Evaluating the outcomes of children undergoing lacrimal surgery for congenital nasolacrimal duct obstruction with the aim of developing a patient pathway for children presenting to a tertiary paedatric service with epiphora.

OBJECTIVE: Nasolacrimal duct obstruction is the most common cause of epiphora in children. Management has classically focused on probing and syringing followed by stent insertion and dacryocystorhinostomy either externally or endonasally. This study aimed to assess the effectiveness and safety of these procedures. METHODS: This was a retrospective analysis of the outcomes of all patients under the age of 16 who have undergone a surgical lacrimal intervention for nasolacrimal duct obstruction under general anaesthetic over a 10-year period. Outcomes included success rates and complications. RESULTS: A total of 432 patients were identified. Primary probe and syringing had a success rate of 83 per cent. Primary endonasal dacryocystorhinostomy had a success rate of 71 per cent, and revision surgery had a success rate of 82 per cent. CONCLUSION: Endonasal dacryocystorhinostomy is an effective and safe technique for the treatment of epiphora, and this study has provided a suggested protocol for management of patients presenting with epiphora.

Aerosolisation during tracheal intubation and extubation in an operating theatre setting.

Aerosol-generating procedures such as tracheal intubation and extubation pose a potential risk to healthcare workers because of the possibility of airborne transmission of infection. Detailed characterisation of aerosol quantities, particle size and generating activities has been undertaken in a number of simulations but not in actual clinical practice. The aim of this study was to determine whether the processes of facemask ventilation, tracheal intubation and extubation generate aerosols in clinical practice, and to characterise any aerosols produced. In this observational study, patients scheduled to undergo elective endonasal pituitary surgery without symptoms of COVID-19 were recruited. Airway management including tracheal intubation and extubation was performed in a standard positive pressure operating room with aerosols detected using laser-based particle image velocimetry to detect larger particles, and spectrometry with continuous air sampling to detect smaller particles. A total of 482,960 data points were assessed for complete procedures in three patients. Facemask ventilation, tracheal tube insertion and cuff inflation generated small particles 30-300 times above background noise that remained suspended in airflows and spread from the patient's facial region throughout the confines of the operating theatre. Safe clinical practice of these procedures should reflect these particle profiles. This adds to data that inform decisions regarding the appropriate precautions to take in a real-world setting.

Cross-sectional follow-up of voice outcomes in children who have a history of airway reconstruction surgery.

OBJECTIVES: This study reports vocal function in a cross-section of children with subglottic stenosis. Each child had a history of laryngotracheal reconstruction and/or cricotracheal resection surgery. Vocal function was measured using laryngoscopy, acoustic analysis, perceptual evaluation and impact of voice on quality of life. DESIGN: All patients aged >5 years with history of laryngotracheal reconstruction and/or cricotracheal resection surgery at the Scottish National Complex Airways service were invited to participate. SETTING: Data were gathered in the Royal Hospital for Children in Glasgow in a single outpatient appointment. PARTICIPANTS: Twelve of 56 former patients (aged 5-27) provided a voice sample and eleven consented to awake laryngoscopy. All consented for detailed evaluation of their medical records. MAIN OUTCOME MEASURES: Acoustic analysis of fundamental frequency and pitch perturbation was conducted on sustained vowel [a]. Perceptual evaluation was conducted by 4 trained listeners on a series of spoken sentences. Impact on quality of life was measured using the paediatric voice-related quality of life questionnaire. Laryngeal function was descriptively evaluated. RESULTS: Four children had normal voice acoustically, perceptually and in relation to voice-related quality of life. One of these had vocal fold nodules unrelated to surgical history. Two other children had "near normal" vocal function, defined where most voice measurements fell within the normal range. CONCLUSIONS: Normal or "near normal" voice is a possible outcome for children who have had this surgery. Where there is an ongoing complex medical condition, voice outcome may be poorer.

