Should paediatric tonsillar asymmetry be an indication for tonsillectomy? A single centre experience.

BACKGROUND: Paediatric tonsillar lymphoma (TL) is a rare diagnosis. Historically, the presence of clinical features such as tonsillar asymmetry, grossly abnormal tonsil appearance and cervical lymphadenopathy raise concern for this diagnosis. Tonsillar asymmetry is considered to be the most concerning clinical feature; however, asymmetry is often apparent due to differences in depth or shape of tonsillar fossa and tonsillar pillars, rather than a true difference in volume. There is debate whether a tonsillectomy is required in all cases of tonsil asymmetry to exclude lymphoma, and what clinical features should raise concern. The aim of this study was to establish whether the presence of clinical asymmetry can be deemed a reliable marker for genuine tonsil size discrepancies. We also sought to evaluate the clinical and examination characteristics that are concerning for lymphoma. METHODS: Retrospective review of clinical records for paediatric tonsil specimens sent for histological evaluation between 1 January 2012 and 1 January 2023 driven by a clinical suspicion of lymphoma at Starship Children's Hospital, New Zealand. Patient demographics and clinical data were recorded. A comparison was made between tonsil size asymmetry on clinical examination (Brodsky criteria) and tonsil volume difference based on dimensions given in pathology reports. RESULTS: One hundred and forty-three patients had tonsillectomies between 2012 and 2022 at Starship Children's Hospital due to concern for lymphoma. Of these, three were positive for lymphoma. Presence of pain and abnormal tonsil appearance were predictors for lymphoma (p<0.02). Interrater reliability agreement between clinical size difference and tonsil volume was poor, Kappa= -0.13 p<0.05. CONCLUSION: Clinical size difference is a poor predictor for true tonsil volume difference. We advise that assessment of tonsil size should be performed in conjunction with the examination of gross visual abnormalities and lymphadenopathy to guide clinical decision making.

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 epidemiology of Scottish trauma: A comparison of pre-hospital and in-hospital deaths, 2000 to 2011.

AIMS: To characterise the temporal trends and urban-rural distribution of fatal injuries in Scotland through the analysis of mortality data collected by the National Records of Scotland. METHODS: The prospectively collected NRS database was queried using ICD-10 codes for all Scottish trauma deaths during the period 2000 to 2011. Patients were divided into pre-hospital and in-hospital groups depending on the location of death. Incidence was plotted against time and linear regression was used to identify temporal trends. RESULTS: A total of 13,100 deaths were analysed. There were 4755 (36.3%) patients in the pre-hospital group with a median age (IQR) of 42 (28-58) years. The predominant cause of pre-hospital death related to vehicular injury (27.8%), which had a decreasing trend over the study period (p = 0.004). In-hospital, patients had a median age of 80 (58-88) years and the majority (67.0%) of deaths occurred following a fall on the level. This trend was shown to increase over the decade of study (p = 0.020). In addition, the incidence of urban incidents remained static, but the rate of rural fatal trauma decreased (p < 0.001). CONCLUSIONS: Around a third of Scottish trauma patients die prior to hospital admission and the predominant mechanism of injury is due to road traffic accidents. This contrasts with in-hospital deaths, which are mainly observed in elderly patients following a fall from standing height. Further research is required to determine the preventability of fatal traumatic injury in Scotland.

Acute upper airway obstruction and emergency front of neck access in an achondroplastic patient.

Dwarfism is defined as a failure to attain a height of 148 cm in adulthood. Achondroplasia is the most common form of short-limbed dwarfism. Although this condition is relatively rare, with an incidence of 0.5-1.5 per 10,000 live births, most medical professionals will come across the achondroplastic dwarf (AD) during their career. Faulty endochondral ossification produces the characteristic short stature phenotype, as well as severe craniofacial, central nervous system, spinal, respiratory and cardiac anomalies. These unusual characteristics may present airway management difficulties in elective as well as emergency situations. Within the literature there is very little information regarding the emergency insertion of a surgical airway in an adult AD. We present our experience of this situation in the form of a case report and a review of the relevant literature.