Long-term Airway Outcomes and Interventions in Children With Oesophageal Atresia With Tracheoesophageal Fistula: A 20-year Single Centre Observational Study.

BACKGROUND: Airway anomalies, symptoms and interventions are commonly reported in children with oesophageal atresia with tracheoesophageal fistula (OA/TOF). The purpose of this study was to assess the incidence of these airway pathologies and those requiring interventions in the long-term. METHODS: A retrospective case note review of all patients admitted to the Neonatal Unit at the Royal Hospital for Children, Glasgow between January 2000 and December 2015 diagnosed with OA/TOF. Included patients had a minimum of 5 years follow-up. RESULTS: 121 patients were identified. 118 proceeded to OA/TOF repair. 115 patients had long-term follow-up data. Ninety-five (83%) children had one or more airway symptom recorded. Thirty-six (31%) neonates underwent airway endoscopy at the time of their initial OA/TOF repair. Forty-six (40%) children underwent airway endoscopy at a later date due to airway symptoms. Airway pathologies identified included airway malacia, thirty-two (28%), subglottic stenosis, eleven (10%), tracheal pouch, twenty-five (22%), laryngeal cleft, seven (6%) and recurrent fistula, five (4%). Airway interventions included endoscopic division of tracheal pouch, ten (9%), tracheostomy, seven (6%), aortopexy, six (5%), repair of recurrent fistula, five (4%), endoscopic repair of laryngeal cleft, three (3%) and four (3%) required open airway reconstruction for subglottic stenosis. One child (1%) remains tracheostomy dependent. CONCLUSIONS: Long-term airway pathologies are common in children with OA/TOF. Many of these are remediable with surgical intervention. Clinicians should be cognisant of this and refer to Airway Services appropriately.

Outcomes and complications of simple layered closure of persistent tracheocutaneous fistula after tracheostomy in childhood.

BACKGROUND: Up to half of all children who have a tracheostomy will develop a persistent tracheo-cutaneous fistula (TCF) after decannulation. Surgical closure of the TCF is technically easy but post-operative complications can be immediate and life-threatening. These include air leak from the tracheal repair leading to massive surgical emphysema or pneumothorax. We reviewed our experience of TCF closure to try to identify potential risk factors for complications. METHOD: Retrospective case record review of all children (0-16 years) who underwent surgical TCF closure between January 2010 and December 2021 following development of a persistent TCF after decannulation of a tracheostomy. RESULTS: We identified 67 children. They ranged in age from 14 months to 16 years (median 3 years 10 months) at the time of the TCF closure. Major medical comorbidities were present in 90%. Pre-operative pulse oximetry with the fistula occluded was used in 29 children (43%). An underwater leak test was performed in 28 (42%). A non-suction drain was used in 29 children (43%). Prophylactic antibiotics were prescribed for 30 children (45%). Post-operative complications occurred in 15 children (22%). Life-threatening air leak occurred in the immediate post-operative period in 2 children (3%). Respiratory distress occurred in 3 children (4%) in the recovery area immediately after surgery. None required re-tracheostomy. Three children suffered post-operative pneumonia (4%), and wound infections occurred in 8 children (12%). We were unable to show a significant association between patient or surgical factors and complications. DISCUSSION: Complications for TCF closure are unfortunately common and it is unclear from the available evidence how best to prevent them. Further research is required.

Tracheostomy decannulation at the Royal Hospital for Sick Children in Glasgow: Predictors of success and failure.

BACKGROUND: Tracheostomy techniques, indications and care are extensively covered in the literature. However, little is written about the process of removing the tracheostomy tube. At the Royal Hospital for Sick Children in Glasgow we use a stepwise ward-based protocol for safe tracheostomy decannulation. Our aim therefore was to review all the paediatric tracheostomy decannulations that we attempted over the last 3 years to evaluate our protocol, to determine our success rate and to see whether any modifications to the protocol are required. METHOD: We reviewed all patients who had undergone ward decannulation between January 2012 and May 2015. We extracted data from clinical records including patient characteristics, indications for tracheostomy, timing of decannulation and success or failure of the process. RESULTS: The 45 children in the study underwent 57 attempts at decannulation during the study period. 25 were male (56%) and 20 were female (44%), and they were aged between 1 day and 16 years 6 months at the time of the original tracheostomy operation. 33 attempts were successful (58%). 10 children had more than one attempt at decannulation. Children were found to fail at every stage of the protocol, with the commonest point of failure being day 2 when the tracheostomy tube was capped. DISCUSSION: We have demonstrated that our current protocol for ward decannulation is effective and safe, and that all five days of the protocol are required.

