Congenital Diaphragmatic Hernia

Abstract

Despite advances in neonatal and surgical care, the management of congenital diaphragmatic hernia (CDH) remains challenging with no definitive standard treatment guidelines. Several centers report mortality rates as low as 20%, but if extracorporeal membrane oxygenation (ECMO) support is required, the mortality rate rises to 50%. The disease severity is related to the degree of pulmonary hypoplasia and pulmonary hypertension that occurs with CDH. Both conditions decrease the infant's ability to ventilate and oxygenate adequately at delivery. These physiologic conditions that impair gas exchange are the important determinants of morbidity and mortality in CDH infants. Presently, delivery of infants with CDH is recommended close to term gestation. The focus of care includes gentle ventilation, hemodynamic monitoring, and treatment of pulmonary hypertension followed by surgery for the defect. Extracorporeal membrane oxygenation (ECMO) is considered after failure of conventional medical management for infants ≥ 34 weeks' gestation or with weight >2 kg and no associated major lethal anomalies. This chapter discusses long-term follow-up recommendations for survivors, which should involve a multidisciplinary approach, as there are many surgical and nonsurgical consequences to the disease process. Clinical strategies that address these multifaceted aspects of care, from prenatal to long-term follow-up, may further reduce the high mortality rate for these infants.

A new method of ventilation inhomogeneity assessment based on a simulation study using clinical data on congenital diaphragmatic hernia cases

“…In the most severe cases with only one functional lung (often underdeveloped), achieving acceptable gas exchange is extremely challenging. In CDH, the compressed, ipsilateral lung is smaller than expected, with less developed respiratory structures and blood vessels 3 . Both lungs are hypoplastic and differ in resistive-compliance properties that causes VI.…”

“…It is known, that liver herniation significantly increases the risk of mortality in CDH infants 3 , 7 , 34 , 35 . The higher percentage of the liver in the thorax (%LH), the higher the risk of therapy failure.…”

Repair of Congenital Diaphragmatic Hernia and Eventration Of Diaphragm Without Ventilatory Support

“…The earliest description of gross anatomy and pathophysiology associated with CDH was by McCaulay in the proceedings of the Royal College of Physician in 1754 9 . Coopel in 1827 10 and Laennec in 1834 11 reported clinical description gross pathology of CDH and suggested laparotomy as the proper approach for reduction and correction of hernia. Bowditch in 1847 12 first made the bedside diagnosis of CDH and emphasized the clinical criteria for diagnosis.…”