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題 名 | Tracheal Agenesis with Multiple Congenital Anomalies: A Case Report=氣管發生不全伴隨多重先天性異象:一病例報告 |
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作 者 | 王壯銘; 陳淑貞; 陸振翮; 黃碧桃; | 書刊名 | 中華醫學雜誌 |
卷 期 | 61:1 1998.01[民87.01] |
頁 次 | 頁48-52 |
分類號 | 417.132 |
關鍵詞 | 軸中胚層發生不良; 氣管發生不全; VACTERL症候群; Axial mesodermal dysplasia spectrum; Tracheal agenesis; VACTERL association; |
語 文 | 英文(English) |
中文摘要 | 氣管發生不全是一種罕見的先天性異常,而且患兒無法長期存活,它可以單獨發生或伴隨多重先天性異象。一足月男性新生兒出生時有發紺現象、肌肉低張、無哭聲且呼吸窘迫。氣管插管非常困難,且由喉鏡證實必須食道插管。因此氣管發生不全被強烈懷疑,進一步的支氣管鏡檢查發現有正常上會厭、正常聲帶,但無法看到氣管。病人因心肺功能惡化,死於第二天晚上,屍體解剖證實這個診斷且伴隨多重先天性異常。這些異常包括氣管發生不全,食道、氣管□管、右心室雙出口、雙肺動脈瓣、右肺單葉、肛門閉鎖及直腸尿道□管。我們描述這個病例是氣管發生不全伴隨多重先天性異常,且符合VACTERL症候群的診斷。根據胚胎發育的理論,是由於不正常囊胚發育,引起中胚層缺失引起氣管發生不全和VACTERL症候群。 |
英文摘要 | Tracheal agenesis (TA) is a rare congenital anomaly that is incompatible with prolonged life. It may occur alone or with other associated anomalies. A term infant presented with cyanosis, hypotonia, absence of crying and respiratory distress at birth. Intubation was difficult. Esophageal intubationwas performed under laryngoscopy. As TA was suspected, a bronchoscopy was performed and the infant was found to have a normal epiglottis and vocal cords; however, there was no trachea. Cardiorespiratory deterioration developed and the patient died on the night of the second day at the postnatal age of 41 hours. Tracheal agenesis was confirmed at autopsy. Associated anomalies included bronchoesophageal fistula, double outlet of the right ventricle with ventricular septal defect, bicuspid pulmonary valve, single lobe of the right lung, imperforate anus and a rectourethral fistula. According to development theory, tracheal agenesis and VACTERL (vertebral defects, anal atresia, cardiovascular defects, tracheoesophageal fistula, radial dysplasia or renal defects and limb defects) association may result from a mesodermal deficiency caused by abnormal blastogenesis. |
本系統中英文摘要資訊取自各篇刊載內容。