The incidence of juvenile onset recurrent respiratory papillomatosis at Starship Children's Hospital before and after a national HPV vaccination programme: a retrospective review.

AIM: To review and compare the incidence of juvenile onset recurrent respiratory papillomatosis (JRRP) at Starship Children's Hospital (SSH) before and after the introduction of a national HPV vaccination programme. METHODS: Patients treated for JRRP at SSH were identified retrospectively using ICD-10 code D14.1 over a 14-year period. The incidence of JRRP in the 10-year period prior to the introduction of HPV vaccination (1 September 1998 to 31 August 2008) was compared with the incidence after its introduction. A second comparison was made between the pre-vaccination incidence with the incidence over the most recent 6 years when the vaccination became more widely available. All New Zealand hospital ORL departments that referred children with JRRP to SSH exclusively were included. RESULTS: SSH manages about half of the New Zealand paediatric population with JRRP. The incidence of JRRP before the introduction of the HPV vaccination programme was 0.21 per 100,000 per year in children 14 years of age and younger. This remained stable between 2008 and 2022 (0.23 vs 0.21 per 100,000 per year). The mean incidence in the later post-vaccination period was 0.15 per 100,000 per year based on small numbers. CONCLUSION: The mean incidence of JRRP before and after the introduction of HPV has remained unchanged in children treated at SSH. More recently, a reduction in incidence has been noted, although this is based on small numbers. The low HPV vaccination rate (≤70%) may explain why a significant reduction in the incidence of JRRP seen overseas has not been observed in New Zealand. Ongoing surveillance and a national study would provide more insight into the true incidence and evolving trends.

Flexible Endoscopic Evaluation of Swallowing in Breastfeeding Infants With Laryngomalacia: Observed Clinical and Endoscopic Changes With Alteration of Infant Positioning at the Breast.

OBJECTIVES: This retrospective cohort study uses endoscopic assessment of the pharyngeal phase of swallowing in infants with laryngomalacia, to ascertain the impact of infant positioning on airway compromise and fluid dynamics during breastfeeding. The study aims to identify whether modification of infant positioning at the breast may improve the possibility of safe, successful breastfeeding in infants with laryngomalacia and concurrent breastfeeding difficulty. METHODS: Twenty-three infants referred for noisy breathing and difficulty feeding were assessed with flexible endoscopic evaluation of swallowing (FEES) during breastfeeding. All had endoscopically confirmed laryngomalacia. During FEES, observations were made of clinical signs of airway compromise as well as endoscopically observable anatomical features and swallowing dynamics during breastfeeding, including tongue base position, view of laryngeal inlet and vocal folds, dynamic supraglottic soft tissue collapse, timing of milk flow into pyriform fossae/hypopharynx relative to sucking, and presence of penetration and/or aspiration. If airway and/or swallowing compromise was present, the infant's initial position at the breast was altered from supine or semi lateral decubitus position to semi-prone, with a description of the clinical and endoscopically observable changes that subsequently occurred. RESULTS: Signs of dynamic airway obstruction and/or compromised airway protection with swallowing were present in 20 of the 23 infants (87%) in their initial supine or semi lateral decubitus position. These 20 infants were repositioned to semi-prone, with improvement and/or resolution of stridor and an improved ability to maintain latch in all infants. Continued endoscopic evaluation following positional change was possible in 16 infants, identifying anterior positioning of the tongue base, reduced dynamic supraglottic tissue collapse, reduced volume of milk flow into pyriform fossae during pauses in sucking and resolution of penetration and aspiration. CONCLUSION: This study has shown how alteration of breastfeeding position to semi-prone may improve dynamic airway obstruction and reduce aspiration risk in infants with laryngomalacia.

In vitro Inhibition of Clinical Isolates of Otitis Media Pathogens by the Probiotic Streptococcus salivarius BLIS K12.

Otitis media is a common childhood infection, frequently requiring antibiotics. With high rates of antibiotic prescribing and increasing antibiotic resistance, new strategies in otitis media prevention and treatment are needed. The aim of this study was to assess the in vitro inhibitory activity Streptococcus salivarius BLIS K12 against otitis media pathogens. Efficacy of the bacteriocin activity of S. salivarius BLIS K12 against the otitis media isolates was assessed using the deferred antagonism test. Overall, 48% of pathogenic isolates exhibited some growth inhibition by S. salivarius BLIS K12. S. salivarius BLIS K12 can inhibit the in vitro growth of the most common pathogens.