Current and future therapies for Pseudomonas aeruginosa infection in patients with cystic fibrosis.

Pseudomonas aeruginosa opportunistically infects the airways of patients with cystic fibrosis and causes significant morbidity and mortality. Initial infection can often be eradicated though requires prompt detection and adequate treatment. Intermittent and then chronic infection occurs in the majority of patients. Better detection of P. aeruginosa infection using biomarkers may enable more successful eradication before chronic infection is established. In chronic infection P. aeruginosa adapts to avoid immune clearance and resist antibiotics via efflux pumps, ß-lactamase expression, reduced porins and switching to a biofilm lifestyle. The optimal treatment strategies for P. aeruginosa infection are still being established, and new antibiotic formulations such as liposomal amikacin, fosfomycin in combination with tobramycin and inhaled levofloxacin are being explored. Novel agents such as the alginate oligosaccharide OligoG, cysteamine, bacteriophage, nitric oxide, garlic oil and gallium may be useful as anti-pseudomonal strategies, and immunotherapy to prevent infection may have a role in the future. New treatments that target the primary defect in cystic fibrosis, recently licensed for use, have been associated with a fall in P. aeruginosa infection prevalence. Understanding the mechanisms for this could add further strategies for treating P. aeruginosa in future.

Validation of a family-centred outcome questionnaire for pinnaplasty: a cross-sectional pilot study.

OBJECTIVES: Comparison of pinnaplasty techniques is difficult due to variation in the reporting of outcomes. We aimed to develop a family-centred outcome questionnaire for use after pinnaplasty and assess it for reliability and validity. DESIGN: Cross-sectional study. SETTING: Tertiary referral paediatric otolaryngology centre. PARTICIPANTS: 20 prospective and 71 retrospective pinnaplasty patients and their parent(s). METHODS: The Post-Operative Pinnaplasty Questionnaire (POPQ) was developed after semi-structured interview with families of children undergoing pinnaplasty. Children aged 4-16 were recruited. Three different ear measurements (auriculocephalic angle, helix-mastoid distance and Walker's ratio) were performed pre-operatively. Children were reviewed 3 months post-operatively and asked to complete a POPQ and Glasgow Children's Benefit Inventory with their parent(s). Ear measurements were repeated and data collected on surgical technique and complications. 200 retrospective pinnaplasty patients were posted a POPQ and Glasgow Children's Benefit Inventory and data collected on surgical technique, complication rate and Walker's angle. MAIN OUTCOMES MEASURES: Validity and reliability of the POPQ. RESULTS: Age range was 4-16 (median 12). POPQ correlated well with Glasgow Children's Benefit Inventory (Spearman's rho = 0.776, P < 0.001). There was no correlation of POPQ scores with age, sex, complication or surgical technique. POPQ score was not associated with severe prominence pre-op nor change or degree of prominence post-op. POPQ displayed good internal consistency (Cronbach's alpha 0.850) and ease-of-use scores. CONCLUSIONS: We have developed a family-focused outcome tool for pinnaplasty that displays good face validity, internal consistency and correlation with health-related quality of life and that is simple and easy to use. No correlation was seen with more traditional measures of outcome such as complications or ear measurements. Further refinement and testing of validity and reliability on a larger sample is planned.

An interventional airway delivery service for congenital high airway obstruction.

BACKGROUND: Congenital airway obstruction is rare but potentially fatal. We developed a complex airways interventional delivery team to manage such cases. Antenatal imaging detects airway compromise at an early stage and facilitates the planning of delivery procedures ('ex utero intrapartum treatment' and 'operation on placental support') which maintain feto-placental circulation whilst an airway is secured. METHOD: A retrospective review was performed of cases in which ENT input was required at birth for airway obstruction. RESULTS: Four neonates were delivered before implementation of the service: two were intubated and another two underwent tracheostomy but died in the peri-natal period. Seven neonates were delivered after implementation of the service: six were intubated and one underwent immediate tracheostomy. Five subsequently underwent tracheostomy (three have since been decannulated). One child with multiple congenital anomalies died due to respiratory failure. Airway obstruction was caused by lymphatic malformation, teratoma, costo-craniomandibular syndrome and choristoma. CONCLUSION: In the absence of other anomalies, interventional airway delivery led to reduced mortality and improved outcomes.

