Alabdulgader Abdullah, Patten David, Harder Joyle, Thevenen Cynthia
King Fahad Hospital, P.O. Box 9596, Prince Sultan Cardiac Centre, Hofuf 31982, Eastern Province, Saudi Arabia.
Ann Diagn Pathol. 2005 Dec;9(6):323-6. doi: 10.1016/j.anndiagpath.2005.05.006.
The laryngotracheoesophageal cleft is marked by the absence of the anatomic separation of the esophagus and the larynx. The cleft functionally can be restricted to involve the interarytenoid musculature (type 1), cricoid involvement (type 2), the proximal laryngotracheoesophagus (type 3), or missing of the whole tracheoesophageal septum (type 4). Association with significant congenital heart disease is uncommon. In this report, we are presenting a 2-week-old preterm girl presented with frequent aspiration pneumonitis, cyanosis, and heart murmur. Mechanical ventilation was extremely difficult because of endotracheal tube air leak and excessive secretions. Endoscopic evaluation revealed type 3 laryngotracheoesophageal cleft. Echocardiogram revealed double outlet right ventricle with subpulmonic ventricular septal defect. Such unique combination with severe airway disease and complex congenital heart disease was thought to constitute very high morbimortality if intervention is contemplated. It was elected to proceed with compassionate care.
喉气管食管裂的特征是食管和喉之间缺乏解剖学上的分隔。从功能上讲，裂隙可局限于杓间肌组织（1型）、环状软骨受累（2型）、近端喉气管食管（3型），或整个气管食管隔缺失（4型）。与严重先天性心脏病相关的情况并不常见。在本报告中，我们介绍了一名2周大的早产女婴，她出现频繁的吸入性肺炎、发绀和心脏杂音。由于气管插管漏气和分泌物过多，机械通气极其困难。内镜评估显示为3型喉气管食管裂。超声心动图显示右心室双出口并伴有肺动脉下室间隔缺损。如果考虑进行干预，这种严重气道疾病和复杂先天性心脏病的独特组合被认为具有极高的病残率和死亡率。因此选择给予姑息治疗。
