Chronic Rhinosinusitis in Children Clinical Presentation Hector Stone-Aguilar, M.D. Pediatric Allergy & Immunology Hospital San Jose de Hermosillo Universidad del Valle de Mexico

Clinical presentation of CRS in Children The problem: To fully define chronic sinusitis has been difficult There is a wide variation in clinical expression of the disease Discordance between patient symptoms and objective findings No one set of diagnostic criteria has been agreed on by all specialty groups

Clinical presentation of CRS in Children The problem: Clinical criteria to diagnose CRS, as well as the predictive value of these criteria, are not well defined, specially in children Historically, the diagnosis of CRS was based on several clinical symptoms, similar to acute RS, but usually less severe However, none of these symptoms are specific to sinusitis

Definition of Sinusitis Inflammation of 1 or more of the paranasal sinuses Acute Sinusitis: less than 4 weeks/duration Subacute Sinusitis: 4 to 12 weeks/duration Chronic Sinusitis: longer than 12 weeks Some guidelines also requiring : Failure to respond to treatment One positive imaging study Dykewicz M, JACI Feb 03

Definition of Rhinosinusitis Inflammation of the nose and paranasal sinuses characterized by two or more symptoms, one of which should be either nasal blockage/obstruction/congestion or nasal discharge (anterior/posterior nasal drip) ± facial pain/pressure ± reduction or lost of smell EPOS Guidelines, Rhinology 2007

Rhinosinusitis OHNS , 1997

Definition of Chronic Rhinosinusitis More than 12 weeks of symptoms without complete resolution Can be subdivided in: Chronic Rhinosinusitis with Nasal Polyps Chronic Rhinosinusitis without Nasal Polyps CRS also may be susceptible to exacerbations EPOS Guidelines, Rhinology 2007

CRS: Symptom-based Diagnosis 73.15% of the nonallergic patients with symptom based diagnosed CRS 65.34% of the allergic patients with symptom-based diagnosed CRS Had No CT and endoscopic pathology (Endoscopic score 0 + CT score 0) Tahamiler R, Allergy 2007

Chronic Rhinosinusitis in Children In general : The main symptoms associated with rhinosinusitis in children are rhinorrhea, nasal obstruction, mouth breathing, hyponasal speech, and snoring but…

Diagnosing CRS in Children: Special issues Infants and Pre-school children Signs/symptoms difficult to evaluate: Congestion (very subjective/indirect/parent’s biass) Only anterior rhinorrhea is reported Symptoms impossible to evaluate: Posterior discharge Sense of smell Headache / toothache / facial pain Symptoms very unspecific : Cough, irritability, fever, fatigue/decreased activity, etc.

Diagnosing CRS in Children: Special issues Infants and Pre-school children Anterior Rhinoscopy : Limited data Anterior third of nasal cavity Osteomeatal zone difficult to reach, even w/use of topical decongestant Nasal Endoscopy: Ideal but impossible to perform without sedation or anesthesia CT scan: Also requieres sedation or anesthesia Sedation/anesthesia: increases costs and risks Increased value of plain X-Rays at this age ??

Severity of Sinusitis Disease severity can be divided into: Mild (0-3 points) Moderate (4-7 points) Severe (8-10 points) Using a 10-point scoring system or Visual Analogue Scale (VAS) EPOS Guidelines, Rhinology 2007

Clinical presentation of CRS in Children Diagnosis must be based in a combination of: Clinical symptoms and evolution Age-group related Previous treatments (type and duration) Likelihood of allergy involvement: Family history, allergy stigmata, personal history of other allergic diseases (AD or Asthma) Clinical Signs Anterior rhinoscopy and/or Nasal endoscopy Imaging support Plain X-Rays CT scans MRI

Chronic Rhinosinusitis in Children By definition, needs to be at least 12 weeks old (3 m.o.) Ethmoid and maxillary sinuses present at birth Clinical presentation strongly related to the specific pediatric age group: Infants: Persistent or recurrent rhinorrhea after an acute febrile URIs ( ± AOM, Rhinopharyngitis, Bronchitis) Pre-schoolars: Persistent rhinorrhea and nasal congestion w/adenoid and tonsil hypertrophy, serous OM, allergies and asthma. Scholars and adolescents : Nasal obstruction, headaches, sore throath, hyposmia, irritability, sleep disturbances, etc. (PAR or PNAR)

Clinical presentation of CRS in Children In infants and preschool childrens, most cases of CRS are a chronologic extension of acute infectious sinusitis (viral bacterial) In contrast, in older children or adolescents most CRS cases are not an infectious disease but an inflammatory disease, much akin to asthma. Jones NS, Curr Opinion Pulm Med, 2000

Clinical evolution of Viral URI’s

When to suspect CRS in INFANTS Continuous or intermittent RHINORRHEA Anterior, posterior or both Usually clear initially (days or weeks) Colored (greenish or yellowish) more dense secretions It can alternate clear and colored secretions Nasal CONGESTION Mild at the beginning Worsening in an intermittent pattern in absence of appropriate treatment Not as bad as other age groups Objective findings: mouth breathing, snoring

When to suspect CRS in INFANTS COUGH : A prominent feature of sinusitis at this age Starts as “Dry” cough usually for several days Can continue with “wet” cough all the way Intermittent along the day, not very intense Can start or worse at night or bedtime Usually associated with posterior rhinorrhea Also associated with coarse and audible ronchi Maybe a better predictor than rhinorrhea about the outcome

When to suspect CRS in INFANTS FEVER: Usually present at the beginning of the clinical picture Low or mid grade Fades away after few days (with or without treatment) Can not be present at all Can relapse in the course of the disease (worsening) Its absence doesn’t rule out the possibility of chronic infection