Early identification of pituitary dysfunction in congenital nasal pyriform aperture stenosis: recommendations based on experience in a single centre.

BACKGROUND: Congenital nasal pyriform aperture stenosis (CNPAS) is an increasingly recognised cause of upper airway obstruction associated with midline abnormalities. Studies have described pituitary dysfunction in 40% of patients. We aimed to develop guidelines for: (a) the early identification of pituitary insufficiency to minimise surgical risk and (b) to stratify patients for follow-up. METHODS: Retrospective case note review of patients with CNPAS between 2000 and 2014 in a tertiary paediatric unit. RESULTS: 20 patients (12 female:8 male) were analysed; 16 were diagnosed during the neonatal period while 4 were diagnosed later. There was no consistent approach in the evaluation of the pituitary axis at diagnosis. Pituitary dysfunction was identified in 3 (15%) patients, 2 of whom were found during evaluation of short stature in mid-late childhood. Hypoglycaemia and conjugated hyperbilirubinaemia, but not the degree of stenosis, were highly predictive of pituitary dysfunction (p < 0.05). Available height standard deviation score (SDS) data at 1 year of 70% of our patients identified both of the late-diagnosed growth hormone-deficient patients, with SDS of -2.6 and -3.6, respectively. CONCLUSION: All CNPAS patients should have MRI of the brain and baseline endocrine investigations at diagnosis. Growth monitoring for at least 1 year is recommended as low, or falling, height SDS at 1 year is a good predictor of pituitary dysfunction.

Management of congenital cartilaginous sleeve trachea in children.

AIMS: Children with congenital tracheal cartilaginous sleeve may present to otolaryngology services with airway problems. We wish to describe our overall management in a series of four children with this very rare anomaly. METHODS: Retrospective case note review of children diagnosed with congenital tracheal cartilaginous sleeve presenting to our department between 2006 and 2014. RESULTS: Four patients were seen. One had Opitz G syndrome, two had Pfeiffers syndrome and one had no associated anomalies. Two children were successfully managed with laryngeal reconstruction using an anterior costal cartilage graft, while the third and fourth required a short period of tracheostomy only. All four are well and currently asymptomatic from an airway point of view. CONCLUSION: Congenital tracheal cartilaginous sleeve is a very rare and potentially challenging problem. Otolaryngologists should be aware that it can occur in children with syndromes other than craniosynostosis (and indeed, those with no syndrome) and that it can be successfully treated using established airway management techniques. ETHICAL APPROVAL: Registered with Clinical Governance Committee.

Otolaryngological presentations of Cornelia de Lange syndrome.

AIM: Children with Cornelia de Lange syndrome frequently present to otolaryngology services with hearing problems. Airway problems have not previously been reported. We wish to describe our experience of the overall management in a series of children with Cornelia de Lange syndrome. METHODS: Retrospective case note review of children diagnosed with Cornelia de Lange syndrome presenting to our department between 2005 and 2014. RESULTS: Six patients were seen. Airway problems consisted of laryngeal overspill with severe gastroesophageal dysmotility and reflux despite structurally normal airway (1 case), laryngomalacia requiring supraglottoplasty (2 cases), reflux laryngitis with secondary laryngomalacia and coincidental tracheal diverticulum (1 case) choanal atresia requiring stents (1 case) and obstructive sleep apnoea (1 case). Supraglottoplasty produced a dramatic improvement in feeding and breathing in both children who underwent the procedure. Two children had palatal anomalies and one underwent cochlear implantation for a profound sensorineural hearing loss. CONCLUSION: Children with Cornelia de Lange syndrome have multifaceted ENT problems. Airway pathology has not previously been described in Cornelia de Lange syndrome but has been common in our experience. We wish to highlight that laryngomalacia in Cornelia de Lange syndrome responds well to supraglottoplasty.

The management of laryngeal clefts.

