Acquired Tracheomalacia - A Case Report

Tracheomalacia is a condition characterized by weakness of the tracheal walls and supporting cartilage. It may be congenital, occurring in association with other lesions, such as vascular ring, tracheoesophageal fistula, dyschondroplasia, congeuital cysts or tumors, or it may be acquired, most commonly as a result of protracted endotracheal intubation and less often of irradiatiou, trauma or neoplasm. We present a case of acquired tracheomalacia in a 15-year-old boy, a victim of Proteus syndrome (hemihypertrophy, subcutaneous tumors, and macrodactyly), who had congenital scoliosis with secondary chronic restrictive pulmonary disease, for which he was admitted to our hospital for surgical correction of the spine. Unfortunately, his conditions, particularly of respiratory, worsened after the operation, which called for an emergent tracheostomy. During the procedure, high airway pressure and CO_2 retention developed, and an extreme narrowing of the tracheal lumen was revealed by fiberoptic bronchoscopy. Acquired tracheomalacia was diagnosed. The probable mechanisms, diagnostic tests, and treatments of tracheomalacia are hereunder discussed.

關鍵字

Tracheal disease: tracheomalacia ； Complications: intraoperative ； Neoplasms, multiple primary: Proteus syndrome

並列摘要

氣管軟化症是一種因氣管壁與軟骨缺損而造成氣管塌陷的一種疾病。其原因可以是先天性的，如血管環、氣管食道瘻、軟骨發育不良、先天性囊腫或腫瘤所造成的；也可以是後天性的，如長時間的氣管插管、放射治療、外傷或腫瘤所造成。本文報告一位十五歲患有Proteus syndrome（半側肥大、皮下腫瘤與多指）的男孩，因合併先天性脊柱側彎與慢性限制性肺病，至本院做脊柱矯正手術。因術後病情惡化，於施行氣管造口術時，發生氣道壓力昇高與二氧化碳滯留的現象。軟式支氣管鏡檢查顯示非常狹窄的氣管管腔，為後天性氣管軟骨軟化症。本文討論此病症發生的可能機轉、診斷與處理的方法。

並列關鍵字

氣管疾病：氣管軟骨軟化；併發症：術中；贅生物，多重原發性：變形徵候群

國際替代計量

Acquired Tracheomalacia - A Case Report

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