The Great Ormond Street Hospital paediatric cochlear implant programme 1992-2004. A review of surgical complications.

The authors present a review of surgical complications following cochlear implantation at Great Ormond Street Hospital, since inception of the programme in 1992 until June 2004. Complications are defined as major (resulting in re-operation, explantation, re-implantation or which resulted in permanent serious morbidity or mortality) or minor (where the implant was not threatened). A comparison of surgical complication rates is made both with an earlier study in the same institution and also with other cochlear implantation centres worldwide. The decrease in surgical complication rates is discussed along with the difficulties inherent in cochlear implantation in the paediatric population with coexisting medical complaints.

Adult croup.

Adult croup is a distinct disease entity that probably represents a heterogeneous clinical syndrome. Three cases of adult laryngotracheitis characterized by upper airway infection and progression to airway obstruction are illustrated. Close observation and prompt decisions regarding airway intervention are critical in effective management, and complete resolution is expected.

Otologic and laryngologic manifestations of mucopolysaccharidoses after bone marrow transplantation.

INTRODUCTION: Children with mucopolysaccharidosis have significant functional impairment of a number of systems because of deposition of glycosaminoglycans. The otolaryngologic system is affected, resulting in conductive and sensorineural hearing loss and voice abnormality. METHOD: Eleven children who had undergone bone marrow transplantation had their hearing and voices assessed. Unmatched data from patients who had not undergone transplantation were collected for nonstatistical comparison. RESULTS: The hearing loss in children who had undergone bone marrow transplantation was variable but on average was less severe than in the younger children who had not undergone transplantation. The incidence of otitis media with effusion was diminished in the transplanted group. Approximately half had normal voices. DISCUSSION: Diminution of the progression of hearing loss and voice abnormality probably occurs in patients after treatment by bone marrow transplantation.

Cimetidine treatment for recurrent respiratory papillomatosis.

Effective adjuvant treatment for recurrent respiratory papillomatosis (RRP) is at present limited to alpha-interferon, which may have significant side effects including rebound growth of papillomata following its withdrawal, is given by injection and is expensive. High dose cimetidine is known to have immunomodulatory side effects and has been reported as a useful treatment for cutaneous warts. We report a case of very advanced RRP with tracheo-bronchial-pulmonary involvement treated with adjuvant cimetidine at a dose of 40 mg/kg for 4 months. The patient enjoyed a remarkable improvement in her clinical condition following treatment. The literature regarding cimetidine treatment for cutaneous warts is reviewed.

Disordered breathing during sleep in patients with mucopolysaccharidoses.

OBJECTIVE: Obstructive sleep apnoea (OSA) has been reported as a feature of children with mucopolysaccharidoses (MPS). However, the incidence and severity of OSA with respect to disease type is poorly defined. The aim of the present study was to measure objectively the degree of OSA in a group of children with a range of MPS syndromes. METHODS: In a cross-sectional study, cardiopulmonary sleep studies were performed during unsedated sleep in 26 children with MPS over a period of 2 years. Scores of OSA severity based upon clinical history and upon objective sleep study data were made in each case and compared. RESULTS: OSA was present in 24/26 patients, and ranged in severity from mild to severe. OSA was most marked in MPS type IH (Hurler syndrome) followed by types IHS (Hurler--Scheie syndrome) and II (Hunter syndrome). Frequent arousals and poor sleep quality, not suspected clinically, were noted in several patients. There was agreement between the clinical and objective scoring systems in only 17/26 patients (65%) with clinical history scores tending to underestimate the most severe cases (5/26 cases) and overestimate the severity in the mild cases (4/26 cases). CONCLUSIONS: Obstructive respiratory problems are frequent in MPS patients and there are differences in severity of OSA between the different MPS types. Assessments of the severity of OSA based upon clinical history alone are inadequate. Our results suggest that objective sleep studies are necessary to evaluate these cases, to monitor clinical outcome and to assess the effects of therapeutic intervention. Prospective studies in larger numbers of patients are needed to validate these observations.

Maxillary myxoma: a rare midfacial mass in a child.

The case of a maxillary myxoma in a 13-month-old child is presented. This case highlights the difficulties associated with managing such cases. The tumour was found at operation to be unencapsulated and infiltrative. This was contrary to the preoperative CT scan findings and necessitated a change from a sublabial to a lateral rhinotomy approach. This case illustrates a potential pitfall of CT scanning in such cases: it is likely that a MRI scan would have demonstrated the invasive nature of this tumour. To the authors' knowledge this is the second youngest reported case of maxillary myxoma in the literature.

Diagnostic value of infantile stridor: a perceptual test.

