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Abstracts 2 |
The Outcome of Primary Endoscopic Repair of Laryngeal Clefts
IA Bruce, S Broomfield, MP Rothera Royal Manchester Children’s Hospital
Introduction
Laryngeal clefts result from abnormal tracheoesophageal septum development. Smaller clefts may be closed endoscopically with larger clefts necessitating an open anterior approach. We report the presentation, evaluation and outcome following endoscopic surgical repair of a series of laryngeal clefts.
Methods
Retrospective study of children treated in a tertiary referral centre since 2003. The presenting symptoms, patient demographics, cleft type, surgical outcome and complications were evaluated.
Results
7 children (4 boys and 3 girls) underwent primary endoscopic repair of their laryngeal clefts (4 Benjamin Type III and 3 Type II). The average age at first surgery was 15 months (range 1 – 51 months). Associated congenital anomalies included oesophageal atresia/ TOF and Opitz syndrome (2 children). Presenting symptoms included stridor, cough and cyanosis with feeds, swallowing problems, weak cry and recurrent LRTI. Ultimately treatment was successful in 6 of 7 children, with treatment ongoing in the remaining child who has undergone revision surgery via an open approach. 2 children subsequently required a second endoscopic repair and 2 underwent an open repair of a residual defect. 1 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 when presented with persistent aerodigestive tract symptoms as described here. In addition, certain associated congenital anomalies may point to the presence of a laryngeal cleft.
IN-VITRO EVIDENCE OF GROWTH STIMULATORY EFFECTS OF CIDOFOVIR ON HPV E6 TRANSFECTED CELLS AND IMPLICATIONS FOR RECURRENT RESPIRATORY PAPILLOMATOSIS.
Donne AJ1,3, Hampson, L1, He XT1, Rothera MP2, Homer JJ3 and Hampson IN1 1 University of Manchester Gynaecological Oncology Laboratories, St Mary’s Hospital, Hathersage Road, Manchester M13 OJH. 2 Department of Otolaryngology, Royal Manchester Children’s Hospital, Pendlebury, Manchester, M27 4HA. 3 University Department of Otolaryngology-Head & Neck Surgery, Manchester Royal Infirmary, Oxford Road, Manchester, M13 9WL.
INTRODUCTION
Recurrent Respiratory Papillomatosis (RRP) is caused by human papillomavirus (HPV) 6 and 11. Cidofovir is currently the most contemporary adjuvant treatment for RRP. E6 is a pivotal HPV protein that controls growth. To date, the effects of Cidofovir on non-malignant HPV E6 transfected cell lines have not been tested.
METHOD
HPV types 6 and 16 E6 expressing cell lines were derived from telomerase immortalized human keratinocytes and E6 expression confirmed by RT-PCR. E6 expressing cells and vector control cells were incubated with either of two different doses of Cidofovir or plain medium for either 2 or 4 days. The effects of this treatment on growth rate and long term survival was estimated by AQ96 and clonogenic assays.
RESULT
E6 mRNA expression was confirmed by RT PCR. Clonogenic long term survival assays showed that initial 2 days of exposure to 5"g/ml of Cidofovir caused a marked increase in the survival of HPV16 (p=0.013) and HPV6 (p=0.0007) E6 expressing cells only and not vector control cells. Despite a longer initial exposure (4 days) AQ96 growth assays still demonstrated stimulated growth in HPV6 E6 expressing cells only.
Treatment of paediatric laryngeal papiloma – Service evaluation from British Association Of Paediatric Otolaryngologist (BAPO)-Web based survey
Manickavasagam J, Wu K, Bateman N Sheffield Children’s Hospital, UK
Introduction
Juvenile onset recurrent laryngeal papillomas are benign epithelial tumours that are caused by infection with the human papilloma virus (HPV). They are the most common benign neoplasm affecting the larynx and upper respiratory tract in children. We have prepared the questionnaire to evaluate the current trend in the management of paediatric laryngeal papilloma and indication of Cidofovir use in children with laryngeal papilloma
Methods
We have conducted this short questionnaire web survey amongst all the members of The British Association of Paediatric Otorhinolaryngology.