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Management of suprastomal collapse in the pediatric population.

INTRODUCTION: Suprastomal Collapse (SuStCo) is a common complication of prolonged tracheostomy in children. There is a paucity of literature on this subject, especially regarding how to manage significant suprastomal collapse that prevents safe decannulation. OBJECTIVE: Provide a definition, classification system, and recommend management options for significant suprastomal collapse in children with tracheostomy. METHODS: Members of the International Pediatric Otolaryngology Group (IPOG) who are experts in pediatric airway conditions were surveyed and results were refined using a modified Delphi method. RESULTS: Consensus was defined as > 70% agreement on a subject. The experts achieved consensus: CONCLUSION: This consensus statement provides recommendations for medical specialists who manage infants and children with tracheostomies with significant Suprastomal Collapse. It provides a classification system to facilitate diagnosis and treatment options for this condition.

Understanding the Lingual Frenulum: Histological Structure, Tissue Composition, and Implications for Tongue Tie Surgery.

Lingual frenotomy has become an increasingly common surgical procedure, performed for a broad range of indications from birth through adulthood. This study utilizes histology to define the structure and tissue composition of the lingual frenulum and floor of mouth (FOM) fascia. En bloc specimens of anterior tongue, lingual frenulum, and FOM tissues were harvested from ten embalmed adult cadavers. An additional three fresh tissue cadaveric specimens were frozen with the tongue supported in an elevated position, to enable harvesting and paraffin embedding of the elevated lingual frenulum as a discrete specimen. All 13 specimens were prepared as ten-micron coronal sections using stains to determine the general morphology of the lingual frenulum, its relationship to neighbouring structures (Mason's Trichrome), presence of elastin fibers (Verhoeff-van Gieson), and collagen typing (Picrosirius Red). Our results have shown a submucosal layer of fascia spanning horizontally across the FOM was present in all specimens, with variability in fascial thickness and histologic composition. This FOM fascia suspends the sublingual glands, vessels, and genioglossus from its deep surface. The elevated lingual frenulum is formed by a central fold of this FOM fascia together with the overlying oral mucosa with variability in fascial thickness and composition. With tongue elevation, the fascia mobilizes to a variable extent into the fold forming the frenulum, providing a structural explanation for the individual variability in lingual frenulum morphology seen in clinical practice.

Imaging the breastfeeding swallow: Pilot study utilizing real-time MRI.

OBJECTIVE: Knowledge of the breastfeeding swallow is limited by practical challenges. Radiation exposure to both mother and infant and the radiolucent properties of breastmilk make videofluoroscopy an unsuitable imaging modality. Furthermore, ultrasound is not ideal for capturing the complex 3-dimensional functional anatomy of swallowing. In this study we explore the feasibility of using real-time MRI to capture the breastfeeding swallow. METHODS: Prospective observational study: Review of imaging from 12 normal infants (<5 months of age) and their mothers while breastfeeding using real-time MRI. RESULTS: Static images were successfully captured in 11 infants and dynamic images in nine infants. This imaging modality confirms the dorsal surface of the infant's tongue elevates the maternal nipple to the hard palate, closing the space around the nipple with no air visible in the oral cavity during sucking and swallowing. We obtained dynamic imaging of mandibular movement with sucking, palatal elevation and pharyngeal constriction with swallowing, diaphragm movement with breathing and milk entering the stomach. Breastmilk was easily visualized, being high intensity on T2 sequences. Technical challenges were encountered secondary to infant movement and difficulties acquiring and maintaining midsagittal orientation. The similarity in tissue densities of the lips, tongue, nipple and hard palate limited definition between these structures. CONCLUSION: Real-time MRI imaging was successful in capturing dynamic images of the breastfeeding swallow. However, technical and practical challenges make real-time MRI unlikely at present to be suitable for swallow assessment in clinical practice. Advances in technology and expertise in dynamic image capture may improve the feasibility of using MRI to understand and assess the breastfeeding swallow in the near future. LEVEL OF EVIDENCE: 4.

Pediatric tracheostomy decannulation: When can decannulation be performed safely outside of the intensive care setting? A 10 year review from a single tertiary otolaryngology service.

