Presentation of first branchial cleft anomalies: the Sheffield experience.

Abnormalities of the first branchial cleft are rare. They may present with a cutaneous defect in the neck, parotid region, external auditory meatus or peri-auricular area, or with inflammatory or infective lesions at these sites. A retrospective case note review of the patients treated by the senior author is presented. This group consisted of 18 patients and represents the largest published UK series to date. Eleven patients (65 per cent) had undergone incomplete surgery prior to referral. Over half the patients had a clinically apparent lesion in relation to the external auditory meatus. There was a variable relationship between the tract and the facial nerve, which was identified at surgery in 15 cases. These findings are consistent with those of previously published series. Clinicians should keep this diagnosis in mind when assessing patients with infected lesions in the neck and parotid area. Surgeons should be familiar with parotid surgery, in children where appropriate, and be prepared to expose the facial nerve before embarking on the surgical management of these lesions.

Grommet insertion: a novel technique.

We describe a new technique for inserting grommets which is both easy to master and provides better visualization of the tympanic membrane. This is particularly helpful in patients with narrow ear canals.

Paediatric airway disorders.

Impairment of the airway in infants and children causes anxiety to the patient, the parents and the health-care staff responsible for the child. This article outlines and explains paediatric airway impairment and aims to improve understanding of the various diseases.

Fine needle aspiration cytology in the management of a parotid mass: a two centre retrospective study.

OBJECTIVES: There is a diversity of opinion regarding the role of fine needle aspiration cytology (FNAC) in the pre-operative evaluation of the parotid mass. This study further investigates the role of FNAC from the standpoint of the clinician attempting to resolve one or more clinical issues. METHODS: A retrospective study conducted at two UK Hospitals with no overlap of cytopathologists or surgeons. Patients undergoing parotidectomy at each institution were identified from Pathology department databases. The definitive histopathological diagnosis was compared with any pre-operative FNAC diagnosis. Cytology results were classified as suggestive, non-diagnostic, sampling error, or misleading. SETTING: The study was conducted in a District General Hospital and a University Teaching Hospital providing secondary care for each community. RESULTS: For the University Teaching Hospital the sensitivity in distinguishing malignant from benign disease was 79% (95% CI 61-97%) with a specificity 84% (95% CI 73-95%). However, three of eight patients with a primary parotid salivary gland malignancy were reported as having benign disease on FNAC. For the participating District General Hospital the sensitivity in distinguishing malignant from benign disease was 38% (95% CI 13-63%) and specificity 95% (95% CI 73-95%). CONCLUSIONS: Fine needle aspiration cytology does not reliably distinguish a benign from a malignant primary salivary gland neoplasm in the participating institutions. Where clinical teams use FNAC in an attempt to resolve this clinical problem, the results should be interpreted with caution and an ongoing audit of performance is required.

Blocked nose and snoring in a 6-year-old boy.

Nasal obstruction, mouth-breathing, snoring and deafness are common symptoms seen in children presenting to the otorhinolaryngology clinic. The aetiopathologies are frequently due to adenotonsillar hypertrophy and otitis media with effusion. Rarely however, nasal obstruction and snoring may be associated with more sinister pathology. We report a case of a child with nasopharyngeal B-cell Non-Hodgkin's lymphoma whose initial symptoms were of nasal obstruction and snoring.

Marginally excised parotid pleomorphic salivary adenomas: risk factors for recurrence and management. A 12.5-year mean follow-up study of histologically marginal excisions.

The aim of the study was to identify recurrence risk factors in surgically excised parotid pleomorphic salivary adenomas. We reviewed the case histories and histological findings for all cases of marginal or inadequate excision of pleomorphic salivary adenomas at the Royal Hallamshire Hospital, Sheffield, UK, between 1980 and 1995. A total of 83 cases with complete records were identified, with a mean follow-up period of 12.5 years. The histological slides were reviewed in each case. The overall recurrence rate was 6.0%. Where tumour was present at the margin, recurrence occurred in 17.6% of cases. However, cases conventionally regarded as marginally excised and likely to recur (tissue margin < 1 mm) showed recurrence in only 1.8% of cases. Intraoperative capsular rupture, microscopic capsular invasion by tumour and several other surgical factors were not predictive of recurrence. Adequate excision of pleomorphic salivary adenomas, in the sense of minimal recurrence risk, does not require more than a fraction of a millimetre of surrounding tissue. Only pleomorphic salivary adenomas with tumour actually at the excision margin require prolonged follow-up or consideration of radiotherapy. Provided that the tumour can be removed intact, the surgical approach for pleomorphic salivary adenomas should be guided by the need to preserve vital structures rather than by an attempt to remove a cuff of normal tissue with the tumour.

