Familial congenital laryngotracheal stenosis: A systematic review.

BACKGROUND: Congenital laryngotracheal stenosis (CLS) is a rare cause of stridor among newborns. Evidence has shown that several family members can be affected by CLS. Knowledge of the pathophysiology of familial congenital laryngotracheal stenosis (FCLS) will enable more effective therapeutic strategies. OBJECTIVE: To determine the clinical course and outcome of familial congenital laryngotracheal stenosis (FCLS). METHODS: A literature search was conducted over a period of one month (September 2023) by searching several databases to identify studies published from inception to 31st August 2023. RESULTS: Of 256 papers identified, five articles met the inclusion criteria. A total of 17 patients with slight female predominance (59 %) were identified. Familial congenital tracheal stenosis was reported in female twins (100 %). A variety of clinical presentations were listed. An endoscopic airway study was performed on all patients. 64.8 % of the included children were managed surgically. Genetic studies performed on 41 % of children could not locate genetic abnormalities. CONCLUSION: Consanguinity, twin births, and female gender could be predisposing factors for FCLS, although the quality of evidence is low due to the rarity of the condition.

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.

Late airway complications following pediatric liver transplantation: A case series.

BACKGROUND: Late airway complications, as consequence of immunosuppression following pediatric liver transplantation are uncommonly reported. METHODS: In this retrospective case series, we describe two young children presenting with symptoms of airway obstruction, secondary to differing pathologies in the supraglottic airway, as a result of immunosuppression following liver transplantation. RESULTS: Case 1, a 2-year-old girl who presented with stridor 12-months following liver transplantation, was found to have a proliferative soft tissue mass involving the supraglottic larynx. Biopsies were consistent with infiltrative eosinophilic laryngitis and associated eosinophilic esophagitis. Case 2, a 12-month-old female who presented with stridor 5-months following liver transplantation, was found to have an exophytic soft tissue mass involving the supraglottis and hypopharynx. Biopsies revealed polymorphic Epstein-Barr virus (EBV) driven post-transplant lymphoproliferative disease (PTLD). Case 1 was managed with local resection and high dose oral corticosteroids. Case 2 responded to debulking of the necrotic supraglottic mass, reduction of immunosuppression and rituximab. CONCLUSION: A high index of suspicion needs to be maintained for complications of immunosuppression for appropriate diagnosis of airway presentations following pediatric liver transplantation. Further research is necessary to improve early detection and consolidate management strategies for these airway lesions.

The learning curve of BiZact™ tonsillectomy.

OBJECTIVE: To evaluate the learning curve of BiZact™ tonsillectomy amongst trainees and fellows as a further technical skill. METHODS: Prospective audit of consecutive BiZact™ tonsillectomies undertaken by a trainee and fellow at Starship Children's Hospital. Primary outcomes measured were operative time from gag open to gag closed, readmission and post tonsillectomy bleed rates. Secondary outcomes included need for bipolar rescue dissection, bipolar haemostasis including site and side, tonsil size (Brodsky grade 1-4) and depth (shallow or deep). Operative time was plotted against number of procedures performed and moving averages was used to investigate the learning curve. Generalised linear model was applied to examine the association between operation time and tonsil depth. RESULTS: Mean operative time for the trainee and fellow was similar (07 min 34 s versus 07min and 37 s). We observed a trend of decreased operative time over number of procedures performed by the trainee but the learning curve was short. The fellow's initial time was lower than the trainee's and remained similar over time. No bipolar rescue dissection was required. Additional bipolar haemostasis was required 95% of the time, but the majority involved the superior pole only (87.5%, p = 0.0001). The overall operative time for shallow tonsils was shorter than for deep tonsils (06 min 31 s versus 08 min 09 s, p = 0.0066). A learning curve was evident for both shallow and deep tonsils for both surgeons combined but was steeper for deep. There were no primary bleeds and the secondary haemorrhage rate was 5.8% (3/51 cases) with no returns to theatre. CONCLUSION: This is the first study to investigate the BiZact™ tonsillectomy learning curve. BiZact™ tonsillectomy is safe and easy to learn. We observed a clear trend of shallow tonsils being quicker to remove than deep tonsils using this technique. The secondary bleed rate of 5.8% is comparable to the previously published Starship rate of 4.7% for all tonsillectomies performed over a 10 year period. Moving averages is a useful method to assess operative learning curves during training and beyond.

Recurrent Pneumococcal Meningitis Secondary to Nasoethmoidal Meningocele.

Since the widespread introduction of the conjugate pneumococcal vaccine and subsequent decline of invasive pneumococcal disease in children, a significant proportion of children with pneumococcal meningitis are identified as having an underlying immunodeficiency or predisposing anatomic abnormality. We present 3 cases of recurrent pneumococcal meningitis where detailed neuro-imaging revealed subtle anterior base of skull defects not detected on initial investigations.

