USA Surgery Today

Based on the knowledge gained from the advances in the aforementioned diagnostic modalities, perinatal management recommendations have been formulated for select fetuses with life-threatening anomalies (Table 3-1). More precisely, the natural history, pathophysiology, associated anomalies, and therapeutic options have become the basis for the multidisciplinary fetal treatment team, which has enabled the approach to the fetus as patient (13:Calan). Prenatal intervention has, in large part, been predicated on those anomalies that result in either low- or high-output cardiac failure resulting in hydrops (skin and/or nuchal edema or fluid accumulation in two of three body cavities (pleura, pericardium, peritoneum)]. Hydrops resulting from a specific lesion results in nearly 100% fetal mortality. Examples include fetuses with hydrops from a CCAM, sacrococcygeal teratoma (SCT:Calan), pericardial teratoma, select heart defects, tension hydrothorax, twin–twin transfusion syndrome (TTTS:Calan), and twin reversed arterial perfusion (TRAP) sequence. Pulmonary and kidney failure can be predicted in a subset of fetuses with urinary tract obstruction (e.g., posterior urethral valves:Calan) and may benefit from US-guided vesicoamniotic shunt placement.

Select fetuses with threatened postnatal airway obstruction from a cervical teratoma or lymphangioma may benefit from the ex utero intrapartum treatment (EXIT:Calan) strategy in which a cesarean delivery approach is used, but myometrial bleeding is controlled and the umbilical cord is not cut until an airway is obtained—by orotracheal intubation, tracheostomy, or mass resection followed by intubation or tracheostomy. Selective reduction for complications of monochorionic twinning has been used with increasing frequency given the low relative risk and ease of the procedure. Presently, a percutaneous, needle-based, US-guided approach using radiofrequency energy can perform this procedure under local anesthesia in under 5 minutes. Controversial areas of maternal–fetal intervention include nonlethal anomalies such as laser therapy for amniotic band syndrome and prenatal repair of a myelomeningocele. There are a host of nonlethal fetal anomalies whose outcomes are enhanced by prenatal diagnosis and counseling since the timing, mode, and venue of delivery are often important variables that directly affect postnatal outcome. Examples include hydrocephalus, gastroschisis, omphalocele, and urinary tract obstruction.