OBJECTIVES: Pediatric tracheostomy is performed in a variety of complex, comorbid patients. Tracheostomy involves a significant burden of care for families and a risk of life-threatening complications. There is little research regarding the ideal location and protocol for safe tracheostomy decannulation. This study aims to determine patient factors that may be predictive of trial of tracheostomy decannulation being able to take place safely outside of the intensive care setting. METHODS: A 10-year retrospective review of all decannulation trials at our institution is used to assess for patient factors associated with a higher risk of decannulation failure. The timing of failure and the interventions required to secure the patient's airway are reviewed. This data is used to inform recommendations regarding location of tracheostomy decannulation trial and length of inpatient stay, aiming to rationalize the use of resources while maintaining safe tracheostomy decannulation practices. RESULTS: One hundred and fifty-eight decannulation events occurred in 131 children over the study period, resulting in 132 successful decannulations (83.5%). Twenty-six failed episodes (16.5%) occurred in 16 patients (12.2%). Ten of these patients were successful on a second decannulation attempt and six had two or more failed decannulation attempts (4.6%). Failed decannulation was higher in patients with upper airway obstruction as the indication for tracheostomy (20.3% failure rate versus 0%). History of prematurity was significantly associated with failure of decannulation. Nine decannulation failures occurred immediately, with a further 9 failures occurring within the first 24 hours. A further 3 failures occurred in hospital and 5 following discharge. No mortality or significant morbidity occurred during any decannulation trial. CONCLUSIONS: Our study identified a higher rate of decannulation failure in patients with upper airway obstruction, suggesting that decannulation trials for this subgroup should occur in the intensive care unit. Patients with tracheostomy for other indications may be safe to decannulate in a ward setting. Early failures demonstrated more rapid deterioration. Further research is recommended on the utilization of capping trials or polysomnography prior to decannulation to help guide the ideal location and timing for trial decannulation.

Complications following frenotomy for ankyloglossia: A 24-month prospective New Zealand Paediatric Surveillance Unit study.

AIM: To investigate the incidence and characteristics of complications arising from frenotomy for ankyloglossia (tongue-tie) in New Zealand. METHODS: Prospective surveillance among hospital-based paediatricians of complications arising from frenotomy for ankyloglossia to children <1 year old was conducted by the New Zealand Paediatric Surveillance Unit for 24 months, from August 2016 to July 2018, inclusive. RESULTS: A total of 16 cases of complications arising from frenotomy were reported. The overall average annual incidence rate was 13.9/100 000. Geographic variation was noted with a peak of 85.6/100 000 in one region. Complications reported: poor feeding (44%), respiratory events (25%), pain (19%), bleeding (19%) and weight loss (19%). Three children (19%) also had delayed diagnosis of an underlying medical condition initially overlooked in favour of treating their ankyloglossia, this has not previously been reported. The majority (75%) of cases required admission to hospital. Treatments given included supplementary feeds (44%), surgical intervention (25%), breastfeeding support (19%), analgesia (13%) and blood products (13%). A total of 25% of children had one or more frenotomies; 50% were treated for two or more of: 'anterior' ankyloglossia, 'posterior' ankyloglossia or 'lip tie'; 50% had their frenotomies performed out of the hospital. Dentists were the most common performing practitioner (31%). CONCLUSIONS: Frenotomy rates in New Zealand are unknown. Poor feeding, pain, bleeding, weight loss and delayed diagnosis of an alternative underlying medical condition are important complications that require hospital assessment and admission. Practitioners and parents/families need to be aware of these possibilities. Centralised guidelines with access to specialist second opinions should be developed.

Defining the anatomy of the neonatal lingual frenulum.

The lingual frenulum is recognized as having the potential to limit tongue mobility, which may lead to difficulties with breastfeeding in some infants. There is extensive variation between individuals in the appearance of the lingual frenulum but an ambiguous relationship between frenulum appearance and functional limitation. An increasing number of infants are being diagnosed with ankyloglossia, with growing uncertainty regarding what can be considered "normal" lingual frenulum anatomy. In this study, microdissection of four fresh tissue premature infant cadavers shows that the lingual frenulum is a dynamic, layered structure formed by oral mucosa and the underlying floor of mouth fascia, which is mobilized into a midline fold with tongue elevation and/or retraction. Genioglossus is suspended from the floor of mouth fascia, and in some individuals can be drawn up into the fold of the frenulum. Branches of the lingual nerve are located superficially on the ventral surface of the tongue, immediately beneath the fascia, making them vulnerable to injury during frenotomy procedures. This research challenges the longstanding belief that the lingual frenulum is a midline structure formed by a submucosal "band" or "string" and confirms that the neonatal lingual frenulum structure replicates that recently described in the adult. This article provides an anatomical construct for understanding and describing variability in lingual frenulum morphology and lays the foundation for future research to assess the impact of specific anatomic variants of lingual frenulum morphology on tongue mobility. Clin. Anat. 32:824-835, 2019. © 2019 The Authors. Clinical Anatomy published by Wiley Periodicals, Inc. on behalf of American Association of Clinical Anatomists.