Traqueomalacia pediátrica / Pediatric tracheomalacia

RESUMEN La malacia de la vía aérea central puede afectar la tráquea y/o los bronquios, haciéndola susceptible al colapso de sus paredes durante el ciclo respiratorio. Puede clasificarse como primaria o secundaria y clínicamente se manifiesta por síntomas respiratorios recurrentes o persistentes (especialmente en espiración), infecciones recurrentes y en casos severos, episodios de hipoventilación con cianosis. El diagnóstico se establece mediante broncoscopía flexible o rígida. Los estudios de imágenes se consideran complementarios, siendo especialmente útiles en casos de duda diagnóstica, estudio de causas secundarias o para la planificación preoperatoria. Su tratamiento depende de distintos factores como la severidad de los síntomas, su etiología, ubicación y extensión. En casos leves, una observación activa y tratamiento médico en espera de la resolución espontánea en los primeros 2 años, suelen ser suficientes. En casos moderados, la ventilación a presión positiva podría ser de utilidad. En casos severos está indicado el tratamiento quirúrgico, mediante distintas técnicas que buscan dar soporte a la estructura traqueobronquial debilitada, incluyendo traqueostomía, suspensiones directas (traqueopexias) o indirectas (aortopexia), tutores externos o stents intraluminales. En este articulo se revisarán las principales causas y tratamientos disponibles para la traqueomalacia pediátrica. Ya que su diagnóstico y manejo son complejos, es fundamental el trabajo de equipos médicos multidisciplinarios familiarizados con esta patología.

ABSTRACT Central airway malacia can affect the trachea and/or the main bronchi, making their walls susceptible to collapse during the respiratory cycle. It can be classified as primary or secondary, and clinically presents with recurrent or persistent respiratory symptoms (especially on expiration), recurrent infections and in severe cases, episodes of hypoventilation with cyanosis. The diagnosis is established by flexible or rigid bronchoscopy; imaging studies are considered as complementary, especially in cases of unclear diagnosis, secondary causes or for preoperative planning. Treatment depends on different factors such as the severity of the symptoms, their etiology, location and extension. In mild cases, active observation and medical treatment waiting for spontaneous resolution may be enough, which usually occurs in the first 2 years of age. In moderate cases, positive pressure ventilation could be useful. In severe cases, surgical treatment is indicated. Different techniques aiming to provide support to the weakened tracheobronchial structures are available, including tracheostomy, direct (tracheopexies) or indirect suspensions (aortopexy), external splints and intraluminal stents. In this article we present the main etiologies and available treatments for pediatric tracheomalacia. Given that diagnosis and management of these patients is complex, the work of multidisciplinary teams familiar with this pathology is of paramount importance.

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.

Recurrent post tonsillectomy bleeds: Presentation and characteristics in the paediatric population.

OBJECTIVE: To evaluate the incidence of, features and risk factors for recurrent post tonsillectomy bleeding. METHODS: Audit of all patients 0-15 years of age undergoing tonsillectomy between 01/2004-12/2014 with selection of those with recurrent bleeding post tonsillectomy for subanalysis. RESULTS: Of 5400 single centre tonsillectomies, 234 (4.8%) presented with bleeding and 9 (0.17%) presented with recurrent bleeding. 13 patients in total were treated, 10 surgically. Average patient age: 8.5 years with median time to presentation of the first bleed: 5 days and second bleed: 11 days. There was no association with abnormal coagulation, NSAID usage, severity of the first bleed or surgical technique. The risk of developing a second bleed following treatment for one post tonsillectomy bleeding incident is approximately 1 in 25. CONCLUSION: Recurrent post tonsillectomy bleeds are rare, and cannot be predicted preoperatively. The mainstay of management is surgical cautery, but other interventions are occasionally needed.

Retrotracheal Extraskeletal Ewing's Sarcoma: Case Report and Discussion on Airway Management.

Extraskeletal Ewing's sarcoma is a rare tumor, and the management of airway compromise in case of cervical Ewing's sarcoma has not been established. This report describes the case of a patient with retrotracheal Ewing's sarcoma and discusses a successful approach to airway management. A 12-year-old male presented with a 2-week history of sore throat and sleep-disordered breathing and 48 hours of stridor. Imaging confirmed a retrotracheal soft tissue mass with airway compromise. A planned and controlled approach to his airway management resulted in a secure airway prior to definitive treatment.

Neonatal airway obstruction in bilateral congenital dacryocystocoele: Case report and review of the literature.

Bilateral nasal obstruction due to simultaneous bilateral dacrocystocoeles is a rare and potentially life threatening condition. We present a five day old girl with this condition who presented with respiratory distress without any eye signs or symptoms associated with dacryocystocoeles. She was successfully managed with surgery and the clinical and radiological features of this condition are presented here with a review of the literature.

