Chapter 29

### Key Points

1. Treatment of bronchopulmonary malformations may vary somewhat depending on the time of diagnosis and the presentation, but in most cases, complete resection is the desired therapy.

2. New minimally invasive techniques allow treatment with much less pain and morbidity and long-term consequence for the infant or child.

3. Bronchopulmonary sequestration (BPS) lesions may be asymptomatic, but postnatal resection should be considered because of the risks of infection, hemorrhage, and malignant transformation.

4. The surgical approach to BPS is straightforward, with the exception of the management of anomalous blood supply. Intraoperative death due to hemorrhage from unrecognized anomalous vessels has been reported.

5. Congenital pulmonary airway malformation (CPAM) is increasingly being diagnosed by routine prenatal ultrasound, allowing for prenatal consultation and planning. The need for fetal intervention is rare and limited to cases with severe hydrops and a predicted mortality of near 100%.

6. The treatment of choice in infants with CPAM is complete resection of the CPAM, usually by lobectomy.

7. The treatment for patients with symptomatic congenital lobar emphysema (CLE) is surgical resection; in most cases, a complete lobectomy.

8. The operative approach to resecting bronchogenic cysts depends on their location and involvement of adjacent structures, such as the trachea or mainstem bronchi, which may require repair.

### Introduction

There is a broad spectrum of bronchopulmonary malformations that present in early infancy and childhood. These include bronchopulmonary sequestrations (BPS), congenital pulmonary airway malformation (CPAM), congenital lobar emphysema (CLE), and bronchogenic cysts. These lesions may be detected by prenatal diagnosis, present as acute respiratory distress in the newborn period, or may remain undiagnosed and asymptomatic until late in life. Treatment may vary somewhat depending on the time of diagnosis and the presentation, but in most cases, complete resection is the desired therapy. New minimally invasive techniques now allow this to be done with much less pain, morbidity, and long-term consequences for the infant or child.

### Bronchopulmonary Sequestration

Bronchopulmonary sequestration is a rare congenital malformation of the lower respiratory tract. It consists of a nonfunctioning mass of lung tissue that lacks normal communication with the tracheobronchial tree and receives its arterial blood supply from the systemic circulation. There appears to be a spectrum of sequestration with, at one extreme, an abnormal vessel supplying a nonsequestered lung and, at the other extreme, abnormal pulmonary tissue but without anomalous vascular supply.

The majority of sequestrations fall into 2 categories: intralobar sequestration (ILS) and extralobar sequestration (ELS). ILS, defined as a lesion that lies within the normal lobe and lacks its own visceral pleura, accounts for 75% of BPS in infants and children. ELS is a mass located outside the normal lung and has its own visceral pleura. ELS accounts for 25% of cases in infants and children and may be either intrathoracic or subdiaphragmatic (Savic et al 1979; Collins et al 1987).

Terminology has become increasingly complicated. The term “malinosculation” describes the ...

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