Preface

The guiding principle for the inclusion of a topic in Fetal and Neonatal Surgery and Medicine is that not only can the condition be diagnosed prenatally but also it is either amendable to fetal or neonatal intervention (either medical or surgical) or that prenatal counseling is an important component of the management and involves more than one discipline. For example, a diagnosis of a ventricular septal defect as an isolated cardiac defect does not impact the prenatal course of the pregnancy, requires no intervention, and can be fully counseled by a pediatric cardiologist. In contrast, any diagnosis that would have implications for the rest of the pregnancy, may require fetal intervention, or require multiple disciplines to adequately counsel the mother, ie, cloacal exstrophy, would be a candidate for inclusion in this book and defines the scope of fetal and neonatal surgery. To be included, a condition must be one which can be diagnosed prenatally, is either amenable to treatment in utero, or for which extensive counseling prenatally is advisable for postnatal management, often involving multiple disciplines, and requiring intervention in the newborn period.

The scope and definition of the field of fetal and neonatal surgery and medicine is in no way intended to remove or minimize the role of maternal–fetal medicine specialist from their role in fetal surgery. As has happened in pediatric surgery, there is a subset of maternal–fetal medicine specialists and, in Europe, obstetricians who have chosen to focus their practice on prenatal diagnosis and treatment including fetal surgery. Similarly, in the field of neonatology, especially in freestanding children’s hospitals, there has been a shift in focus from care of premature babies to the care of surgical newborns. In quaternary care children’s hospitals, a significant proportion of neonates are admitted due to anomalies and may or may not be premature. However, the focus of neonatal-perinatal medicine continues to be on the care of premature infants. Further, the focus of the Neonatal Resuscitation Program (NRP) is on the care of well newborns at the time of delivery. There is a paucity of information related to neonates with congenital anomalies. The use of prenatal imaging and genetic studies to guide recommendations for the perinatal team on the best mode of delivery, anticipated challenges in the delivery room, and the NICU is presented in this textbook. This reference will be useful to neonatologists to guide prenatal consultation and provide continuity between the prenatal diagnosis and the NICU. Knowledge of the multidisciplinary care required for the surgical neonate is essential for neonatologists caring for these unique patients.

The scope of this book reflects the evolution in all three fields. Pediatric surgery has seen the development of the subspecialty of fetal and neonatal surgery in which pediatric surgeons have chosen to focus their practice entirely on surgery of the fetus and newborn in contrast to general pediatric surgery which has become more focused on general surgery for children and adolescents. Maternal–fetal medicine has seen the divergence of specialists focusing on medical conditions of the mother while others have focused on prenatal diagnosis, a subset of which, have devoted themselves to the care of mother and baby undergoing fetal interventions. In neonatology, while the focus remains the care of premature infants, a subset of neonatologists have devoted themselves to prenatal diagnosis and counseling of structural anomalies and the care of these babies in the delivery room and NICU.

The editors believe strongly in the essential partnerships of maternal–fetal medicine specialists, pediatric surgeons, and neonatologists who consider their primary focus the care and management of the fetal and neonatal patient. By training and experience, these three indispensable disciplines with complementary experience bring to the mother and fetus expertise, knowledge, and capabilities essential to the proper care and management of the mother–baby dyad in a continuum from prenatal diagnosis to postnatal discharge from the hospital. The focus of this book is the vertical integration of care from the time of prenatal diagnosis through neonatal surgery and beyond. The fetal and neonatal surgeon has a unique role in this continuum of care, in partnership on the one hand with maternal–fetal medicine specialists and on the other with neonatologists, providing a link between prenatal and postnatal life.

In pediatric surgery, we have seen the evolution of fetal and neonatal surgery distinguish itself from general pediatric surgery as a primary focus. This is not the strength of the typical general pediatric surgeon. As a result, a concentration of experience and expertise in caring for these babies, before and after birth, has evolved in a manner distinct from the role of the typical general pediatric surgeon. As a result, there is a growing gap in knowledge, expertise, and experience in the surgical care of these complex patients. This gap in knowledge and experience led us to the conclusion that a textbook on surgery and medicine of the fetus and newborn would help fill the gap in knowledge and expertise that would be a resource for not only the general pediatric surgeon but also neonatologists, radiologists, geneticists, nursing staffs, and residents and fellows from a broad range of disciplines involved in the care of these patients.

