pCRS can be difficult to make as children suffer from six to eight viral upper respiratory infections annually. The symptoms of these infections overlap considerably with those of bacterial rhinosinusitis. Distinguishing one from the other is challenging. The signs and symptoms and their duration required for the diagnosis of pCRS are outlined in the introduction section. As the diagnosis is primarily clinically made, the acquisition of a proper history is critical. Nasal obstruction, discolored drainage, hyposmia and facial pressure or pain are common complaints [25]; however, many patients do not present with nasal discharge [26]. Headache and facial pain alone rarely represent sinonasal disease but more commonly have a neurological cause [3 ]. Persistent cough is a frequent complaint. Although rhinosinusitis is not often the direct cause of the cough, cough is present because the same inflammatory process is affecting both the upper and lower airways [27]. Numerous studies have supported the idea that the upper and lower airways exist as an interconnected immunologic and reactive network [28]. To appreciate this ‘unified airway’ concept is to be aware that complete treatment of CRS requires recognition and treatment of both upper and lower airways inflammation. Finally, history should also assess for allergic rhinitis, asthma and reflux, all of which have been associated with pediatric CRS. Physical examination and nasal endoscopy are also important. Nasal endoscopy can be considered &&
Volume 22 Number 1 February 2014
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Pediatric chronic rhinosinusitis Rizzi and Kazahaya
to diagnose the specific site of nasal airway obstruction, such as enlarged adenoids or turbinates, nasal polyps, unsuspected masses or foreign bodies [29]. Endoscopy is also superior to lateral neck radiograph in evaluating adenoid size and degree of occlusion of the nasopharynx [30]. Although flexible nasal endoscopy is usually possible in young children, it is often difficult because of fear and intolerance of the procedure. Finally, imaging is of value in selected patients. CT scanning is the gold standard modality because of its clear resolution of bone, soft tissue and air. In interpreting pediatric sinonasal CT scans, it is important to recognize that abnormalities are often present in the absence of disease. Children without rhinosinusitis have an average Lund McKay score of 2.8 [31], and a score of 5 has been proposed to be required for a CT to be considered abnormal [31,32]. It is now clear that CT scans in children increase the risk of leukemia and brain cancer, and efforts should be made to minimize the number of studies performed and the dose of radiation delivered [33 ]. CT imaging should be reserved for patients in whom endoscopic sinus surgery is imminent, when a complication of sinusitis is suspected, or for patients with atypical presentations, such as unilateral obstruction [34]. It is not appropriate for screening or disease surveillance, even in patients known to be at risk for CRS [35 ]. &&
&
Medical treatment
maxillary sinuses may be an effective treatment strategy for patients who fail oral medical therapy [1]. Topical nasal corticosteroids are also considered first-line therapy for pCRS [3 ]. Nasal corticosteroids may also relieve nasal obstruction in patients with moderate-to-severe adenoid hypertrophy [41]. Nasal saline irrigations appear to be effective and well tolerated in the pediatric population [42], although it is likely an underutilized modality [26]. Antihistamines may be used for relief of symptoms of allergic rhinitis in applicable patients, but their efficacy in the treatment of CRS is not proven. &&
Surgical treatment The European Position Paper on Rhinosinusitis and Nasal Polyps 2012 summarized that a logical surgical algorithm for pCRS begins with adenoidectomy with possible antral irrigation or balloon dilation of the maxillary sinuses, with FESS reserved for treatment failures [3 ]. Symptomatic patients with sinonasal polyposis, CF, allergic fungal rhinosinusitis (AFRS), PCD or antrochoanal polyps are more likely to require FESS for disease control. &&
Adenoidectomy First-line surgical therapy for pCRS is adenoid removal. Multiple studies have shown that this treatment modality is effective in the majority of patients [3 ,43,44]. In a meta-analysis that included nine studies, Brietzke and Brigger [45] concluded that 69.3% of patients experienced significant improvement following adenoidectomy. Size of the adenoid does not influence success of adenoidectomy [46,47] perhaps explained by the biofilm concept [24]. In a study examining adenoid tissue, biofilms were identified by scanning electron microscopy in all seven individuals who underwent adenoidectomy for CRS but in zero of nine patients who underwent adenoidectomy for the treatment of obstructive sleep apnea [24]. There is a high correlation between the bacteria present within the adenoids and within the middle meatus in children with CRS [48]. The addition of middle meatal irrigation or balloon dilation may increase the efficacy of adenoid removal in the treatment of pCRS. Balloon catheter dilation of the maxillary ostium has been shown to be safe in children [49]. Ramadan and Terrell [50] reported that balloon therapy when combined with adenoidectomy was more effective than adenoidectomy alone. In this study, some patients also underwent sinus irrigation making the contribution of the balloon less clear. In a separate study, these authors demonstrated successful &&