Paediatric tracheostomy-An 11 year experience at a Scottish paediatric tertiary referral centre.

AIMS: The aim of this paper was to review the indications, complications and outcomes for tracheostomy at a Scottish paediatric tertiary referral hospital. METHODS: All patients undergoing tracheostomy between January 2001 and September 2012 were identified. A retrospective case note analysis was performed. RESULTS: 111 tracheostomies were done in the study period. The mean number per year was 11 (3-12). Full data was available for 95 patients. There were 56 (59%) males and 39 (41%) females. Age at time of tracheostomy ranged from one day to 15 years, the mean age of tracheostomy insertion was 69 weeks. The majority of patients, 75 (79%), were under one year old when they had their tracheostomy. The most common indication was long-term ventilation (20%), followed by craniofacial abnormality causing airway obstruction (18%), followed by subglottic stenosis (14%). 37% of patients were decannulated. CONCLUSIONS: This series reflects current trends in the indications for paediatric tracheostomy, with chronic lung disease of prematurity being the most common indication.

Socioeconomic influence on orofacial cleft patient care.

BACKGROUND: Cleft lip and palate is the most common craniofacial birth defect in the UK. Orofacial clefts have functional and aesthetic implications requiring intensive multi-disciplinary follow-up to optimise development. Failure to attend follow-up is likely to have a negative impact on patient outcomes. The aim of this retrospective audit is to establish if socioeconomic status influences attendance, DNA and cancellation rates in cleft patients. METHODS AND RESULTS: A retrospective audit of 74 orofacial cleft patients born and operated on at the Royal Hospital for Sick Children Glasgow between 2006 and 2007. There was higher rate of DNA in more deprived social groups-21% (SIMD 1) against 10% (SIMD 5). A higher rate of DNA in cleft lip and palate patients was noted. This group of patients showed a marked difference in attendance between SIMD 1 (38%) and SIMD 5 (78%). CONCLUSION: More deprived areas have a higher outpatient DNA rate for cleft patients resulting in suboptimal follow-up. Ultimately, causation of poorer outcomes in this group is likely to be multi-factorial but the financial implication of travelling to multiple clinics should be considered and it may be that resource reallocation is the answer to address the current inequality of health care provision.

Risk factors for failure of supraglottoplasty.

OBJECTIVES: To assess for identifiable risk factors for failed surgical intervention in children with laryngomalacia. METHODS: Retrospective case note review between September 2007 and March 2012. Case notes were reviewed for demographic data, symptoms, co-morbidities, operative technique, postoperative recovery, complications, length of hospital stay including intensive care unit (ICU) care, and resolution of symptoms. RESULTS: 148 children underwent supraglottoplasty. Case notes were available for 115 (78%) patients. 35% (41/115) of cases were females and 65% (74/115) were male. A bimodal age distribution was observed with peaks at 3 months and 3.5 years. Those over one year of age were more likely to have complications (p = 0.035). There was no significant difference in outcomes for age (p > 0.05). In patients less than one year, reflux symptoms were significantly associated with a higher likelihood of failure of the operation (p = 0.013). Patients under one year with pre-operatively identified comorbid conditions were less likely to have an improvement in breathing (p = 0.002). Cold steel was used in 55% (63/115) of cases, laser only in 17% (20/115) cases and a combination of the two techniques in 28%, (32/115). There was no association between the surgical technique used and complications (p = 0.558). There was no association between improvement in symptoms and surgical technique used (p = 0.560). There was a significant association between delayed post-operative neurological diagnosis and failure of the operation (p < 0.001). 21 (18%) patients required a second procedure. CONCLUSIONS: Pre-operative predictors of failure were patients with reflux symptoms (p = 0.013). Patients that required a second procedure were 37 times more likely to have a delayed diagnosis of an underlying neurological condition. Failure of symptoms to improve after supraglottoplasty should alert the clinician to the possibility of an underlying neurological disorder.

