Management of paediatric sleep-disordered breathing.

Paediatric sleep-disordered breathing is a common condition which varies in severity from snoring to obstructive sleep apnoea. Paediatric sleep-disordered breathing is usually diagnosed clinically, with investigations such as polysomnography reserved for more complex cases. Management can involve watching and waiting, medical or adjunct treatments and adenotonsillectomy. National working groups have sought to standardise the pathway for surgery and improve the management of surgical and anaesthetic complications. Current guidelines use age, weight and comorbidities to stratify risk for these surgical cases. This article summarises these recommendations and outlines the important factors that indicate cases that may be more suitable for management in secondary and tertiary units. Appropriate case selection will reduce pressure on tertiary units while maintaining training opportunities in district general hospitals.

The changing face of paediatric airway endoscopic surgery: An 8-year single surgeon review.

INTRODUCTION: As a recently established division, we sought to reflect on the development of our paediatric airway surgery service, and prospectively examine the diagnoses that underwent microlaryngobronchoscopy (MLB) to help quantify the evolving population demographics of paediatric airway disorders. MATERIAL AND METHODS: This was a prospective longitudinal study conducted of all paediatric MLBs performed by a single surgeon in a tertiary paediatric ENT centre between 2012 and 2019. RESULTS: A total of 1040 MLBs were performed in 498 patients at the paediatric ENT centre of the Royal London Hospital. Median age at first procedure was 19 months. Median follow-up was 48 months. Primary diagnoses were laryngomalacia (21%), subglottic stenosis (SGS - 18%), laryngeal cleft (13%), and normal anatomy (28.3%). Repeat procedures were needed in 39.1% patients, who underwent a median of 2 repeat procedures. SGS (57.7%) constituted majority of the repeat category, followed by laryngeal cleft (12.36%), laryngomalacia (10.15%), unilateral/bilateral vocal cord palsy(4.24%) and laryngeal papilloma(4.24%). Laryngeal papilloma constituted largest number of procedures per patient (Median = 4, IQR = 5.75), followed by subglottic web and SGS. Mean length of stay(LOS) was 0.67 ± 0.96 days(d), with laryngeal cleft cases recording longest mean LOS. There was a steady increase in proportion of day-surgeries across study period [6.9% (2012) vs 59%(2019)]. CONCLUSION: SGS constitutes the major bulk of paediatric airway surgery, reflective of increasing number of premature births and prolonged intubation among neonates. Day-case MLB is a safe and feasible option in selected patients. This long-term data provides useful information to accurately prognosticate patients regarding potential number of repeat procedures for each diagnosis.

The role of antibiotics in the surgical management of paediatric obstructive sleep apnoea (OSA): a cohort study.

PURPOSE: Obstructive sleep apnoea (OSA) describes an irregular night-time breathing pattern that is present in approximately 1.8% of children and can have a negative impact on quality of life. The use of antibiotics postoperatively is controversial. They are commonly prescribed; however, they can also be associated with side effects and resistance. This study explores the role of antibiotics in the post-operative management of children with OSA in a cohort of children. METHODS: We conducted a retrospective cohort study of children undergoing surgery for OSA or sleep disordered breathing (SDB) at a tertiary paediatric ENT referral centre from November 2018 to November 2019. RESULTS: This study identified 382 children who had undergone surgical treatment for OSA or sleep disordered breathing (SDB); 319 underwent adenotonsillectomy, 53 adenoidectomy and 10 tonsillectomies. Antibiotics were given post-operatively to 158 (41%) patients and 18 (11%) of these patients presented to hospital with post-operative complications. A higher number of patients re-presented to hospital from the group who did not receive antibiotics (p = 0.982). Bleeding (p = 0.886) and infection (p = 0.823) were also more common in those children who did not receive antibiotics. CONCLUSION: Antibiotics led to fewer complications and re-presentations to hospital in children undergoing operative management of OSA; however, this trend was not found to be statistically significant.

Prematurity and associated future paediatric airway pathology: experience from a single tertiary paediatric ENT centre.

