Bilateral maxillary mucoceles: an unusual presentation of cystic fibrosis A QUREISHI, P LENNOX, I BOTTRILL Department of Otolaryngology and Head and Neck Surgery, John Radcliffe Hospital, Oxford, UK Abstract Objective: We report a rare case of bilateral maxillary mucoceles. This was the primary presentation in a child with an underlying diagnosis of cystic fibrosis. Case report: A five-month-old infant presented with isolated symptoms of nasal congestion. Initial examination and investigation with computed tomography identified the cause to be bilateral maxillary mucoceles. The child was investigated for cystic fibrosis and found to be positive. Given the young age and anatomy, a modified surgical approach was used to treat the mucoceles, comprising endoscopic and bilateral uncapping of the maxillary mucocele, uncinectomy, and middle meatal antrostomy. The child was then followed up with appropriate treatment for cystic fibrosis. At six-week follow up, there was no recurrence of symptoms. Conclusion: Maxillary mucoceles are extremely rare but can present in patients with chronic sinusitis or cystic fibrosis. To our knowledge, there are no prior reports of this condition being the primary indicator of underlying cystic fibrosis in the absence of upper respiratory tract symptoms. Doctors should be aware that mucoceles can present in very young individuals and may indicate an underlying diagnosis of cystic fibrosis. A high index of clinical suspicion will avoid delays in diagnosis and long-term management. Key words: Cystic Fibrosis; Child; Maxillary Sinus; Mucoceles Introduction Cystic fibrosis (CF) is one of the UK’s commonest inherited life-threatening diseases, affecting over 8500 individuals. 1 The disease is an autosomal recessive genetic disorder result- ing in exocrine gland dysfunction with various clinical manifestations. Although targeted newborn screening is usually effective in identifying those with the condition, children are also often diagnosed following the development of respiratory or digestive tract symptoms. Sinonasal symptoms are almost universal, resulting in chronic sinusitis or polyposis. 2 However, these symptoms themselves are rarely the disorder that results in a diagnosis of CF. We present here the case of a five-month-old child diag- nosed with CF following an initial presentation with bilateral maxillary mucoceles. Case report A five-month-old baby girl was referred to our ENT depart- ment with symptoms of nasal congestion. The symptoms appeared following her first vaccination and improved, but then deteriorated again 10 days following her second routine vaccination. Despite being constantly ‘snuffly’ the child was reasonably well, feeding and gaining weight nor- mally, with no morphological abnormalities. She had recently recovered from an ear infection, and examination of the ears was normal. Flexible nasendoscopy showed her nasal airway to be patent bilaterally. However, both middle turbinates were grossly enlarged, occluding the middle portion of the nasal cavity and touching the septum. A computed tomography (CT) scan was arranged to assess the extent of the problem. The scan (Figure 1) demonstrated bilateral maxillary mucoceles. Considering the rarity of this diagnosis, there was a clinical suspicion of CF, and the child was referred for a sweat conductivity test. This confirmed the diagnosis of CF. Of interest, a paediatric review confirmed that the child was doing well otherwise, and there were no other symptoms or family history. The child weighed 8.88 kg, which was between the 75th and 91st percentiles for her age. The child was treated for her maxillary mucoceles by endoscopic bilateral uncapping of the maxillary mucocele, uncinectomy and middle meatal antrostomy. In the anaesthetic room, the nasal mucosa was pre- operatively prepared with 1:30 000 topical adrenaline on neurosurgical patties. During surgery, a 0° view, 2.7 mm diameter paediatric endoscope with a camera system was used to visualise the surgical field. Bilaterally, the mucoceles were seen to bulge through both anterior and posterior fontanelles, obscuring the view of the uncinate process. An endoscopic uncinectomy was impractical given limited space. An anterior inferior incision was made through the anterior fontanelle to uncap the mucocele, using a ‘lancet’ blade myringotomy knife (Figure 2). Mucus was drained from the mucocele, con- firming the diagnosis and origin. Suction was used to clear the field, and a paediatric sinus back-biting rongeur was used to perform the uncinectomy and antrostomy. Accepted for publication 3 August 2011 First published online 12 January 2012 The Journal of Laryngology & Otology (2012), 126, 319–321. CLINICAL RECORD © JLO (1984) Limited, 2012 doi:10.1017/S002221511100329X