Cholesteatoma has a high prevalence in Turner syndrome, highlighting the need for earlier diagnosis and the potential benefits of otoscopy training for paediatricians.

AIM: Girls with Turner syndrome are prone to cholesteatoma, a serious suppurative middle ear disease. We aimed to confirm its high prevalence in Turner syndrome, identify risk factors and suggest possible strategies for earlier detection. METHODS: We reviewed 179 girls with Turner syndrome between 1989 and 2012 to identify cases of cholesteatoma. RESULTS: Seven girls (3.9%) had cholesteatoma (index girls) and each was compared with three age-matched girls without cholesteatoma (comparison girls). All the index girls had either the 45,X or 45,X/46X,i(Xq) karyotypes. Nine ears were initially affected, with three recurrences in two girls. Median age at first cholesteatoma presentation was 11.9 years (range: 7.5-15.2), with otorrhoea for three (range: one to seven) months in all 12 affected ears. Index girls had a significantly higher proportion of previous recurrent acute (p = 0.007) and chronic otitis media (p = 0.008), chronic perforation (p = 0.038) aural polyps (p < 0.0001) and tympanic membrane retraction (p = 0.0001) than comparison girls. CONCLUSION: Cholesteatoma has a high prevalence in Turner syndrome. Risk factors include 45,X and 46,XiXq karyotypes; a history of chronic otitis media, tympanic membrane retraction and persistent otorrhoea; and older age. Earlier recognition of ear disease is needed and otoscopy training for paediatricians caring for Turner syndrome patients may be beneficial.

Post-operative respiratory distress following primary cleft palate repair.

INTRODUCTION: Infants are obligate nasal breathers. Cleft palate closure may result in upper airway compromise. We describe children undergoing corrective palatal surgery who required unplanned airway support. SETTING: Tertiary referral unit. METHOD: Retrospective study (2007-2009) of 157 cleft palate procedures (70 primary procedures) in 43 patients. Exclusion criteria comprised combined cleft lip and palate, secondary palate procedure, and pre-existing airway support. RESULTS: The children's mean age was 7.5 months and their mean weight 7.72 kg. Eight children were syndromic, and eight underwent pre-operative sleep studies (five positive, three negative). Post-operatively, five developed respiratory distress and four required oxygen, both events significantly associated with pre-operative obstructive sleep apnoea (p = 0.001 and 0.015, respectively). Four desaturated within 24 hours. Five required a nasopharyngeal airway. Hospital stay (mean, 4 days) was significantly associated with obstructive sleep apnoea (p = 0.002) and nasopharyngeal airway insertion (p = 0.017). DISCUSSION: Pre-operative obstructive sleep apnoea correlated significantly with post-operative respiratory distress, supplementary oxygen requirement, nasopharyngeal airway insertion and hospital stay. We recommend pre-operative sleep investigations for all children undergoing cleft palate repair, to enable appropriate timing of the procedure.

Cricoid split for acute subglottic injury in the older child.

OBJECTIVES: To describe our experience of cricoid split in the older child for acquired subglottic stenosis secondary to chemical or thermal burns. METHODS: A retrospective case series. RESULTS: We describe two patients, both two years old, who benefitted from the procedure and had a return to a normal-sized airway. Neither child required a tracheostomy or further airway intervention after the cricoid split. CONCLUSIONS: Laryngotracheal reconstruction (LTR) is the standard treatment for subglottic injuries with associated subglottic stenosis in children, infants and (where possible) neonates. We have found the cricoid split a useful technique in carefully selected older children with acute subglottic injury and associated early subglottic stenosis, where LTR or ballooning is not feasible, where there is limited experience of ballooning, and/or ballooning has failed in the early stages of treatment. Cricoid split is a technique that is part of the airway surgeon's open operative repertoire and therefore should be remembered as a management option.

