Overview
Definition:
Chronic rhinosinusitis with polyps (CRSwNP) is a complex inflammatory condition of the nasal and paranasal sinuses characterized by the presence of nasal polyps, leading to prolonged sinonasal inflammation and airflow obstruction
In pediatrics, it often requires a thorough workup to identify underlying conditions like Cystic Fibrosis (CF) and the Aspirin Triad (asthma, nasal polyps, and aspirin sensitivity).
Epidemiology:
CRSwNP is less common in children than adults, with prevalence estimates varying
Pediatric CRSwNP is often associated with underlying conditions
Cystic Fibrosis is a significant underlying cause, affecting a substantial proportion of CF patients
Aspirin-exacerbated respiratory disease (AERD) is rare in young children but should be considered in older children and adolescents.
Clinical Significance:
Accurate diagnosis and management of CRSwNP in children are crucial for improving quality of life, preventing complications such as asthma exacerbations and vision loss, and addressing potentially life-threatening underlying conditions like CF
Early identification of the Aspirin Triad allows for appropriate avoidance strategies and medical management.
Clinical Presentation
Symptoms:
Nasal obstruction or blockage
Chronic nasal discharge (anterior or posterior)
Reduced or lost sense of smell
Facial pain or pressure
Postnasal drip
Cough, especially nocturnal
In children, may also present with snoring, mouth breathing, and irritability.
Signs:
Visible nasal polyps on anterior rhinoscopy or nasal endoscopy
Pale, boggy nasal mucosa
Purulent or mucoid nasal discharge
Signs of asthma (wheezing, decreased breath sounds)
Facial tenderness on palpation.
Diagnostic Criteria:
The European Position Paper on Rhinosinusitis and Nasal Polyps (EPOS) 2020 guidelines define adult CRSwNP as: persistent sinonasal symptoms (nasal blockage, discharge, facial pain/pressure, reduced sense of smell) for >12 weeks, AND objective evidence of inflammation (endoscopic signs: polyps, mucopurulent discharge
OR CT scan: mucosal changes in osteomeatal complex or sinuses)
Pediatric criteria are adapted, often requiring objective confirmation due to symptom variability in children.
Diagnostic Approach
History Taking:
Detailed symptom history including duration, severity, and impact on daily activities
Past medical history: atopy, asthma, allergies, previous sinonasal surgery, immune deficiencies
Family history: CF, allergic diseases, autoimmune conditions
Medication history: especially aspirin or NSAID use, and response or lack thereof
History of recurrent respiratory infections.
Physical Examination:
Complete Ear, Nose, and Throat (ENT) examination
Nasal endoscopy is key to visualize polyps, assess their extent, and identify associated inflammation or purulence
Oropharyngeal examination for postnasal drip
Auscultation of the lungs for wheezing or signs of asthma
General examination for signs suggestive of CF (e.g., failure to thrive, clubbing).
Investigations:
Nasal endoscopy: primary tool for visualization and biopsy if needed
Allergy testing (skin prick tests or serum IgE): to identify comorbid allergic rhinitis
Sweat chloride test: essential for ruling out Cystic Fibrosis, with values > 60 mEq/L highly suggestive
Blood tests: CBC (eosinophilia in AERD/CRSwNP), IgA, IgG, IgM (for immunodeficiency)
Imaging: CT scan of paranasal sinuses (coronal view) to assess extent of disease, bony involvement, and rule out other pathologies
typically reserved for refractory cases or surgical planning in children
Spirometry: to assess lung function and diagnose asthma in older children, especially if AERD is suspected.
Differential Diagnosis:
Allergic rhinitis, non-allergic rhinitis with eosinophilic mucin (NARES), pediatric fungal rhinosinusitis, sinonasal tumors (benign or malignant), juvenile nasopharyngeal angiofibroma (in adolescents), foreign body, primary ciliary dyskinesia, adenoid hypertrophy, dental infections.