The mainstay of treatment for pCRS is medically derived. Surgery is often described as being reserved for individuals who fail ‘maximal medical treatment’ although there is no consensus to define the extent of such therapy. Antibiotics are the mainstay of first-line therapy despite there being only weak evidence supporting their efficacy [36]. Penicillin resistance is common [37 ] and is more likely in patients attending daycare, in patients less than 2 years old and in those who underwent recent treatment with antibiotics [38]. Amoxicillin-clavulanate is a commonly cited first-line agent [39,40]. In penicillin allergic patients, a cephalosporin or macrolide can be considered. If methicillin-resistant Staphylococcus aureus is suspected, clindamycin and Bactrim may be effective. Recommended antibiotic duration is commonly described as being between 3 and 6 weeks. In a 2006 review, Wu et al. [32] suggested that such therapy should include extended use of a broad-spectrum antibiotic and nasal steroid spray with or without adjunctive use of an oral antihistamine, an oral mucolytic agent, oral steroids or nasal saline irrigation. Intravenous antibiotics, when combined with irrigation of the &&
1068-9508 ß 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins
www.co-otolaryngology.com
29
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Nose and paranasal sinuses
use of balloon sinuplasty in 81% of patients who previously failed adenoidectomy as measured by change in Sn5 scores [51]. Patients with persistent symptoms despite medical management and adenoid removal as above are candidates for CT scan and potentially FESS. Consideration for work-up of immunodeficiency and other comorbidities should be undertaken in these patients if this has not yet been done.
FUNCTIONAL ENDOSCOPIC SINUS SURGERY The efficacy of FESS in children has been previously reported. In 1998, a meta-analysis by Hebert and Bent [52] revealed favorable outcomes in 88–92% of patients with a mean follow-up of 3.7 years. These results have been replicated consistently since that time. Siedek et al. [53] reported a 77% improvement in symptoms and a 74% improvement in quality of life in children after FESS as measured by patient self-assessment. In a 2007 review, Rudnick and Mitchell [54] showed that the benefits of FESS may extend further than 6 months postsurgery. In 2012, El Sharkawy et al. [55] published a retrospective review of 87 patients who underwent FESS for CRS after failing medical treatment. A detailed description defining medical failure in their individuals was not provided. In this study, an overall success rate, defined as improved endoscopic or CT appearance as well as improved symptoms, was 87.69%. Concerns about impedance of midfacial growth have been allayed by studies of long-term follow-up of patients who underwent FESS at a young age [56]. In 2013, Makary and Ramadan [57 ] performed a review of the literature with the aim of analyzing outcomes from FESS in children with CRS. Their review excluded studies that included patients with CF, polyposis, immunodeficiency and PCD if the original reports did not analyze these patients separately. Eleven studies published between 1993 and 2012 were included. The authors report a success rate from surgery ranging from 82 to 100% . However, the authors noted in their conclusion that the question of when sinus surgery is indicated in children remains unanswered because of lack of adequately powered studies that compare FESS to other surgical options or to medical therapy alone [57 ]. &
&
SURGICAL MANAGEMENT IN SPECIFIC POPULATIONS CF, AFRS, aspirin exacerbated respiratory disease (AERD) and PCD are disease processes which are 30
www.co-otolaryngology.com
associated with refractory CRS and in which surgery is likely to play a more primary adjunctive role to medical therapy than it does in patients with isolated CRS. Polyps are often present in these patients. Even in the absence of these systemic illnesses, polyps are associated with more persistent disease. Lee et al. [58] showed that sinonasal polyps were an independent risk factor for persistent symptoms in patients who underwent FESS. Tsukidate et al. [59] report that 50% of children who underwent FESS for bilateral nasal polyposis were either unchanged or worse at 1-year follow-up but that the proportion of patients improved increases with time beyond that point. In children, sinonasal polyps should be thought to represent underlying CF until proven otherwise. CF is an autosomal recessive disorder that involves mutation of the CFTR gene, which codes for a chloride channel that indirectly affects water transport across cell membranes. The thick, inspissated secretions generated within the airways of these patients leads to a chronic colonized state [60]. Initial colonization and evolution of antibiotic resistance may occur within the paranasal sinuses and transition to the lower airways from there [61]. CF involves the sinuses in 90% of afflicted patients [62] although many do not complain of symptoms [24]. Pseudomonas aeruginosa is a common pathogen of the upper and lower airways in older children, whereas staphylococcal species are often seen in younger patients [63]. There are no established indications for proceeding to surgery in patients with CF. FESS