PURPOSE: Enhanced management of the pre-term patient has resulted in improved survival rates in increasingly premature patients. Although prematurity predisposes to congenital airway pathology, there is also increased risk of endotracheal intubation, and therefore acquired subglottic pathology. We sought to evaluate airway pathology in children outside the neonatal period with a history of prematurity to explore the relationship between prematurity and upper airway pathologies. METHODS: Data for patients undergoing elective microlaryngobronchoscopy (MLB) at our centre were collected prospectively over a 5-year period. Patients identified as premature were sub-classified by the grade of prematurity. RESULTS: 339 patients over 1 month of age underwent MLB, of which 56 (16.5%) were born prematurely. Of those with identified airway pathology, 49 (23.4%) were born prematurely, accounting for 32.6% of subglottic stenosis (n = 30), 24% of laryngomalacia (n = 13) and 19% of laryngeal cleft diagnoses (n = 16). 49 premature patients (87.5%) had one or more airway pathologies diagnosed. Multi-level airway pathology was seen in twelve premature infants (21.4%), demonstrating a statistically significant association (odds ratio 3.396; 95% CI 1.697-6.842; p value < 0.0016). Incidence of airway pathology, the severity of airway disease and multi-level airway pathology were not related to the grade of prematurity. CONCLUSIONS: Premature patients account for a significant proportion of the workload within our tertiary centre due to improving neonatal care and survival in pre-term infants. We suggest early paediatric ENT evaluation for ex-premature patients with symptoms of airway pathology, with a low threshold for MLB. Improving neonatal survival rates in ever-increasing prematurity will require the further provision of specialist paediatric ENT services to manage their ongoing care.

Paediatric aneurysmal bone cyst: not as easy as ABC.

Aneurysmal bone cysts (ABCs) are expansile cystic lesions that can affect any bone of the body. Whilst these lesions are histologically benign, the lesions are locally aggressive and can affect the integrity of the affected bone as well as surrounding structures. ABCs arising in the head and neck region, particularly the paranasal sinuses are rare and they are limited to case reports in the literature. Due to the proximity of critical anatomical structures and the visual apparatus, the potential complications can be devastating. The present article discusses both the clinical and radiological findings of an ABC arising from the ethmoid sinuses in a 6-year-old child and the potentially challenging diagnosis with its complex ensuing surgical management. The identification of an ABC arising in the paranasal sinuses is both a diagnostic and surgical challenge and ideally requires complex management in a joint paediatric ENT and craniofacial unit.

Paediatric subglottic stenosis - Have things changed? Our experience from a developing tertiary referral centre.

BACKGROUND: Paediatric airway disorders are common, particularly in the context of improved ventilation methods for neonates in intensive care units. Management is not standardised. OBJECTIVES: To assess the number, severity, management and outcomes of all patients diagnosed with subglottic stenosis at a developing tertiary referral centre. STUDY DESIGN: 19 month prospective longitudinal study. STUDY POPULATION: All patients who underwent microlaryngobronchoscopy (MLB) were included. Subglottic stenosis (SGS) was graded intraoperatively using the Myer-Cotton classification. RESULTS: 102 patients underwent MLB during this period. 33 of 102 patients (32.4%) were diagnosed with SGS+/-other co-pathologies. Mean and median age at diagnostic procedure were 24.7 months (SD 23.5) and 18 months, respectively. At their first MLB, 22 of 33 patients (66.7%) were found to have a Grade 1 SGS, 7 of 33 (21.2%) were Grade 2 and the remaining 4 of 33 (12.1%) were Grade 3. We had no patients with Grade 4 SGS. During this period, these patients with SGS underwent 73 MLBs+/-interventions (2.21 per patient) such as incision and balloon dilatation, tracheostomy (2 of 33) or ultimately, laryngotracheal reconstruction (LTR) (2 of 33). A further 3 patients have since undergone LTR. No significant unexpected events occurred. CONCLUSIONS: These findings suggest that subglottic stenosis may be evolving in terms of its presentation and management. Management can more often be endoscopic and perhaps avoid tracheostomy or laryngotracheal reconstruction. Further long term prospective studies are required.

The changing face of the paediatric microlaryngobronchoscopy (MLB): A two year prospective study.

BACKGROUND: Changes in the management and survival of paediatric patients with airway complaints combined with improving survival rates of premature babies have resulted in a different patient population for the paediatric airway surgeon than that previously described in the literature. OBJECTIVES: To examine the presentation, diagnosis, clinical course and outcomes for patients undergoing microlaryngobronchoscopy (MLB). STUDY DESIGN: 2 year prospective longitudinal study. STUDY POPULATION: 210 microlaryngobronchoscopy examinations were performed on a total of 102 patients. Mean age at initial examination was 29.4 months with a male preponderance (68%). RESULTS: 72 (71%) patients had other documented medical co-morbidities with 30 children having no previous medical history. Of the 102 patients the primary diagnoses were: Subglottic Stenosis (29.4%), Laryngomalacia (20.6%), Laryngeal Cleft (16.7%), Normal Anatomy (11.8%) and Vocal Cord pathology (5.9%). The average rate of diagnoses per patient for the whole cohort was 1.57. Of those patients with a diagnosis on examination, 40 had a solitary diagnosis whilst 50 patients (55.5%) were found to have multiple diagnoses, equating to 2.35 diagnoses per patient. Children with a history of prematurity accounted for 18.6% of our cohort with a 100% rate of laryngo-tracheal pathology on examination and an average number of diagnoses per child of 2.21. CONCLUSION: Our cohort illustrates the varied population served by today's paediatric airway surgeon alongside common diagnoses and co-pathologies affecting our patients.

