Pain in children with Bell's palsy: secondary analysis of a randomised controlled trial.

OBJECTIVE: To describe the prevalence and severity of pain experienced by children with Bell's palsy over the first 6 months of illness and its association with the severity of facial paralysis. METHODS: This was a secondary analysis of data obtained in a phase III, triple-blinded, randomised, placebo-controlled trial of prednisolone for the treatment of Bell's palsy in children aged 6 months to <18 years conducted between 13 October 2015 and 23 August 2020 in Australia and New Zealand. Children were recruited within 72 hours of symptom onset and pain was assessed using a child-rated visual analogue scale (VAS), a child-rated Faces Pain Score-Revised (FPS-R) and/or a parent-rated VAS at baseline, and at 1, 3 and 6 months until recovered, and are reported combined across treatment groups. RESULTS: Data were available for 169 of the 187 children randomised from at least one study time point. Overall, 37% (62/169) of children reported any pain at least at one time point. The frequency of any pain reported using the child-rated VAS, child-rated FPS-R and parent-rated VAS was higher at the baseline assessment (30%, 23% and 27%, respectively) compared with 1-month (4%, 0% and 4%, respectively) and subsequent follow-up assessments. At all time points, the median pain score on all three scales was 0 (no pain). CONCLUSIONS: Pain in children with Bell's palsy was infrequent and primarily occurred early in the disease course and in more severe disease. The intensity of pain, if it occurs, is very low throughout the clinical course of disease. TRIAL REGISTRATION NUMBER: ACTRN12615000563561.

Agreement of Clinician-Administered and Modified Parent-Administered House-Brackmann Scales in Children with Bell's Palsy.

Objective: Currently there is no parent administered scale for facial nerve function in children. We set out to assess the agreement between a newly developed parent-administered modified version of the House-Brackmann (HB) scale and the standard clinician-administered HB scale in children with Bell's palsy. Study Design: Secondary analysis of a triple-blind, randomized, placebo-controlled trial of corticosteroids to treat idiopathic facial paralysis (Bell's palsy) in children (6 months to <18 years). Setting: Multicenter study at pediatric hospitals with recruitment in emergency departments. Methods: Children were recruited within 72 hours of symptom onset and assessed using the clinician-administered and the parent-administered modified HB scales at baseline, and at 1, 3, and 6 months until recovered. Agreement between the 2 scales was assessed using intraclass coefficient (ICC) and a Bland-Altman plot. Results: Data were available for 174 of the 187 children randomized from at least 1 study time point. The mean ICC between clinician and parent HB scores across all time points was 0.88 (95% confidence interval, CI: 0.86, 0.90). The ICC for the data collected at baseline was 0.53 (95% CI: 0.43, 0.64), at 1 month was 0.88 (95% CI: 0.84, 0.91), at 3 months was 0.80 (95% CI: 0.71, 0.87) and at 6 months was 0.73 (95% CI: 0.47, 0.89). A Bland-Altman plot indicated a mean difference between the 2 scores (clinician-reported minus parent-reported) of only -0.07 (95% limits of agreement -1.37 to 1.23). Conclusion: There was good agreement between the modified parent-administered and the clinician-administered HB scales.

Transfer of patients from a regional centre to a tertiary paediatric hospital for acute otolaryngology management: When is it necessary?

AIM: Transfer of patients from a regional to a tertiary paediatric facility is sometimes required for management of otolaryngological conditions when the expertise available locally is insufficient to deal with the complexity of the case. METHODS: A retrospective analysis was carried out over a 3-year period of all patients under 18 years of age who were transferred to the Royal Children's Hospital Melbourne, primarily for otolaryngology management, from a regional hospital with an inpatient paediatric service and located 100 km or far from the Royal Children's Hospital. RESULTS: Thirty-nine patients (30 male, 9 female) were identified. The majority of patients (29, 74%) required surgical management, with the most common procedures being rigid bronchoscopy with or without foreign body removal (7 patients, 24%), drainage of deep neck space infection (7 patients, 24%) and bilateral choanal atresia repair (4 patients, 13%). CONCLUSION: Notwithstanding the cost and inconvenience, transfer was necessary in essentially all cases in this study for either specialist surgical management or medical management of conditions that could have otherwise escalated in acuity. By inference, it would appear that most routine acute paediatric otolaryngology conditions are managed successfully in a regional setting.