INTRODUCTION: stridor is the most common presenting symptom of pediatric airway obstruction, yet its value in clinical diagnosis is undetermined. The purpose of this study was to determine what diagnostic information was available to clinicians based entirely on listening to infantile stridor. METHOD: ten taped samples of stridor and corresponding clinical summaries were independently scored by Pediatric Otolaryngologists and their trainees. Respondents were asked to score the 'perceived' severity of the stridor based on their aural perception. Participants were recruited by an advertisement on the Internet Pediatric Otolaryngology forum. Thirty-eight consultants and 22 trainees responded. The responses were compared to known outcomes in the ten patients presented. RESULTS: listening to stridor in isolation resulted in respondents performing at the level of chance when their score of 'perceived' severity was compared to the known clinical outcome. When the clinical summaries (of the same patients) were scored, the level of performance significantly improved (P < 0.001). There was no difference in the level of performance when responses of trainees were compared with consultants, although there was a trend towards improved performance by the consultants. CONCLUSION: the importance of infantile stridor judged in isolation is of limited diagnostic value. Clinical information contributes significantly to the rating of stridor severity. The combination of both clinical and auditory information will likely improve diagnostic acumen.

Caustic soda ingestion -- a case presentation and review of the literature.

We present a case report of a 2-year-old child with a hypopharyngeal stricture secondary to caustic soda ingestion. We discuss the initial and long-term management of caustic soda ingestion. In this case the hypopharynx was successfully reconstructed using a pedicled pectoralis major flap. We demonstrate the versatility of this flap, which is traditionally known for its use in adult head and neck surgery.

Management and outcome of choanal atresia correction.

OBJECTIVES: Outcome analyses of factors that may either maximize success or predict a better outcome following choanal atresia correction. METHODS: A retrospective review of children undergoing choanal atresia correction at Great Ormond Street Hospital for Children, London between January 1990 and April 1998. Children with unilateral or bilateral choanal atresia were studied. In all cases, correction was by a transnasal approach under endoscopic control. A 120 degrees Hopkins rod telescope was used to visualize the atretic plate from the nasopharynx. Straight urethral sounds were used to perforate the plate followed by use of an air drill to remove the bony component. Portex endotracheal tubes were subsequently inserted as nasal stents. RESULTS: Sixty-five children (19 M, 46 F: age range 1 day to 17 years) presented with choanal atresia and the outcomes for 46 were included in the study. Twenty-six patients (40%) had other major anomalies. In children with unilateral atresia neither duration of stenting nor presence of facial anomalies had an impact on outcome. Of those children with bilateral choanal atresia and associated facial anomalies (n=9), 56% were asymptomatic following correction. In children with isolated bilateral choanal atresia (n=19), 74% were asymptomatic following correction; 29% (n2.3 kg (n3. 5 mm (n=6) had an 83% chance of a good outcome. Those patients stented with a tube

Benign thyroid disease and vocal cord palsy.

The case notes of 2453 consecutive patients admitted for thyroid surgery and with successful preoperative laryngoscopy were examined retrospectively. Of the 2408 patients who had not had previous operations on the gland, 2321 proved to have benign pathology. A total of 29 patients had a preoperative vocal cord palsy of which 22 were associated with benign disease. Return of cord movement after surgery occurred in 89% of the patients with a benign goitre. We advocate routine preoperative laryngoscopy to detect vocal cord paresis. Such a finding with a goitre does not necessarily indicate malignancy. The recurrent laryngeal nerve should therefore be identified at surgery and preserved to allow for recovery of vocal cord movement.

The value of head dressings for middle ear surgery.

A prospective trial was performed to ascertain the value of head dressings in the post-operative management of patients undergoing middle ear and mastoid surgery. One hundred consecutive patients were randomly allocated to a head dressing or no head dressing group after wound closure. Nine patients in the head dressing group developed a wound complication as opposed to four patients in the no head dressing group. The application of a pressure dressing following middle ear and mastoid surgery is unnecessary and may contribute to increased wound morbidity.

Long-term results of submandibular duct transposition for drooling.

This study examines the long-term results and morbidity of submandibular duct transposition in drooling children. Twenty-two patients, aged 3 to 18 years, with neurological dysfunction and excessive drooling underwent submandibular duct transposition between 1984 and 1987. In January 1990, 20 patients were reviewed. Their degree of drooling pre-operatively, immediately post-operatively and currently was assessed. The rate of improvement and the occurrence of complications were noted. Drooling was 'much better' in the early post-operative period in 17 of the 20 patients, and this improvement was invariably noted within three weeks. In the three other patients drooling was 'better'. Deterioration occurred in only three patients over the entire follow-up period. Complications all occurred in the first 18 months following surgery; they consisted of salivary retention cysts in four and transient submandibular gland swelling in a fifth patient.

