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Introduction

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Survival of infants born with congenital diaphragmatic hernias (CDHs), as with many other anomalies in pediatrics, has steadily improved over the last three decades owing to advances in critical care medicine, as well as surgical technique. While early attempts to repair CDH were made in emergent fashion, recognition that the life-threatening issue was not mechanical compression of the lungs or mediastinal structures, but disordered pulmonary vascular reactivity, has been the major advance in improving survival and outcomes. There is wide variation in the technical approach to the repair of these anomalies including variability in pre- and postoperative care, timing of the operation, abdominal or thoracic operative approach, and choice of prosthetic material for closure. The intent of this chapter is to provide the adult thoracic surgeon with a reference for review of the current management of CDH and discuss the impact of having these diaphragmatic defects repaired as an infant or child on general thoracic anomalies/procedures in the adult.

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Types of Cdh

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The most common type of CDH is the Bochdalek hernia (Fig. 153-1). These occur posterolaterally and most commonly on the left. In general, Bochdalek hernias are categorized on the basis of size. Small defects are usually triangular in shape, with the apex medially and the base on the chest wall. Small defects most often are repaired primarily. Medium-sized defects have a larger segment of missing diaphragm, often leading to inability to close by primary repair. These defects may require a prosthetic patch, which usually includes sutures placed around the ribs laterally. The largest defects are commonly referred to as agenesis of the hemidiaphragm, and require nearly complete replacement of the hemidiaphragm with prosthetic patch material. The medium and large defects tend to have vanishingly small amounts of tissue along the esophagus and aorta to which the patch can be sewn. Consequently, gastroesophageal reflux (GERD) is more common in the medium and large defect forms of CDH leading these patients to be more likely to require a fundoplication to control their reflux. Another reason that patients with a patch repair have more reflux is that the patch does not grow as the patient does, and may, therefore, pull on the esophageal hiatus as the patient grows.

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Figure 153-1

Common locations of congenital diaphragmatic hernias.

Graphic Jump Location
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Morgagni hernias are located in the midline just posterior to the sternum. These defects can have little to no symptoms. Consequently, Morgagni hernias are most commonly diagnosed with older patients who have chest x-rays for other reasons and a mediastinal mass is seen. These Morgagni defects are commonly closed primarily; however, on rare occasion, a patch may be required. Morgagni defects are also seen in pentalogy of Cantrell, and in that case, are usually bilateral.

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Eventration of the diaphragm (Fig. 153-2) occurs in the congenitally ...

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