Unplanned inter-hospital transfers following elective paediatric surgery in a private hospital.

BACKGROUND: A significant number of surgeries in children are being performed in the private setting. Our aim was to determine the rate of unplanned inter-hospital transfers (IHTs) for paediatric patients undergoing elective surgical procedures in a private hospital without a paediatric intensive care unit to a tertiary hospital, and to investigate the reasons for these transfers. METHODS: A retrospective clinical audit was performed searching hospital coded data of all patients aged 18 years or less at the date of admission, who underwent elective surgery between 1 January 2013 and 31 October 2018 at St Vincent's East Melbourne Private Hospital. RESULTS: A total of 17 366 patients were identified, of whom 23 required IHT, with an overall transfer rate of 0.13%. Adenotonsillectomy had the highest IHT rate of 0.26%; however, operative specialty had no statistical correlation with IHT (P = 0.24) with a comparable transfer rate across all specialties. Hypoxia was the most frequent reason for IHT and was the cause in 16 out of 23 transfers (69%). Nine cases (39%) were transferred due to hypoxia while awake and seven (30%) due to hypoxia only while asleep. Three patients requiring IHT were identified as having preoperative acute respiratory illness. CONCLUSION: Elective paediatric surgery undertaken at St Vincent's East Melbourne Private Hospital is safe and has a low IHT rate, with surgery involving the upper airway having a higher risk. In the paediatric population, hypoxia while awake is the most frequent cause for IHT.

Skull base anatomy and surgical safety in isolated and CHARGE-associated bilateral choanal atresia.

INTRODUCTION: Bilateral choanal atresia (BCA) is associated with a high incidence of congenital abnormalities that include skull base anomalies and defects. Surgical repair of BCA is necessary in the early neonatal period and any altered anatomy of the adjacent skull base will heighten the risk of intracranial injury. This risk may be further increased in patients with CHARGE syndrome. OBJECTIVES: To measure surgically relevant nasal and skull base dimensions in neonates with BCA in order to determine whether any difference exists between isolated and CHARGE syndrome associated subgroups, thereby optimizing the safety of surgical repair. METHODS: A retrospective review of medical charts and computed tomography was undertaken at a tertiary pediatric hospital of all neonates diagnosed with BCA between 2004 and 2016. Isolated and CHARGE syndrome subgroups of BCA were identified from clinical records and CT data was analyzed and compared between the two. The skull base parameters measured were choanal width, choanal height, mid-nasal skull base height and skull base slope. RESULTS: Of the 13 patients included, 3 had CHARGE syndrome and 10 had isolated BCA. Whilst the difference in mid-nasal height approached significance for the two groups, numbers were too small for a statistical difference to be identified. The mean value for choanal width in the isolated BCA group was significantly less the largest series of normative data available in the literature for comparison (p < 0.001). No skull base anomalies were noted in either group. CONCLUSION: While this is a small study with limited numbers, it is the first that has attempted to identify and measure the posterior nasal and skull base anatomy most pertinent to avoiding skull base injury in the surgical management of BCA.

An approach to successful slide tracheoplasty in the low birth weight neonate with single lung.

The advent of the slide tracheoplasty technique and a multi-disciplinary approach has improved outcomes of congenital tracheal stenosis. However, tracheal surgery in younger patients with pulmonary malformations, especially low birth-weight neonates, has been associated with increased mortality. Patients with very low birth-weight, pulmonary malformations and prematurity may be palliated prior to definitive tracheal surgery due to the poor prognosis. We report a successful and unique approach of delaying tracheal reconstruction to allow growth and development in the premature, very low birth-weight neonate (1046g) with left lung agenesis.

Targeted Tonsillar Biopsy for Unilateral Tonsillar Enlargement.