PCV7- and PCV10-Vaccinated Otitis-Prone Children in New Zealand Have Similar Pneumococcal and Haemophilus influenzae Densities in Their Nasopharynx and Middle Ear.

Otitis media (OM) is a major reason for antibiotic consumption and surgery in children. Nasopharyngeal carriage of otopathogens, Streptococcus pneumoniae and nontypeable Haemophilus influenzae (NTHi), is a prerequisite for development of OM, and increased nasopharyngeal otopathogen density correlates with disease onset. Vaccines can reduce or eliminate otopathogen carriage, as demonstrated for pneumococcal serotypes included in pneumococcal conjugate vaccines (PCV). The 10-valent PCV (PCV10) includes an NTHi carrier protein, and in 2011 superseded 7-valent PCV on the New Zealand Immunisation Program. Data are conflicting on whether PCV10 provides protection against NTHi carriage or disease. Assessing this in otitis-prone cohorts is important for OM prevention. We compared otopathogen density in the nasopharynx and middle ear of New Zealand PCV7-vaccinated and PCV10-vaccinated otitis-prone and non-otitis-prone children to determine PCV10 impact on NTHi and S. pneumoniae carriage. We applied qPCR to specimens collected from 217 PCV7-vaccinated children (147 otitis-prone and 70 non-otitis-prone) and 240 PCV10-vaccinated children (178 otitis-prone and 62 non-otitis-prone). After correcting for age and day-care attendance, no difference was observed between NTHi density in the nasopharynx of PCV7-vaccinated versus PCV10-vaccinated otitis-prone (p = 0.563) or non-otitis-prone (p = 0.513) children. In contrast, pneumococcal nasopharyngeal density was higher in PCV10-vaccinated otitis-prone children than PCV7-vaccinated otitis-prone children (p = 0.003). There was no difference in otopathogen density in middle ear effusion from PCV7-vaccinated versus PCV10-vaccinated otitis-prone children (NTHi p = 0.918; S. pneumoniae p = 0.415). When pneumococcal carriage was assessed by vaccine serotypes (VT) and non-vaccine serotypes (NVT), there was no difference in VT density (p = 0.546) or NVT density (p = 0.315) between all PCV7-vaccinated versus all PCV10-vaccinated children. In summary, PCV10 did not reduce NTHi density in the nasopharynx or middle ear, and was associated with increased pneumococcal nasopharyngeal density in otitis-prone children in New Zealand. Development of therapies that prevent or reduce otopathogen colonisation density in the nasopharynx are warranted to reduce the burden of OM.

What is a tongue tie? Defining the anatomy of the in-situ lingual frenulum.

Surgical release of the lingual frenulum (frenotomy) has become an increasingly common procedure, performed from birth through to adulthood. Surprisingly, detailed anatomy of the in-situ lingual frenulum has never been described, and no anatomical basis has been proposed for the individual variability in frenulum morphology. The lingual frenulum is frequently referred to as a "cord" or "submucosal band" of connective tissue, yet there is no evidence to support this anatomical construct. This paper aims to describe the anatomy of the in-situ lingual frenulum and its relationship to floor of mouth structures. Fresh tissue microdissection of the lingual frenulum and floor of mouth was performed on nine adult cadavers with photo-documentation and description of findings. The lingual frenulum is a dynamic structure, formed by a midline fold in a layer of fascia that inserts around the inner arc of the mandible, forming a diaphragm-like structure across the floor of mouth. This fascia is located immediately beneath the oral mucosa, fusing centrally with the connective tissue on the tongue's ventral surface. The sublingual glands and submandibular ducts are enveloped by the fascial layer and anterior genioglossus fibers are suspended beneath it. Lingual nerve branches are located superficially on the ventral surface of the tongue, immediately deep to the fascia. The lingual frenulum is not a discrete midline structure. It is formed by dynamic elevation of a midline fold in the floor of mouth fascia. With this study, the clinical concept of ankyloglossia and its surgical management warrant revision. Clin. Anat. 32:749-761, 2019. © 2019 The Authors. Clinical Anatomy published by Wiley Periodicals, Inc. on behalf of American Association of Clinical Anatomists.

