Wegener 肉芽腫症による軟口蓋全欠損に対しスピーチエイドを装着した1例

Abstract

We report a case of a total soft palate defect caused by Wegener’s granulomatosis, treated by application of a speech aid. The patient was a 25-year-old man with a tumor accompanied by an ulcer and tenderness of the soft palate. The ulceration was extensive, and the soft palate was perforated. Finally, the soft palate became totally involved and showed an extensive defect. We obtained a biopsy specimen from the granulomatous reddish gingiva, so called “strawberry gums.” Clinical appearance, a positive finding for PR3-ANCA, and histopathological findings led to a diagnosis of the upper respiratory tract-limited form of Wegener’s granulomatosis. Although the symptoms such as ulceration and tenderness were improved by administration of steroids and immunosuppressants, nasal leakage of food and hypernasality were severe. We decided to apply a speech aid. The obturator was adjusted by functional impression of the pharyngeal area, so that the space between the obturator and pharyngeal posterior wall would be narrowed. It took only 2 weeks to complete the speech aid fabrication. Remarkable improvement of velopharyngeal function and speech intelligibility was confirmed on logopedic and phoniatric evaluation. We considered application of a speech aid to be effective for a patient with a total soft palate defect caused by Wegener’s granulomatosis who had vascular abnormalities and required long-term administration of steroids and immunosuppressants, because it could be fabricated and applied within a short time, without an invasive procedure.