Results
We have 35 responses. 23 responders treating paediatric laryngeal papilloma 19 responders prefer microdebrider, 11 responders prefer laser and 5 responders prefer cold steel along with either laser or microdebrider One Half of surgeons use Cidofovir in selected patients and other half do not use Cidofovir in any patients Amongst the Cidofovir users, 7 people consider Cidofovir after 0-4 procedures, 4 people consider Cidofovir after 4-6 procedures and 6 people consider Cidofovir after 6 procedures With regard to consent, 11 people warn the patient about the side effects of Cidofovir and 5 people do not warn their patients Interferon, Zinc supplements and Gardasil are the other adjuvant treatments.
Conclusion
It appears that there is probably a small number of people treating RRP in children on a regular basis. The microdebrider is widely used although some people are still using the laser. No consensus as to when it would be appropriate to use Cidofovir. However small numbers of people are giving it without adequately informed consent. Fairly, poorly understood drug with potentially serious side effects and there should be some guidelines as to its use.
Upper Airways Obstruction In Apert Syndrome
Scott Maskell1, Damian Marucci2, Richard Hayward2, David Dunaway2, Michelle Wyatt1 1ENT Dept, Great Ormond Street Hospital for Children, London. 2The Craniofacial Unit, Great Ormond Street Hospital for Children, London.
Introduction
Apert syndrome is characterised by craniosynostosis, syndactyly and midfacial hypoplasia. These individuals are at a high risk for developing obstructive sleep apnoea syndrome. This paper reviews our experience of upper airways obstruction and the variety of treatment modalities involved.
Methods
Retrospective case note and sleep study analysis of children born between 1992 and 2007 was undertaken. Sleep study oximetry data was analysed both pre and post intervention. Interventions included placement of a nasopharyngeal airway (NPA), adenotonsillectomy, nasal continuous positive airways pressure (CPAP), tracheostomy and facial bipartition.
Results
Fifty-four patients were identified, with 41 requiring airway intervention. Nine patients had NPA insertion (at mean age 1.5 years), 19 patients had an adenotonsillectomy (at mean age 4.4 years), 14 patients had CPAP (at mean age 6.0 years), six patients had formation of a tracheostomy (at mean age 2.3 years) and three patients had a facial bipartition (average 7.6 years). Paired sleep study oximetry data showed statistically significant improvement with adenotonsillectomy and CPAP, less so for NPA insertion. There was little effect on airway obstruction with facial bipartition.
Conclusion
Our department’s policy is to undertake regular monitoring of patients with Apert syndrome from birth onwards allowing early detection of airway problems. Interventions are varied and tailored to each child. Our aim is to treat airway obstruction effectively with the least invasive technique available. Generally either a NPA or adenotonsillectomy are first line considerations followed by CPAP if symptoms persist. Tracheostomy is performed when other modalities have failed or are considered inappropriate.
Inter and intra-rater reliability of the reflux finding score (RFS) in the paediatric larynx
David D Pothier, Shiraz Ahmed, Michael W Saunders Bristol Children’s Hospital, Bristol
Introduction
Laryngopharyngeal reflux (LPR) has been associated with many otolaryngological disorders. The gold standard of pH monitoring is not commonly undertaken, but physical signs of LPR have been described and collated as a reflux finding score1 (RFS). There has been no validation of these physical findings in children; we set out to determine inter and intra-rater reliability for these findings.
Methods
A group of 35 ENT surgeons was shown a series of twenty digital video clips of paediatric rigid laryngoscopies taken directly from a Karl Storz endoscopic stack system. The first ten were unique clips, but the second series of ten were repeats of the original ten clips that had been rotated through 180 degrees on a horizontal and vertical plane. The participants were asked to record all RFS findings for each clip.