in CF patients significantly relieves symptoms of nasal obstruction and discharge and may improve overall quality of life [64]. A further benefit is improved access for application of antibiotic irrigation [3 ]. Controversy exists as to whether FESS may be beneficial with regard to the pulmonary status of patients with CF although most studies agree that FESS has no effect on pulmonary function tests [65–69]. Nasally inhaled aerosolized DNase postoperatively may improve symptoms, mucociliary clearance and the endoscopic and radiologic appearance of the sinuses in patients with CF [70,71]. Additionally, surgery is beneficial in patients with medically refractory CRS postlung transplantation [66,72 ]. AFRS is a syndrome characterized by type 1 hypersensitivity, nasal polyposis, eosinophilia, characteristic CT findings of unilateral or bilateral soft tissue density with expansile and erosive features and often marked by scattered hyperdensity within the sinuses and the presence of noninvasive fungal elements in pathologic tissue specimens [73]. The underlying cause is unknown although it has &&
&
Volume 22 Number 1 February 2014
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Pediatric chronic rhinosinusitis Rizzi and Kazahaya
been described as analogous to allergic bronchopulmonary aspergillosis of the lungs [74]. AFRS is commonly unilateral. Medical treatment involves reducing inflammation within the nose and sinuses with corticosteroids. Allergen immunotherapy aimed at typically implicated fungi may also be beneficial [75]. The use of antifungal drugs is not widely accepted. Surgery is often necessary for complete relief of symptoms. The goals of surgery are to decrease the burden of fungal antigen by thorough removal of polyps and eosinophilic mucin and to widely open the sinuses to allow for both ventilation and access for diagnostic endoscopy and topical therapy in the future. PCD is an autosomal recessive disorder that affects approximately 1 : 15 000 patients. Diagnosis is often delayed, and many patients receive suboptimal management. Approximately, 30% of patients have nasal polyps [76 ]. Diagnosis can be difficult because of the heterogeneity of these patients. Many have normal ciliary morphology on electron microscopy. Identification of known genetic abnormalities can be helpful, but the entire genetic spectrum of the disease is not known. Identification of decreased nasal nitric oxide is supportive of the diagnosis but not specific as low nitric oxide levels are seen is some patients with CF or sinusitis [77]. Ultimately, diagnosis must come from astute clinical vigilance. Eighty percent of patients with PCD have a history of neonatal respiratory distress. Daily nasal congestion, year-round wet cough and the presence of situs inversus or situs ambiguous strongly suggest PCD. Patients commonly have chronic ear disease. Evidence-based guidelines for proceeding to sinus surgery in these patients do not exist, and many patients are treated in a similar manner to CF. AERD is characterized by aspirin sensitivity, asthma and CRS. This combination portends severe, protracted sinonasal disease. Many patients have polyps. Treatment involves topical and systemic corticosteroid therapy, and many patients will require sinus surgery although the disease is likely to persist despite such interventions [3 ,8,78]. Aspirin desensitization and maintenance therapy appears to be helpful in some patients, and surgery has been recommended as an adjunct to reduce disease burden prior to this intervention [40]. &&
&&
CONCLUSION The question of when treatment should progress to surgery has not adequately been answered in the literature. Nevertheless, surgery is safe and effective, and it should be considered for children with CRS in the following scenarios:
(1) Patients with CRS with or without nasal polyposis who have failed maximal medical treatment. As no consensus for maximal medical therapy exists in the literature, therapy must remain individualized but should at least include: at least one round of a 3–6 week course of broad spectrum antibiotics coupled with topical or systemic corticosteroid therapy, nasal saline irrigations and concomitant treatment of allergic rhinitis or gastroesophageal reflux should these conditions be present. (2) Patients with nasal polyposis not adequately responsive to medical therapy in which polyps are leading to symptomatic obstruction of the nasal airway or sinus outflow tracts. (3) Patients with CF, in which removal of polyps is indicated for relief of symptoms of nasal obstruction, headache or anosmia that persist despite medical therapy. Sinus surgery is beneficial in patients with CRS postlung transplant. (4) The timing and role of surgical intervention for rhinosinusitis in patients with CRSwNP, AFRS, AERD or PCD is not clear from the literature. Patients with these conditions can be expected to be more likely to require surgery because of the potentially aggressive nature of their disease. CT scan should only be performed in the following scenarios: to evaluate anatomy and disease extent prior to FESS, in patients with persistent symptoms despite adenoidectomy and subsequent medical therapy, if a complication of rhinosinusitis is suspected or for atypical symptoms and signs, such as unilateral obstruction. Acknowledgements None. Conflicts of interest The authors have no conflicts of interest to report relating to this article.
