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A 2½12 year old girl with cystic fibrosis (CF) was seen with painless exophthalmos of the left eye with increased tearing and redness. She had severe pulmonary exacerbations and had been colonised by Pseudomonas aeruginosa since the age of 4 months. She had no fever, but an obstructed nose with purulent secretions. There was mild left exophthalmos. The periorbital skin was normal, without swelling. The mobility and reaction to light was intact and the child was able to fix with both eyes and to close the eye correctly. Fundoscopy was normal. A computed tomography (CT) scan was performed (figs 1 and 2). There appeared to be a tumour in the left orbit with erosion of the medial lamina, dislocating the bulbus anterolaterally.
Surgical intervention was performed and intraoperatively a fibrosing mucocoele was found and resected, together with the anterior and middle part of the ethmoidal cells. Histopathological analysis revealed enlarged goblet cells, accumulation of lymphocytes, plasma cells, fibrinous material, and blood. Microbiological analysis showed P aeruginosa. Diagnosis of a P aeruginosa containing mucocoele of the left ethmoidal sinus with subsequent exophthalmos of the left eye was made. Her exophthalmos disappeared immediately and she recovered within 10 days.
Chronic sinusitis and nasal polyps are well known in CF, but reports about mucocoele are sparse. Sharma et al reported three patients, in whom the mucocoele had dramatically infiltrated the wall of the frontal sinus.1 Robertson and Henderson reported a 16 month old child with proptosis of the right eye, in which the correct diagnosis was initially excluded because mucocoele was not believed to occur in infancy.2 Thome et al reported a child of 10 months with CF, having bilateral ethmoidal mucocoele.3 Information about long term outcome is provided by Alvarez et al, who found no recurrence after 18 months.4
The traditional treatment for paranasal mucocoeles in children is to perform surgical drainage via an external incision. In adults endonasal surgical techniques are increasingly being used. Hartley and Lund report a series of seven pediatric patients without CF, successfully treated with endoscopic intranasal surgical drainage.5 In our case the surgeons judged the external approach to be the safest. The orifice of the ethmoidal sinus into the nasal cavity was enlarged to prevent any recurrence of the mucocoele. Our patient had suffered from abundant, purulent, pulmonary and nasal secretions since infancy. Excessive amounts of mucus might not be cleared from the nasal cavity and paranasal sinuses in infants and young children unable to blow their nose.