Decannulation in children affected by congenital central hypoventilation syndrome: A proposal of an algorithm from two European centers

Abstract

Rationale Long‐life ventilatory assistance is necessary for survival in pediatric patients with congenital central hypoventilation syndrome (CCHS). Invasive mechanical ventilation (IMV) through tracheostomy is the most used method, especially in the first years of life when the central nervous system is maturing. Nevertheless, IMV via tracheostomy is not ideal because tracheostomy in children is associated with impaired speech and language development, also frequent infections of the lower airway tract occur. Objective Only few reports describe the transition from IMV to the noninvasive method, ending with decannulation in CCHS affected patients. We aim to provide our experience regarding decannulation program in CCHS affected children and to describe a proposal of an algorithm concerning transition from invasive to noninvasive ventilation (NIV) in CCHS patients. Methods The study has been conducted retrospectively. Four children from two European centers underwent tracheostomy removal and decannulation, upon request of patients and their families. Results All children were trained to carry out tracheostomy capping before decannulation and underwent endoscopic assessment of upper and lower airway. Subsequently they started training to NIV at mean age of 106.25 months (±40.7 SD). Decannulation occurred 12 months after and no patients needed the reintroduction of tracheal cannula in either short or long term follow up. Conclusions: our study shows that effective liberation from IMV, the transition to NIV and decannulation are possible in CCHS affected children and offers a proposal of an algorithm which can be applied in selected centers.

Guidelines for diagnosis and management of congenital central hypoventilation syndrome

“…Transition from tracheostomy to mask ventilation may be considered at varying ages, mainly for those with adequate ventilation during wakefulness [66]. Some authors consider the optimal time for transition as being older children (e.g.…”

Trastornos De Hipoventilación Central Del Sueño en Pediatría: Causas Poco Frecuentes

“…La ventilación por traqueostomía es el modo más eficáz en los primeros años de vida, garantiza una adecuada ventilación y oxigenación (3,9) hasta que los niños alcancen una adecuada maduración del SNC y aparato respiratorio (16,23,41); la Sociedad Americana de Tórax (American Thoracic Society-ATS) recomienda la ventilación por traqueostomía durante los primeros 6 a 8 años de vida en CCHS, para optimizar un desarrollo neurocognitivo adecuado (16,17,19). La traqueostomía debe ser realizada antes del mes de vida, las cánulas usadas no tienen balón y son de menor tamaño, para permitir una fuga lo suficientemente adecuada como para usar válvulas de habla unidireccional (17,19,23).…”

“…Al respecto hay poco descrito en niños con ROHHAD, casos aislados reportan con éxito el tratamiento de la hipoventilación alveolar utilizando AVAPS (42). La transición de ventilación invasiva por traqueostomía a VNI, ocurre principalmente en pacientes con fenotipos leves de CCHS que ventilan adecuadamente durante la vigilia (17,18,43); sin embargo hasta la fecha no existen indicaciones concretas sobre el momento óptimo para la transición, porque es una opción que está muy vinculada a las características propias de cada paciente y su entorno familiar; pero culminar un programa de decanulación antes de la adolescencia puede ser una buena opción (41,44). Aunque se han reportado casos aislados de éxito donde se logra la transición a edades más tempranas (3 años), enfatizando que todo caso debe ser analizado de manera personalizada (18).…”

Long Term Non-invasive Ventilation in Children With Central Hypoventilation

“…An increasing number of children with CCHS have been successfully transitioned from invasive ventilation to BiPAP ventilation and recently a proposal of an algorithm for decannulation was published (35). By the moment there are not specific indications about optimal time to switch from tracheostomy to NPPV because it is a choice closely linked to the patient and his family, but ending the decannulation program before adolescence can be a good option.…”