Results
Scores varied considerably from a minimum of 3 to a maximum of 24 for the same series of clips (mean = 10.9 (SD=2.44). Inter rater reliability was poor for all domains (Krippendorff’s alpha ranged from r=0.06 for erythema and r=0.32 for vocal cord oedema). Intra-rater reliability was better, ranging from r=0.19 (for erythema) to r=0.46 (for granuloma), far less than the level required for a reliable staging system (r=0.8).
Conclusion
RFS scores varied considerably for identical clips, domains within the system were subject to very substantial inter and intra-rater variability. These data suggest that the RFS cannot be applied to children in a consistent way.
References 1) Belafsky PC, Postma GN, Koufman JA. The validity and reliability of the reflux finding score (RFS). Laryngoscope. 2001 Aug;111(8):1313-7.
Quality of Life in Obstructive Sleep Apnoea Following Adenotonsillectomy
Steven Powell1, Mike Tremlett2, Derek Bosman1 1 Department of Otolaryngology, 2 Department of Anaesthesia, James Cook University Hospital, Middlesbrough
Introduction
Assessments of quality of life in obstructive sleep apnoea are not widely used in the UK. The OSA-18 is a quality of life tool which has been previously validated and correlates with polysomnographic data. The aims of this study were to use the OSA18 in the assessment of UK children with OSA who were planned for adenotonsillectomy, and to determine the change scores and effect sizes for the tool post surgery.
Methods
25 children were recruited. A clinical history consistent with obstructive sleep apnoea or polysomnographic diagnosis was required. The OSA-18 was administered to the child’s parent pre-operatively, then at the post operative visit. The OSA-18 score range is 1 to 7. Data was analysed using a paired t-test for change score and effect sizes calculated with 95% confidence intervals.
Results
22 children had complete data (mean age 61 months). 11 had pre-operative polysomnography. 21 children had adenotonsillectomy (1 tonsillectomy). Median follow up was 8 weeks (inter-quartile range 6 to 11). Overall change score mean improvement following surgery was 2.57, p<0.0001 effect size 2.35 (95% confidence interval 1.91 to 2.80). There were significant improvements in each of the individual domains: sleep disturbance (change score mean 3.85, p<0.0001), physical suffering (2.23, p<0.0001), emotional distress (2.05, p=0.0001), daytime problems (1.76, p=0.0001), caregiver concerns (2.63, p<0.0001).
Conclusion
The OSA-18 is a useful adjunct to clinical and polysomnographic assessment of children with obstructive sleep apnoea. It has shown significantly improved change score means and effect sizes across all domains in this sample.
Tracheobronchial foreign body aspiration in children: an audit of 22 cases at the Royal Hospital for Sick Children, Glasgow
Joana Riley1, Haytham Kubba2, 1Glasgow University; 2Royal Hospital for Sick Children, Glasgow
Introduction
Foreign body aspiration (FBA) is a significant problem in children, and official figures suggest a not insignificant mortality rate. Previous studies have shown clinical findings and investigations do little to predict the presence of a true foreign body in suspected cases. Our aim was to evaluate the clinical characteristics of children with suspected FBA in our centre, and identify the negative bronchoscopy rate in this group. We also aimed to identify any factors which may help predict a positive finding.
Methods
A retrospective review of case records of children admitted following suspected foreign body aspiration who underwent diagnostic bronchoscopy between January 2005 and June 2007.
Results
A total of 22 bronchoscopies were examined and a foreign body was identified in 6 cases. The negative bronchoscopy rate was 73%. The most common presenting symptom was that of a choking crisis. The presence of wheeze (sensitivity 67%, specificity 100%) and abnormal auscultation (sensitivity 83%, specificity 81%) were the most reliable predictive factors for true FBA. In children where the type of suspected foreign body was unknown and who had a normal physical examination, bronchoscopy was negative in 100%.
