Neonatal surgery epitomizes the cooperation that must exist among the pediatrician, pediatric anesthesiologist, and pediatric surgeon to provide the foundation for a successful pediatric surgical team. The specialty of pediatric surgery is a reasonably recent development. The first pediatric surgeons were general or thoracic surgeons who chose to restrict their practice to the pediatric age group. Almost every country has had visionary leaders who laid the foundations of this specialty. Ladd at the Boston Children's Hospital was one of the first. Dennis Browne at Great Ormond Street Hospital for Sick Children in London, England, Dodd in Edinburgh, Russell Howard at the Royal Children's Hospital in Melbourne, Australia, and other leaders in France, Italy and Scandinavia contributed to this newly emerging specialty. Each of these leaders trained a cadre of surgeons who have spread the concept of pediatric surgery as a recognized subspecialty throughout the world. The rate of this expansion accelerated markedly immediately after the Second World War.
Neonatal surgery is an integral part of pediatric surgery. Because of the rarity of most of the major neonatal surgical emergencies (diaphragmatic hernia and esophageal atresia both occur only about once per 5,000 live births), centralizing neonatal surgery to designated regional centers would seem to be a logical concept. The first neonatal surgical intensive care unit was set up by Rickham in Liverpool in 1953.[1] It drew neonates with surgical problems from western England and northern Wales. The first neonatal surgical intensive care unit in the United States was not set up until 1962, when Koop established a unit in the Children's Hospital in Philadelphia. In many areas, regional centers for neonatal surgery have still not yet been established because of local political obstacles. This is in spite of their proven efficacy in decreasing perinatal mortality due to neonatal surgical emergencies. Logically, neonatal surgical units must be an integral part of the Level III neonatal nurseries.
Advances in neonatal surgery are closely tied to parallel advances in neonatology. These are adequately discussed elsewhere in this volume. Improved surgical results are also closely allied to advances in the field of anesthesiology. The first major article on pediatric anesthesia was written by Robson in 1936.[2] Ayre[3] introduced the "T-piece" circuit (the standard by which other circuits are still measured, and which is still used today) in 1937. The first text in pediatric anesthesia was written by Leigh and Belton[4] and published in 1948. Demming in Philadelphia proved the safety of endotracheal intubation in children, and in the 1940's Smith introduced the concept of the precordial stethoscope which remains an essential device for continuous monitoring of the progress of a pediatric anesthetic even today. These early leaders and subsequent progress in the field of pediatric anesthesia are extensively reviewed by Smith.[5] Such fundamental advances as miniaturized endotracheal tubes, fine intravenous and arterial cannulae, and the development of safe (nonexplosive) anesthetic agents have all contributed tremendously to the advances in pediatric anesthesia and to our ability to safely anesthetize even small premature babies. More important, perhaps, has been a better understanding of neonatal physiology and how it is altered by surgical diseases as well as by the operations used in their correction.
Another major advance was the development of successful total parenteral nutrition. Dudrick[6] proved that puppies grew normally if adequate calories were supplied parenterally. Application of variations of this technique has allowed the neonatal surgeon to delay feeding babies with gastroschisis, omphalocele or bowel atresia until normal oral feedings are tolerated, following surgical repair of the defect.
Each major congenital anomaly has especially intrigued some pediatric surgeons enough to encourage them to make major contributions to diagnosis and management, and others to clarify embryology and pathophysiology. Some of these major contributors will be highlighted below; however, such a list cannot be complete. Any major contributions that have been missed in this summary can be blamed on a combination of the limitations of space and this author's inadvertent oversight.
Congenital Diaphragmatic Hernia. The first successful repair of this lesion was carried out by Heidenhain or Aue in 1902 and reported by Heidenhain in 1905[7] and by Aue in 1920.[8] By 1940 Ladd and Gross[9] were advocating routine repair on diagnosis. They reported 9 survivors in 16 pediatric patients, of which at least 5 were neonates. However, it was not until 1946 that Gross[10] reported the successful repair of a diaphragmatic hernia in a neonate on the first day of life. Reported mortality in diaphragmatic hernia reached a minimum in 1953 when Gross" reported eight deaths in 72 cases. All of the deaths were among the 63 cases of Bochdaleck type of diaphragmatic hernia. These results have never been surpassed. However, close comparison of Bowditch's paper in 1853[12] with that of Harrison et al.[13] in 1975 reveals that the true mortality for this lesion has not altered at all in over 100 years. The pathophysiology of the pulmonary insufficiency associated with congenital diaphragmatic hernia is currently under active and intensive study.
Esophageal Atresia and Tracheoesophageal Fistula. Richter's transthoracic ligation of a tracheoesophageal fistula in 1913[14] set the stage for the first direct or staged repairs attempted by Lanman[15] and by Shaw[16] in the late 1930's. The first survivors with this lesion were operated on in 1939 within 24 hours of each other by Leven[17] and Ladd[18] in two separate centers. Haight, in 1941,'9 was the first to successfully perform a primary anastomosis. Waterston et al.[20] suggested a prognostic classification based on the baby's weight and the presence of associated anomalies. The babies in the group with poor prognosis were staged, with ligation of the fistula and cervical esophagostomy and later colonic interposition. Howard and Myers[21] introduced a new concept in staging with division of the fistula (but no cervical esophagostomy) and delayed esophageal anastomosis in babies with "long-gap" esophageal atresia. This delayed primary anastomosis was later extended to babies in Waterston's groups B and C. In 1953, Berman and Berman[22] introduced an end-to-side anastomosis with ligation of the distal tracheoesophageal fistula. This approach was popularized by Duhamel[23] in France and by Beardmore[24] in North America but has still not achieved universal acceptance. Livaditis[25] introduced the circular myotomy in 1969, allowing primary esophageal anastomosis in many babies with "long gap" atresia.