REFERENCES AND RECOMMENDED READING Papers of particular interest, published within the annual period of review, have been highlighted as: & of special interest && of outstanding interest 1. Adappa ND, Coticchia JM. Management of refractory chronic rhinosinusitis in children. Am J Otolaryngol 2006; 27:384–389. 2. Lanza DC, Kennedy DW. Adult rhinosinusitis defined. Otolaryngol Head Neck Surg 1997; 117:S1–S7. 3. Fokkens WJ, Lund VJ, Mulol J, et al. European position paper on rhinosinusitis && and nasal polyps 2012. [in special issue: EPOS2012]. Rhinology 2012; 50 (Supplement 23):1–298. This evidence-based review of rhinosinusitis by a multidisciplinary panel is the most thorough analysis in the literature published in recent years. It covers all important aspects of pediatric and adult sinonasal disease. 4. Sobol S, Samadi S, Kazahaya K, Tom LW. Trends in the management of pediatric chronic sinusitis: survey of the American Society of Pediatric Otolaryngology. Laryngoscope 2005; 115:78–80.
1068-9508 ß 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins
www.co-otolaryngology.com
31
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Nose and paranasal sinuses 5. Kennedy DW, Senior BA, Gannon FH, et al. Histology and histomorphometry of ethmoid bone in chronic rhinosinusitis. Laryngoscope 1998; 108:502– 507. 6. Perloff JR, Gannon FH, Bolger WE, et al. Bone involvement in sinusitis: an apparent pathway for the spread of disease. Laryngoscope 2000; 110:2095–2099. 7. Park JH, Kim YJ, Sung KJ, et al. Correlation between radiologically identified osteitis and prognosis in chronic rhinosinusitis. Korea J Otolaryngol 2005; 48:998–1003. 8. Mendelsohn D, Jeremic G, Wright ED, Rotenberg BW. Revision rates after endoscopic sinus surgery: a recurrence analysis. Ann Otol Rhinol Laryngol 2011; 120:162–166. 9. Brook I. The role of anaerobic bacteria in sinusitis. Anaerobe 2006; 12:5–12. 10. Orobello PW Jr, Park RI, Belcher L, et al. Microbiology of chronic sinusitis in children. Arch Otolaryngol Head Neck Surg 1991; 117:980–983. 11. Muntz HR, Lusk RP. Bacteriology of the ethmoid bullae in children with chronic sinusitis. Arch Otolaryngol Head Neck Surg 1991; 117:179–181. 12. Goldenhersh MJ, Rachelefsky GS, Dudley J, et al. The microbiology of chronic sinus disease in children with respiratory allergy. J Allergy Clin Immunol 1998; 85:1030–1039. 13. Wald ER, Byers C, Guerra N, et al. Subacute sinusitis in children. J Pediatr 1989; 115:28–32. 14. Erkan M, Ozcan M, Arslan S, et al. Bacteriology of antrum in children with chronic maxillary sinusitis. Scand J Infect Dis 1996; 28:283–285. 15. Baier G, Stopper H, Kopp C, et al. Respiratory diseases and genotoxicity in tobacco smoke exposure. Laryngorhinootologie 2002; 81:217–225. 16. Wang LF, White DR, Andreoli SM. Cigarette smoke inhibits dynamic ciliary beat frequency in pediatric adenoid explants. Otolaryngol Head Neck Surg 2014; 146:659–663. 17. Ramadan HH, Hinerman RA. Smoke exposure and outcome of endoscopic sinus surgery in children. Orolaryngol Head Neck Surg 2002; 127:546– 548. 18. Sih T. Correlation between respiratory alterations and respiratory diseases due to urban pollution. Int J Pediatr Otorhinolaryngol 1999; 49:261–267. 