The management of subglottic stenosis in patients with wegener's granulomatosis

Abstract

Wegener's granulomatosis (WG) is a multisystem inflammatory disease characterized by vasculitis, granuloma formation, and necrosis. Among 158 patients treated at the National Institutes of Health during the past 24 years, 145 (92%) had an otolaryngologic manifestation of their disease and 25 (16%) had subglottic stenosis (SGS). SGS varied from asymptomatic to life-threatening. Sixteen (80%) of 20 patients with fixed SGS required surgical intervention, including manual dilations, carbon-dioxide laser resections, and laryngotracheoplasty (LTP). LTP was performed with and without microvascular reconstruction. Thirteen of the patients required tracheostomy and all 13 were ultimately decannulated. Five patients who repeatedly failed dilations and/or endoscopic laser surgery underwent LTP. Since 1987, two patients have undergone LTP with microvascular free flaps. Both patients were subsequently decannulated. The authors' experience demonstrates that management of SGS in WG is complex, requiring individualized frequent multimodality interventions to achieve satisfactory results. Microvascular laryngotracheal reconstruction should be considered in the surgical armamentarium for patients with persistent stenoses.

Airway manifestations in childhood granulomatosis with polyangiitis (Wegener's)

“…It has been recognized that in distinction from adults, childhood GPA patients develop nasal deformity and SGS more frequently (4 -6,8,10,11). Large adult GPA studies have found a 24% prevalence of nasal deformity and a 10% prevalence of SGS (4,11). In concurrence with previous pediatric studies, 29% of our patients developed nasal deformity (aforementioned range 20 -48%) and 43% developed SGS (historical range 41-48%) (4 -6).…”

“…Details regarding airway manifestations in childhood GPA have been limited in prior studies (4 -9). Differences between adult and childhood GPA include statistically higher prevalences of nasal deformity and subglottic stenosis (SGS) (4,6,8,10,11). Additionally, it has been proposed that GPA may encompass 2 diseases: limited and generalized (12).…”

Estenose subglótica como manifestação clínica da granulomatose de Wegener em adolescentes: relato de caso e revisão de literatura

“…1 Ainda, 85% das manifestações clínicas iniciais ocorrem em maiores de 20 anos de idade, sendo estas raras na população pediátrica, cuja incidência varia de 6 a 14%. 2,3 As formas clínicas podem variar de localizadas ao acometimento multissistêmico. Critérios para definição de doença incluem: comprometimento nasal ou oral; alterações radiográficas características; microhematúria ou cilindros hemáticos em exames de urina e alteração histológica compatível com inflamação granulomatosa de parede arterial ou perivascular.…”

“…5,6 Os protocolos de tratamento medicamentoso incluem a utilização de glicocorticóides e/ou imunossupressores, dentre eles a ciclofosfamida, permitindo índices satisfatórios de remissão da doença. 1,3,5,7,8 As manifestações otorrinolaringológicas têm elevada prevalência, tanto em apresentações clínicas iniciais, como fazendo parte do curso habitual da doença. Sintomas nasofaríngeos e laríngeos podem estar presentes em até 73% dos casos.…”

“…Sintomas nasofaríngeos e laríngeos podem estar presentes em até 73% dos casos. 3 Rinite resistente ao tratamento medicamentoso habitual incide em 65% das fases iniciais da Granulomatose de Wegener. 1 Labovics et al 3 relataram sinusopatia com incidência de 85%.…”