Congenital Lesions

Tracheal agenesis or atresia is usually fatal at birth. The larynx may form normally. The lungs may or may not be normal, and with or without bronchial communications to the esophagus (Figure 6-2). These malformations are extremely rare. The most common presentation is with normal bronchi, communicating centrally to the esophagus.2 Other congenital anomalies are common in these patients. Fonkalsrud and colleagues described a newborn of type C, who survived for a short term by using the esophagus as an airway.3 A major bronchus may also communicate directly with the esophagus while the balance of the lung is served by anomalous bronchi from a partly stenotic trachea. Microgastria is a common concomitant feature. No systematic surgical treatment has evolved, doubtlessly due to the rarity and variations in the anomalies as well as the complexity of the defects.

Hiyama and colleagues described two such patients in whom diagnosis was suspected due to respiratory distress without audible cry and difficulty in intubation.4 One infant was successfully treated by the

figure 6-2 Tracheal agenesis, redrawn according to Faro and colleague's classification.2 In addition to types diagrammed, type A (8%) shows total pulmonary agenesis and type G (5%) has short segment tracheal agenesis. In type B, both main bronchi connect to esophagus separately, whereas in type C, bronchi are fused but a bronchoesophageal fistula (BEF) is present. Type D additionally has an atretic band (dashed line) from larynx to BEF. Type E has a tracheoesophageal communication, but in type F, communication with the esophagus is absent. Distribution of types in Faro and colleague's collected series of 39 patients is noted.

figure 6-2 Tracheal agenesis, redrawn according to Faro and colleague's classification.2 In addition to types diagrammed, type A (8%) shows total pulmonary agenesis and type G (5%) has short segment tracheal agenesis. In type B, both main bronchi connect to esophagus separately, whereas in type C, bronchi are fused but a bronchoesophageal fistula (BEF) is present. Type D additionally has an atretic band (dashed line) from larynx to BEF. Type E has a tracheoesophageal communication, but in type F, communication with the esophagus is absent. Distribution of types in Faro and colleague's collected series of 39 patients is noted.

following procedures: gastrostomy and abdominal esophageal banding, translaryngeal and esophageal ventilation by endotracheal tube, tracheostomy and later T tube, pharyngeal sump drainage followed by establishment of cervical esophagostomy (proximal tracheal segment present), and esophageal reconstruction by colonic interposition at age 3.

In the more common anomaly of tracheoesophageal fistula, the tracheal problem is usually managed by division and closure of the communication. Congenital TEF and the complexities of esophageal atresia have been well described and categorized. The reconstructive challenge is principally esophageal, and congenital TEF is not further detailed here. Rarely, there is accompanying tracheal stenosis. Adjacent local tracheomalacia, especially after repair of TEF, may cause respiratory problems, and is described later. Less commonly found is an H-type congenital tracheoesophageal fistula without concomitant esophageal atresia, often high in the trachea and usually small in diameter. It is sometimes discovered in the adult and is usually managed by transcervical division and closure (Figure 6-3).5,6 Coughing is the usual symptom, especially after ingestion of liquids. A large fistula may be treated by limited tracheal resection and anastomosis, with esophageal closure.

Congenital fistula between biliary and respiratory tracts is extraordinarily rare.7 Respiratory problems begin with cough and progress to intractable pneumonia. The most common location of the fistula is at the carina, but right and left main bronchial connections have been noted. Yellow fluid is identified bron-choscopically. Contrast will identify a long paraesophageal tract connecting to a hepatic duct. It has also been seen in a young adult.8 Excision of the intrathoracic segment with closure at the carina (or bronchus) and at the diaphragmatic level cures the problem.

Congenital bronchoesophageal fistula is a rare anomaly with fewer that 150 cases reported (see Chapter 12, "Acquired Tracheoesophageal and Bronchoesophageal Fistula").9 Symptoms may not occur until figure 6-3 H-type tracheoesophageal fistula in a 49-year-old woman. A, Fistula demonstrated (arrow) by barium contrast swallow. The trachea is clearly outlined. B, Computed tomography scan showing the small fistula. The fistula was divided and repaired easily through a low collar incision. Also, see Figure 6-21B. (Courtesy of Dr. Hon Chi Suen.)

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