Congenital nasal pyriform aperture stenosis: a report of 10 cases and literature review.

INTRODUCTION: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of neonatal upper airway obstruction. This study is to review the presentation and management of this uncommon condition in a tertiary paediatric ENT centre in Scotland, United Kingdom. METHODS: Retrospective case note review between 2003 and 2011. A literature search was performed using MEDLINE and EMBASE between April and June 2011 using search terms 'pyriform aperture' in combination with diagnosis and treatment. Suitable references were also selected and reviewed. RESULTS: A total of 10 cases were identified and 50% of cases were managed surgically. Surgical widening of the pyriform aperture was performed at an average of 14 days at birth. Associated malformations include holoprosencephaly, hypopituitarism, cardiac and urogenital malformations. CONCLUSION: CNPAS is a treatable cause of upper airway obstruction. Early recognition is vital to appropriate management. Treatment is either conservative or surgical and depends on the severity of symptoms.

A metabolomics study of cultivated potato (Solanum tuberosum) groups Andigena, Phureja, Stenotomum, and tuberosum using gas chromatography-mass spectrometry.

Phytochemical diversity was examined by gas chromatography-mass spectrometry in tubers of genotypes belonging to groups Andigena, Phureja, Stenotomum, and Tuberosum of the potato, Solanum tuberosum. Polar extracts (mainly amino acids, organic acids, sugars, and sugar alcohols) and nonpolar extracts (mainly fatty acids, fatty alcohols, and sterols) were examined. There was a large range in levels of metabolites, including those such as asparagine, fructose, and glucose, that are important to tuber quality, offering considerable scope for selecting germplasm for breeding programmes. There were significant differences in the levels of many metabolites among the groups. The metabolite profiles of genotypes belonging to Phureja and Stenotomum were similar and different from those of Tuberosum and the majority of Andigena genotypes. There was some agreement with the phylogeny of the groups in that Stenotomum is believed to be the ancestor of Phureja and they are both distinct from Tuberosum. Andigena genotypes could be partially distinguished according to geographical origin, Bolivian genotypes being particularly distinct from those from Ecuador. Biosynthetic links between metabolites were explored by performing pairwise correlations of all metabolites. The significance of some expected and unexpected strong correlations between many amino acids (e.g., between isoleucine, lysine, valine, and other amino acids) and between several nonpolar metabolites (e.g., between many fatty acids) is discussed. For polar metabolites, correlation analysis gave essentially similar results irrespective of whether the whole data set, only Andigena genotypes, or only Phureja genotypes were used. In contrast, for the nonpolar metabolites, Andigena only and Phureja only data sets resulted in weaker and stronger correlations, respectively, compared to the whole data set, and may suggest differences in the biochemistry of the two groups, although the interpretation should be viewed with some caution.

Retrograde viral vector-mediated inhibition of pontospinal noradrenergic neurons causes hyperalgesia in rats.