BACKGROUND: Unilateral tonsillar enlargement (UTE) is not an uncommon incidental finding. Lymphoma is the most common malignancy of the tonsils in children and presents with tonsillar enlargement. METHODS: This is a retrospective case series of all cases of histopathological analysis of tonsillectomy specimens at a single pediatric tertiary hospital between 1996 and 2015. Patients either had a preoperative indication (n = 53) for pathological testing or indications based on intraoperative findings (n = 88). Of the preoperative group, 17 patients had tonsillectomy for UTE alone. In 72% of cases, the side of perceived unilateral enlargement was confirmed at histopathological assessment. RESULTS: Zero cases of suspected malignancy were identified on testing. CONCLUSION: There is a very low prevalence of unsuspected and suspected malignancy in routine and targeted examination of tonsillar specimens. It is reasonable to test based on other clinical findings and risk factors such as immunosuppression.

The Kölliker-Fuse nucleus: a review of animal studies and the implications for cranial nerve function in humans.

To review the scientific literature on the relationship between Kölliker-Fuse nucleus (KF) and cranial nerve function in animal models, with view to evaluating the potential role of KF maturation in explaining age-related normal physiologic parameters and developmental and acquired impairment of cranial nerve function in humans. Medical databases (Medline and PubMed). Studies investigating evidence of KF activity responsible for a specific cranial nerve function that were based on manipulation of KF activity or the use of neural markers were included. Twenty studies were identified that involved the trigeminal (6 studies), vagus (9), and hypoglossal nerves (5). These pertained specifically to a role of the KF in mediating the dive reflex, laryngeal adductor control, swallowing function and upper airway tone. The KF acts as a mediator of a number of important functions that relate primarily to laryngeal closure, upper airway tone and swallowing. These areas are characterized by a variety of disorders that may present to the otolaryngologist, and hence the importance of understanding the role played by the KF in maintaining normal function.

Magnetic resonance imaging findings in children with tinnitus.

OBJECTIVE: Tinnitus in adults is generally investigated by contrast-enhanced magnetic resonance imaging (MRI) to rule out the diagnosis of acoustic neuroma. Acoustic neuroma is rare in children and, therefore, the role of MRI in children with tinnitus is unclear. This study was undertaken to determine the value of MRI in the investigation of tinnitus in children. METHODS: Retrospective study of children younger than 18 years who underwent MRI for the investigation of tinnitus over a 10-year period. RESULTS: Sixty-five patients were identified, but there were only 34 who had also undergone audiologic assessment. Among the 25 patients with normal audiology, MRI abnormalities were present in 9, but these were all thought to be nonspecific. Nine patients had abnormal audiograms and the MRI was abnormal in 4 of these cases, which included 3 children who were found to have multiple sclerosis. CONCLUSION: Magnetic resonance imaging would appear to be mandatory in the investigation of tinnitus in children who are found to have sensorineural hearing loss, particularly to rule out the diagnosis of multiple sclerosis. Although our study does not support the routine use of MRI in children with normal audiology, the numbers in our series are too small for a conclusive recommendation.

Magnetic resonance imaging findings in pediatric bilateral vocal fold dysfunction.

OBJECTIVES: We studied the findings of brain magnetic resonance imaging (MRI) in infants with idiopathic congenital bilateral vocal fold dysfunction (CBVFD). METHODS: We performed a retrospective investigation of a case series. RESULTS: We identified 26 children (14 male, 12 female) over 11 years. Three children were excluded. Thirteen patients required airway interventions, including continuous positive airway pressure (4 patients), endotracheal intubation (1), and tracheostomy (8). The findings on brain MRI were abnormal in 8 patients (35%). Tracheostomy was required in 3 patients (38%) with abnormal MRI findings, as compared with 5 of 15 patients (33%) with normal MRI findings. The MRI abnormalities involved evidence of white matter injury (2), abnormal white matter signal (1), subdural blood (3), cerebral swelling (1), and perisylvian polymicrogyria (1). The cranial ultrasound findings were abnormal in 4 of 11 patients. The MRI findings were abnormal in 2 of 7 children in whom the cranial ultrasound findings were normal, and in 2 of the 4 patients in whom the cranial ultrasound findings were abnormal. CONCLUSIONS: The MRI abnormalities were nonspecific; however, they may indicate unrecognized perinatal intracranial injury as being related to CBVFD. In addition, MRI may reveal an underlying structural brain anomaly. Cranial ultrasound has poor sensitivity and specificity. Hence, MRI should be considered as part of the routine assessment of neonates with CBVFD.