Conclusion
Despite the low threshold for investigation, the overall number of cases of suspected FBA is low. While the workload of bronchoscopy performed for FBA is probably not unnecessarily high, from this series no one variable can predict true FBA with full certainty. However, in a child with wheeze and a positive choking history the diagnosis should be strongly suspected.
Sclerotherapy versus Surgery in Paediatric Cystic Hygroma
Simone Boardman, Marlene Soma, Lesley Cochrane, Benjamin Hartley Great Ormond St Hospital for Children, London
Introduction
Cystic hygromas are lymphatic malformations that may affect any anatomic site in the human body. Most lesions are clinically evident by the age of 2, with approximately 75% of lesions occurring in the head and neck region. Morbidity is related to the site, size and nature of the lesion. In addition to cosmetic disfigurement, children with this condition may suffer from airway compromise, feeding issues and speech impairment, recurrent infective exacerbations and impingement on adjacent structures.
Methods
Retrospective case note review of the management of children with cystic hygroma between 2001 and 2008.
Results
108 patients were identified (60 male, 48 female) with the diagnosis of cystic hygroma during the study period. 15% of patients had been treated at other centres prior to their presentation at Great Ormond St Hospital. The majority of patients presented with neck involvement and varying degrees of extension into adjacent sites (face, floor of mouth, tongue, parotid region and mediastinum). The predominant disease type was macrocystic. Supportive management included tracheostomy, gastrostomy formation and CO2 laser to mucosal surfaces. Definitive treatment was individualised for each patient depending on the site and severity of the symptoms: observation only (12%), sclerotherapy alone (24%), surgery alone (45%) or combined therapy (19%). The outcome of these management groups is analysed.
Conclusion
Paediatric cystic hygroma is a challenging condition to manage. We advocate a stepwise approach to treating children with this diagnosis to improve their quality of life and to minimise potential harm.
Paediatric lipomas and lipoblastomas – a case series
Rebecca Dawson1, Marlene Soma1, Ben Hartley1 1Great Ormond Street Hospital for Children, London
Introduction
Lipoblastoma are rare tumours arising from embryonic fat, which can be locally expansive, infiltrative and recurrent. This case series explores clinical and radiological features in order to aid identification.
Methods
A retrospective analysis of a series of 9 patients with lipomas or lipoblastomas excised at GOSH.
Results
5 male and 4 female patients aged 4 to 52 months (mean 1 year 9 months) were identified, of whom 6 had lipoma and 3 lipoblastoma identified on histology. All presented with masses of the head and neck -7 lesions in the neck, 2 in the nasal septum and one in the parotid -some of which proved difficult to diagnose clinically or radiologically. Features of presentation, radiological assessment and histology are discussed, as well as a literature review of paediatric otolaryngological lipoblastoma.
Conclusion
Though rare, lipoblastoma is an important differential diagnosis to consider in the paediatric age group. The potential for local infiltration as well as site-specific problems resulting from expansion mean that these lesions require aggressive management. Surgical excision is the treatment of choice, allowing histological confirmation of diagnosis and clearance. However this can be difficult due to site and limited access, as well as potential risk to head and neck structures.
Hypercapnia monitoring – a new tool in the otolaryngologists repertoire.
Donne AJ, Pal P, Arnavout L, Hore I. Evelina Children’s Hospital, St.Thomas’ Hospital, London
Introduction
Transcutaneous CO2 monitoring is a relatively new technology, allowing CO2 to be monitored in a similar way to O2 saturations. A probe similar to an ECG dot is placed on the skin, connected to a portable monitor. Trends including hypercapnic peaks are captured. Four complex airway cases are presented where its use has directly guided management.
Method Case note review.