Management
Initial Management:
Medical management is the mainstay
Aim is to reduce inflammation and polyp size
Nasal saline irrigation: to clear secretions and improve mucociliary clearance
Intranasal corticosteroids: first-line therapy, delivered via sprays or rinses, for at least 3 months
Dose and frequency should be age-appropriate.
Medical Management:
Oral corticosteroids: Short courses may be used for severe symptoms or significant polyp burden to induce remission, with careful monitoring for side effects
Antibiotics: Reserved for acute exacerbations of bacterial sinusitis
typically broad-spectrum for 10-14 days based on culture and sensitivity if available
Antihistamines: May help if comorbid allergic rhinitis is present
Aspirin desensitization: Considered for confirmed AERD in select cases after initial stabilization, performed under strict medical supervision
Management of CF: Standard CF therapies including airway clearance, mucolytics, and antibiotics for pulmonary exacerbations
Management of Asthma: Proper bronchodilator and inhaled corticosteroid therapy.
Surgical Management:
Surgical intervention (Functional Endoscopic Sinus Surgery - FESS) is indicated for refractory cases unresponsive to maximal medical therapy, significant nasal obstruction, or complications
Pre-operative workup is crucial, especially for CF and Aspirin Triad
Surgery aims to enlarge sinus ostia, remove polyps, and improve aeration
Extensive pre-operative assessment and post-operative care are essential
Multidisciplinary approach with ENT, Pulmonology, and Allergy/Immunology is vital.
Supportive Care:
Patient and parent education on disease process, medication adherence, and nasal hygiene
Regular follow-up to monitor symptom control, polyp recurrence, and response to therapy
Nutritional support for children with CF
Prompt management of comorbidities like asthma and allergies.
Complications
Early Complications:
Bleeding post-operatively
Infection (sinusitis, orbital cellulitis)
Synechiae formation
Anosmia or hyposmia persistence.
Late Complications:
Recurrence of polyps
Asthma exacerbations
Development or worsening of Aspirin sensitivity
Orbital complications (proptosis, vision loss)
Intracranial complications (rare, e.g., meningitis, abscess).
Prevention Strategies:
Optimal medical management with sustained intranasal corticosteroid use
Regular nasal saline irrigation
Strict avoidance of NSAIDs and aspirin in patients with Aspirin Triad
Prompt recognition and management of comorbidities
Careful surgical technique and meticulous post-operative care.
Prognosis
Factors Affecting Prognosis:
Presence and severity of underlying conditions like CF and asthma
Adherence to medical therapy
Extent and aggressiveness of surgical intervention
Early diagnosis and initiation of appropriate management.
Outcomes:
With appropriate management, symptom control and improved quality of life can be achieved in most pediatric patients
However, CRSwNP, especially when associated with CF or AERD, can be a chronic and relapsing condition
Long-term surveillance for polyp recurrence and management of comorbidities is essential.
Follow Up:
Regular follow-up appointments with ENT, and potentially pulmonology and allergy specialists, are crucial
Frequency of follow-up depends on disease severity and response to treatment, ranging from every few months to annually
Monitoring for signs of recurrence, new onset symptoms, or complications of treatment.
Key Points
Exam Focus:
Always consider Cystic Fibrosis and the Aspirin Triad (asthma, nasal polyps, aspirin sensitivity) in pediatric CRSwNP
Sweat chloride test is paramount for CF
Nasal endoscopy is essential for diagnosis
Intranasal corticosteroids are first-line medical therapy
FESS is for refractory cases.
Clinical Pearls:
In pediatric CRSwNP, a high index of suspicion for underlying systemic disease is warranted
Differentiate between allergic and eosinophilic causes
Aspirin desensitization in AERD is a specialized procedure
Remember that nasal polyps in children can be a sign of serious underlying pathology.
Common Mistakes:
Underestimating the severity of CRSwNP in children
Failing to perform a sweat chloride test in suspected cases
Relying solely on CT scan without nasal endoscopy
Inadequate duration or incorrect use of intranasal corticosteroids
Not considering AERD in asthmatic children with nasal polyps and recurrent sinusitis.