19. Celedon JC, Litonjua AA, Weiss ST, Gold DR. Day care attendance in the first year of life and illness of upper and lower respiratory tract in children with a familial history of atopy. Pediatrics 1999; 104:495–500. 20. Phipps CD, Wood WE, Gibson WS, Cochran WJ. Gastroesphageal reflux contributing to chronic sinus disease in children: a prospective analysis. Arch Otolaryngol Head Neck Surg 2000; 126:831–836. 21. Gutman M, Torres A, Keen KJ, Houser SM. Prevalence of allergy in patients with chronic rhinosinusitis. Otolaryngol Head Neck Surg 2004; 130:545– 552. 22. Roberston JM, Friedman EM, Rubin BK. Nasal and sinus disease in cystic fibrosis. Paediatr Respir Rev 2008; 9:213–219. 23. Knowles MR, Daniels LA, Davis SD, et al. Primary ciliary dyskinesia: recent advances in diagnostics, genetics and characterization of clinical disease. Am J Respir Crit Care Med 2013; 188:913–922. 10.1164/rccm.2013010059CI. 24. Zuliani G, Carron M, Gurrola J, et al. Identification of adenoid biofilms in chronic rhinosinusitis. Int J Pediatr Otorhinolaryngol 2006; 70:1613–1617. 25. Wood AJ, Douglas RG. Pathogenesis and treatment of chronic rhinosinusitis. Postgrad Med J 2010; 86:359–364. 26. Pham V, Sykes K, Wei J. Long-term outcome of once daily nasal irrigation for the treatment of pediatric chronic sinusitis. Laryngoscope 2013; doi: 10.1002/lary.24224. [Epub ahead of print] 27. Chang AB. Chronic cough in children. Paediatr Child Health 2008; 18:333– 339. 28. Krouse JH. The unified airway: conceptual framework. Otolaryngol Clin North Am 2008; 41:257–266. 29. Kubbba H, Phil M, Bingham BJ. Endoscopy in the assessment of children with nasal obstruction. J Laryngol Otol 2001; 115:380–384. 30. Mlynarek A, Tewfick MA, Hagr A, et al. Lateral neck radiography versus direct video rhinoscopy in assessing adenoid size. J Otolaryngol 2004; 33:360– 365. 31. Hill M, Bhattacharyya N, Hall TR, et al. Incidental paranasal sinus imaging abnormalities and the normal Lund score in children. Arch Otolaryngol Head Neck Surg 2004; 130:171–175. 32. Wu AW, Shapiro NL, Bhattacharyya N. Chronic rhinosinusitis in children: what are the treatment options. Immunol Allergy Clin N Am 2009; 29:705– 717. 33. Pearce MS, Salotti JA, Little MP, et al. Radiation exposure from CT scans in && childhood and subsequent risk of leukaemia and brain tumours: a retrospective cohort study. Lancet 2012; 380:499–505. This long-term study retrospectively analyzed a large cohort of patients who underwent CT imaging as children and found a statistically significant increase in the rate of leukemia and brain cancer attributable to CT. 34. Wald ER, Bordley WC, Darrow DH, et al. Clinical practice guidline: management of sinusitis. Pediatrics 2001; 108:798–808. 35. Cavel O, Quintal MC, Marcotte JE, et al. Restricting indications for sinonasal & computed tomography in children with cystic fibrosis. JAMA Otolaryngol Head Neck Surg 2013; 139:54–58. This article focuses on the utility of sinus CT scans in patients with CF. The findings do not report benefit from the use of CT for simple disease surveillance.