OBJECTIVE: To review the clinical presentation and management of all infants and children presenting with laryngeal clefts to a tertiary pediatric ENT centre and to identify changes in practice over time. PATIENTS AND METHODS: A retrospective case note review of the management of all infants and children with a diagnosis of a laryngeal cleft identified in our Department between 01/11/2003 and 31/12/2010. RESULTS: Twelve children with laryngeal clefts were identified. Six clefts were grade 1, five grade 2 and one grade 3b. All grade 1 clefts were managed conservatively. Of the grade 2 clefts, four required surgery with one being managed conservatively. Two were repaired using an open technique and two using an endoscopic technique. The grade 3b cleft was repaired endoscopically. Two cleft repairs broke down post-operatively requiring further surgery. CONCLUSIONS: Conservative management remains the management of choice for lower grade clefts. Where a laryngeal cleft requires repair there has been a trend towards the endoscopic over open technique, even of more extensive clefts.

Laser division of congenital complete tracheal rings.

Congenital complete tracheal rings are a rare and life threatening problem in young children and they are often challenging to manage. Whilst historically associated with high mortality rates increasing experience with this tracheal pathology has led to much improved survival rates and slide tracheoplasty has become the treatment of choice. We present 3 cases in which an open procedure was not deemed possible and they underwent laser division (CO2, KTP) of their complete rings. Two patients subsequently required stent insertion. All patients are alive and well at a mean follow up of 3 years and 5 months. Whilst laser division of complete tracheal rings has only been described in a small number of cases it may provide an alternative approach in patients who are not able to undergo an open procedure or in an emergency situation.

Unilateral vocal cord paralysis following patent ductus arteriosus ligation in extremely low-birth-weight infants.

OBJECTIVE: To determine if unilateral vocal cord paralysis (UVCP) following patent ductus arteriosus (PDA) ligation is associated with respiratory and swallowing morbidities in extremely low-birth-weight (ELBW) infants. DESIGN: Case-control study. SETTING: Tertiary care neonatal intensive care units and pediatric hospital. PARTICIPANTS: Twenty-three infants undergoing PDA ligation (subdivided into the main study group of 12 infants with UVCP and 11 without paralysis) and 12 weight- and gestational age-matched ELBW controls. MAIN OUTCOME MEASURES: Incidence of UVCP, time requiring supplemental oxygen and ventilatory support, length of hospital stay, incidence and duration of tube feeding following discharge, and incidence of chronic lung disease. RESULTS: The overall incidence of UVCP was 52% (12/23), increasing to 67% (12/18) in ELBW infants. Infants without UVCP following PDA ligation were heavier (P = .006), with a more advanced gestational age (P = .03). Patients with UVCP required longer tube feeding (relative risk, 8.25; 95% confidence interval, 1.93-46.98; P = .003), supplemental oxygen (P = .004), and ventilatory support (P = .001) and had a longer hospital stay (P < .001). In comparison to matched controls, infants with UVCP required longer tube feeding (relative risk, 9.00; 95% confidence interval, 2.08-51.30; P = .003), supplemental oxygen (P = .03), and ventilatory support (P = .002) and had a longer hospital stay (P < .001). CONCLUSIONS: There was a high incidence of occurrence of UVCP (67%) associated with PDA ligation in ELBW infants. Unilateral vocal cord paralysis following PDA ligation does seem to be associated with increased requirements for tube feeding, respiratory support, and hospital stay in these ELBW infants.

Laser epiglottopexy for laryngomalacia: 10 years' experience in the west of Scotland.

OBJECTIVES: To determine the results of laser epiglottopexy and to compare them with other surgical techniques for severe laryngomalacia. DESIGN: A retrospective 10-year case note review of laser epiglottopexy for severe laryngomalacia. SETTING: Royal Hospital for Sick Children, Glasgow, Scotland. PATIENTS: The study population comprised 52 male and 24 female children who underwent surgery for laryngomalacia between January 1, 1993, and December 31, 2002. In all children except 2, the indication for surgery was stridor associated with poor feeding and failure to thrive. The age at presentation ranged from 5 days to 32 months (mean, 16 weeks). Three had neurological problems, in 3 a syndrome was diagnosed, and 2 were described as dysmorphic and no syndrome was diagnosed; 5 had a cardiac abnormality. Complete follow-up data were available for 59 children (78%). Intervention Laser epiglottopexy. MAIN OUTCOME MEASURE: Resolution of stridor and growth velocity. RESULTS: Resolution of stridor was complete in 34 children, with mild residual stridor in 4 and persistent stridor but good weight gain in 5 (73% improved sufficiently with 1 procedure). Improvement was documented objectively by measuring growth velocity. Two children required a brief period of intubation postoperatively, and 4 had postoperative respiratory tract infections. Seven children (12%) required revision endoscopic laser surgery to control symptoms. There were no cases of supraglottic stenosis. One child died of cardiac abnormalities, and 8 (14%) required a tracheostomy, of whom 6 had neurological or syndromal abnormalities and 2 had associated tracheomalacia. CONCLUSIONS: We describe a technique of laser epiglottopexy for laryngomalacia and present objective evidence of its efficacy by means of growth velocity charts in a retrospective review of laryngomalacia surgery over a 10-year period. We believe this to be a safe method for treating a self-limiting condition. We have had comparable success to other surgical techniques without having the risks of permanent scarring to the supraglottis.

