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.

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.

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.

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.

Nasogastric tube misplacement into Eustachian tube.

Paediatric nasogastric tube placement can occasionally be difficult to perform. A unique case in a child is described where the tip of the nasogastric tube managed to travel via the Eustachian tube through the attic and into the mastoid cavity of the middle ear. To the best of the authors' knowledge, this has never been reported in the literature before.