Scientific and clinical aspects of the use of cidofovir in recurrent respiratory papillomatosis.

OBJECTIVE: Cidofovir is the most contemporary adjuvant treatment for recurrent respiratory papillomatosis (RRP) and its use is increasing. Cidofovir is potentially harmful. Otolaryngologists should understand the science of cidofovir and review the current published data on the effects of this therapy. METHOD: Pubmed was searched using the terms cidofovir and papillomatosis. Comparisons were made between published articles. RESULTS: Thirteen articles were identified between 1998 and 2006, representing the treatment of 142 patients. Cidofovir did result in a significant improvement of papillomatous lesions in the majority (60%) of patients despite the use of different regimes of cidofovir administration. There was no unifying protocol in use. A partial response was demonstrated in 29% of patients. 7.5% had no response however, an additional 3.5% patients were lost to follow-up. No malignant change was reported. CONCLUSION: Cidofovir does appear to be effective in improving the outcome of patients with RRP. There are no reports of malignant transformation despite concerns raised by toxicology studies.

A tubeless anaesthetic technique for paediatric laryngeal laser surgery.

Laser surgery on the paediatric larynx requires cooperation between the surgeon and anaesthetist to carry out the procedure in the safest manner possible. Over a period of 3 years, 45 laser procedures have been undertaken on the upper airway of 14 patients at our institution. All procedures were performed with volatile gas anaesthesia supplied via a nasopharyngeal tube in a spontaneously ventilating patient, thus allowing the surgeon an unobstructed view of the larynx. In only one case, was a problem experienced with the technique, that of laryngospasm. The technique provides an unrivalled view of the larynx whilst allowing safe anaesthesia.

Otorhinolaryngological manifestations of the mucopolysaccharidoses.

The mucopolysaccharidoses (MPS) are a family of related inherited metabolic disorders where, due to specific lysosomal enzyme deficiencies, partially degraded glycosaminoglycans (GAGs) accumulate in the body's cells. Due to the ubiquitous nature of GAGs in the body this deposition can occur in many tissue types and may interfere with cellular function. Although these conditions are rare, there is a propensity for the disease process to cause problems with the function of the ears, noses and throats of affected patients. In this review, we present an overview of the clinical manifestations of MPS in general and highlight the problems specifically presenting in the field of otorhinolaryngology.

Customising the length of the Montgomery T-tube whilst retaining a bevelled edge.

BACKGROUND: The Montgomery T-tube is used in a number of conditions that require safe tracheal stenting. Specific lengths of T-tube limbs are occasionally needed in patients with complex airway anatomy or differing neck proportions; this requires customisation of the T-tube limbs. This is done either by pre-ordering customised T-tubes from the manufacturer (which needs to be planned ahead of time) or using a tube cutter in the operating theatre. However, the latter does not provide a 'factory like' bevelled edge when shortened, which increases the risk of mucosal trauma and granulation formation. OBJECTIVE: This paper reports a novel technique for customising the length of existing Montgomery T-tubes, with preservation of the bevelled edges. This technique can be easily performed with basic equipment available in operating theatres.

The application of computer-enhanced imaging to improve preoperative counselling and informed consent in children considering bone anchored auricular prosthesis surgery.

OBJECTIVES: Bone anchored auricular prostheses have become a valuable option in the treatment of congenital and acquired deformities of the pinna. However, preoperative counselling and informed consent remains a challenging issue. Until recently it has been difficult to provide the child with a realistic prediction of their own postoperative appearance. This is particularly relevant when a remnant pinna needs to be excised prior to the second stage. The potential for psychological repercussions and the possibility that remnant excision might compromise future autologous tissue reconstruction make it imperative that the decision to proceed with surgery is founded on the best possible information. METHODS: The authors describe the use of computer enhanced images using the Adobe Photoshop (Apple Mac. Inc.) software package to provide such a preview. This technique is used in the outpatient clinic as an adjunct to counselling provided by clinic staff and is reinforced by meeting children who have already enrolled on to the implant programme. Children are encouraged to follow the stages of their planned operation on the computer screen, providing an accurate insight into the physical consequences of surgery. RESULTS: Our experiences suggest that this approach has encouraged a better qualitative understanding of implant surgery which has helped to foster the on-going commitment that is required to maintain a long-lasting, trouble-free implant site. CONCLUSIONS: This application of the Adobe Photoshop package has strengthened our basis for a personal informed consent and has provided an opportunity to lessen the adverse psychological consequences of such irreversible surgery. It is commended for its simplicity as it employs established software to enhance photographic prints or slides taken from the child's clinical records.

