Laryngotracheal reconstruction outcomes in hypotonic children

IMPORTANCE: Children with poor muscle tone may demonstrate upper airway obstruction due to several mechanisms including obstructive sleep apnea, laryngopharyngeal reflux, and laryngomalacia. Though hypotonia has been shown to compromise the pediatric airway, and some authors suggest that neurologic deficits can compromise the success of laryngotracheal reconstruction (LTR), to our knowledge no studies have evaluated the effect of neurologic diagnoses or hypotonia on outcomes in LTR. OBJECTIVE: To determine whether hypotonic children with subglottic stenosis have lower rates of successful decannulation after LTR compared with children without neurologic deficit. DESIGN, SETTING, AND PARTICIPANTS: A retrospective medical chart reviewwas conducted for 27 children aged 0 to 6 years, who underwent LTR for subglottic stenosis between December 2007 and December 2012 at a tertiary care children's hospital. Children were classified based on documented neurologic findings. Group 1 comprised those children without neurologic impairment (n = 16). Group 2 included those children with a documented neurocognitive or neuromuscular diagnosis but without evidence of hypotonia (n = 7). Group 3 comprised hypotonic children (n = 4). INTERVENTIONS: Laryngotracheal reconstruction. MAIN OUTCOMES AND MEASURES: The number of procedures performed after LTR to optimize the airway and whether the child was successfully decannulated. RESULTS: All 16 of the neurologically intact patients (100%) were decannulated. Among children with a neurologic deficit, 5 of 7 (71%) were ultimately decannulated. No hypotonic children 0 of 4 were decannulated. The difference in rates of decannulation between unaffected and normotonic children with a neurologic deficit was not statistically significant (P = .08). However, the difference in outcomes between hypotonic children and neurologically intact patients was statistically significant (P < .001). CONCLUSIONS AND RELEVANCE: Findings from this study suggest that hypotonic children may experience poorer rates of post-LTR decannulation compared with children without neurologic deficit. Dynamic upper airway obstruction may be unappreciated in hypotonic children. Future research may be directed at the appropriate evaluation and treatment of children with poor muscle tone and subglottic stenosis.