Results
In a 6 week old with chromosome 1q36 deletion, and multiple level airway obstruction, a decision to proceed to tracheostomy was guided when despite daytime random blood gases recording CO2 <7kPa, trancutaneous CO2 monitoring identified a peak of 12 kPa at night. In a 15 month old with very severe obstructive sleep apnoea (initial oxygen saturation dips to 35%), a decision not to proceed to tracheostomy was confirmed when nasopharyngeal airway not only improved saturations, but also kept transcutaneous CO2 peaks overnight at <7kPa. In a 2 year old on intermittent Bi-PAP via a tracheostomy for previous bronchiectasis, a decision was made not to decannulate yet as transcutaneous monitoring of CO2 off Bi-PAP found peaks of 8kPA despite normal oxygen saturations. In a 12 year old on intermittent Bi-PAP via a tracheostomy for congenital central hypoventilation syndrome, it was possible to allow her out of the hospital during the day, coming back when her CO2 started to rise.
Conclusion
Hypercapnia monitoring is valuable tool that is easy to interpret and use. We envisage an increasing role of such monitoring.
The management of first branchial cleft anomalies
Jerome Lim, Marlene Soma, Benjamin Hartley Great Ormond Street Hospital, London
Introduction
First branchial cleft anomalies are a group of uncommon congenital malformations of the head and neck. The clinical manifestations of these anomalies often involve infection, and inadequate treatment frequently leads to recurrent problems. Surgical treatment of these lesions carries the risk of injury to the facial nerve. We present our experience of treating patients with first branchial cleft anomalies.
Methods
A retrospective review of patients referred to Great Ormond Street Hospital for management of their first branchial cleft anomalies between 1997 and 2008.
Results
15 patients were identified and we will discuss their presentation, investigation and treatment.
Conclusion
Incomplete closure of the first branchial cleft may result in the formation of a sinus, cyst or fistula. The variety of symptoms and signs associated with these anomalies may lead to misdiagnosis, and careful clinical assessment combined with imaging is necessary to define their true nature and extent. Successful treatment of first branchial cleft anomalies generally requires complete surgical excision. An understanding of the embryology and anatomy of these lesions will provide the basis for successful surgical removal whilst minimising the risk of iatrogenic injuries.
A cross-sectional survey of chronic rhinosinusitis symptoms and disease-related quality of life in patients with primary ciliary dyskinesia
F. Kay Seymour1,2, Matthew Rollins2, Reza Nouraei2, Jonny Harcourt1,2, Claire Hogg1 Royal Brompton Hospital, London1 Charing Cross Hospital, London2
Introduction
Optimal management of chronic rhinosinusitis due to mucous stasis in patients with primary ciliary dyskinesia (PCD) remains debatable. We undertook a cross-sectional study of paediatric PCD patients to quantitatively investigate the clinical impression that these children tend to "grow out" of their sinonasal disease, to help better inform clinical practice.
Methods
Disease specific quality of life was assessed in a sample of 34 children using the validated ‘Kay and Rosenfeld’3 questionnaire which was administered to the children or their parents. None of the patients had prior surgery. Distributional normality of the data was assessed using Kolmogorov-Smirnov statistic and Spearman’s rank correlation was used to investigate the relationship between age and quality of life across individual instrument domains as well as overall score and quality of life. The results were controlled for oral and topical steroid use and antibiotic use.
Results
There were 6 patients aged 0-3y, 9 patients aged 4-7y, 12 patients between 8 and 11 years of age and 7 patients 12-15y. A significant negative correlation (symptom improvement) was found between age and symptoms of sinusitis (correlation=-0.408, p=0.0189), and the multidimensional questionnaire score (correlation=-0.397, p=0.0227). No correlation was found with quality of life, nasal obstruction, activity limitation, or emotional distress and age.
Conclusion
Paediatric PCD patients experience an improvement in symptoms of sinus infection and mean rhinosinusitis scores with increasing age. The overall quality of life marker was however unchanged, possibly representing progressive pulmonary disease. These results favour a conservative expectant approach to the management of chronic rhinosinusitis associated with PCD. A prospective observational cohort study is needed to confirm these findings.
References
3 – Kay DJ, Rosenfeld RM: Quality of Life for children with persistent sinonasal symptoms. OtolaryngolHead Neck Surg 2003 Jan; 128(1):17-26.