later in life (reported from age 9 to 83 years) but 25% of cases present before age 17. These fistulas connect to the middle or lower esophagus, from right upper lobe, from left lower lobe, from bronchus intermedius, from right middle or lower lobe, and from left upper lobe. The fistula usually slopes downward from the bronchus to esophagus, perhaps accounting in part for the lack of earlier symptoms, but it may also connect from a small diverticulum of esophagus, or on the pulmonary side, to a cyst or to a sequestrated lobe.10 (See Figure 12-10 in Chapter 12, "Acquired Tracheoesophageal and Bronchoesophageal Fistula.") Cough, especially after drinking, is the most common symptom, with respiratory infection and hemoptysis, and even hematemesis (seen less often). Barium esophagogram is the most accurate method of diagnosis. Endoscopy or bronchography are less helpful. Methylene blue in the esophagus aids bronchoscopic identification. The condition is permanently corrected by surgical excision and closure of the fistula, with interposition of healthy tissue between the bronchus and esophagus. Chronic pulmonary infection may dictate limited resection of the lung (see Chapter 12, "Acquired Tracheoesophageal and Bronchoesophageal Fistula" and Chapter 26, "Repair of Acquired Tracheoesophageal and Bronchoesophageal Fistula").
A very rare anomaly is the laryngotracheoesophageal cleft. Varying degrees of incompleteness of the wall between the larynx and trachea and the esophagus present (Figure 6-4).11,12 A minor interarytenoid cleft reaching through cricoid cartilage, a deeper defect reaching to the upper trachea, and a complete cleft extending to the carina have been designated as types I, II, and III, respectively.11 Ryan and colleagues added type IV, where the defect extends into both main bronchi.12 Type I may not require repair. Type II has been repaired through laryngofissure or cervical or cervicomediastinal approach. Types III and IV are best approached laterally transcervically and transthoracically (see Chapter 33B," Repair of Congenital Tracheal Lesions: Larynogotracheoesophageal Cleft Repair"). Donahoe and Gee first successfully repaired a type III cleft, with long-term survival.13
Tracheal webs sometimes occur in the neonatal and juvenile trachea at the cricoid level. Laryngeal webs at the glottic level are more common. The tracheal web does not involve any significant length of airway and is consequently usually treated endoscopically (Figure 6-5). Congenital cartilaginous cricoid stenosis is an extremely rare lesion.14 Subglottic stenosis is more common as a consequence of postintubation injury. Other purely laryngeal congenital lesions, such as glottic and subglottic stenosis and atresia, are not considered here.
Segmental or diaphragm-like congenital main bronchial stenosis, principally on the right, has been very rarely encountered.15 Segmental bronchial resection or even wedge resection in the instance of a thin web is an effective treatment. In the latter case, bronchoscopic management might be considered.
Congenital tracheal stenosis has been classified into three principal types: 1) generalized hypoplasia, 2) funnel-like narrowing, and 3) segmental stenosis (Figure 6-6).16 The stenotic segment is most often composed of completely circular "O" rings of cartilage (Figure 6-7). Alternatively, disorganized cartilages, ridges, or plates of cartilages may occur (Figure 6-8). In type I, the larynx is of normal diameter but the entire trachea, or much of the trachea, is narrowed (1 to 3 mm diameter in the newborn) to a point just above the carina. The main bronchi are often normal in diameter, but may be more transverse than usual and frequently malacic. In some cases, the bronchi are also stenotic and composed of "O" rings of cartilage. In type II, the trachea begins with normal diameter, but then funnels down over a variable length to a tight stenosis. The location of the funneling and of the tight stenosis varies widely. It may be located proximally with a more distal normal segment. In many cases, the stenosis is long, involving more than half of the trachea. In type III,
figure 6-6 Congenital tracheal stenosis. General categories of stenosis are diagrammed. Types I-III redrawn from Cantrell JR and Guild HC.16 A, Type I: All or most of the trachea is stenosed. B, Type II: Funnel stenosis variously located and of variable length. C, Type III: Short segmental stenosis, sometimes below an anomalous right upper lobe bronchus. D, Type IV: Anomalous right upper lobe bronchus with a "bronchus" to horizontally branching bronchi to the rest of the lung. The right upper lobe bronchus is at the normal carinal level. The bridge bronchus is stenotic, and lesser stenosis may involve part of the trachea above. In some cases, the trachea is elongated as shown, but the upper lobe bronchus is absent. Circles indicate locations of left pulmonary artery sling when present.
segmental stenosis may occur at any level in the trachea and be of any length, but it occurs most often in the lower trachea. Bronchial anomalies may be present, such as a misplaced right upper lobe bronchus (bronchus suis), which takes off from the trachea above an area of segmental stenosis. Tracheal stenosis may also be present above an anomalous right upper lobe bronchus, with tighter stenosis or "bridge bronchus" below the lobar bronchus. The right main bronchial anomaly is also found as an isolated variant apart from congenital stenosis, and is then usually closer to the carina. With some frequency, patterns are seen which manifest variable groupings of funnel tracheal stenosis, bronchus suis, a "bridging bronchus" (often the most severely stenotic segment), and distal branching into horizontal bronchi to right lower lobe and left lung, forming an inverted "T" (Figure 6-9).17 The point of relatively transverse bronchial branching is held not to be the true carina, because the trachea plus "bridging bronchus" to this point is much longer for age than normal and contains many more rings than the normal trachea. These patients are more difficult to correct surgically than patients with stenosis in a trachea with a more normal pattern. Repair is described in Chapter 33A, "Repair of Congenital Tracheal Lesions: Tracheoplasty for Congenital Tracheal Stenosis."
In over half of the patients, congenital tracheal stenosis may be accompanied by many other malformations, including cardiac anomalies, hyaline membrane disease, pulmonary anomalies, inguinal hernias, imperforate anus, radial aplasia, and megaureters. Segmental stenosis of the distal trachea may be associated with an aberrant left pulmonary artery, the so-called "pulmonary artery sling" ("ring sling complex") (see Figure 6-9).18,19 The left pulmonary artery originates from the proximal portion of the right artery and passes behind the trachea to the left lung (Figure 6-10). In this course, it indents, but rarely obstructs the
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