Conservative management of bilateral choanal atresia? Bilateral choanal atresia diagnosed in a 5-year-old girl.

Choanal atresia (CA) is an obliteration or blockage of the posterior nasal aperture, limiting or obstructing the nasal airway to the nasopharynx. The presentation of unilateral CA commonly occurs at a later age than those children affected by bilateral CA (BCA) and can often persist undiagnosed into adulthood. BCA, on the other hand, typically presents with respiratory obstruction within the first few days of life, or desaturations with or without cyanosis when feeding or during exertion and traditional teaching is that this is a life-threatening emergency. We present the case of a 5-year-old girl referred to our department with nasal obstruction, snoring and some mild rhinorrhoea. After investigation she was found to have a mixed bony and membranous BCA and no other craniofacial abnormalities. The BCA was subsequently repaired using urethral dilators and a drill and the child is currently asymptomatic of any restenosis. This case prompts discussion of the various presentations and options in management of CA as well as allowing us an opportunity to discuss the literature on the subject.

Paediatric adenotonsillectomy as a daycase for obstructive sleep apnoea: how we do it in a tertiary unit.

BACKGROUND: Paediatric adenotonsillectomy is a common ENT operation. Daycase surgery for uncomplicated, elective procedures is encouraged in order to improve efficiency in healthcare. For patients with obstructive sleep apnoea (OSA), most units advocate an overnight stay for adenotonsillectomy, a procedure usually performed as a daycase in other contexts. METHODS: A retrospective casenote review was carried out from 1st December 2011 to 1st December 2012 for all children undergoing daycase adenotonsillectomy for treatment of OSA at Bart's Children's and the Royal London Hospital. RESULTS: 250 children underwent adenotonsillectomies for OSA as daycase procedures over twelve months. 6% had immediate, unplanned overnight admissions. 3% were readmitted within 30 days. No patients readmitted required surgical intervention. CONCLUSION: For an appropriately selected child, adenotonsillectomy can be safely performed as a daycase procedure in a tertiary centre.

Severe obstructive sleep apnoea due to adenotonsillar hypertrophy after liver transplantation.

Epstein-Barr virus-related adenotonsillar hypertrophy is a precursor to post-transplantation lymphoproliferative disorder. We report a case of a 4-year-old child with severe obstructive sleep apnoea, who had liver transplantation at the age of 7 months. She had gross lymphoid hypertrophy in the oropharynx and supraglottis. We performed an adenotonsillectomy and aryepiglottoplasty which improved her symptoms. We emphasise the importance to consider the diagnosis of post-transplantation lymphoproliferative disorder in post-transplantation immunosuppressed patients who present with adenotonsillar hypertrophy.

The successful use of the nasopharyngeal airway in Pierre Robin sequence: an 11-year experience.

INTRODUCTION: Pierre Robin sequence (PRS) is a congenital anomaly presenting with micrognathia, glossoptosis and a cleft palate. This study describes a decade's experience of the management of upper airway obstruction (UAO) in PRS patients with a nasopharyngeal airway (NPA). METHODS: This study was conducted by paediatric respiratory and otolaryngology departments. Children with PRS referred with UAO were evaluated according to a standard protocol. Data collected included the degree of airway obstruction, method of airway management, polysomnography data before and after intervention, and longer term follow-up. RESULTS: Data were collected on 104 PRS patients referred to us for airway assessment in 2000-2010. 64/104 were aged <4 weeks at referral. Airway symptoms were managed conservatively in 27 patients (25.9%), with an NPA in 63 (60.6%) and a tracheostomy in 14 (13.4%). The average duration of NPA use was 8 months (3 weeks to 27 months). Polysomnography results improved in all 63 patients with an NPA. Fourteen severely obstructed patients underwent a tracheostomy. 86.5% (90/104) of PRS patients were managed conservatively or with the help of an NPA. There were no NPA related complications. CONCLUSION: There is a spectrum of UAO in PRS. This study reports on long-term outcomes in 104 children with PRS and airway obstruction. In most children (86.5%), airway obstruction was managed by conservative measures or with an NPA for a few months. The natural history shows that with normal growth, airway compromise resolves without immediate surgical intervention as advocated by some practitioners. Few PRS children require a tracheostomy.

