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Key Points

  1. Fetal surgery has the potential to alter the natural history of congenital disease.

  2. Patient selection requires detailed understanding of disease pathophysiology.

  3. Potential benefits of prenatal intervention must offset the significant risks for both mother and fetus.

  4. Improvements in imaging technology permit more detailed diagnosis of congenital anomalies and provide better prognostic information.

  5. A multidisciplinary approach is necessary for patient selection and counseling.

  6. Prevention of preterm labor is a focus of preoperative, intraoperative, and perioperative management.

Development of Fetal Surgery

Fetal surgery is a specialty born of clinical necessity. Pediatric physicians and surgeons had long observed that certain congenital anomalies could cause irreversible organ damage before birth, leading to fetal or neonatal death despite all efforts at treatment. As new imaging technologies emerged, our ability to prenatally diagnose anatomic anomalies and to understand the correlation between their prenatal pathophysiology and postnatal outcomes improved. In specific conditions, progressive organ destruction occurred in utero, raising a compelling rationale for fetal surgery. It was the logical progression of thought to consider whether repair of the defect in utero might reverse the pathophysiology, restore normal development, and lead to good quality of life with survival.

Fetal surgery requires several unique considerations, including maternal –fetal monitoring, anesthesia, tocolysis, and methods of hysterotomy and uterine closure, in addition to the technical aspects of each individual procedure. Animal models have played a key role in the development of techniques to allow the mother and fetus to undergo operation safely and successfully. Since its inception, fetal surgery has developed rapidly, driving improvements in prenatal diagnosis, technical innovations, and better understanding of the pathophysiology and natural history of candidate disorders, as well as the unique physiology of the fetus and pregnant mother. The fetus is, in essence, a patient within a patient, protected by layers of maternal abdominal wall, uterine wall, and chorioamniotic membranes, and management of obstetric complications remains critical to the success of fetal interventions.

The first report of successful therapeutic intervention on the fetal patient was the transfusion of a hydropic fetus for Rh disease by Sir A. W. Liley, which remains the treatment for Rh disease today. This transfusion was guided by radiographs and contrast instillation and represents the first acknowledgment of the fetus as a patient. Following a few attempts at open fetal exchange transfusion during the 1960s, modern fetal surgery was conceived and developed by Michael Harrison and colleagues at the University of California, San Francisco (UCSF) during the late 1970s and 1980s. There, the concept of a multidisciplinary fetal treatment program was developed, along with experimental models of fetal disease in sheep and monkeys, which allowed study of the pathophysiologic rationale and technical feasibility of human open fetal surgery. The fetal lamb model allowed evaluation of the pathophysiology of specific fetal defects, while researchers used the primate model to develop the anesthetic, tocolytic, and technical methods required to conduct ...

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