C. M. Bailey

Updated surgical experience with bone-anchored hearing aids in children.

BACKGROUND We present the results of a retrospective review of children undergoing implantation with bone-anchored hearing aids (BAHAs) at the Great Ormond Street Hospital for Children. METHODS The case notes of 71 children undergoing BAHA placement at the Great Ormond Street Hospital for Children between December 1990 and August 2002 were reviewed. Outcome measures included hearing thresholds, incidence of fixture loss, skin reaction and need for revision. Quality of life outcomes were also measured. RESULTS Eighty-five ears had been implanted. Fifty-four per cent of children had experienced no complications, 42 per cent had required revision surgery and 26 per cent had experienced fixture loss at some point. Young age at implantation was associated with an adverse outcome. Trauma and failure of osseointegration had been the commonest reasons for failure. A skin reaction around the abutment had occurred at some point in 37 per cent of children but had persisted for longer than six months in only 9 per cent; this had been associated with fixture loss. The use of fixture site split skin grafts had reduced problems with skin hypertrophy and hair overgrowth. Hearing thresholds when using BAHAs had been comparable to those when using bone conduction hearing aids. However, BAHAs had significant additional benefits in terms of sound quality, ease of use and overall quality of life. CONCLUSION Bone-anchored hearing aids provide significant benefits over other types of hearing aid, both audiologically and in terms of quality of life. Careful selection of candidates and meticulous follow up are required in order to minimize complications.

Aryepiglottoplasty for laryngomalacia: 100 consecutive cases

A retrospective review of the notes of 100 consecutive patients who had undergone aryepiglottoplasty for laryngomalacia, at Great Ormond Street Hospital for Children, was undertaken. Fifty-six were male, 44 female and 47 were under three months of age. Indications for surgery were oxygen desaturation below 92 per cent and feeding difficulties causing failure to thrive. Forty-seven patients had other pathology contributing to their airway compromise or feeding problems. Improvement in stridor after one month was achieved in 86/91 (94.5 per cent) being abolished completely in 50/91 (55 per cent). Of the 25 per cent of patients whose symptoms took more than one week to resolve, 16/22 (63.6 per cent) were later found to have a serious neurological condition. Feeding was improved in 42 of 58 patients (72.4 per cent) who had a pre-operative feeding difficulty. The complication rate was low, with only five out of 86 (10 per cent) experiencing initial worsening of the airway and six per cent having aspiration of early feeds before improvement occurred. Endoscopic aryepiglottoplasty remains the operation of choice for patients with severe laryngomalacia, however, in the presence of neurological disease surgery is less likely to be successful.

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 </=3.5 mm (n=22) had only a 64% success rate. None of the patients who were stented for at least 12 weeks remained symptomatic. CONCLUSIONS Neonates with bilateral choanal atresia who were stented for at least 12 weeks with the largest Portex tube that comfortably passed through the anterior nares had the best outcome. If they had either associated anomalies or low weight at surgery, they were less likely to become asymptomatic. For patients with unilateral choanal atresia, neither the presence of facial anomalies nor stent duration had an impact on outcome.

Laryngeal ultrasound to assess vocal fold paralysis in children

The purpose of this study was to assess the practicality and validity of laryngeal ultrasound to establish vocal fold movement in children with suspected vocal fold palsy. Fifty-five consecutive patients (age range three days to 12 years) with suspected vocal fold palsy underwent both laryngoscopy and laryngeal ultrasound. Ultrasonographic findings correlated with endoscopic findings in 81.2 per cent of cases. This, however, rose to a concordance rate of 89.5 per cent in patients aged over 12 months. Laryngeal ultrasound is well-tolerated, safe and non-invasive and the authors feel that it is a useful adjunct to endoscopy in the diagnosis of vocal fold palsy.

The Great Ormond Street protocol for ward decannulation of children with tracheostomy: increasing safety and decreasing cost

Ward decannulation is the eventual endpoint for the majority of the 40 children managed by tracheostomy at Great Ormond Street Hospital each year. Our previous protocol dictated a 10 day stay in hospital for the 25 children decannulated annually. The aim of this retrospective study was to determine the minimum safe duration for this inpatient stay. In addition, we hoped to identify any factors which might distinguish potentially successful candidates from those likely to fail decannulation. Of 104 patients decannulated between January 1991 and January 1996, complete nursing and medical records were retrieved for 84 children. These notes were retrospectively reviewed and in this group of patients there were 101 attempted decannulations. Information about the initial pathology, interim course and details of the decannulation were collected. Summary statistics were generated. There was no relation between initial pathology or duration of tracheostomy and eventual success or failure of decannulation. Observations by the nursing staff of restlessness, anxiety and recession appeared more frequently in children who eventually failed decannulation. All failures occurred within 48 h of the 24 h trial of tracheostomy blocking. Our results suggest that a safe decannulation protocol can allow discharge after day five.

Plummer-Vinson syndrome--a rare presentation in a child.

Plummer-Vinson (Paterson, Brown-Kelly) syndrome refers to the association of iron-deficiency anaemia with dysphagia secondary to a post-cricoid web. Only seven cases of Plummer-Vinson syndrome in children and adolescents between the ages of 14 and 19 have been reported in the world literature. We report a case of the syndrome occurring in a child of 14 years and provide a short review of the present knowledge concerning the symptom complex.