Long-standing inhaled foreign bodies in children: Characteristics and outcome.

OBJECTIVE: Aspirated foreign bodies in children present a potentially life-threatening condition and can be challenging to diagnose. This study aims to elucidate the characteristics and outcome of children with long-standing aspirated foreign bodies. METHODS: Retrospective case series of all cases of confirmed long-standing aspirated foreign bodies (LSAFB) between January 2003 to December 2015 in a single paediatric tertiary-level institution, defined as more than two weeks from choking episode or beginning of symptoms. RESULTS: Clinical files and operative records on 227 patients were screened and 35 children were confirmed to have been treated for LSAFB as per definition above. Median time to presentation was 4 weeks (mean 8.8 weeks). Eighty-six percent presented with cough and 51% with dyspnoea. Abnormal chest X-ray findings were found in 28 out 31 patients (90%). Organic foreign bodies (22) were more common than inorganic (14). Intraoperative granulation tissue was demonstrated in 89% of patients and in 46% of patients this was regarded as significant (defined as obstructing more than 50% of the involved airway lumen). Mean length of stay was 2.5 days. Nine patients (26%) had 11 respiratory complications; there were no mortalities. CONCLUSIONS: Paediatric LSAFB poses an uncommon diagnostic dilemma as there is often no witnessed history of aspiration event; and signs, symptoms and chest X-Ray findings are often non-specific. Laryngobronchoscopy is made more difficult by the presence of granulation tissue and the sequelae of prolonged non-treatment is a higher rate of chronic respiratory disease.

Botulinum toxin injections for chronic sialorrhoea in children are effective regardless of the degree of neurological dysfunction: A single tertiary institution experience.

OBJECTIVE: To determine the effectiveness of submandibular salivary gland Botulinum Toxin Type-A (BTX-A) injection in the treatment of drooling in children with varying degrees of neurological dysfunction. METHODS: A retrospective review of pre- and post-procedure drooling frequency and severity scores of patients receiving BTX-A between January 2008 and January 2013. Stratification to different subgroups of neurological impairment was performed according to Gross Motor Function Classification System (GMFCS) score. Drooling severity was assessed using Thomas-Stonell and Greenberg symptom questionnaires administered at time of initial consultation and 3 months after treatment. RESULTS: 48 sets of BTX-A injections in 26 patients with an average age of 9.45 years (range 7 months-18 years) were included in the study. Marked improvement in drooling was seen in 60.4% of patients, a marginal or brief improvement was seen in 20.8% and there was no improvement in 18.8%. No adverse events were reported following any of the BTX-A injections. BTX-A was safe and effective in the eight patients with pre-existing swallowing dysfunction. Subsequent drooling surgery was performed in 15 (57.7%) of the cohort, all 15 patients responded to BTX-A injections. In patients with Cerebral Palsy, there was no correlation between the severity of the neurological dysfunction as measured by the Gross Motor Function Classification System (GMFCS) score and the response to BTX-A treatment. CONCLUSIONS: Injection of BTX-A to the submandibular glands of children with neurological disorders is a safe procedure and results in a reduction in drooling in the majority of patients. Children with severe neurological dysfunction respond to BTX-A injections as effectively as their less impaired peers and the degree of response does not appear to be associated with the severity of neurological disability. BTX-A injection is a good initial procedure when drooling surgery is being considered.

The Yield of Multigene Testing in the Management of Pediatric Unilateral Sensorineural Hearing Loss.

OBJECTIVE: To explore the role and yield of multigene evaluation in children recently diagnosed with unilateral sensori-neural hearing loss (SNHL). STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary level pediatric institution. PATIENTS: Sixty children diagnosed with unilateral SNHL between January 2005 and December 2015. INTERVENTION: Targeted mutation analysis was performed in 51 children using DNA microarray for genotyping 11 different genes known to be correlated with nonsyndromic SNHL. MAIN OUTCOME MEASURE: Multigene testing results. RESULTS: Average age at diagnosis was 4.3 years. All children appeared to have nonsyndromic hearing loss (HL). HL was categorized as mild (17 children), moderate (17 children), severe (7 children), or profound (19 children). Genetic testing was performed in 51 (85%) children. Sixteen children (31.3%) were tested positive to connexin 26 (GJB2). One patient (2%) from this subgroup was homozygous and 15 were heterozygous. The average age of GJB2 positive children was 6 years and their average pure tone audiometry (PTA) was 75 dB. Computed tomography (CT) and/or magnetic resonance imaging (MRI) scans were performed in 43 children (71.66%). Out of the children who had a scan, 27 patients (62.8%) had negative findings on either CT or MRI scans. Sixteen (37.2%) patients had positive scan finding/s. CONCLUSIONS: Significant proportion of children with unilateral SNHL may have positive genetic testing while the vast majority of these children present with heterozygous mutations of connexin 26 (GJB2). Findings suggest that genetic evaluation has a role as a complementary modality in HL evaluation for pediatric unilateral SNHL although it may not be necessary to analyze for various abnormalities other than connexin 26 when practising in a limited resources environment.