Hairy polyps of the nasopharynx.

Hairy polyps are rare tumours that can occur anywhere in the body. They are especially rare inthe pharynx. We report two cases of hairy polyps which originated from the nasopharynx. One presented with intermittent obstruction of the airway and the second presented as a visible pedunculated mass protruding from the mouth of a neonate.

Salivary gland stones: diagnosis and treatment.

Salivary calculi are a common cause of salivary gland disorder and may occur in any of the salivary glands and at almost any age. The stones may be small and intraductal or lie within the gland substance when they may become very large. They cause symptoms by obstructing salivary flow. Diagnosis is usually straightforward, and treatment is aimed at stone removal.

Management of non-tuberculous mycobacterial peri-sialadenitis in children: the Sheffield otolaryngology experience.

Non-tuberculous mycobacterial infection (NTM) has been recognized as an important cause of infection in the head and neck in children since 1956. It is important to differentiate NTM from the more serious Mycobacterium tuberculosis (TB) since the management differs significantly. The causative organisms of NTM infection are resistant to the most commonly used anti-tuberculous preparations, though they do show sensitivity to the newer antibiotics such as clarithromycin, ciprofloxacin and azithromycin. Between 1986 and 1997, 15 patients with NTM infection involving the major salivary glands were treated at the Sheffield Children's Hospital Department of Otolaryngology. There were 11 girls and four boys. In all patients the onset of symptoms was between September and April. Resolution occurred in two patients without surgery. The remaining 13 underwent formal surgical exploration with excision of the mass, associated nodes and of the overlying skin if necessary. There were nine parotid explorations. There were no long-term facial nerve deficits as a result of surgery and no recurrence of the disease. Co-operation between the Paediatrician and the Otolaryngologist is important for effective management since NTM may also affect the lungs, soft tissues, bones and joints. Diagnosis relies upon culture, histology, chest radiography, purified protein derivative (PPD) testing, clinical features and skin testing. The use of antibiotics such as azithromycin with ciprofloxacin may be justified while waiting for the results of investigations, especially with small, early lesions, as resolution is possible. In patients who have no history of exposure to TB, are not immunocompromised, have a normal chest radiograph and have a Mantoux test with < 15 mm diameter induration, the treatment should be surgical excision rather than prolonged antibiotic therapy.

Endoscopic division of the ary-epiglottic folds in severe laryngomalacia.

OBJECTIVE: Laryngomalacia is the commonest cause of congenital stridor. The underlying anatomical abnormality associated with this condition is a prolapse of the supraglottic tissues into the laryngeal inlet during inspiration, and may involve the epiglottis, ary-epiglottic folds and the corniculate mounds of the arytenoids. However, it has been noted that the most consistent structural abnormality seen in these cases is the shortening of the ary-epiglottic folds and marked side to side curling of the epiglottis. We describe the follow-up and outcome of 33 cases treated by the simple division of the ary-epiglottic folds. METHOD: All case notes were reviewed with respect to indications of operation, age of operation, endoscopic findings, operative technique, complications and follow-up until resolution of symptoms. RESULTS: Surgical outcomes could only be ascertained in only 32 patients. Twenty-two cases (68.7%) showed complete resolution of stridor and associated complications of laryngomalacia. In the remaining ten cases who could be followed up (31.2%), seven patients (21.8%) showed partial resolution with no further surgery required, two patients (6.2%) required additional excision of redundant mucosa as second procedure, one patient (3%) with associated cleft lip/palate and tracheomalacia had to undergo a tracheostomy. There was improvement in feeding after surgery in all the 12 patients (100%) who had had pre-operative feeding difficulties. All the four patients with cyanosis pre-operatively were cured, but one of the two patients with apnoeic episodes pre-operatively continued to have apnoeic spells despite resolution of their laryngomalacia. CONCLUSION: Simple endoscopic excision of the ary-epiglottic folds is a quick, reliable, highly effective procedure with very few complications. We recommend its use as the first line option in the management of severe laryngomalacia, with more extensive methods reserved for the very occasional case of primary failure.