Otitis media with effusion in paediatric cochlear implantees: the role of peri-implant grommet insertion

INTRODUCTION Otitis media with effusion (OME) is a common disease coincident with the need for cochlear implantation in 44% of the 45 children (mean = 4.8 years) currently implanted at our centre. Our suspicion that peri-implant effusion contributed to complications generated our interest in studying this issue. METHOD Retrospective review of patient records was carried out with the focus on clinical and audiological evidence of OME before, during and after implantation. Operative records were reviewed to determine any effect OME had on the procedure and postoperative complications. RESULTS Of our 45 implanted patients, 10 males and 10 female had OME prior to or after implantation. Pre-implant, 19 children had OME diagnosed and 11 received grommets (6 had multiple sets). Peri-implantation, 11 children had OME. These children with peri-implant effusions had 2 of 3 major, 2 of 2 intermediate and 1 of 2 minor complications. Children with peri-implant effusions also accounted for 3 of 4 cases in which there were bothersome middle ear effusions or granulation tissue at operation. The 6 children in whom grommets were placed within two months of cochlear implantation accounted for only one case of effusion at implantation and their courses were complicated only by a keloid in one child and a late flap disruption in another. SUMMARY Insertion of grommets prior to cochlear implantation in children with peri-implant OME may provide some protection against intra-operative and some post-operative complications. Our management policy in patients with OME now aims at placing grommets such that they are in and patent at the time of implantation.

Laryngotracheal reconstruction and cricotracheal resection in children: Recent experience at Great Ormond Street Hospital

BACKGROUND Surgery for paediatric airway stenosis is constantly evolving. Surgery is the primary treatment modality via either an open or endoscopic approach. The objective of this study was to review the results of laryngotracheal reconstruction (LTR) and cricotracheal resection (CTR) procedures performed at Great Ormond Street Hospital over the past 10 years. METHODS All patients who underwent open airway reconstruction surgery from January 2000 to December 2010 were included in this study. Patients treated entirely endoscopically were excluded. The data was collected using the electronic operating theatre database and the discharge summary database. RESULTS Complete data was available for 199 patients who underwent open airway reconstruction from January 2000 to December 2010. The procedures included single stage LTR (57, 28.6%), two stage LTR (115, 57.7%), single-stage stomal reconstruction (14), single-stage CTR (8) and two-stage CTR (5). The diagnoses at the initial airway endoscopy were laryngeal web (22), subglottic stenosis (151), posterior glottic stenosis (9), suprastomal collapse (15), supraglottic stenosis (1) and tracheal stenosis (1). For those with subglottic stenosis, the stenosis was grade 1 in 1 patient, grade 2 in 26 patients, grade 3 in 117 patients and grade 4 in 6 patients. At the completion of intervention 175/199 (87.9%) patients reported improvement in their symptoms. Amongst the subglottic stenosis group, post LTR success was achieved in 100% with grade 1 stenosis, 92.3% with grade 2 stenosis, 88.1% in grade 3 stenosis and 83.3% in grade 4 stenosis. Of the two-stage LTR procedures, 100/115 (86.9%) had their tracheostomy removed and 15/115 (13.1%) have failed decannulation. Of the single-stage LTR group, 50/57 (87.7%) patients were better both on airway examination and symptomatically postoperatively. Of the single-stage stomal reconstruction group, 13/14 (92.8%) were better symptomatically and on airway examination. Patients who underwent single-stage CTR had a better airway on examination and were symptomatically improved in all cases (8/8). For the patients who underwent two stage CTR, the tracheostomy was removed in 3/5 (60%) and retained in 2/5 (40%). For the whole group, 15/199 (7.5%) patients underwent a revision LTR. On further analysis, revision LTR was required in 4/57 (7.1%) single-stage LTR, 9/115 (7.8%) two-stage LTR, 1/5 (20%) two-stage CTR and 1/8 (12.5%) single-stage CTR. In this study complications occurred in 13/199 (6.5%). CONCLUSIONS Subglottic stenosis in children needs to be approached on the basis of the nature and severity of stenosis and the individual patients general health. Good outcomes are achieved with both LTR and CTR. Good results are obtained both with single-stage and two-stage LTR, but restenosis remains a problem. An individual approach is required for treatment of paediatric airway stenosis to achieve good final outcomes. The overall success rate has increased only marginally in our institution over the last 20 years.

Management of subglottic haemangioma

Two contrasting methods of treatment for paediatric subglottic haemangioma are presented. Dr Hoeve describes his results using intralesional steroids and short-term intubation, and Dr Froehlich discusses his outcomes with open surgical excision. Mr Bailey then reviews the currently available therapeutic options with special reference to each of the above techniques, and suggests an optimum plan of management given our current state of knowledge. The conclusion is that very small haemangiomas may not require treatment, or may be amenable to CO2 laser vaporization. Medium-sized lesions seem suitable for intra-lesional steroids and intubation, but large ones are probably best managed by primary submucous resection. Very large haemangiomas, and especially those which are circumferential or in which magnetic resonance imaging (MRI) shows extension down into the trachea and/or through the tracheal wall into the surrounding tissues, may be more safely dealt with by performing a tracheostomy and awaiting spontaneous involution.

Laryngeal paraganglioma in a five-year-old child--the youngest case ever recorded.

A paraganglioma is a neuroendocrine neoplasm that originates from the paraganglion cells of the parasympathetic system. The average age of presentation is in the fifth decade. We report a case of laryngeal paraganglioma in a five-year-old child, the youngest case ever recorded. The features of paraganglioma which differentiate it from other tumours are also discussed.