Metastatic meningioma in the neck.

Although meningiomas represent 15 per cent of tumours of the central nervous system, they rarely metastasize. A case is presented in which metastasis to a cervical lymph node occurred, together with local recurrence, nine years after initial diagnosis and treatment. This case serves to illustrate that malignant meningioma is a rare cause of a neck mass.

A mixed thyroglossal cyst.

The case is described of a boy who presented at age six months with symptoms and signs of a thyroglossal cyst, which seemed to be confirmed by ultrasound findings. The cyst slowly increased in size, and eventually the patient underwent Sistrunk's procedure at age four years. Histology showed that the cyst was in fact a mixed thyroglossal and dermoid cyst. This casts doubt on the doctrine that thyroglossal cysts and dermoid cysts are anatomically and histologically separate entities, and strengthens the view that these cysts should be more appropriately named 'thyroglossal abnormalities'.

The developing role of a paediatric voice clinic: a review of our experience.

OBJECTIVE: We report our experience in developing a paediatric voice clinic within a tertiary otolaryngology department and describe the emerging role of this specialized clinic. MATERIALS: Currently our referral base is divided between other otolaryngologists within our department who require voice assessment as part of the pre- or post-operative management of laryngeal disorders (e.g. cysts, webs, vocal fold palsies, laryngo-tracheal reconstruction) and other professionals within our hospital who require characterisation of voice within the broader task of defining medical conditions in which voice abnormalities exist (e.g. mucopolysaccharidoses, functional dysphonias). METHODS: The patients were assessed by a team consisting of a paediatric laryngologist and a speech and language therapist. Each patient underwent a perceptual voice assessment and qualitative voice assessment using electrolaryngography. Direct visualisation was attempted and methods of and suitability for, such examination are discussed. RESULTS: Our experience is reviewed and guidelines for the establishment of a paediatric voice clinic are presented.

Styloid apparatus anomaly causing dysphagia.

An unusual case of dysphagia due to anomalous styloid apparatus anatomy is presented. Clinical and radiological findings are documented. Variations in anatomy and clinical syndromes are discussed together with management.

Diffuse tuberculous parotitis.

Parenchymatous parotid tuberculosis diffusely affecting the entire gland is very rare. We present a case, associated with a primary pulmonary focus, that was confirmed after positive identification of alcohol and acid-fast bacilli in gastric washings. Both sites of infection resolved with quadruple anti-tuberculous chemotherapy.

Pseudomonal supraglottitis occurring in a patient with profound neutropenia secondary to virus-associated haemophagocytic syndrome.

We present a case of virus-associated haemophagocytic syndrome following Epstein-Barr virus infection in which a fulminant pseudomonal supraglottitis developed. Increasingly, unusual pathogens have been found in immunocompromised patients. This is the first reported case of pseudomonal supraglottitis.

MRI as a single screening procedure for acoustic neuroma: a cost effective protocol.

Magnetic resonance imaging (MRI) is accepted as the 'gold standard' in diagnosing acoustic neuromas. Limited availability and perceived high costs have prevented clinicians from using it as a first-line investigation. A prospective study was set up in a specially designated screening session to audit the cost effectiveness and accuracy of audiovestibular investigations compared to MRI. Ninety-nine patients with asymmetrical audiovestibular symptoms or signs were investigated. Of these 54 evoked response audiometry tests, and 39 calorics were either not performed or were inconclusive. One patient refused to enter the MRI machine. All others received an unequivocal report after MRI and four tumours (three intracanalicular) were detected. The total cost of the audiovestibular protocol was 12,545 pounds compared to 12,900 pounds for the MRI protocol, which is a diagnostic and well-tolerated procedure. This study shows that MRI can be cost effective, as well as accurate, when used as a single screening procedure for acoustic neuromas.

Swallowing in motor neurone disease.

Ninety-two patients with motor neurone disease have been assessed clinically and radiologically for evidence of swallowing problems. At the time of examination, moderate or severe swallowing difficulty was present in 89% of those whose disease had presented as bulbar palsy, in 45% of those in whom the disease began many months before as progressive muscular atrophy and in 29% of those with amyotrophic lateral sclerosis. Patients with more severe swallowing symptoms appeared more likely to have abnormal findings on videofluoroscopy overall. However, not all patients with an abnormal radiological picture had swallowing difficulties. It is suggested that radiological signs should only be used within the context of clinical symptoms and signs in the selection of patients for palliative surgery. Thirteen patients with pseudobulbar symptoms and signs had a cricopharyngeal myotomy performed: two suffered major post-operative complications. However, the satisfaction rate was 89% and we recommend cricopharyngeal myotomy for such patients. Pharyngostomy was performed for seven patients unable to initiate swallowing, six had post-operative complications.