A new deal for ENT surgeons--The Manchester experience 1992-3.

OBJECTIVES: To reduce junior doctors' hours by introducing an on-call system involving cross-cover at SHO level between two separate ENT units. DESIGN: Prospective collection of data on the cross-cover system introduced in September 1992 with analysis after 12 months. SETTING: ENT departments within Salford and Central Manchester. SUBJECTS: Five SHOs and four registrars within the two ENT units. RESULTS: SHOs work a 1 in 5 rota providing first on-call cover for two adjacent ENT units. The system has proved to be an effective means of decreasing junior doctors' hours, though actual hours exceed the contracted hours by approximately 8 hours per week. Initial problems of contacting SHOs were overcome by the introduction of new bleep arrangements while the system produced a number of unforeseen benefits. The experience in Manchester suggests that major ENT units do not necessarily require resident first on-call staff, though formal arrangements are recommended to cope with acute emergencies requiring immediate attention. CONCLUSIONS: An effective on-call system has been devised which decreases junior doctors' hours by combining the on-call rotas of two adjacent ENT units. Recommendations for the successful implementation of such a system are presented.

An unusual pattern of intubation injury?

Post-intubation laryngeal dysfunction is well documented. Both common and rare patterns of injury have been described in the literature. An unusual pattern of intubation injury is described in this case report. The different patterns of post-intubation laryngeal injury are discussed as well as the possible aetiology in the case described.

Endoscopic balloon dilatation of subglottic stenosis.

Children with subglottic stenosis present a challenging problem to otolaryngologists. In many cases, a tracheostomy is necessary to safeguard the airway, but morbidity and mortality in the tracheostomized child may be significant. Therefore attempts to improve the airway by endoscopic means are often made; unfortunately, these have a variable success rate. Recent encouraging results in the use of balloon dilatation for subglottic stenosis led us to the successful use of this technique in a child whose stenosis had not responded to conventional endoscopic techniques. The reasons for the success are discussed.

Mediastinal tuberculosis in a 10-month-old child.

We report a rare case of mediastinal tuberculosis in a child who presented as a possible inhaled foreign body. A 10-month-old girl was admitted with a five-month history of cough, wheeze and problematic feeding, thought initially to be due to asthma. A clinical deterioration and subsequent X-rays suggested an inhaled foreign body. However, at direct laryngotracheobronchoscopy no foreign body was found and subsequent investigations revealed a subcarinal mediastinal mass. She underwent a thoracotomy and excision of the mass, the histological analysis of which revealed it to be of tuberculous origin. When a patient presents with symptoms of upper airway obstruction which are highly suggestive of a foreign body, other causes such as mediastinal tuberculosis must be borne in mind when no foreign body can be found. Although rare, cases of tuberculosis are apparently increasing and the otolaryngologist must be aware of its various manifestations and submit specimens for appropriate analysis. We also briefly review mediastinal lymphadenopathy due to tuberculosis.

Combined 'trache-stent': a useful option in the treatment of a complex case of subglottic stenosis.

The authors describe the adaptation of a Montgomery T-tube laryngo-tracheal stent to incorporate an uncuffed fenestrated Shiley tracheostomy tube in the management of a complex case of subglottic stenosis. The combined 'trache-stent' provided a secure, unobstructed airway and optimal phonation. The presence of a removable inner tube facilitated cleaning and reduced patient anxiety regarding the perceived risks of stent obstruction with dried secretions. The combined 'trache-stent' was upsized at four weeks with minimal evidence of local granulation formation. The device was removed entirely six weeks later after direct laryngoscopy reconfirmed the above findings. Almost two years later the patient retains a good voice and airway using a simple fenestrated tracheostomy tube and speaking valve. The subglottic area is stable and the patient has returned to full-time employment.

Delayed diagnosis of laryngeal foreign body.

