Lobectomy for congenital pulmonary airway malformations (CPAMs) is one of the most demanding operations in pediatric MAS. Many patients are now diagnosed antenatally, and there is considerable controversy on the management of those cases that are asymptomatic at birth. Early resection and observation both have equally strong advocates. Large cyst lesions may be at higher risk for complications, including late bronchoalveolar carcinoma, and may not be distinguishable from type 1 pleuropulmonary blastoma on imaging alone; therefore the case for resection is stronger.

Most surgeons favor lobectomy rather than segmental or nonanatomic resections for CPAMs because there is less risk of leaving residual abnormality and of a troublesome postoperative air leak. CPAMs most commonly involve the lower lobes, with the middle and upper lobes only rarely affected. Lower lobe resections are illustrated, but the approach to the upper lobes is also discussed.

Some lesions have a systemic arterial supply, as is also the case for extralobar sequestrations. Good preoperative imaging (Fig. 12.1) is critical in demonstrating the anatomy, enabling planning, and avoiding unpleasant surprises!

Single lung ventilation is essential. This may be achieved by endobronchial intubation, a bronchus blocker, or a double-lumen tube, depending on patient size and anesthetic preference/expertise. The patient is positioned in the full lateral position as for a thoracotomy. A roll or bolster placed under the chest helps to open the upper rib spaces. There should be adequate support or fixation of the patient to the table to allow safe lateral tilting. If the operating screens can be positioned independently of the equipment stack, the stack is best placed at the foot of the table to allow unimpeded access to both sides of the chest.

The general approach is cross table, looking from the anterior aspect of the chest posteriorly up the major fissure, with the primary operating screen at the patient’s back. A second screen to allow working from the posterior to the anterior chest may be useful in difficult cases. The first port is placed using a blunt “cut-down” technique in the fourth or fifth interspace between the mid-axillary line and the anterior axillary line (usually just below and a little anterior to the scapula tip). Once the position of the major fissure is confirmed, two operating ports are placed at the anterior axillary line, above and below the fissure. A fourth port can be placed in the posterior axillary line at the same level as the inferior operating port for a retracting instrument if necessary. The angles for the instruments and, in particular, the telescope are quite steep and so the table will need to be low for comfortable handling.

Sometimes the fissures may be incomplete, virtually to the extent of absence. In these cases it may not be possible to adequately demonstrate the anatomy thoracoscopically, and conversion to open thoracotomy may be advisable (Fig. 12.2). An understanding of the spatial relationships at the hilum on pulmonary artery, bronchi, and pulmonary veins on both sides is crucial as is an understanding of the normal segmental pulmonary arterial anatomy and variations.

If the oblique fissure is complete (Fig. 12.3), then the pulmonary artery and its branches are readily identified (Figs. 12.4 and 12.5).