The bacterial species associated with aspirated foreign bodies in children.

OBJECTIVE: Inhaled foreign bodies in children are common and may be complicated by secondary airway tract infection. The inhaled foreign body may act as carrier of infectious material and the aim of this study was to explore the bacterial species associated with aspirated foreign bodies in a cohort of children. METHODS: Retrospective case series of 34 patients who underwent rigid laryngobronchoscopy because of foreign body aspiration. Each patient had a sample taken from tracheobronchial secretions during the procedure. RESULTS: The average patient age was 31.2 months and the average hospital stay was 2.5 days. Of the foreign bodies 24 (71%) were organic in nature and 10 (29%) were non-organic. Twenty eight (82.3%) patients had mixed oropharyngeal flora organisms growth. Fifteen (44%) samples were positive for organisms other than oropharyngeal flora with the most common cultured organisms being: Streptococcus pneumonia (4/12%), Haemophilus influenza (4/12%), Moraxella catarrhalis (4/12%). Four samples (12%) grew a fungus; Candida albicans was cultured in 3 patients and Aspergillus glaucus was identified in one sample. Of the non-oropharyngeal organisms 7(47%) demonstrated antibiotic resistance with four having resistance to amoxycillin, two resistant to penicillin and one resistant to cotrimoxazole. CONCLUSION: Some children who present with aspirated foreign body may be complicated with secondary airway infection. Antibacterial treatment might be considered in some of these cases. The regimen of antibiotics should aim to cover oropharyngeal flora, S. pneumonia, H. influenza and Moraxella catarrhalis.

Pneumococcal vaccine impact on otitis media microbiology: A New Zealand cohort study before and after the introduction of PHiD-CV10 vaccine.

UNLABELLED: We compared the microbiology of middle ear fluid (MEF) in two cohorts of children having ventilation tube (VT) insertion; the first in the era of 7-valent Streptococcus pneumoniae conjugate vaccine (PCV7) and the second following introduction of the ten-valent pneumococcal vaccine (PHiD-CV10). METHODS: During 2011 (Phase 1) and again in 2014 (Phase 2) MEF and NP samples from 325 children and 319 children were taken at the time of VT insertion. A matched comparison group had NP swabs collected with 137 children (Phase 1) and 154 (Phase 2). Culture was performed on all NP and MEF samples with further molecular identification of Haemophilus species, serotyping of S. pneumoniae, and polymerase chain reaction (PCR) testing on all MEF samples. RESULTS: In Phase 2 immunisation coverage with ⩾3 doses of PHiD-CV10 was 93%. The rate and ratios of culture and molecular detection of the 3 main otopathogens was unchanged between Phase 1 and Phase 2 in both MEF and NP. Haemophilus influenzae was cultured in one quarter and detected by PCR in 53% of MEF samples in both time periods. S. pneumoniae and Moraxella catarrhalis were cultured in up to 13% and detected by PCR in 27% and 40% respectively of MEF samples. H. influenzae was the most common organism isolated from NP samples (61%) in the children undergoing VT surgery whilst M. catarrhalis (49%) was the most common in the non-otitis prone group. 19A was the most prominent S. pneumoniae serotype in both MEF and NP samples in Phase 2. Of Haemophilus isolates, 95% were confirmed to be non-typeable H. influenzae (NTHi) over both time periods. CONCLUSION: Following implementation of PHiD-CV10 in New Zealand, there has been no significant change in the 3 major otopathogens in NP or MEF in children with established ear disease. For these children non-typeable H. influenzae remains the dominant otopathogen detected.

The starship children's hospital tonsillectomy: A further 10 years of experience.

