Hiyama E, Yokoyama T, Ichikawa T, Matsuura Y
Department of General Medicine, Hiroshima University School of Medicine, Japan.
J Thorac Cardiovasc Surg. 1994 Nov;108(5):830-3.
Tracheal agenesis is a rare congenital malformation that usually is fatal. This report describes our experience in two such cases. In both cases, the diagnosis was suspected at birth, because the patients had respiratory distress without an audible cry and were difficult to intubate. A gastrostomy and banding of the abdominal esophagus provided effective initial stabilization, in conjugation with respiratory management. The first patient also had complex cardiac malformations, and the infant died of cardiac failure 1 week after birth. The second infant, who had tracheal agenesis with a proximal tracheoesophageal fistula and a bronchoesophageal fistula, was managed successfully. At 9 months of age, a tracheotomy was performed, a long T tube was inserted to maintain the airway patency beyond the proximal tracheoesophageal fistula, and the patient was discharged. At 3 years of age, esophageal reconstruction was performed with a colonic interposition graft. The patient is thriving and developing normally at 4 years of age. Diagnosis at birth and maintenance of airway patency are essential for successful management of tracheal agenesis. Initial surgical interventions are palliative but lifesaving. Subsequent management focuses on improving the quality of life and allowing swallowing and speech.
气管闭锁是一种罕见的先天性畸形，通常是致命的。本报告描述了我们在两例此类病例中的经验。在这两例病例中，出生时就怀疑有诊断，因为患者有呼吸窘迫且无哭声，难以插管。胃造口术和腹部食管束带术与呼吸管理相结合，提供了有效的初始稳定措施。第一例患者还患有复杂的心脏畸形，婴儿在出生后1周死于心力衰竭。第二例婴儿患有气管闭锁并伴有近端气管食管瘘和支气管食管瘘，治疗成功。在9个月大时，进行了气管切开术，插入了一根长T形管以维持近端气管食管瘘上方的气道通畅，患者出院。在3岁时，采用结肠间置移植进行了食管重建。该患者在4岁时茁壮成长且发育正常。出生时的诊断和气道通畅的维持对于气管闭锁的成功治疗至关重要。初始手术干预是姑息性的，但能挽救生命。后续治疗重点在于提高生活质量并实现吞咽和说话功能。
