Michelle Wyatt

Branchial anomalies in children

BACKGROUND Branchial cleft anomalies are the second most common head and neck congenital lesions seen in children. Amongst the branchial cleft malformations, second cleft lesions account for 95% of the branchial anomalies. This article analyzes all the cases of branchial cleft anomalies operated on at Great Ormond Street Hospital over the past 10 years. METHODS All children who underwent surgery for branchial cleft sinus or fistula from January 2000 to December 2010 were included in this study. RESULTS In this series, we had 80 patients (38 female and 42 male). The age at the time of operation varied from 1 year to 14 years. Amongst this group, 15 patients had first branchial cleft anomaly, 62 had second branchial cleft anomaly and 3 had fourth branchial pouch anomaly. All the first cleft cases were operated on by a superficial parotidectomy approach with facial nerve identification. Complete excision was achieved in all these first cleft cases. In this series of first cleft anomalies, we had one complication (temporary marginal mandibular nerve weakness. In the 62 children with second branchial cleft anomalies, 50 were unilateral and 12 were bilateral. In the vast majority, the tract extended through the carotid bifurcation and extended up to pharyngeal constrictor muscles. Majority of these cases were operated on through an elliptical incision around the external opening. Complete excision was achieved in all second cleft cases except one who required a repeat excision. In this subgroup, we had two complications one patient developed a seroma and one had incomplete excision. The three patients with fourth pouch anomaly were treated with endoscopic assisted monopolar diathermy to the sinus opening with good outcome. CONCLUSION Branchial anomalies are relatively common in children. There are three distinct types, first cleft, second cleft and fourth pouch anomaly. Correct diagnosis is essential to avoid inadequate surgery and multiple procedures. The surgical approach needs to be tailored to the type of anomaly of origin of the anomaly. Complete excision is essential for good outcomes.

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 decades 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.

Laryngotracheal reconstruction in congenital laryngeal webs and atresias

OBJECTIVE: To review the role of open laryngotracheal reconstruction (LTR) in congenital laryngeal webs and atresias. STUDY DESIGN AND SETTING: A retrospective chart review was undertaken in a specialist otorhinolaryngology unit in an academic tertiary referral paediatric hospital. Twenty-one patients with congenital laryngeal subglottic stenosis (SGS) underwent LTR between 1993 and 2003. Two groups were identified; one group had SGS alone (n = 6) and the other group had SGS associated with a laryngeal web (n = 15). Information recorded included presenting features, classification of lesion, surgery performed, and whether decannulation was achieved. RESULTS: All grades of stenosis (Myer-Cotton classification) and webs of type 2, 3, and 4 (Cohens classification) were recorded. Fifteen patients had a staged reconstruction and 6 patients had a single-stage LTR. Complications included 2 revision procedures, 3 tracheocutaneous fistulas, and 1 death (cause unrelated). All appropriate patients were successfully decannulated at between 3 and 18 months post-LTR (median, 5 months). CONCLUSION: This series illustrates the effective role of open LTR in both congenital SGS alone and that in association with glottic webbing.

Peri-operative complications after adenotonsillectomy in a UK pediatric tertiary referral centre.