Castleman's disease: An unusually young presentation resulting in delayed diagnosis of a neck mass.

We present a case report of a two year old child with an enlarging neck mass over a two year period. Her work-up included blood investigations, radiological imaging and fine needle aspirations which were non-diagnostic. At age 4 she had excision of her neck mass and histopathologic examination revealed unicentric Castleman's disease. This is one of the youngest reported cases in the literature and highlights the need to include CD in the differential diagnosis of neck masses in young children. Castleman's disease (CD) is a rare disease of benign lymph node enlargement which most commonly occurs in adults. While the multi-centric variant is associated with HIV and HHV-8 infection, the aetiology of unicentric disease remains uncertain.

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.

Safety of paediatric day-stay laryngeal surgery for recurrent respiratory papillomatosis.

OBJECTIVES: Safety assessment of day-stay laryngeal surgery in a cohort of children with recurrent respiratory papillomatosis (RRP). Recurrent respiratory papillomatosis is a chronic debilitating disease which usually requires multiple recurrent interventions under general anaesthesia. Day-stay surgery is an attractive option as it allows avoiding the inconvenience and costs of routine overnight admissions while recovering in the safe environment of the family home. This is the first study to assess the safety of day-stay laryngeal surgery in this cohort of patients. METHODS: Retrospective cohort study of all consecutive RRP procedures performed between December 1998 and May 2015 in a single paediatric tertiary-level hospital. RESULTS: A total of 465 surgical procedures were performed in 20 patients. Average age on diagnosis was 4.5 years. 415 (89.25%) of the procedures were done as day cases without overnight admission. Average number of procedures per patient was 20 and 25 for Children positive to HPV6 and HPV11, respectively. Only one patient after one single procedure (presenting 0.21% of total procedures, 0.24% of day-stay procedures) represented after discharge. CONCLUSIONS: Day-stay surgery for children with RRP has a favourable safety profile in selected cases.

The S/Z ratio: a simple and reliable clinical method of evaluating laryngeal function in patients after intubation.

OBJECTIVES: 1. To determine the consequences of prolonged intubation on laryngeal function. 2. To evaluate simple clinical criteria or tests that could alert the clinician to potential laryngeal pathology requiring ear, nose, and throat/otolaryngology (ENT) referral. DESIGN: A prospective case series. SETTING: A surgical intensive care unit in a tertiary academic hospital in Cape Town, South Africa. PARTICIPANTS: Thirty-two patients who had undergone a period of translaryngeal intubation for a period greater than 12 hours. MAIN OUTCOME MEASURES: 1. Patient subjective voice change rating. 2. Clinician assessment of laryngeal function. 3. S/Z ratio. 4. Presence of laryngeal pathology on endoscopic assessment of the larynx. RESULTS: Upon initial evaluation within 6 hours of extubation, 26 (81%) of patients exhibited symptomatic laryngeal dysfunction. At this stage, 13 (40%) had S/Z ratios greater than 1.4. The degree of dysfunction as described by subjective scoring and the S/Z ratio was proportional to the duration of intubation. After 24 hours, 23 (72%) patients' voices had improved subjectively; and the S/Z ratio exceeded 1.4 in just 6 patients (19%). Of these 6 patients, 4 exhibited laryngeal pathology on flexible nasoendoscopy. These 4 patients were followed up over 1 year, and 1 patient was ultimately offered a vocal cord medialization procedure. The S/Z ratio is 100% sensitive and 93% specific, with an accuracy of 93.75%, in diagnosing laryngeal pathology requiring ENT referral. CONCLUSIONS: 1. A period of laryngeal intubation carries signification risk of laryngeal dysfunction. Most, but not all, dysfunction resolves within 24 hours. 2. Residual laryngeal pathology can be reliably and simply screened for by the use of the S/Z ratio. We recommend that patients with an S/Z ratio greater than 1.4 more than 24 hours after extubation require ENT referral for laryngoscopy.

Magnetic resonance and computed tomography imaging of a grade IV papillary endolymphatic sac tumour.

The endolymphatic sac tumour (ELST) is an adenomatous neoplasm of the papillary pattern originating from the endolymphatic sac's epithelium. We describe the computed tomography and magnetic resonance imaging features of a rare grade IV tumour with extensive skull base, cerebello-pontine and nasopharyngeal spread as well as involvement of the left temporomandibular joint. Papillary ELST may easily be misinterpreted on histopathological and even on immunohistochemical examination with other papillary lesions. Thus the radiological imaging features and localization in conjunction with histopathological features and clinical presentation play a paramount role in making the correct diagnosis.