Surgical aspects of cochlear implantation in syndromic children.

OBJECTIVE: The objective of this study was to report surgical results and outcomes of cochlear implantation in a large series of children with syndromes from one centre. PATIENTS AND METHODS: All syndromic children who underwent cochlear implantation at Great Ormond Street Hospital, from January 2000 to December 2010 were included in this study. The surgical technique was analysed and audiological outcomes were collected. RESULTS: Over the 10-year period of this study, a total of 88 cochleas in 67 children with syndromes were implanted. The common syndromes implanted in this study were Ushers syndrome (23 patients, 33 cochleas), Wardenburgs syndrome (8 patients, 9 cochleas), Pendreds syndrome (4 patients, 4 cochleas), Jervell-Lange-Neilsen syndrome (3 patients, 4 cochleas), Enlarged vestibular aqueduct syndrome (4 patients, 7 cochleas), Cogans syndrome (3 patients, 4 cochleas), CHARGE (5 patients, 6 cochleas), and Branchio Oto Renal syndrome (3 patients, 4 cochleas). Pre-operative radiological inner ear anatomy was found to be abnormal in 28.4% (25/88) cochleas in this study group. Full insertion of the electrode was achieved in 93.1% (82/88) of cochleas, partial insertion in three cochleas, and insertion was abandoned in three cochleas. Early complications were seen in 6.8% (6/88) of implantations. All the 64/67 children who were implanted are still using the implant. CONCLUSION: Cochlear implantation in syndromic children is challenging in both its audiological and surgical aspects. Good surgical results and good audiological and speech outcomes were achieved in this study, and subjective improvement in quality of life was achieved in these patients.

Long-term results with a simple technique of stoma creation after laryngectomy.

Stomal stenosis after laryngectomy has a major impact on patient rehabilitation. The major contributory factor is the operative technique. The goal is to achieve a widely patent stoma. The objective of the study was to review the long-term results of the technique of stoma creation used by the senior author (CJW). The simple technique, using skin triangles for tracheostoma creation after laryngectomy, has been used by the senior author for past 6 years. Review of 59 cases of total laryngectomy with or without partial pharyngectomies in whom this technique was used for stomaplasty. The technique described for stoma creation after total laryngectomy has been used in 59 patients (48 male, 11 female) over 6 years. The follow-up of these patients varies from 6 months to 6 years. Of these patients 54/59 (91.5%) did not have any problems with the stoma. A total of 5/59 (8.5%) patients, the stoma was narrow. Amongst these patients with narrow stoma, only 1 patient (1.7%) has had dilatation done and the others are managing reasonably with a stoma button. None of the patients in this study has required revision procedures on the stoma. The simple technique of stoma creation described in this study has good long-term results.

Clinical outcomes of total laryngectomy for laryngeal carcinoma.

BACKGROUND: Controversy exists as to the management of advanced laryngeal carcinoma. In general primary radical surgery is favoured. OBJECTIVE: The aim of this study was to analyse the clinical outcome of patients having total laryngectomy for cancer of larynx. MATERIALS AND METHODS: This study was a retrospective case note review and questionnaires were used for evaluating voice handicap. These laryngectomies included in this study were performed by the senior author (CJW) from January 2001 till June 2007 at Leeds General Infirmary, Leeds. Some of the patients had partial or total pharyngectomy in addition to total laryngectomy. RESULTS: In this study a total of 59 patients were included. Seventeen (28.8%) of these patients had preoperative radiotherapy and laryngectomy was performed for residual or recurrent disease. The initial TNM staging of the tumour ranged from T(1)N(0) to T(4)N(2C). Tracheoesophageal puncture for speech prosthesis was done in 48/59 (81.4%) patients. Post-operative complications were seen in 30.5% (18/59) patients. In this study group 9 patients (15.2%) developed pharyngocutaneous fistulas. For communication 31/51 (60.8%) patients were using speech valves. In this study 30.4% had minimal, 26.1% moderate and the rest 43.4% feeling severely handicapped with regards to voice use after total laryngectomy. Five year survival after laryngectomy in this study was 65.2%. CONCLUSION: Long term disease control and survival is achievable with total laryngectomy with or without postoperative radiotherapy with minimal risks in patients with advanced carcinoma of larynx.