OBJECTIVES Adenoidectomy and/or tonsillectomy are commonly performed in tertiary pediatric hospitals for the management of obstructive sleep apnea, often in children with significant comorbidities. This study examines the peri-operative course of a large series of complex patients undergoing such surgery at a major pediatric centre, reporting particularly cases of respiratory compromise requiring intensive care admission, both electively and unplanned. METHODS This study was conducted by the pediatric ENT department at Great Ormond Street Hospital. All children undergoing adenoidectomy and/or tonsillectomy from July 2003 to December 2010 were included in this study. This involved a retrospective review of the case notes and hospital databases, with particular emphasis on those children requiring admission to the pediatric intensive care unit. RESULTS A total of 1735 consecutive admissions for adenoidectomy and/or tonsillectomy (1627 individual patients aged 4-197 months, median 46 months) were included between 2003 and 2010 (998 adenotonsillectomies, 182 tonsillectomies and 555 adenoidectomies). In this group, 999/1627 patients (61.4%) had a diagnosis of sleep disordered breathing or sleep apnea, including 258 who had polysomnography. 407/1627 (25.0%) had no specific comorbidities which were felt likely to influence their surgical outcome. Established high risk factors included age less than 24 months (292), Down syndrome (99), neuromuscular problems (314), craniofacial abnormalities (94), storage diseases (23), morbid obesity (20), cardiovascular disease (133), respiratory disease (261), hemoglobinopathy (76) and coagulophathy (34). 300/1735 admissions were day cases and 1082/1735 were observed for one night. 353/1735 required more than one night in hospital (294 for two to three nights). 7/1735 had primary hemorrhage necessitating return to the operating room, all after tonsillectomy. 41/1735 (38 with major comorbidities) required peri-operative intensive care admission, mostly for respiratory support. Of these, 7 were admitted pre-operatively to intensive care, and 17 were planned post-operative transfers. Only 17/1735 required unanticipated post-operative admission to intensive care. Odds ratio analysis suggested a significantly higher chance of PICU admission in children with particular comorbidities (Down Syndrome, cardiac disease, obesity, cerebral palsy, craniofacial anomalies, mucopolysaccharidoses and hemoglobinopathy) when compared to children without comorbidities. Adenotonsillectomy was associated with a higher risk of PICU admission than adenoidectomy alone, but patient age less than 24 months was not associated with significantly higher rates of PICU admission. There were no peri-operative mortalities in this cohort. CONCLUSIONS The peri-operative course was largely uneventful for the majority of children undergoing surgery during this period, particularly given the high prevalence of sleep apnea and other risk factors in this cohort. Major complications were uncommon, with 2.4% of these selected, typically high risk cases requiring peri-operative intensive care admission. Importantly, only 1% of all admissions required unanticipated transfer to intensive care. This has informed changes in peri-operative management in this unit, with implications for other pediatric tertiary referral centres.

Partial adenoidectomy by suction diathermy in children with cleft palate, to avoid velopharyngeal insufficiency☆

OBJECTIVES Adenoidectomy is indicated for the relief of paediatric nasal obstruction, sleep-disordered breathing and otitis media with effusion (OME). Velopharyngeal insufficiency (VPI) is a rare complication. The main risk factor is the presence of pre-existing velopharyngeal closure-impaired mechanisms, including submucosal or overt cleft palate. Despite possible benefits, adenoidectomy is frequently withheld in such children to avoid VPI. This study aims to demonstrate the efficacy and safety of partial adenoidectomy using suction diathermy in children who previously underwent overt cleft palate repair during infancy, to allow selective resection of tissue and symptom resolution without producing VPI. METHODS Since 1994, 18 patients with previously corrected overt cleft palate have undergone partial adenoidectomy at this centre, for the treatment of nasal obstruction or sleep-disordered breathing, with or without OME. Three had existing VPI following their cleft correction surgery. Selective resection of the adenoid was performed transorally under indirect vision, using a malleable suction coagulator. This allowed exposure of the posterior choanae, leaving the remaining adenoid bulk intact. RESULTS Patients were followed up at 4 weeks, and subsequently at regular intervals (total follow up 30-180 months, median 92 months), including perceptual speech assessment in all cases. All demonstrated symptomatic improvement with respect to the original indications for surgery. None developed worsening hypernasal speech or other features of VPI, and there were no cases of symptomatic adenoidal re-growth. CONCLUSIONS Partial adenoidectomy, employing a variety of methods, has been used successfully in children with submucosal cleft palate. This study demonstrates the safe and effective use of suction diathermy to enable partial adenoidectomy in children who have previously undergone surgical correction of overt cleft palate, allowing symptom resolution without producing VPI.

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.

The role of the nasopharyngeal airway for obstructive sleep apnea in syndromic craniosynostosis.

Syndromic craniosynostosis is known to be associated with obstructive sleep apnea (OSA), which can often present in infancy. Although multifactorial, a predominant contributing factor is midface hypoplasia. Nasal continuous positive airway pressure has proven to be an effective treatment modality but may be poorly tolerated in certain cases. This study looks at the effectiveness of bypassing midface obstruction with a nasopharyngeal airway (NPA). Twenty-seven children with syndromic craniosynostosis with confirmed moderate to severe OSA were initially treated with an NPA. The mean age of NPA insertion was 12.3 months (range, 0.5-48 mo). Seventeen had severe OSA, and 10 had moderate OSA preinsertion. Post-NPA insertion, 26 of 27 children (96%) demonstrated an improvement in sleep severity scores, resulting in 3 with moderate OSA and 24 with mild OSA. There was a significant improvement in mean oxygen saturation, mean number of saturation dips greater than 4% per hour, percentage time spent less than 90% SpO2, and number of pulse rate rises per hour. There were no significant differences in mean pulse rate. The NPA was well tolerated by this patient group, with 24 of 26 children retaining it for at least 6 weeks. We believe that an NPA is therefore an effective first-line treatment modality in the management of OSA in children with syndromic craniosynostosis. It is well tolerated by the patient and may obviate the need for continuous positive airway pressure or tracheostomy.

