Wegener's Granulomatosis: Image Findings in Head and Neck

Wegener's肉芽腫是一種全身性壞死性的血管炎，經常以侵犯頭頸部為其最早表現，許多病人不容易在早期被診斷而延誤治療，本文的目的是希望找出Wegener's肉芽腫在頭頸部常見的影像表徵進而協助早期診斷這個病病。我們研究了本院過去十五年來，經由臨床及病理確定診斷為Wegener's肉芽腫的十七個病人的影像表徵及臨床症狀，結果發現常見的臨床症狀包括鼻塞，呼吸困難，聽力及視力受損，眼球凸出及聲音沙啞，其中七個病人檢測血清中c-ANCA濃度，結果均呈陽性反應，十三個病人接受顱部X光檢查，主要的X光檢查發現為副鼻竇及乳突氣室變模糊。所有的十七個病人均接受頭頸部電腦斷層檢查，常見的電腦斷層檢查發現為副鼻竇內黏液積存，副鼻竇或鼻腔的軟組織增厚，聲門下狹窄合併腫塊，構成副鼻竇的骨骼增厚，眼眶內腫塊及乳突氣室內液體積存。有二例接受磁振造影檢查，磁振造影可更清楚的看到病灶。十七個病人中的七人發現有聲門下狹窄合併腫塊，較過去的報告高出許多；眼眶內腫塊常合併眼球凸出及鼻竇或鼻腔的病灶。所以有下列影像學的發現時，我們必須將Wegener's肉芽腫列入鑑別診斷，它們包括：不明原因的聲門下狹窄，反覆發作的鼻竇炎或中耳炎且一般治療無效，眼眶內腫塊合併眼球凸出，鼻腔內破壞性腫塊及合併肺臟，腎臟或其他全身性病灶。此外，Wegener's肉芽腫的頭頸部病灶在磁振造影的T1WI及T2WI均可表現出低訊號，亦具有相當的特徵性。

關鍵字

頭，電腦斷層；頭，磁振造影；頸，電腦斷層；頸，頸磁振造影；血管炎； Wegener's肉芽腫

並列摘要

Wegener's granulomatosis is a systemic necrotizing granulomatous vascuiltis that in its earliest presentation frequently involves the head and neck. Offen it is not diagnosed at its initial stage so management of the disease is delayed. We believe in determining the common image findings of Wegener's granulomatosis will help in early diagnosis of this disease. In this study, we retrospectively review 17 cases of clinically and pathologically proved Wegener’s granulomatosis seen in our hospital from Sep 1982 to Apr 1997. The clinical findings, plain films, CT scan and MRI were reviewed. Serum titers of c-ANCA were tested in 7 of the 17 patients. The results showed that the common clinical presentations were nasal obstruction, dyspnea, hearing impairment, visual impairment, proptosis, and hoarseness. All of the 7 cases tested with serum titers of c-ANCA showed positive results. The major findings of the plain films were obliteration of paranasal sinuses or mastoid air cells. The common CT findings were fluid collection in the paranasal sinuses, soft tissue thickening along the inner wall of paranasal sinuses or nasal chamber, subglottic stenosis with enhanced soft tissue mass, orbital mass lesion, sclerotic change of the wall of paranasal sinuses and fluid collection in the mastoid air cells. MRI findings in 2 patients detected the extension of the lesion more clearly. Subglottic stenosis with mass lesions were present in 7 of our 17 cases (41%) and the ratio was higher than in those previously reported in the literature. Mass lesions or infiltrations in orbital cavity were frequently associated with proptosis and disorders of the paranasal sinuses or the nasal chamber. The image findings which alerted us to initiated Wegener’s granulomatosis into differential diagnosis included: unexplained subglottic stenosis; recurrent sinusitis or otitis refractory to management; mass lesion in orbital cavity with proptosis; destructive nasal mass lesion; and accompanying renal, pulmonary or other systemic lesions. Hypointense lesions on T2WI of MRI in the head and neck were also highly suggestive of Wegener's granulomatosis.

並列關鍵字

Head, CT ； Head, MRI ； Neck, CT ； Neck, MRI ； Vasculitis ； Wegener's granulomatosis

國際替代計量

Wegener's Granulomatosis: Image Findings in Head and Neck

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