Embryological anomalies of the first branchial cleft are uncommonly encountered. They usually present as cysts, swellings, or fistulas in the pre-auricular or post-auricular area or high in the neck, which may become infected.
Failure to recognise these unusual cases may result in misdiagnosis, inadequate treatment, and subsequent recurrence. Further definitive surgery may thus be complicated. A case is reported of a patient who attended accident and emergency on three occasions with an infected post-auricular cyst, which was treated by incision and drainage. It was subsequently found to be a first branchial cleft anomaly.
- branchial region
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A 15 year old boy presented with a two year history of a recurrently infected cyst behind his right ear. He had initially presented to the accident and emergency (A&E) department with an infected swelling behind his right ear. This was treated in the department with incision and drainage under local anaesthetic. It recurred six months later and another presentation to the A&E department resulted in further incision and drainage under local anaesthetic. This lesion recurred again three months later and was treated in the A&E department with excision under local anaesthetic. The lesion had become infected on two further occasions but had settled with a course of antibiotics from the general practitioner.
On presentation to the ear, nose, and throat clinic, there was a cystic swelling in the right post-auricular region inferiorly, with evidence of some scarring (fig 1). Examination of the external auditory meatus revealed the presence of a pit on the postero-inferior wall. The remainder of the ENT examination including examination of the neck was normal. A provisional diagnosis of a first branchial cleft anomaly was made. Computed tomography with contrast was performed, which showed the extent of the lesion with an associated tract running along the posterior wall of the external auditory meatus.
The patient underwent an exploration of the lesion, which confirmed the presence of a sinus communicating between the pit seen on examination and the cyst, thus confirming the diagnosis. Histological examination revealed the tract to be lined with stratified squamous epithelium thus establishing the ectodermal nature of the lesion.
First branchial cleft anomalies are uncommon and comprise 1%–8% of all branchial cleft anomalies.1 They often present in the first two decades of life and present a clinical challenge as they can easily be misdiagnosed and thus inappropriately treated. They are thought to arise as a result of developmental abnormalities of the branchial apparatus and may take the form of a cyst, sinus, or fistula.2
Clinically they may present with repeated episodes of infection of the lesion. This may manifest itself with a cystic swelling or discharge from a fistulous opening either pre-auricularly or post-auricularly, in the cheek, or high in the neck. A thorough otological examination should be performed in all cases and may reveal a pit visible in the external canal at the site of entrance of a sinus or fistula. Such a lesion may result in otorrhoea or otitis externa with infective exacerbations. The meatus may be found to be partially or completely obstructed by bulging of the canal wall because of a cystic swelling. Such patients may complain of hearing loss, as may those with oedema associated with an otitis externa. There may however be a complete absence of signs in the external auditory canal. The patient may also give a history of having to repeatedly undergo incision and drainage of an apparent abscess around the ear because of infective exacerbations of the lesion that has not resolved.
Early diagnosis and treatment are needed to avoid recurrent infection as scarring may cause difficulties with surgical dissection.3 Acute infective episodes should ideally be treated conservatively with antibiotics. However, this may not always be successful in settling the acute condition and referral for incision and drainage may still be required in some situations.
Computed tomography with contrast will help define the extent of the lesion and identify extension into the middle ear.
In conclusion, cysts, sinuses, or fistulous tracts, which are pre-auricular or post-auricular or high in the neck may represent a branchial cleft anomaly. A high index of suspicion is required and a thorough otological examination and careful history should be undertaken. If suspected the patient should be referred for a specialist otological opinion as attempted drainage may complicate further definitive surgery.