OBJECTIVES/HYPOTHESIS: Tonsillectomy as a day-stay procedure remains controversial, although it is an established procedure in New Zealand. We reviewed our last 10 years' experience. METHODS: A prospective audit was used to determine unplanned conversion from day-stay to overnight hospital admission rates and the incidence of postoperative complications. RESULTS: There were 5,400 tonsillectomies performed over the 10-year study period (January 2004-January 2015); 71% as outpatients. The unplanned conversion rate to overnight stay was 0.4%. The median age of day-stay patients was 6.5 years (range 13 months-15 years) compared with those admitted for overnight stay (5 years; range 8 months-15 years). The primary postoperative bleed rate was 0.5% (confidence interval [CI] 0.3%-0.7%), and the combined primary and secondary posttonsillectomy bleed rate was 4.3% (CI 3.8%-5.0%). The rate of patients returning with postoperative complications within 1 month of surgery was 6.3% (CI 5.6%-7.0%). CONCLUSION: Day-stay tonsillectomy in the pediatric population is safe when performed using the described guidelines in a facility with appropriate resources. LEVEL OF EVIDENCE: 4. Laryngoscope, 126:E416-E420, 2016.

What is behind the ear drum? The microbiology of otitis media and the nasopharyngeal flora in children in the era of pneumococcal vaccination.

AIM: This study aims to describe the microbiology of middle ear fluid (MEF) in a cohort of children vaccinated with Streptococcus pneumoniae conjugate vaccine (PCV7) having ventilation tube insertion. Nasopharyngeal (NP) carriage of otopathogens in these children is compared with children without history of otitis media. METHODS: Between May and November 2011, MEF and NP samples from 325 children aged <3 years were collected in three major centres in New Zealand at the time of ventilation tube insertion. An age-matched non-otitis-prone comparison group of 137 children had NP samples taken. A questionnaire was completed by both groups. RESULTS: Immunisation coverage with at least one dose of PCV7 was 97%. Haemophilus influenzae was cultured in 19.4% of MEF and was polymerase chain reaction (PCR) positive in 43.4%. S. pneumoniae and Moraxella catarrhalis were cultured in <10% of MEF samples but were PCR positive for 23.1% and 38.7%, respectively. H. influenzae was the most common organism isolated from NP samples (60%) in the grommet group, while M. catarrhalis (56%) was the most common in the non-otitis prone group. S. pneumoniae was more commonly found in the nasopharynx of children with ear disease (41% vs. 29%). 19F was the most prominent S. pneumoniae serotype in NP samples of both groups, but no serotype dominated in MEF. Ninety-five per cent of H. influenzae isolates were confirmed to be non-typeable H. influenzae. CONCLUSION: In this cohort of children with established ear disease requiring surgical intervention, non-typeable H. influenzae is the dominant pathogen in both the nasopharynx and MEF.

A heuristic approach to foreign bodies in the paediatric airway.

OBJECTIVES: This retrospective study reviews the clinical presentation and management of children with airway FBs in our centre. It suggests a safe and reliable guideline to help differentiate which patients should proceed to investigation with rigid laryngobronchoscopy. METHODS: A retrospective review of all case notes of laryngobronchoscopies performed for suspected FB aspiration from January 2003 to August 2013 at a tertiary paediatric institution was undertaken. Patient characteristics, history, clinical examination, radiological findings and outcomes were analysed. RESULTS: 158 patients underwent rigid laryngobronchoscopy for suspected FB aspiration between January 2003 and August 2013. The baseline population demographics, the location and type of FBs retrieved were comparable to other similar studies; however, there is a statistically significant higher proportion of Pacific, Maori and Middle Eastern/Latin American/African children compared with the baseline population. Two or more positive findings in the presence of an acute history, any examination or radiology findings is a good indicator to proceed to laryngobronchoscopy with over 99% sensitivity. CONCLUSION: In a hospital presentation population, this retrospective study suggests that a guideline to proceed to laryngobronchoscopy in a case of suspected FB aspiration is two out of the three positive findings in the presence of an acute history, any examination or radiology findings. Patients who are stable and who do not have two of the three broad category findings can be considered for conservative management and observed on the ward, however, this is a guideline and must be combined with the clinical expertise of the paediatric airway specialist. Further studies are recommended to investigate contributing factors for the disproportionately higher incidence amongst Pacific, Maori and Middle Eastern/Latin American/African children.

To drain or not to drain - management of pediatric deep neck abscesses: a case-control study.