Outcomes of submandibular duct re-implantation for sialorrhoea.

Drooling is an important factor affecting the quality of lives of neurologically impaired children, and surgery often has to be performed. Submandibular duct relocation is a relatively simple procedure with a low complication rate, and has been carried out on 37 patients at the Sheffield Children's Hospital between 1986 and 1998. To determine the short-term and long-term outcome of this procedure, a questionnaire was sent to the parents/wards of the patients. Feedback was also obtained on complications, parents'/carers' perceptions and main concerns about the operation, particularly in those cases which did not improve. Both short-term (82.4%) and long-term (76.5%) control rates were high, and there were few complications, none of which had any long-term adverse effects. The most significant area of parental/carer dissatisfaction and concern in 'failed' cases was 'pain', and it was felt that proper preoperative counselling about analgesia and chances of failure is very important. We conclude that this is a safe, highly successful procedure which significantly improves the quality of lives of the majority of drooling children.

Isolated juvenile xanthogranuloma of the subglottis: case report.

BACKGROUND: Juvenile xanthogranulomatosis (JXG) is a relatively rare macrophage proliferative disorder. It usually presents as a localized cutaneous lesion but may affect other organs. Until now it has never been described in the subglottic region of the larynx. METHODS: We report the first case of juvenile xanthogranulomatosis (JXG) in the subglottis in a 3 year old child. RESULTS: The localization in the subglottis caused airway obstruction requiring tracheostomy to secure the airway. On the basis that most cutaneous lesions regress spontaneously the lesion was managed expectantly and regressed over a period of 28 months allowing decannulation of the child. CONCLUSION: JXG should be considered in the differential diagnosis of subglottic lesions. Once the airway has been secured, JXG of the subglottis can be managed conservatively. Long-term follow-up is required because of the possibility of relapse at other sites.

Congenital facial nerve agenesis.

We present a case of a seven-year-old child with a congenital facial palsy, diagnosed at birth, who subsequently developed a non-tuberculous mycobacterial (NTM) infection of the ipsilateral parotid gland. This required parotid exploration to treat the NTM disease with the intention of identifying and protecting the facial nerve to preserve any residual facial nerve function. At operation, thorough exploration revealed the complete absence of the nerve both at the stylomastoid foramen and more peripherally within the substance of the parotid gland. Exploration of the facial nerve for congenital facial paralysis is not normally indicated. Surgical treatment, if required, tends to involve the use of techniques such as cross facial nerve and free vascularized muscle grafting. To our knowledge this is the first reported case of complete congenital facial nerve agenesis, diagnosed incidentally during a surgical procedure for an unrelated condition.

Salivary gland neoplasia in childhood.

Salivary gland tumours, benign or malignant, are rare in childhood and as a result may be misdiagnosed and treated inappropriately. Ten children with salivary gland tumours have been treated by the author over a period of 18 years. Four tumours were benign and six malignant. Treatment in all cases was by surgical excision. There has been one recurrence, treated by further surgery, in an acinic cell carcinoma. Three cases had inadequate surgery or biopsy before referral. These findings reflect other series, which demonstrate that a solid tumour in a salivary gland in childhood is more likely to be malignant than to be benign.

Paediatric subglottic stenosis.

Most reports on paediatric subglottic stenosis have arisen from tertiary referral centres. At the Sheffield Children's Hospital, referrals come from the surrounding region such that almost all paediatric airway problems from this area are seen. This allows an opportunity to look at the overall picture of this condition. A retrospective study was performed to identify all children with subglottic stenosis requiring a tracheostomy since 1979 and to examine the management and eventual outcome in relation to the severity of their stenosis. Forty-two children were identified. There have been four mortalities, two of which have been tube related. Twenty-two laryngotracheoplasty procedures have been performed in 17 patients. Successful decannulation has been achieved in 82%. In the majority of children with more minor stenoses conservative management has lead to successful decannulation. However, with increasingly severe stenosis the necessity of undergoing a laryngotracheoplasty procedure to provide an adequate airway becomes more likely.

Unusual case of tracheal agenesis.

We describe the clinical presentation and pathological features of an unusual case of tracheal agenesis. The axial derivatives of the primitive foregut between the larynx and stomach were represented by a single structure featuring sequential segmentation into regions showing exclusively tracheal or esophageal differentiation in a pattern that is not easily classified by existing nosologic systems nor explained by the conventional hypothesis of dysontogenesis.