Perinatal airway management of neonatal cervical teratomas.

Cervical teratomas are rare but life-threatening neonatal tumors and management of the fetus with a cervical teratoma that threatens the airway remains a clinical challenge. This has been revolutionized by advances in fetal imaging and management of the airway at delivery including the use of Ex-utero Intrapartum Treatments (EXIT procedures). We present a retrospective case series of three neonates managed over a 12-month period. Following pre-natal fetal MRI and a multi-disciplinary management approach, two newborns were managed by prompt post-natal endotracheal intubation while an EXIT procedure was required in one. All three underwent surgical resection in the first few days of life. A decision regarding the best means by which to manage the airway in fetal cervical teratoma requires fetal MRI and a multi-disciplinary team approach to determine whether EXIT, or a safer approach from a maternal perspective can be employed. We also recommend routine endotracheal intubation at birth, due to the risk of spontaneous intra-tumoral hemorrhage. The need for surgery should be planned early, as rapid growth of the tumor can threaten the viability of the overlying skin and surrounding structures.

Bilateral choanal atresia repair in neonates--a single surgeon experience.

OBJECTIVE: To evaluate the success of the transnasal endoscopic repair of bilateral choanal atresia (BCA) repair in neonates based on a single surgeon's experience. METHODS: A retrospective case review was carried out of all neonates with BCA who underwent transnasal endoscopic repair by the senior author (RGB) between September 1993 and September 2009 at the Royal Children's Hospital Melbourne, Australia. RESULTS: Twenty three neonates (18 female and 5 male) aged between 0 and 32 days (mean age, 7.4 days) underwent surgery for BCA between September 1993 and September 2009. Twenty of these patients met the inclusion criteria to calculate success rate of primary surgery. All patients were stented using modified endotracheal tubes for a mean of 75 days (range, 5-158 days). Of the 20 with adequate follow up, 14 patients had successful primary repair (70%). Of the six requiring revision surgery, three had one revision procedure, and three had two revision procedures. All patients were free of recurrence at last follow up. Mean follow up after removal of primary stent was 25.2 months (range, 6.6-77.9 months). CONCLUSION: Endoscopic transnasal repair is a relatively safe and successful technique. Stenting for 3 months was found to reduce the chance of early restenosis.

A population-based study and systematic review of hearing loss in children with cerebral palsy.

AIM: The aims of this study were to estimate the frequency of hearing loss in children with cerebral palsy (CP), to examine factors associated with hearing loss, and to describe aspects of hearing in a population sample of children with CP and hearing loss. METHOD: A systematic review of the international literature was undertaken, and data on the frequency of hearing loss or severe hearing loss were extracted from 14 data sets based on previously devised criteria. Six hundred and eight-five children with CP (406 males, 279 females) born in Victoria, Australia, between 1999 and 2004 were identified from the Victorian Cerebral Palsy Register. Children were included if they had an established post neonatal cause for their CP before the age of 2 years. Additional information was collected on 48 children with documented hearing loss based on a four-tone pure tone average in the better ear. RESULTS: There was considerable variation in the definitions and proportions of hearing loss (range 4-13%) and severe hearing loss (range 2-12%) reported by CP registries in developed countries. In Victoria, 7% of individuals with CP had bilateral hearing loss of a moderate to profound degree, whereas the subgroup with a severe-profound degree of loss constituted 3% to 4% of the CP population. INTERPRETATION: These population-based data are likely to more accurately reflect the true frequency of defined hearing loss in children with CP than previous reviews.

Airway complications of pediatric extracorporeal membrane oxygenation.

