UCSF

Congenital Diaphragmatic Hernia (CDH) is a life-threatening developmental defect of the diaphragm. In CDH, a hole is formed in the diaphragm during fetal life and the abdominal organs pass through it, limiting the space the lungs have to grow. Roughly one in 3000 children born in the United States suffer from this disease, and one third of these will die as an infant. Those that do survive are often met with lifelong disability.

Over the last decades, fetal surgery---surgery performed on an unborn fetus---has been developed as an attempt to treat CDH. Specifically, the airway of a developing fetus can be obstructed with a balloon or other removable device, trapping the natural fluid of lung development inside the fetal lungs. While this has been shown to reverse some deleterious effects of CDH, there has not yet, to date, been a randomized controlled study demonstrating the efficacy of this treatment. This dissertation presents the first steps toward an evolution of this fetal therapy.

First is a discussion of the epidemiology and history of CDH and its fetal therapies. This is followed by a primer to those concepts of pulmonary physiology relevant to CDH and fetal surgery. Subsequently, a novel fetal therapeutic device is discussed, representing both a new therapy and a window of scientific inquiry into the origins of CDH. The creation of specific scientific instruments to measure pulmonary development is then expounded, followed by methods of analyzing data from these instruments. The dissertation is concluded with a physiologic analysis of these data, and the proposal of a novel design methodology for sensors for clinical studies.