Pontospinal noradrenergic neurons form a component of an endogenous analgesic system and represent a potential therapeutic target. We tested the principle that genetic manipulation of their excitability can alter nociception using an adenoviral vector (AVV-PRS-hKir(2.1)) containing a catecholaminergic-selective promoter (PRS) to retrogradely transduce and inhibit the noradrenergic neurons projecting to the lumbar dorsal horn through the expression of a potassium channel (hKir(2.1)). Expression of hKir(2.1) in catecholaminergic PC12 cells hyperpolarized the membrane potential and produced a barium-sensitive inward rectification. LC neurons transduced by AVV-PRS-hKir(2.1) in slice cultures also showed barium-sensitive inward rectification and reduced spontaneous firing rate (median 0.2 Hz; n = 19 vs control 1.0 Hz; n = 18, p < 0.05). Pontospinal noradrenergic neurons were retrogradely transduced in vivo by injection of AVV into the lumbar dorsal horn (L4-5). Rats transduced with AVV-PRS-hKir(2.1) showed thermal but not mechanical hyperalgesia. Similar selective augmentation of thermal hyperalgesia was seen in the CFA-inflammatory pain model after AVV-PRS-hKir(2.1). In the formalin test, rats transduced with hKir(2.1) showed enhanced nocifensive behaviors (both Phase I and II, p < 0.05, n = 11/group) and increased c-Fos-positive cells in the lumbar dorsal horn. Transduction with AVV-PRS-hKir(2.1) before spared nerve injury produced no change in tactile or cold allodynia. Thus, the selective genetic inhibition of approximately 150 pontospinal noradrenergic neurons produces a modality-specific thermal hyperalgesia, increased nocifensive behaviors, and spinal c-Fos expression in the formalin test, but not in the spared nerve injury model of neuropathic pain, indicating that these neurons exert a selective tonic restraining influence on in vivo nociception.

Acute paediatric neck abscesses.

Acute neck swellings in a paediatric setting are often treated with antibiotics, proceeding to incision and drainage if an abscess is suspected. A variety of investigations are available and the causative organism can vary. A five-year retrospective study analysing trends in microbiology, antibiotic sensitivity, clinical evaluation and management of children presenting with an acute cervical abscess of four weeks duration or less was performed. The case notes of 175 children admitted between January 1996 and December 2000 to the acute surgical admission unit at the Royal Hospital of Sick Children, Glasgow were studied. During this period there were 90 males and 85 females with a mean age of three years (range: one month to 13 years). One hundred and twenty three (70%) children underwent surgery with pus being confirmed in 114 (93%) of cases. Ultrasound was performed in 70 (40%) patients with 48 proceeding to surgery. Positive culture of pus from abscess cavities revealed Staphylococcus aureus in 46% (85% sensitive to both flucloxacillin and erythromycin) and Streptococcus pyogenes in 15% (80% sensitive to penicillin and 75% to erythromycin). The role of investigations and the available treatment options are discussed.

Labial pit and ectopic salivary gland tissue.

We report the unusual case of a girl who presented with a lesion on her soft palate and subsequently was found to have a sinus in her labial sulcus. This case report discusses investigation and management of the patient. Further to this we discuss the postulated embryological development of both the parotid gland and labial pit.

Endonasal laser dacryocystorhinostomy: its role in anticoagulated patients.

OBJECTIVE: To report the results of endoscopic laser-assisted dacryocystorhinostomy in anticoagulated patients. STUDY DESIGN: A retrospective study of 16 consecutive anticoagulated patients with distal nasolacrimal duct obstruction treated by endoscopic laser-assisted dacryocystorhinostomy. METHODS: A case note review was made of all patients treated with endoscopic laser-assisted dacryocystorhinostomy who were taking coumadin in two centers between 1993 and 2000. The parameters of age, gender, indications for surgery, surgical findings, complications, and outcome were analyzed. The mean follow-up time was 14 months (range, 9-26 mo). RESULTS: Fifteen of the 16 patients who were treated had an eventual successful outcome, but 6 patients required revision surgery. The patient whose symptoms were not improved was shown to have functional epiphora. No patient had a problem with primary or secondary epistaxis, and no patient required admission. A major benefit was the lack of disruption of anticoagulant therapy. CONCLUSIONS: Endoscopic laser-assisted dacryocystorhinostomy is a safe, efficient technique for the relief of distal nasolacrimal duct obstruction in anticoagulated patients. Not only does it avoid any disruption to their anticoagulant therapy, but it also can be performed as an outpatient procedure.