The book is divided into four parts. Part I, Prenatal Diagnosis, focuses on the state of the art of prenatal screening and prenatal diagnosis from imaging with ultrasound and MRI to the use of molecular genetic techniques. Part II, Fetal Intervention: Medical and Surgical Techniques, discusses the techniques used in fetal intervention both medical and surgical, presenting the full armamentarium of fetal treatment techniques from transplacental medical therapy, to ultrasound guided techniques, fetoscopic surgical techniques, and open fetal surgery. Part III, Special Considerations in the Fetus and Neonate, covers red cell alloimmunization and transitional physiology of the newborn and management of newborns with congenital anomalies including resuscitation, nutritional support, and ventilator support and palliative care. Part IV, Fetal and Neonatal Anomalies, is devoted to prenatal and postnatal management of specific conditions which may be treatable before birth or in the newborn period. Each chapter has been divided into sections on Prenatal Management, Delivery Management, and Postnatal Management.

The purpose of writing this new book is to address a significant and growing gap in knowledge and expertise in fetal and neonatal care. As this field has grown and the complexity of management has expanded, there has developed a paradoxical regression in information that is available in standard texts and reference materials. The characteristic of this paradox is the superficial, even cursory, nature of coverage of fetal and neonatal surgical conditions in standard textbooks of maternal–fetal medicine, pediatric surgery, and neonatology. This often is a reflection of the experience and the expertise of the authors of these chapters who while often well regarded in their fields may be generalists lacking in-depth experience with management of the problems they have been tasked with writing about. These chapters are written at a level that would be suitable for a medical student but not for physicians actually taking care of these fetal and neonatal patients.

The other problematic aspect of fetal and neonatal conditions are their relative rarity. It is difficult for any single institution, let alone a single clinician to gain adequate experience with a complex fetal or neonatal condition unless they have devoted their clinical practice to this field. The number of maternal–fetal medicine specialists, fetal and neonatal surgeons, and neonatologists who have devoted their practice to this subset of fetal and neonatal patients is still relatively small. This problem is compounded by the growth in size and complexity of freestanding children’s hospitals along with the size of their neonatal and pediatric surgical staff. In major children’s hospitals today, it is not uncommon to have 25 to 35 neonatal attendings and 15 to 20 pediatric surgical attendings covering the NICU. This, paradoxically, has diluted the experience of each individual with complex neonatal conditions because any individual neonatologist or pediatric surgeon now sees fewer of these complex conditions. These influences have created a more pressing need for a definitive reference that can marshal the disparate sources of data on fetal and neonatal surgical conditions.

The maternal–fetal medicine specialists, pediatric surgeons, and neonatologists who are confronted with the challenge of counseling a mother carrying a baby with a structural anomaly find it difficult to cull together everything they need in a single reference or resource. The goal of filling this niche is the purpose of fetal and neonatal surgery and medicine. The maternal–fetal medicine specialists may be very familiar with the sonographic features of a particular condition and making a prenatal diagnosis, but may not be familiar with current approaches to prenatal treatment, much less the neonatal natural history, treatment, and long-term outcome of the condition. Similarly, pediatric surgeons and neonatologists may have a rudimentary understanding of the prenatal natural history of a fetal anomaly and what treatment options may be available before birth. In addition, general textbooks in maternal–fetal medicine, pediatric surgery, and neonatology rarely devote more than a single overview chapter to prenatal diagnosis and fetal intervention. These chapters all too often present only superficial discussion with limited information about the natural history, treatment options, and outcomes for many conditions which are diagnosed in utero and treated in the newborn intensive care unit. It is our hope that this textbook will prove to be a comprehensive resource for any professional counseling mothers about prenatal findings or treatment options for a fetal or neonatal condition.