UNLABELLED: Optimal management of deep neck abscesses has been the subject of debate for more than a century: surgical drainage has been the mainstay of treatment, but recently many centres have reported successful non-operative management in selected cases. OBJECTIVES: Our objective was to review the management of deep neck abscesses in our institution and to identify characteristics that would predict successful non-operative management. METHODS: A retrospective chart review from January 2001 to August 2010 was performed. Children up to age fifteen years with a CT-confirmed diagnosis of retropharyngeal or parapharyngeal abscess were included. A case-control study of small deep space neck abscesses (≤ 25 mm maximal diameter) was performed, comparing antibiotic treatment alone with antibiotics plus abscess drainage. RESULTS: 54 children met the inclusion criteria, of whom half had abscesses ≤ 25 mm diameter. Younger children within the group with smaller abscesses were more likely to need surgical drainage (p<0.05). Of 13 children requiring operative management, ten underwent a period of antibiotic treatment and observation prior to surgery, eight (80%) had fever beyond 48 h compared with three (23%) in the non-surgical group (p<0.01). 27 children had an abscess > 25 mm diameter on CT scan, four (15%) of whom responded quickly to antibiotics and were managed non-operatively, while the rest underwent surgery. There were no significant differences between the surgical and non-surgical group characteristics with larger abscesses. CONCLUSION: High dose intravenous antibiotics are an effective treatment for deep space neck abscesses and may obviate the need for surgical drainage, particularly in smaller abscesses. Children who do not respond quickly to antibiotics are more likely to require surgery to achieve resolution. Children with larger abscesses may respond to antibiotic therapy alone but should be closely observed. A trial of high dose intravenous antibiotics in stable children with close observation is warranted as first line treatment, especially for small deep space neck abscesses.

Congenital stridor with feeding difficulty as a presenting symptom of Dok7 congenital myasthenic syndrome.

OBJECTIVE: The congenital myasthenic syndromes (CMS) are a group of genetic disorders of neuromuscular transmission causing fatigable weakness. Symptoms may be present from birth, but diagnosis is often delayed for several years, notably in post-synaptic CMS due to mutations in the DOK7 gene. Recently, we noted a subgroup of children with CMS in whom congenital stridor and bilateral vocal cord palsy predated other symptoms. All had mutations in the DOK7 gene. The purpose of this study was to review our population of DOK7 CMS patients with congenital stridor and assess whether there were other phenotypic features which might raise suspicion of a diagnosis of CMS in the neonatal period, in the absence of limb weakness and ptosis and prompt earlier referral for neurophysiological investigation, genetic diagnosis and appropriate treatment. METHODS: A retrospective case review of 11 DOK7 CMS patients at a tertiary referral centre. RESULTS: Six patients were identified with DOK7 mutations and congenital stridor, four requiring intubation soon after birth. Four patients had a diagnosis of bilateral vocal cord palsy and three required tracheostomy, successfully decannulated in one after 3 years. All six patients had difficulty with feeding, with weak suck and swallow necessitating nasogastric feeding in five, two of whom required gastrostomy. Despite all six children having had neonatal symptoms, the mean age at CMS diagnosis was 5 years and 9 months. CONCLUSION: CMS, particularly caused by mutations in the DOK7 gene, is a rare but treatable cause of congenital stridor in the neonate. A combination of congenital stridor, especially with an apparently idiopathic bilateral vocal cord palsy and weak suck and swallow should alert the clinician to the possibility of CMS and prompt early referral for neurophysiology and genetic investigations. Confirmation of a CMS diagnosis enables treatment to be initiated, informed management of the VCP and anticipation of myasthenic symptoms, particularly life-threatening respiratory decompensation. Treatment may allow early decannulation or possible avoidance of tracheostomy. At least 12 genes are known to cause CMS; the presence of congenital stridor may help target genetic diagnosis.

Successful treatment of isolated subglottic haemangioma with propranolol alone.

Subglottic haemangioma is a rare but potentially life threatening condition which requires intervention. Many different treatments have been described with varying degrees of success and complications. Recently, successful treatment with propranolol has been reported in 11 cases of cutaneous haemangiomas and then in two cases of subglottic haemangiomas with extensive cutaneous lesions in conjunction with other treatment modalities. We describe the successful treatment with propranolol, of a stridulous four-month-old child with a 95% obstructing subglottic haemangioma. This was achieved without the need for tracheostomy or any other surgical intervention, and with no reported side effects. We now believe the new discovery of a dramatic response to propranolol allows treatment in the acute setting and following further study may render surgical treatment of subglottic haemangioma obsolete.