32
www.co-otolaryngology.com
36. Mandal R, Patel N, Ferguson BJ. Role of antibiotics in sinusitis. Curr Opin Infect Dis 2012; 25:183–192. 37. Cazzavillan A, Castelnuovo P, Berlucci M. Management of chronic rhinosi&& nusitis. Pediatr All and Immunol 2012; 23 (Suppl 22):32–34. This article provides a complete, thorough review of pediatric sinusitis, focusing both on medical and surgical treatment. 38. Swapna CK, Higgins TS. Pediatric rhinosinusitis: definitions, diagnosis and management: an overview. Am J Rhinol Allergy 2013; 27:S16–S19. 39. Clement P, Bluestone C, Gordts F, et al. Management of rhinosinusitis in children. Int J Pediatr Otorhinolaryngol 1999; 49:S95–S100. 40. Lieser JD, Derkay CS. Pediatric sinusitis: when do we operate? Curr Opin Otolaryngol Head Neck Surg 2005; 13:60–66. 41. Zhang L, Mendoza-Sassi RA, Cesar JA, Chanda NK. Intranasal corticosteroids for nasal airway obstruction in children with moderate to severe adenoidal hypertrophy. Cochrane Database of Systematic Reviews 2008; CD006286. DOI:10.1002/14651858.CD006286.pub2. 42. Wei JL, Sykes KJ, Johnson P, et al. Safety and efficacy of once-daily nasal irrigation for the treatment of pediatric chronic rhinosinusitis. Laryngoscope 2011; 121:1989–2000. 43. Vandenberg SJ, Heatley DG. Efficacy of adenoidectomy in relieving symptoms of chronic sinusitis in children. Arch Otolaryngol Head Neck Surg 1997; 123:675–678. 44. Ramadan HH. Surgical management of chronic sinusitis in children. Laryngoscope 2004; 114:2103–2109. 45. Brietzke SE, Brigger MT. Adenoidectomy outcomes in pediatric rhinosinusitis: a meta-analysis. Int J Pediatr Otorhinolaryngol 2008; 72:1541–1545. 46. Gates GA. Adenoidectomy for otitis media with effusion. Ann Otol Rhinol Laryngol 1994; 163:54–58. 47. Maw AR. Age and adenoid size in relation to adenoidectomy in otitis media with effusion. Am J Otolaryngol 1985; 63:245–248. 48. Elwany S, El-Dine AN, El-Medany A, et al. Relationship between bacteriology of the adenoid core and middle meatus in children with sinusitis. J Laryngol Otol 2011; 125:279–281. 49. Ramadan HH. Safety and feasibility of balloon sinuplasty for treatment of chronic rhinosinusitis in children. Ann Otol Rhinol Laryngol 2009; 118:161–165. 50. Ramadan HH, Terrell AM. Balloon catheter sinuplasty and adenoidectomy in children with chronic rhinosinusitis. Ann Otol Rhinol Laryngol 2010; 119:578–582. 51. Ramadan HH, Bueller J, Hester ST, Terrell AM. Sinus balloon catheter dilation after adenoidectomy failure for children with chronic rhinosinusitis. Arch Otolaryngol Head Neck Surg 2012; 138:635–637. 52. Hebert RL, Bent JP. Meta-analysis of outcomes of pediatric functional endoscopic sinus surgery. Laryngoscope 1998; 108:796–799. 53. Siedek V, Stelter K, Betz CS, et al. Functional endoscopic sinus surgery: a retrospective analysis of 115 children and adolescents with chronic rhinosinusitis. Int J Pediatr Otorhinolaryngol 2009; 73:741–745. 54. Rudnick EF, Mitchell RB. Long-term improvements in quality-of-life after surgical therapy for pediatric sinonasal disease. Otolaryngol Head Neck Surg 2007; 137:873–877. 55. El Sharkawy AA, Elmorsy SM, Eladl HM. Functional endoscopic sinus surgery in children: predictive factors of outcome. Eur Arc Otorhinolaryngol 2012; 269:107–111. 56. Bothwell MR, Piccirillo JF, Lusk RP, Ridenour BD. Long-term outcome of facial growth after functional endoscopic sinus surgery. Otolaryngol Head Neck Surg 2002; 126:628–634. 57. Makary CA, Ramadan HH. The role of sinus surgery in children. Laryngoscope & 2013; 123:1348–1352. This article represents a thorough review of the current literature pertaining to FESS in children. 58. Lee T, Liang C, Chang P, Huang C. Risk factors for protracted sinusitis in pediatrics after endoscopic sinus surgery. Auris Nasus Larynx 2006; 36:655– 660. 