Impact of long-term nasopharyngeal airway on health-related quality of life of children with obstructive sleep apnea caused by syndromic craniosynostosis.

Obstructive sleep apnea in children with syndromic craniosynostosis is a well-documented entity, and airway management in these group of children is difficult, with well-documented cardiorespiratory and neurodevelopment consequences. Numerous well-documented techniques are available for airway management in this group of children. In our center, the nasopharyngeal airway (NPA) is the first-line treatment. This study aimed to evaluate the improvement in health-related quality of life in this group of children using the Glasgow Childrens Benefit Inventory.We conducted a retrospective postal study of 24 patients with craniofacial disorders who had an NPA inserted as part of their airway management. A 79.2% (n = 19) response rate was obtained. Mean (SD) age was 5.8 (4.1) years. Patients were classified into 3 main groups: Crouzon (n = 11), Apert (n = 6), and Pfeiffer (n = 2) syndromes.There was improvement in 3 of the 4 domains, namely, learning (P = 0.006), vitality (P = 0.003), physical (range, −3 to 8; mean, 2; P = 0.005). There was significant improvement in the sleep study parameters; however, no correlation was found between this and the Glasgow Childrens Benefit Inventory findings. Parents also preferred an NPA as opposed to a tracheostomy when given a choice.Our study shows that NPA confers significant improvement not only in health-related quality of life but also in severity of obstructive sleep apnea as judged by sleep study parameters. The NPA is also well tolerated by patients and is much preferred by parents for airway management for their children as opposed to having a tracheostomy. We think that this should be considered in other centers as first-line management in children with syndromic craniosynostosis.

International Pediatric ORL Group (IPOG) laryngomalacia consensus recommendations.

OBJECTIVE To provide recommendations for the comprehensive management of young infants who present with signs or symptoms concerning for laryngomalacia. METHODS Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). RESULTS Consensus recommendations include initial care and triage recommendations for health care providers who commonly evaluate young infants with noisy breathing. The consensus statement also provides comprehensive care recommendations for otolaryngologists who manage young infants with laryngomalacia including: evaluation and treatment considerations for commonly debated issues in laryngomalacia, initial work-up of infants presenting with inspiratory stridor, treatment recommendations based on disease severity, management of the infant with feeding difficulties, post-surgical treatment management recommendations, and suggestions for acid suppression therapy. CONCLUSION Laryngomalacia care consensus recommendations are aimed at improving patient-centered care in infants with laryngomalacia.

MRI brain abnormalities in cochlear implant candidates: How common and how important are they?

OBJECTIVE To investigate the incidence of abnormal findings on brain MRI in paediatric cochlear implantation candidates. METHODS Retrospective review of brain MRI scans of cochlear implant patients between 2000 and 2009 who underwent MRI brain as part of their pre-operative work-up. RESULTS MRI scans of 162 patients were reviewed (76 female patients and 86 male patients). The mean age at time of MRI scan was 3 years 8 months. Abnormalities were detected/ reported in 49 patients (30%). The total number of abnormalities detected was 51 (two patients had two separate abnormalities each). Of the abnormalities 82% could be related to known pre-existing conditions. 18% of the abnormalities were incidental/unexpected. Incidental/unexpected abnormalities were found in 9 patients (6%). Four of the patients with incidental abnormalities required referral and further investigations (2.5%). The most common abnormality detected was white matter changes (70%). All the white matter changes were related to pre-existing known medical conditions. CONCLUSION At our institution abnormalities detected by pre-operative brain MRI scans on cochlear implant candidates are common (30%). The majority of abnormalities are related to known pre-existing medical conditions. Incidental findings are rare (4%) and approximately half of them required further investigation or referral.