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Current Opinion in Otolaryngology & Head & Neck Surgery:
doi: 10.1097/MOO.0000000000000018
NOSE AND PARANASAL SINUSES: Edited by Samuel S. Becker and Nithin D. Adappa

Pediatric chronic rhinosinusitis: when should we operate?

Rizzi, Mark D.a,b; Kazahaya, Kena,b

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Author Information

aDivision of Pediatric Otolaryngology, Children's Hospital of Philadelphia

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: rizzim@E-Mail.chop.edu

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Abstract

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.

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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.

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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 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.

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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▪▪].

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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 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▪].

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Medical treatment

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 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.

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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.

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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 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.

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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▪].

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SURGICAL MANAGEMENT IN SPECIFIC POPULATIONS

CF, AFRS, aspirin exacerbated respiratory disease (AERD) and PCD are disease processes which are 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 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].

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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.

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Acknowledgements

None.

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Conflicts of interest

The authors have no conflicts of interest to report relating to this article.

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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

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Keywords

chronic rhinosinusitis; functional endoscopic sinus surgery; pediatric

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