THE ROLE OF ENDOSCOPIC SINUS SURGERY IN PEDIATRIC PATIENTS WITH CYSTIC FIBROSIS

Cystic fibrosis (CF) is the most common life threatening autosomal recessive disorder affecting Caucasians. Molecular genetic mutations in the CF transmembrane conductance regulator (CFTR) gene alter function of CFTR protein, which leads to dysfunction in chloride channel on epithelial surface of exocrine glands. In the head and neck area, the pathology involves mostly paranasal sinuses starting from childhood. Chronic and recurrent rhinosinusitis with or without nasal polyposis and mucocele of sinuses are the main disorders observed in CF patients. About 20-25% of these patients will require sinus surgery. Regarding surgical treatment, endoscopic sinus surgery (ESS) for management of paranasal sinus diseases in CF patients is undoubtedly a challenge for the surgeon. Moreover, evidence-based guidelines regarding the precise indications for surgery are currently lacking. Even if ESS is undeniably needed for management of sinonasal complications and can improve CF-related nasal symptoms, its role with regards to timing of surgery and consequent improvement of pulmonary function is not well described. Moreover, underlying medical issues such as acquired coagulopathies and advanced pulmonary disease, raise perioperative morbidity in these patients, with consequent and prolonged hospitalization. Indeed, the surgeon is often faced with distorted anatomy, due to anomalous development of nasal and paranasal structures or multiple revision operations. Therefore, multidisciplinary management of paranasal sinus disease in patients affected by CF is pivotal to achieve the correct surgical indication, prepare the patient for surgery, and address possible perioperative complications. The authors describe the indications of ESS in CF patients, analyzing personal experience in the diagnosis and treatment of CF-related sinus disorders. Finally, data on follow-up after ESS, pulmonary function, and nutritional status will be presented.