Tracheal Agenesis: Report of One Case

氣管發生不全是新生兒期極罕見且致命的先天性異常疾病之一，其發生率小於0.002%。自1900年以來，在文獻上僅有61例報告且只有極少數在死前診斷出來。我們報告一個病例，本病例爲一足月兒，出生外觀無異常，但明顯發紺，心跳減慢及哭不出聲，經由多次急救後失敗，故轉送到本院新生兒加護病房。在本院新生兒加護病房給予重新氣管插管多次均失敗，在一次似科成功的插管後，病人轉爲紅潤，但胸部X光證實爲插入食道，此時即高度懷疑本病人爲一氣管發生不全合併氣管食道瘻管，以緊急電腦斷層攝影證實，其呼吸道在聲帶下5mm以下完全逸失，而氣管分叉處，支氣管及肺實質均正常，在家屬無積極治療意願下，病人於第六天死亡，經解剖證實本病人爲氣管發生不全第二型合併氣管食道瘻管。氣管發生不全是一種極罕見的病例，其特徵爲出生即發紺，插管困難及哭不出聲，雖病人可籍由食道插管再經氣管食道瘻管保持肺部通氣，但終非長久之計，目前文獻上有報告嘗試外科療法，但不幸尚無病例存活，爲一致命之疾病，又因僅有少數於死前診斷出本病，我們建議可配合喉鏡及電腦斷層攝影幫助此一診斷的建立。

關鍵字

氣管發生不全；氣管食道瘻管

並列摘要

In this report, we present a newborn male infant with tracheal agenesis. At birth the term baby was cyanotic, bradycardic, and had a failure to cry. His Apgar scores were 3 and 3 at 1 and 5 minutes after birth. Immediately after birth, an endotracheal intubation was unsuccessfully attempted; however, a chest excursion was visible with intubation through the esophagus. Tracheal agenesis with esophago-tracheal fistula was highly suspected. Direct endoscopy and emergency computed tomography were performed and revealed tracheal agenesis. The baby died on day 6, and an autopsy confirmed the diagnosis. Tracheal agenesis (TA) is a rare cause of respiratory distress in the newborn. Cyanosis at birth, difficulty to perform an endotracheal intubation and a failure to cry are the characteristics of TA. Although continuous mechanical support through the esophagus can maintain vital functions, there are no effective medical or surgical method to correct the congenital abnormality currently. The longest survivor of all of the infants with TA had no more than 6 wks of live. In a review of the current literature, only a few cases have had the diagnosis established antemortem. We report a case of tracheal agenesis diagnosed by emergency computed tomography (CT). The role of CT in establishing this diagnosis has been very useful.

並列關鍵字

tracheal agenesis ； tracheoesophageal fistula

國際替代計量

Tracheal Agenesis: Report of One Case

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