figure 6-11 Congenital tracheomalacia causing difficulty early in life. Malacia of the lower 45% of the trachea and of the left main bronchus was identified at 6 months. At age 12, a 12 mm silicone Y stent was placed to allow the patient to be more active. A 14 mm Y stent was fitted at age 15. Minor granulation tissue in the left main bronchus was removed and that limb of a new stent was shortened at age 19. The stent continues to be well tolerated. Resection and primary reconstruction would be too hazardous. The lack of visible cartilage discourages posterior wall splinting, and the location deters the use of external circumferential splinting. A, Bronchoscopic view at carina. Note the absence of a visible ring structure in the anterior tracheal wall. The left main bronchus shows collapse. The right maintains its patency. B, Tomogram demonstrating lower trachea and left main bronchus, held patent by a silicone Y stent. Also, see Figure 3 in the Tracheobronchial Endoscopic Atlas (Color Plate 12).
of the trachealis muscle. The anterior tracheal wall may become indented as the rings fold backward (see Chapter 15, "Tracheobronchial Malacia and Compression").
Vascular rings are associated with tracheal and esophageal compression and, if of long enough duration, with tracheomalacia. Tracheal compromise is seen most commonly with double aortic arch, right aortic arch with retroesophageal left subclavian artery and left ligamentum arteriosum, and right arch with mirror image brachiocephalic artery and left ligamentum (Table 6-1) (Figure 6-13). The multiple patterns of vascular rings that occur have been well described and will not be cataloged here again.33,34 Right aberrant subclavian artery from left aortic arch may be associated with dysphagia due to esophageal compression (dysphagia lusoria), but infrequently causes tracheal symptoms. Aneurysm of an aberrant artery late in life, however, can cause tracheal compression.
In infants, severe respiratory symptoms may result from vascular rings, with stridor, "crowing," and repeated respiratory infections. Dysphagia and aspiration may occur. Diagnosis was traditionally made by barium esophagogram. However, vessels are now identified by computed tomography (CT) with contrast or by magnetic resonance imaging (MRI). Division of the ring and ductus or ligamentum arteriosum plus excision of a Kommerell's diverticulum, if present, usually serves to correct the basic constriction.35-39
figure 6-12 Symptomatic tracheal diverticulum remaining after repair of a congenital tracheoesophageal fistula. Although the cartilaginous rings were not malacic, the combination of a patulous membranous wall and a diverticular pouch created obstruction. A, Bronchoscopic view of superior (arrow) and inferior lips of the wide-mouthed diverticulum, both of which extend obliquely across the field of view. The distal trachea is above and the patulous membranous tracheal wall below the slit-like mouth of the diverticulum (arrow). B, Diverticulum exposed by sternotomy. Penrose drains encircle and retract the trachea, allowing the posterior diverticulum to protrude (arrows). Forceps point to the diverticulum. A vascular loop encircles the brachiocephalic vein. The diverticulum was excised and the membranous wall was closed in a linear fashion, pulling the splayed rings into normal configuration to provide a normal tracheal lumen. Symptoms were relieved.
An aberrant subclavian artery may be best divided and reimplanted or possibly just divided in an infant. Continued tracheal obstruction after surgery may be due to malacia and require intubation or splinting. This must be differentiated from residual obstruction due to an unresected aortic diverticulum or continued vascular compression, as described below.
Congenital Vascular Rings Which Obstruct Trachea
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