Ear, nose, and throat disorders in Cystic Fibrosis

The Leeds Method of Management. April, 2008. Ear, nose and throat disorders in Cystic Fibrosis [online]. Leeds Regional Adult and Paediatric Cystic Fibrosis Units, St James's University Hospital, Leeds, UK. Available from http://www.cysticfibrosismedicine.com

Introduction

Patients with CF often suffer from ear, nose, and throat (ENT) disorders particularly those affecting the nose and sinuses that are parts of the breathing (respiratory) system. The lining of the nose is shared with the sinuses. This lining is a respiratory mucosa and is affected by the same basic defect present in CF that affect the lungs.

The nasal lining is often swollen. This is known as rhinitis. The rhinitis in CF is slightly different to that found in asthma and allergy. In CF the lining is often constantly swollen and contains many neutrophils (white cells) rather than eosinophils (different white cells). There is also thicker mucus within the nose and this is associated with local infection. The thick mucus may fill the sinuses and colonisation of the nose with bacteria such as P. aeruginosa and S. aureus lead to constant infection. This is known as chronic rhinosinusitis.

Chronic rhinosinusitis is characterised by constant green and snotty nasal discharge either anteriorly from the front of the nose or posteriorly into the throat (catarrh or post nasal drip). There is often a sensation of fullness or heaviness within the face but not usually any severe pain.

Rhinosinusitis is often associated with the development of nasal polyps (nasal polyposis). Nasal polyps are bags of swollen nasal lining that develop from the rhinitis and start to hang down within the nose. Polyps develop mostly at the top of the nose in and around the ethmoid sinuses. Polyps add to nasal blockage and discomfort. They start small but may grow to the size of jelly babies. Occasionally they can project out of the front of the nose or hang backwards into the pharynx or the back of the mouth. Polyps block the nose and slow the drainage of the sinuses and the thickened mucus adding to infected nasal discharge.

Rhinitis is not curable but may be helped by the regular use of nasal steroid sprays (Flixonase® or similar), washing through the nose with salty water (saline douching) and the intermittent use of antibiotics.

Regular douching through the nose can be very helpful in controlling infected nasal discharge and sometimes sinus washouts are required to clean out a reservoir of infection from the sinuses. This small operation can be performed with local anaesthetic or under a brief general anaesthetic.

Troublesome chronic rhinosinusitis associated with complications or nasal polyps can be treated with keyhole surgery through the nostrils using small cameras and fine instruments. This surgery is known as FESS or Functional Endoscopic Sinus Surgery and allows the blocked sinuses to be opened and polyps to be removed. This reduces blockage and may improve sinus drainage but is not a cure for the underlying CF rhinitis. Despite careful removal it is typical for polyps to recur.

Despite the frequent nasal and sinus infections and thickened secretions in CF patients, the frequency of ear problems such as glue ear may be no more than normal. This is surprising and might be due to frequent antibiotic use.

 

Key points

• Problems with the nose and sinuses are common in CF

• Rhinosinusitis may result in nasal polyps

• Rhinitis may be relieved by nasal steroid sprays, washing with salty water, and antibiotics

 

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