OBJECTIVES: Prolonged intubation is a risk factor for the development of laryngotracheal stenosis. Children who undergo extracorporeal membrane oxygenation (ECMO) usually remain intubated for an extended period. It is unclear whether the impaired cardiorespiratory status that necessitated ECMO places these children at a higher risk of laryngotracheal stenosis. This study was performed to assess the incidences of laryngotracheal stenosis and tracheostomy in children who undergo ECMO. METHODS: We identified all patients under 18 years of age who underwent ECMO over a 10-year period concluding July 1, 2009, by use of the extracorporeal life support database of Royal Children's Hospital, Melbourne. All children in this database who underwent either a diagnostic or a therapeutic surgical procedure on the airway were identified. RESULTS: The 218 patients included in the study had an overall survival rate of 51.4%. A total of 14 patients (6.4%) required a surgical procedure on the airway, and 11 of these (5.0%) needed tracheostomy. Ten of these 14 patients (71.4%) survived; of these, 2 presented with congenital laryngotracheal stenosis, 3 developed clinically significant laryngotracheal stenosis as a likely consequence of ECMO, and 5 required tracheostomy alone for long-term ventilation. The rate of airway stenosis was 2.7% in survivors. CONCLUSIONS: The rate of laryngotracheal stenosis in children who require ECMO is acceptably low.

The temporal relationship between non-respiratory burst activity of expiratory laryngeal motoneurons and phrenic apnoea during stimulation of the superior laryngeal nerve in rat.

A striking effect of stimulating the superior laryngeal nerve (SLN) is its ability to inhibit central inspiratory activity (cause 'phrenic apnoea'), but the mechanism underlying this inhibition remains unclear. Here we demonstrate, by stimulating the SLN at varying frequencies, that the evoked non-respiratory burst activity recorded from expiratory laryngeal motoneurons (ELMs) has an intimate temporal relationship with phrenic apnoea. During 1­5 Hz SLN stimulation, occasional absences of phrenic nerve discharge (PND) occurred such that every absent PND was preceded by an ELM burst activity. During 10­20 Hz SLN stimulation, more bursts were evoked together with more absent PNDs, leading eventually to phrenic apnoea. Interestingly, subsequent microinjections of isoguvacine (10 mm, 20­40 nl) into ipsilateral Bötzinger complex (BötC) and contralateral nucleus tractus solitarii (NTS) significantly attenuated the apnoeic response but not the ELM burst activity. Our results suggest a bifurcating projection from NTS to both the caudal nucleus ambiguus and BötC, which mediates the closely related ELM burst and apnoeic response, respectively. We believe that such an intimate timing between laryngeal behaviour and breathing is crucial for the effective elaboration of the different airway protective behaviours elicited following SLN stimulation, including the laryngeal adductor reflex, swallowing and cough.

Acquired cholesteatoma in children following congenital cholesteatoma surgery.

OBJECTIVES: To determine the incidence of severe atelectatic otitis media and acquired cholesteatoma (AC) in children treated for congenital cholesteatoma (CC). METHODS: Retrospective chart review of 15 children who underwent primary surgery for CC over a 15 year period by a single surgeon. RESULTS: The mean postoperative follow up was 3.1 years. Significant tympanic retraction occurred in 6 children, included a retraction pocket that required T-tube insertion (3), and AC requiring tympanomastoid surgery (3). There was no complication related to retraction pocket in 9 children however 2 developed residual disease. In comparing the two groups, those with and without subsequent significant tympanic retraction, both groups had similar gender, age, extent of CC (median Potsic grade of 2), bone erosion, and surgical technique. Differences were noted in air-bone gap at presentation (PTA 32.4 and 17.25), otitis media with effusion in the contralateral ear (3/6 and 1/9), smaller mastoid volume ratio compared with the contralateral ear (0.74 and 1.21), and longer average timing for second surgery (14.8 months and 8 months). CONCLUSIONS: Acquired middle ear disease, including cholesteatoma, can follow surgical removal of CC, and long term follow up of all patients is required. Factors at initial evaluation indicative of risk of AC include a significant air-bone gap, otitis media with effusion in the contralateral ear and a smaller mastoid cavity ratio. The use of composite grafts at the time of CC surgery should be considered. Additionally, our findings suggest that the mastoid volume plays a causative role in the development of AC.

Neuronal mechanisms underlying the laryngeal adductor reflex.