Aspiration of a foreign body is a recognized cause of accidental death in children. Paediatricians are aware of the symptoms of inhaled foreign bodies in the lower respiratory tract. However, symptoms which suggest impaction in the larynx do not appear to raise the same index of suspicion of a foreign body. One case of laryngeal foreign body is described with a delay in diagnosis of five days. The clinical presentation, investigations and management are discussed.

Bones of contention. The supply of temporal bones for dissection: the legalities, problems and solutions.

Temporal bone dissection is considered to be an important aspect of the otological training of the Specialist Registrar with dissection skills being formally assessed in the Intercollegiate Fellowship Examination. However the procurement of cadaveric specimens suitable for dissection may be fraught with difficulties. The authors take an historical perspective to clarify the existing legal issues and outline the means available to improve supply.

Relapsing polychondritis associated with monoclonal gammopathy in a patient with myelodysplastic syndrome.

Relapsing polychondritis is a rare condition characterized by inflammation and subsequent degeneration of cartilages. Deformity of the pinna, nasal saddling and stridor due to involvement of the cartilages of the respiratory tract may lead to patients being referred to the otolaryngologist for initial assessment and further management. Recent observations have suggested that relapsing polychondritis may occur as a paraneoplastic phenomenon in cases of myelodysplasia. The case of a patient with relapsing polychondritis, myelodysplastic syndrome and a monoclonal gammopathy is presented. The authors highlight the apparent existence of this association and encourage otolaryngologists to consider such possible links when cases of relapsing polychondritis present to the outpatients department.

Upper airway obstruction secondary to anticoagulant induced haemorrhage necessitating a tracheostomy.

The incidence of major haemorrhagic complications from oral anticoagulants is between 4.4% and 8.2%. Life-threatening haemorrhage is usually either intracranial or gastro-intestinal, but haemorrhage into the upper airways is a distinctly uncommon occurrence. We report the case of an elderly lady who developed an anticoagulant induced haemorrhage into the parapharyngeal area resulting in upper airway obstruction and necessitating an emergency tracheostomy.

Case series: Endoscopic management of fourth branchial arch anomalies.

INTRODUCTION: Fourth branchial arch anomalies represent <1% of all branchial anomalies and present as recurrent neck infections or suppurative thyroiditis. Traditionally, management has consisted of treatment of the acute infection followed by hemithyroidectomy, surgical excision of the tract and obliteration of the opening in the pyriform fossa. Recently, it has been suggested that endoscopic obliteration of the sinus tract alone using laser, chemo or electrocautery is a viable alternative to open surgery. OBJECTIVES: To determine the results of endoscopic obliteration of fourth branchial arch fistulae in children in our institute. METHODS: Retrospective case note review of all children undergoing endoscopic treatment of fourth branchial arch anomalies in the last 7 years at the Royal Manchester Children's Hospital. Patient demographics, presenting symptoms, investigations and surgical technique were analysed. The primary and secondary outcome measures were resolution of recurrent infections and incidence of surgical complications, respectively. RESULTS: In total 5 cases were identified (4 females and 1 male) aged between 3 and 12 years. All presented with recurrent left sided neck abscesses. All children underwent a diagnostic laryngo-tracheo-bronchoscopy which identified a sinus in the apex of the left pyriform fossa. This was obliterated using electrocautery in 1 patient, CO2 laser/Silver Nitrate chemocautery in 2 patients and Silver Nitrate chemocautery in a further 2 patients. There were no complications and no recurrences over a mean follow-up period of 25 months (range 11-41 months). CONCLUSION: Endoscopic obliteration of pyriform fossa sinus is a safe method for treating fourth branchial arch anomalies with no recurrence.

Radiofrequency ablation in the treatment of paediatric microcystic lymphatic malformations.

OBJECTIVE: Congenital lymphatic malformations are a challenging clinical problem. There is currently no universally accepted treatment for the management of microcystic disease. We describe the novel use of an existing technology (radiofrequency ablation, also termed Coblation) for the debulking of paediatric microcystic lymphatic malformations involving the upper aerodigestive tract. METHODS: Five children with microcystic or mixed-type lymphatic malformations were included in this retrospective case series. RESULTS: Each child had a satisfactory outcome following radiofrequency debulking, with improved oral intake and airway symptoms. No serious complications were reported. These findings constitute level IV evidence. CONCLUSION: We recommend radiofrequency ablation as a safe, viable alternative to existing techniques for the treatment of paediatric microcystic lymphatic malformations of the upper aerodigestive tract. Radiofrequency ablation achieves effective debulking of microcysts whilst avoiding excessive bleeding and thermal damage to surrounding tissues. This paper constitutes the first report of successful treatment of airway obstruction due to paediatric laryngopharyngeal microcystic disease, using radiofrequency ablation.

