先天性鼻梨狀口狹窄－三例報告

Congenital Pyriform Aperture Stenosis – Report of Three Cases

李振川(Jehn-Chuan Lee) ； 李國森(Kuo-Sheng Lee) ； 黃榮貴(Jon-Kway Huang) ； 陳立盛(Lih-Sheng Chen) ； 翁韶嶽(Shau-Yueh Wong) ； 楊政謙(Cheng-Chien Yang) ； 張克昌(Ko-Chang Chang)

《中華民國耳鼻喉科醫學會雜誌》 33卷1期 (1998/02) Pp. 81-85

https://doi.org/10.6286/1998.33.1.81

摘要

先天性鼻梨狀口狹窄是造成鼻部阻塞的罕見疾病。臨床表現為鼻塞、呼吸窘迫、周期性發紺等症狀，其症狀：與後鼻孔閉鎖極為相似。先天性鼻梨狀口狹窄可能非單一異常，可能與中臉成骨不全（midfacial dysostosis）合併腦下垂體內分泌軸及中樞神經系統異常有關。單一上門齒是重要的臨床表徵，可作為早期診斷的線索。電腦斷層攝影可作正確診斷並描述其他相關部位的異常。治療須先考慮整體預後及狹窄程度，初期可以McGovern氏奶嘴維持呼吸道，若重度狹窄的話，應以唇下路徑（sublabial approach）去除過度增生之骨性外壁。本報告報告3例先天性鼻梨狀口狹窄之臨床表現及療程，並討論其病因與治療方法。

關鍵字

單一上門齒 ； 先天性鼻梨狀口狹窄

並列摘要

Congenital pyriform aperture stenosis is an uncommon cause of nasal airway obstruction in infants. This anomaly may produce manifestations of nasal airway obstruction, respiratory distress, and cyclic cyanosis similar to those seen in bilateral choanal atresia. It may represent more than an isolated congenital abnormality of the airway, and eventually may be a midfacial dysostosis with associated endocrine and central nervous system abnormalities. A solitary maxillary central incisor is the most important clue to make early diagnosis. Computed tomography confirms the diagnosis and delineates the anomaly. Management depends firstly on overall prognosis of the patient and secondly on the severity of obstruction. The airway may be managed initially with McGovern nipple and severely affected patients may undergo repair with a sublabial approach to excise hypertrophic bony portion of the aperture. This article reports clinical presentation of three cases with congenital pyrifrom aperture stenosis and discusses its pathogenesis and treatment.

並列關鍵字

Solitary maxillary central incisor ； congenital nasal pyiform aperture stenosis