Pediatric Chronic Rhinosinusitis

Definition

Chronic rhinosinusitis (CRS) in children is defined as an inflammation of the nose and the paranasal sinuses characterized by two or more symptoms ( Box 21.1 ). The diagnosis also requires objective signs of disease documented by endoscopy and/or computed tomography (CT) scan. In general, CRS refers to symptoms that last 12 weeks or longer without symptom-free periods.

Clinical Symptoms

The four most common clinical symptoms are cough, rhinorrhea, nasal congestion, and postnasal drip, with a slightly higher predominance of chronic cough. ^, Tatli and colleagues found that 66% of children who undergo evaluation for chronic cough had CT scan abnormalities of the paranasal sinuses. The clinical diagnosis of CRS in children is challenging, related to the overlap of symptoms with other common childhood nasal diseases such as viral upper respiratory tract infections, adenoid hypertrophy/adenoiditis, and allergic rhinitis. Furthermore, the history is limited to the subjective evaluation by the child’s parents, and some younger children may not tolerate nasal endoscopy. It is often very difficult to differentiate CRS from adenoid hypertrophy/adenoiditis in young children. A thorough history of the timing of symptoms is critical to attempt to define the category of disease that best applies to each patient. A very common clinical scenario in children who present to the office is that of CRS with upper respiratory tract infection–induced acute exacerbations.

Quality of Life

In a study of children with chronic and recurrent rhinosinusitis who failed medical treatment and required surgical intervention, Cunningham and colleagues showed significant impairment of generic quality-of-life (QOL) measures. In these children, QOL scores were significantly lower than those of children with other common chronic diseases such as asthma, attention-deficit/hyperactivity disorder, juvenile rheumatoid arthritis, and epilepsy.

The SN-5 survey is a disease-specific tool completed by parents to reflect the previous 4 weeks. It consists of five domains that include sinus infection, nasal obstruction, allergy symptoms, medication use, emotional distress, and activity limitations. The survey correlates with CT scan scores in patients with CRS and was validated as a measure of change over time in sinonasal symptoms. ^, However, limited evidence of improvement of SN-5 scores was noted in patients with CRS after surgical intervention.

Genetic Factors

Orb and colleagues explored the familial risk of CRS in children by mining the Utah population database and identifying 496 children (12 years or younger) with the diagnosis of CRS, and 4959 ethnically matched controls ( Box 21.2 ). Siblings of patients with CRS demonstrated a 57.5-fold increased risk of having pediatric CRS compared with controls. First cousins had a 9.0-fold increased risk and second cousins a 2.9-fold increased risk of pediatric CRS, all significant associations. Parents, first- and second-degree relatives, and first cousins of pediatric cases demonstrated significant increased risks of having adult CRS. This suggests a significant familial risk associated with CRS. Purkey and colleagues hypothesized that potassium channel genes are associated with CRS, genotyping single nucleotide polymorphisms in 828 children with CRS and 5083 healthy controls from the Children’s Hospital of Philadelphia. A variant in the KCNMA1 gene was found to be significantly associated with CRS in white children. There was borderline evidence for association in a variant in the KCNQ5 gene with CRS in African American children. In an earlier study in 58 white children with CRS and no known cystic fibrosis (CF), 12.1% were found to carry heterozygous mutations in the CF transmembrane regulator (CFTR) gene, higher than the expected rate of 3% to 4% in the same ethnic group. These results suggest a predisposition to CRS in children who are carriers for CFTR mutations.