Congenital nasal pyriform aperture stenosis; our experience of 34 cases.

OBJECTIVES: To study our population of patients with congenital nasal pyriform aperture stenosis (CNPAS) in terms of incidence and socioeconomic status; the effect of pyriform aperture size, gestational age, birth weight, and whether congenital abnormalities are associated with surgical requirement. METHODOLOGY: Retrospective case note review of all patients treated for CNPAS at a single tertiary paediatric referral site was undertaken. Diagnosis was made on the basis of a pyriform aperture of <11 mm on CT scanning; patient demographics were collected to explore risk factors for surgery and surgical outcomes. RESULTS: 34 patients were included in the series, 28 (84%) of whom underwent surgery. 58.8% of subjects had an associated mega central incisor. A smaller pyriform aperture size was seen in neonates requiring surgery (4.87 mm ± 1.24 mm vs 6.55 mm ± 1.41 mm, p = 0.031). There was no difference in gestational age in neonates requiring surgery (p = 0.074). Requirement for surgery was not associated with co-existing congenital anomalies (p = 0.297) or lower birth weight (p = 0.859). Low socioeconomic status was not significantly associated with requiring surgery but a potential link between CNPAS and deprivation was identified (p = 0.0583). CONCLUSION: These results suggest that a pyriform aperture of less than 6 mm requires surgical intervention. Associated birth anomalies add additional management considerations but in this cohort were not associated with increased need for surgery. A potential association between CNPAS and low socioeconomic status was identified.

Paediatric tracheobronchomalacia: Incidence, patient characteristics, and predictors of surgical intervention.

OBJECTIVES: Tracheobronchomalacia (TBM), a condition where an abnormality of the tracheal walls causes collapse during the respiratory cycle, is a common cause of airway obstruction in childhood. TBM can present with a large spectrum of disease severity and underlying pathologies that may be managed medically and surgically, and it is not always clear which patients would most benefit from surgical intervention. We aim to describe the incidence, patient characteristics, and predictors of surgical intervention in a large cohort of paediatric patients. METHODS: We performed a retrospective review of all children diagnosed with TBM to a paediatric Otolaryngology unit in the west of Scotland between 2010 and 2020. Odds ratios for clinical predictors of surgery were calculated using logistic regression with uni- and multivariate analysis. RESULTS: 249 patients were identified of which 219 proceeded to data collection. Primary malacia was noted in 161 (73.5%) and secondary in 58 (26.5%). Causes of secondary malacia included compression by the innominate artery (11%) and vascular rings (7.8%). Surgical interventions were performed in 28 patients (12.8%) including division of vascular ring, aortopexy, and surgical tracheostomy. Multivariate analysis showed secondary TBM, acute life-threatening events, and difficulty weaning from mechanical ventilation were independent risk factors for surgical intervention. CONCLUSIONS: TBM can present with a myriad of airway symptoms and is frequently associated with other airway and mediastinal pathologies necessitating multiple interventions. Children aged <1 year present with a more severe form of the disease and the presence of particular independent risk factors may indicate a need for surgical intervention.

The prevalence of airway problems in children with Down's syndrome.

AIM: Airway disorders are common in children with Down's syndrome. We report the findings on airway endoscopy in a birth cohort of children from a well-defined geographical area, in order to estimate true population prevalence of airway problems in children with Down's syndrome. METHOD: Retrospective case note review over a 20-year period between 1993 and 2013 for all children in Greater Glasgow born with Down's syndrome, identified through the hearing surveillance programme. All children undergoing airway endoscopy under general anaesthesia for investigation of potential airway symptoms (stridor, hoarseness, recurrent croup and difficulties with intubation/extubation) were studied in detail to identify the number with laryngeal, tracheal or bronchial pathology. RESULTS: All 239 children (F:M=1.15:1) were reviewed. Of these, 39 (16.3%) underwent microlaryngoscopy-bronchoscopy under general anaesthesia for airway symptoms. The main presentations were stridor (9), extubation problems (12) and exacerbations of recurrent croup (7). Thirty-three were found to have at least one airway diagnosis (13.8%) including trachaeobronchomalacia (17), laryngeal cleft (2), laryngomalacia (2), tracheal compression (2), vocal cord paralysis (1), acquired tracheal stenosis (2) and symptomatic subglottic stenosis (14). CONCLUSION: Laryngo-tracheo-bronchial pathology is much more common in children with Down's syndrome than in the general population, particularly subglottic stenosis and tracheal problems.