Rhabdomyosarcoma of the trachea: first reported case treated with proton beam therapy.

OBJECTIVE: We report a case of rhabdomyosarcoma of the trachea in a 14-month-old child, and we present the first reported use of proton beam therapy for this tumour. CASE REPORT: A 14-month-old girl presented acutely with a seven-day history of biphasic stridor. Emergency endoscopic debulking of a posterior tracheal mass was undertaken. Histological examination revealed an embryonal rhabdomyosarcoma with anaplasia. Multimodality therapy with surgery and chemotherapy was administered in the UK, and proton beam therapy in the USA. CONCLUSION: Only three cases of rhabdomyosarcoma of the trachea have previously been reported in the world literature. This is the first reported case of treatment of this tumour with proton beam therapy. Compared with conventional radiotherapy, proton beam therapy may confer improved long-term outcome in children, with benefits including reduced irradiation of the spinal cord.

Balloon dilatation to treat plasmacytosis of the supraglottic larynx.

OBJECTIVE: We discuss the use of balloon dilatation to relieve supraglottic stenosis caused by mucous membrane plasmacytosis. CASE REPORT: A 54-year-old man with a known diagnosis of mucous membrane plasmacytosis presented with dysphonia and worsening airway obstruction which required a tracheostomy. He underwent balloon dilatation of the supraglottic larynx using an angioplasty balloon within sequentially sized endotracheal tubes. This enabled successful decannulation, with minimal re-stenosis at eight-month follow up. CONCLUSION: To our knowledge, this is the first reported case of supraglottic stenosis caused by plasmacytosis to be successfully treated using this method. We have shown that this minimally invasive technique deals effectively with a complex airway and minimises re-stenosis.

Bone-anchored hearing aids and chronic pain: a long-term complication and a cause for elective implant removal.

OBJECTIVES: To report a case series of elective removal of bone-anchored hearing aid implants, and reasons for removal. DESIGN: Retrospective review of a prospectively collected database. SETTING: Two tertiary referral centres in the Manchester area: Manchester Royal Infirmary and Salford Royal University Hospital. PARTICIPANTS: A series of 499 adults and children who had undergone a total of 602 implant insertions (1984-2008). MAIN OUTCOME MEASURES: Implant removal rates, and reasons. RESULTS: Twenty-seven of the 602 implants (4.5 per cent) required removal. Of these, 12 were due to pain (2.0 per cent), seven to persistent infection (1.2 per cent), three to failure of osseointegration (0.5 per cent), three to trauma (0.5 per cent) and two to other reasons (0.4 per cent). CONCLUSION: Chronic implant site pain represents the main reason why implants are removed electively, and affects 2 per cent of all implants. This complication has important medico-legal implications and should be discussed when obtaining informed consent for implantation.

Primary endoscopic repair of intermediate laryngeal clefts.

INTRODUCTION: Traditionally, small laryngeal clefts may be closed endoscopically, while larger clefts necessitate an open anterior approach. We report the presentation, evaluation and outcome following endoscopic surgical repair of a series of laryngeal clefts. METHOD: Retrospective study of children treated in a tertiary referral centre between 2003 and 2008. The presenting symptoms, patient demographics, cleft type, surgical outcome and complications were evaluated. RESULTS: Seven children underwent primary endoscopic repair of their laryngeal clefts (four Benjamin-Inglis type III clefts and three type II clefts). Presenting symptoms included stridor, cough and cyanosis with feeds, swallowing problems, weak cry, and recurrent lower respiratory tract infection. Treatment was ultimately successful in six of the seven children; treatment was ongoing for the remaining child, who underwent subsequent revision surgery via an open approach. Two children went on to require a second endoscopic repair, and two underwent an open repair of a residual defect. One child required a tracheostomy for failed extubation in the post-operative period. CONCLUSION: Endoscopic repair is a safe, useful technique in the management of laryngeal clefts. Laryngeal clefts must be excluded in a child presenting with persistent aerodigestive tract symptoms, as described here.