A prospective randomized single-blind trial comparing ultrasonic scalpel tonsillectomy with tonsillectomy by blunt dissection in a pediatric age group.

OBJECTIVE: To determine if there was a difference in postoperative pain and dietary intake when comparing ultrasonic scalpel (US) with blunt dissection tonsillectomy (BD) in children. STUDY DESIGN AND SETTING: A prospective single-blind randomized controlled trial comparing US and BD tonsillectomy in children aged between 5 and 13 years who undergo tonsillectomy for recurrent acute tonsillitis in a specialized pediatric hospital. RESULTS: One hundred and twenty-two children were enrolled. Ninety-three (76%) patients completed dietary and pain scales. Pain was significantly greater in the US group on postoperative days 1 and 3 (P = 0.0466, P = 0.0084) and overall (P = 0.0003). Dietary intake scores were significantly better in the US group on days 1, 5, 7, and 9, however, these scores were inconsistent with dietary intake scores declining in both groups throughout the study. Blood loss was statistically significantly greater in the BD group (33.1 ml vs 3.0 ml, P < 0.0001). There were no significant differences for all other observed outcomes. CONCLUSIONS: US tonsillectomy causes significantly more pain overall and on postoperative days 1 and 3 when compared to BD tonsillectomy. US tonsillectomy causes less intraoperative blood loss, the clinical significance of which is questionable. SIGNIFICANCE: US tonsillectomy appears to cause more postoperative pain than BD tonsillectomy in children aged 5 to 13 years undergoing tonsillectomy for recurrent acute tonsillitis.

Endoscopic carbon dioxide laser division of congenital complete tracheal rings: a new operative technique.

A carbon dioxide laser was used through a bronchoscope to split the posterior aspect of complete tracheal rings in the distal trachea of a 16-month-old boy previously palliated for cyanotic congenital heart disease. After laser division of the complete tracheal rings, the patient was successfully extubated. Subsequently, the boy had granulation tissue develop, which required bronchoscopic resection, and then severe posterior tracheal impingement developed from the esophageal herniation, which required placement of a distal tracheal stent. Although unsuccessful in this case, carbon dioxide laser division of complete tracheal rings may be a safe and effective method of treating congenital tracheal stenosis in selected cases.

Implementation by Scottish otolaryngologists of the Scottish Intercollegiate Guidelines Network document Management of Sore Throats and the Indications for Tonsillectomy: four years on.

A postal survey was undertaken to assess otolaryngologists' awareness, implementation and perceived weaknesses of the Scottish Intercollegiate Guidelines Network (SIGN) clinical guideline for the Management of Sore Throat and Indications for Tonsillectomy. The reply rate was 77 per cent. There was high awareness of the guideline and while 84 per cent of the correspondents felt that they followed the guideline, or a guideline based on it, only 35 per cent were able to accurately recall the four main criteria recommended for listing for tonsillectomy. Twenty-five per cent of the surgeons were in departments that had ongoing implementation programmes for these guidelines but only 10 per cent of surgeons were in departments that had audited their compliance. The most common criticism of the guideline (22 per cent) was that it failed to consider the impact of the disease process on the patient's quality of life. Suggestions made to improve the guideline are included.

Intramuscular hemangioma of the posterior belly of the digastric muscle failing to highlight on magnetic resonance imaging.

Intramuscular hemangiomas of the digastric muscle are rare. We present the fifth case reported and the second to be imaged in vivo with magnetic resonance imaging. Accurate preoperative diagnosis of these lesions is difficult, but may be facilitated by a high degree of clinical suspicion and imaging. Magnetic resonance imaging using T2-weighted sequencing has been reported to effectively highlight these lesions. We present a case in which magnetic resonance imaging failed to highlight an intramuscular hemangioma of the posterior belly of the digastric muscle. Possible reasons for this failure are discussed.