ABSTRACT
OBJECTIVE: To examine the prevalence of airway involvement in children with Wegener's granulomatosis (WG) at our institution and to evaluate the utility of a treatment paradigm involving a multidisciplinary team. DESIGN: Retrospective medical chart review. SETTING: Tertiary children's hospital. PATIENTS: Pediatric subjects with WG treated at a tertiary children's hospital over the past 15 years. MAIN OUTCOME MEASURES: The medical records of all subjects with airway lesions were reviewed for clinical characteristics of airway involvement and for the medical and surgical treatment regimens. RESULTS: Seven of 28 pediatric patients with WG were identified to have airway lesions (25%), including vocal fold granuloma, subglottic stenosis, and multilevel stenoses. Three of these patients had isolated and limited lesions. The 4 remaining patients are the focus of this study. One patient underwent a cricotracheal resection. All patients underwent repeated surgical treatment from combined services (otolaryngology and pulmonology) with flexible and rigid endoscopy, dilatation, and corticosteroid injection to manage subglottic, tracheal, and bronchial stenoses. Operative findings were communicated to the rheumatology service for manipulation of medical therapy. The 3 most recent patients received the anti-CD20 chemotherapeutic agent rituximab. This treatment approach led to temporary remission in some of the patients. CONCLUSIONS: Airway involvement in pediatric patients with WG is known to occur, but multilevel airway involvement is rare and clinically challenging. We advocate a combined surgical approach involving otolaryngology, pulmonology, and rheumatology in managing aggressive cases of multilevel airway involvement in WG.
Subject(s)
Granulomatosis with Polyangiitis/complications , Laryngeal Diseases/etiology , Laryngeal Diseases/therapy , Adolescent , Child , Combined Modality Therapy , Female , Granuloma/etiology , Granuloma/therapy , Humans , Laryngostenosis/etiology , Laryngostenosis/therapy , Male , Patient Care Team , Prevalence , Retrospective Studies , Treatment Outcome , Young AdultABSTRACT
OBJECTIVE: To determine whether unilateral or bilateral cochlear implantation affects the functional balance of children when compared with children with severe-to-profound bilateral hearing loss without a cochlear implant. DESIGNS: An observational case control study. METHODS: Three cohorts of children aged 4 to 17 years were studied: those with unilateral cochlear implants, those with bilateral cochlear implants, and nonimplanted children with severe-to-profound bilateral hearing loss. Each participant's balance was assessed with the Bruininks-Oseretsky Test, Second Edition (BOT2). Raw test scores and age- and sex-matched scale scores were obtained and used to compare each of the 3 groups with one another as well as with standardized norms. RESULTS: Sixty-four children were tested. Twelve had a unilateral cochlear implant, 26 had bilateral cochlear implants, and 26 were nonimplanted. The mean scale scores for the unilateral cochlear implant group, the bilateral cochlear implant group, and the children with severe-to-profound hearing loss were 10.16, 11.31, and 11.15, respectively. The mean scale scores for the 3 groups did not differ significantly from each other. When compared with the standardized norms, all 3 groups had significantly lower scores on the BOT2 than the accepted mean score. CONCLUSION: Balance, as measured by the BOT2, is not significantly different in children with cochlear implants versus a hearing-impaired population. However, all 3 tested groups scored lower than the validated mean score for a healthy population of children.