59. Tsukidate T, Haruna S, Fukami S, et al. Long-term evaluation after sinus surgery for chronic pediatric sinusitis with polyps. Auris Nasus Larynx 2012; 39:583–587. 60. Gibson RL, Burns JL, Ramsey BW. Pathophysiology and management of pulmonary infections in cystic fibrosis. Am J Respir Crit Care Med 2003; 168:918–951. 61. Hansen SK, Rau MH, Johansen HK, et al. Evolution and diversification of Pseudomonas aeruginosa in the paranasal sinuses of cystic fibrosis children have implications for chronic lung infection. ISME J 2012; 6:31–45. 62. Gentile VG, Isaacson G. Patterns of sinusitis in cystic fibrosis. Laryngoscope 1996; 106:1005–1009. 63. Lavin J, Bhushan B, Schroeder JW. Correlation between respiratory cultures and sinus cultures in children with cystic fibrosis. Int J Pediatr Otorhinolarygol 2013; 77:686–689. 64. Jones JW, Parsons DS, Cuyler JP. The results of functional endoscopic sinus (FES) surgery on the symptoms of patients with cystic fibrosis. Int J Pediatr Otorhinolaryngol 1993; 28:25–32. 65. Rosbe KW, Jones DT, Rahbar R, et al. Endoscopic sinus surgery in cystic fibrosis: do patients benefit from surgery? Int J Pediatr Otorhinolaryngol 2001; 61:113–119. 66. Oomen KP, April MM. Sinonasal manifestations in cystic fibrosis. Int J Pediatr Otorhinolaryngol 2012; 2012:789572. doi: 10.1155/2012/789572. Epub.
Volume 22 Number 1 February 2014
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Pediatric chronic rhinosinusitis Rizzi and Kazahaya 67. Umetsu TU, Moss RB, King VV, Lewiston NJ. Sinus disease in patients with severe cystic fibrosis: relation to pulmonary exacerbation. Lancet 1990; 335:1077–1078. 68. Madonna D, Isaacson G, Rosenfeld RM, Panitch H. Effect of sinus surgery on pulmonary function in patients with cystic fibrosis. Laryngoscope 1997; 107:328–331. 69. Osborn AJ, Leung R, Ratjen F, James AL. Effect of endoscopic sinus surgery on pulmonary function and microbial pathogens in a pediatric population with cystic fibrosis. Arch Otolaryngol Head Neck Surg 2011; 137:542–547. 70. Cimmino M, Nardone M, Cavaliere M, et al. Dornase alfa as postoperative therapy in cystic fibrosis sinonasal disease. Arch Otolaryngol Head Neck Surg 2005; 131:1097–1101. 71. Mainz JG, Schiller I, Ritschel C, et al. Sinonasal inhalation of dornase alfa in CF: a double-blind placebo-controlled crossover pilot trial. Auris Nasus Larynx 2011; 38:220–227. 72. Vital D, Hofer M, Boehler A, Holzmann D. Posttransplant sinus surgery in lung & transplant recipients with cystic fibrosis: a single institutional experience. Eur Arch Otorhinolaryngol 2013; 270:135–139. The authors of this study provide support for attentive and thorough sinonasal medicine and surgery in patients with CF who have undergone lung transplant.
73. Bent JP, Kuhn FA. Diagnosis of allergic fungal sinusitis. Otolaryngol Head Neck Surg 1994; 111:580–588. 74. Manning S, Vuitch F, Weinberg A, et al. Allergic aspergillosis: a newly recognized form of sinusitis in the pediatric population. Laryngoscope 1989; 99:681–685. 75. Marple BF. Allergic fungal rhinosinusitis: current theories and management strategies. Laryngoscope 2001; 111:1006–1019. 76. Campbell R. Managing upper respiratory tract complications of primary && ciliary dyskinesia in children. Curr Opin Allergy Immunol 2012; 12:32– 38. The authors provide a thorough and current review of the medical and surgical treatment of sinonasal and otologic disease in patients with PCD. 77. Leigh MW, O’Callaghan C, Knowles MR. The challenges of diagnosing primary ciliary dyskinesia. Proc Am Thorac Soc 2011; 8:434–437. 78. Awad OG, Lee JH, Fasano MB, Graham SM. Sinonasal outcomes after endoscopic sinus surgery in asthmatic patients with nasal polyps: a difference between aspirin-tolerant and aspirin-induced asthma? Laryngoscope 2008; 118:1282–1286.
1068-9508 ß 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins
www.co-otolaryngology.com
33
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