When to suspect CRS in INFANTS Other possible symptoms: Irritability Bad appetite Sleep disturbances: Trouble to got sleep Restless sleeping Nocturnal awakenings Halitosis Reduced general activity

When to suspect CRS in INFANTS Physical signs, NASAL : Rhinorrhea (anterior) Pale and enlarged turbinates Mucosal edema Hyperemic mucosa Middle meatus colored discharge

Rhinoscopy

Muco-purulent discharge in the Sinus Ostium zone Middle turbinate Lateral nasal wall Purulent mucus Septum

When to suspect CRS in INFANTS Physical signs, GENERAL : Posterior rhinorrhea Mouth breathing Pallor Dark infra-orbital shiners Halitosis Tympanic opacity, retraction or hyperemia Enlarged tonsils Granular (cobblestone) adenoid tissue in the pharynx “rude” breathing Coarse rhonchi on chest examination

Serous Otitis Media

Enlarged Adenoids: Cause or consequence ?

Chronic Rhinosinusitis in PRE-SCHOLARS Not necessarily associated to respiratory infection Mostly related to allergies and asthma Difficult to distinguish from PAR. Same sort of signs and symptoms Usually considered a “complication” of allergic rhinitis Nasal or sinusal polyps not frequent at this age

Chronic Rhinosinusitis in PRE-SCHOLARS Differences with CRS in Infants Congestion more prominent than rhinorrhea Cough frequently related to asthma or BHR Headaches, frequently mild or intermittent Hyposmia rarely reported Halitosis Clear or thick mucoid rhinorrhea Paler and more enlarged turbinates Intense edema of nasal mucosa

Chronic Rhinosinusitis in School children and adolescents Moderate to severe nasal congestion/obstruction: Snoring Sleeping problems Dry mouth and sore throat at mornings Headaches: Mild to severe Frequent or intermittent Frontal, maxillary or occipital Rhinorrhea: Posterior > anterior Halitosis

Chronic Rhinosinusitis in School children and adolescents Daytime somnolence Tiredness Poor concentration: altered school performance Hyposmia Dysgeusia Middle ear: Hypoacusia, Popping, Buzzing Polyps: More frequent than the other pediatric groups

Consequences of chronic nasal congestion Snoring Oral breathing Hyponasal speech Sleep disturbances Obstructive Sleep Apneas (OSA) Dry mouth Sore throath Headaches Daytime somnolence Poor concentration Tiredness Facial and dental changes

CRS Diagnosis: Plain X Rays: Useful?

Plain X-rays vs. CT scan in Sinusitis  The sensitivity of Plain X-Ray compared to CT was: 77% (30/39) The specificity of the radiograph to CT was 81% (25/31).  The positive likelihood ratio is 4.05 and The negative likelihood ratio is 0.28. Conclusions - The difference between radiographs and CT for diagnosing sinus disease in this population is relatively small but favors CT exam. Garcia, DP Radiographic imaging studies in pediatric chronic sinusitis  J Allergy Clin Immunol, 94:523-530, 1994.

CRS Diagnosis: CT scan: Gold standard ?

‘Limited’ CT Scan Garcia D, JACI sept 1994

Sinusitis severity Index (grading): (Glicklich) Grade 0: mucosal thickening of ≤ 2 mm in any sinusal wall Grade 1: Any unilateral disease or abnormality Grade 2: Bilateral disease limited to ethmoid or maxillary sinuses Grade 3: Bilateral disease with frontal or sphenoidal involvement (any) Grade 4: Pansinusitis. Emmanuel IA, Otolaryngology Head Neck Surg 2000

CRS Diagnosis: CT scan: Gold standard ? HWANG et al, OHNS april, 2003

CRS Diagnosis: CT scan: Gold standard ? Unilateral involvement of the right maxillary sinus and structural abnormalities: MT concha bullosa and paradoxical curvature of middle turbinate, stretching the OMC

Nasal Endoscopy

Clasification of the severity of polyposis by endoscopy 0 - No visible polyps 1 - Polyps confined to the middle meatus 2 - Polyps beyond middle meatus but did not occlude the nasal cavity 3 - Polyps obstructing completely the nasal cavity Mackay IS y Lund VJ, 1997

Nasal / Sinusal Polyposis in Children If nasal polyps are present in young children, MUST rule out: Aspirin Exacerbated Respiratory Disease (AERD) Cystic Fibrosis (CF) Genetic involvement But still most probably related to Perennial or Persistent Allergic Rhinitis Polyps related to Perennial Non-Allergic Rhinitis are rare at this age

Etiology of CRS in Children Infection: Viral/Bacterial Biofilms Fungal? Allergy Allergic Rhinitis: Persistent > Intermittent Gastroesophageal Reflux Obstruction /Structural Adenoid > Tonsils Hypertrophy Septal deviation Other: concha bullosa, Haller cells, agger nasi cells

Etiology of CRS in Children Immunodeficiency IgA deficiency Transient Hipogammaglobulinemia IgG sub-class deficiency ( IgG2 + IgG4) Selective (polysaccaride) IgG deficiencies CVI Cystic Fibrosis Ciliary Dyskinesia Aspirin Exacerbated Respiratory Disease Other: very uncommon

Hamilos D, JACI oct 2011

Conclusions: CRS is frequent in children No one set of diagnostic criteria has been agreed on by all specialty groups CRS in children have special features that are different of CRS in adult population There are differences also in the clinical presentation of the different pediatric age groups The diagnosis of CRS in children is based almost exclusively in clinical data. Use CT or endoscopy in selected cases. There are very few controlled clinical studies of CRS in children. All Guidelines based in adult studies and transpolated to children. The most common causes are bacterial infections and/or allergies. Other causes are really not frecuent or rare, but still have to rule out them if not responsive