OBJECTIVES: Electromyographic studies of the laryngeal adductor reflex, glottal closure occurring in response to laryngeal stimulation, have demonstrated an early ipsilateral response (R1) and a late bilateral response (R2). To better define the physiologic properties of these responses, we recorded responses from expiratory laryngeal motoneurons (ELMs) in rats during stimulation of the superior laryngeal nerve (SLN). METHODS: Single unit extracellular recordings were obtained from 5 ELMs, identified by their antidromic responses to recurrent laryngeal nerve stimulation and postinspiratory firing pattern, in 4 Sprague-Dawley rats. RESULTS: Unilateral stimulation of the SLN (at 20 Hz) stopped both phrenic nerve inspiratory activity and ELM postinspiratory activity. However, the ELMs displayed robust tonic firing, consisting of non-respiratory burst activity and single action potentials. The single action potentials were identified as short-latency ones (5 to 10 ms) activated by ipsilateral SLN stimulation, with an occurrence rate of 90%, and long-latency ones (20 to 50 ms) activated by bilateral SLN stimulation, with occurrence rates of 47% on the ipsilateral side and 58% on the contralateral side. CONCLUSIONS: The R1 response appears to be the result of the short-latency action potentials, orthodromically activated by ipsilateral stimulation of the SLN. The R2 response is likely to be a result of the long-latency action potentials that can be recorded from ELMs on both sides.

The generation of post-inspiratory activity in laryngeal motoneurons: a review.

Breathing is a vegetative function that is altered during more complex behaviours such as exercise, vocalisation and respiratory protective reflexes. Recent years have seen recognition of the importance of respiratory pattern generation in addition to rhythm generation. Respiratory-modulated cranial motoneurons (laryngeal, pharyngeal, hypoglossal, facial) offer a unique insight into the control of respiration since: (1) they receive rhythmic respiratory inputs but; (2) their respiratory-modulated firing pattern differs to that of phrenic neurons to suit their function, (for example, hypoglossal motoneurons begin firing and thus the tongue depresses before the onset of phrenic nerve discharge and diaphragmatic during inspiration) and; (3) their activity is often altered in parallel with changes in respiration during stereotypical non-respiratory behaviours such as coughing, swallowing and sneeze. Here we review some mechanisms that modulate the respiratory-related activity of laryngeal motoneurons with an emphasis on the generation of post-inspiratory activity.

Cough in the pediatric population.

Children with cough, in particular chronic cough, are sometimes referred to otolaryngologists for assessment, diagnosis, and management. Although the likely diagnoses encountered by otolaryngologists are rhinosinusitis, foreign body aspiration, and tracheomalacia, otolaryngologists should be cognizant of the many other possible diagnoses and the evidence for and against their association. This article highlights and focuses the discussion on the cough issues relevant to otolaryngologists.

Respiration-related laryngeal electromyography in children with bilateral vocal fold paralysis.

We present 2 case reports to demonstrate the relationship between laryngeal muscle activity and respiration in children with bilateral vocal fold paralysis (BVFP) by simultaneous laryngeal electromyography (EMG) with recording of chest wall movement and intercostal muscle EMG. Laryngeal EMG was performed together with recording of chest wall movement in a 55-day-old girl who was undergoing tracheostomy for idiopathic congenital BVFP. Normal phasic activity was observed, i.e., the thyroarytenoid (TA) muscle was active during expiration and the posterior cricoarytenoid (PCA) muscle during inspiration, suggesting a good prognosis for recovery. The child was decannulated at 11 months. Laryngeal EMG together with recording of chest wall movement and intercostal EMG in a 5-year-old girl who was tracheostomy-dependent following tracheoesophageal fistula repair due to BVFP showed phasic activity during expiration for both the TA and PCA muscles, indicating aberrant regeneration of the PCA motor nerve. The timing of laryngeal muscle activity with respiration in the assessment of pediatric congenital BVFP is essential to demonstrate the presence of normal or abnormal medullary respiratory neuronal input to laryngeal motoneurons. In cases in which BVFP is due to recurrent laryngeal nerve injury, respiration-related laryngeal EMG will identify aberrant regeneration. Laryngeal EMG should be combined with intercostal muscle EMG in the evaluation of children with significant vocal fold dyfunction of either central or peripheral origin.