Omphalocele and Gastroschisis. Initially, no distinction was made in the literature between these two lesions, and there are still those (especially Shaw and Moore[26,27]) who dispute whether these two lesions are distinct or not. Even in the early part of this century the two lesions were not often discussed separately, and both were usually referred to as "gastroschisis." In 1953 Moore and Stokes[28] could find only five cases of gastroschisis in the literature. By this time, gastroschisis had been redefined and this definition now excludes all cases of omphalocele and ruptured omphalocele. The embryology of both lesions continues to be debated in the literature.
The poor prognosis of patients with omphalocele was first highlighted by Ambroise Pare.[29] The first successful surgical closure is attributed to Hey[30] in 1803. The first successful treatment by painting the sac of an omphalocele with alcohol was reported by Ahlfeld[31] in 1899.
In 1930 Williams[32] suggested staged closure of large omphaloceles. Gross[33] in 1948 devised a technique whereby the intact omphalocele sac was covered by widely mobilized skin flaps. While this technique allowed the survival of many infants who almost certainly would otherwise have died, the later repair of the huge ventral hernia was extremely tedious, and quite risky.
The importance of achieving a sound fascial closure when possible was not widely recognized until the 1950's, and it was not until 1967 that Schuster[34] published his technique utilizing prosthetic material to achieve either primary or delayed primary closure of the defect. Various modifications of this technique are now widely used in patients with large omphaloceles, although Firor[35] has shown that nonoperative management of babies with large omphaloceles (especially when there are major associated anomalies) gives better results. The majority of babies with gastroschisis now have the defects closed primarily after aggressive stretching of the abdominal wall and emptying the colon of meconium. In most cases these two maneuvers allow return of the gut to the abdomen and fascial closure to be satisfactorily achieved.
Routine total parenteral nutrition has vastly improved the results achieved in both omphalocele and gastroschisis.
Pyloric Stenosis. This entity was first described by Hirschsprung[36] in 1888. Various attempts at surgical repair met with minimal success. In 1908 Fredet[37] suggested that the pyloric muscle be split without opening the mucosa, with the muscle layers being closed transversely. Then in 1912 Ramstedt[38] serendipitously developed pyloromyotomy, a surgical procedure which is so successful and carries so little morbidity or mortality that it has yet to be improved upon.
Malrotation. The first lucid description of the embryology of the midgut was written by Mall in 1898.[39] Dott[40] in 1923 first correlated the embryology with the clinical entity. In 1936, Ladd[41] first described the surgical therapy for this anomaly, which is still used today. Bill, in an excellent chapter reviewed the history and the current understanding of the embryology of this lesion.[42]
Duodenal Atresia and Stenosis. The first case report of duodenal atresia is attributed to Calder[43] in 1733. Ernst[44] reported the first successful operation to relieve congenital duodenal obstruction in 1916. Over the next 36 years, only 57 survivors were reported.[45]
The first understanding of the embryology of these lesions was based on the observations of Trandle,[46] who in 1902 observed a solid stage in the embryonic duodenal precursor, which was followed by recanalization. Duodenal atresia was attributed to a failure of recanalization. This observation, which was originally confined to the duodenum, was later transposed caudally and erroneously proposed as the cause of jejunoileal atresia. Boyden, Cope and Bill[47] clarified a confused picture with an excellent paper on the embryology of duodenal atresia and stenosis.
With modern surgical management, the majority of these infants now survive. Associated anomalies remain the major cause of mortality in these children today.
Intestinal Atresia. The embryology of this lesion was disputed for many years. In 1912 Spriggs[48] was one of the first to suggest that atresias could result from an in utero vascular accident. Webb and Wangensteen,[49] in 1931, reviewed the theories as to the etiology of this lesion, but it was not until 1955 that Louw and Barnard[50] were able to prove that some intestinal atresias are the result of such a vascular accident. New questions have recently been raised as to the etiology of intestinal atresia, by the work of Molenaar[51] and his group in Rotterdam.
Louw,[52] Nixon,[53] Benson,[54] and Thomas[55] have all made outstanding contributions to the surgical therapy of intestinal atresia.
Meconium Ileus. Landsteiner[56] and later Fanconi et al.[57] linked this lesion to mucoviscidosis. Andersen[58] in 1938 described this lesion as an early sign of cystic fibrosis. Gross,[59] Grob,[60] Swenson,[61] Bishop and Koop,[62] and Santulli[63] were leaders in the surgical therapy for these neonates. Santulli's method was originally suggested as treatment for intestinal atresia, and' modified by others for use in meconium ileus. However, the gastrografin enema introduced by Noblett[64] has meant that surgery is now seldom required for the uncomplicated case of meconium ileus.
Hirschsprung's Disease. The first report of this entity is attributed by many to Ruysch[65] in 1691. However, as Ehrenpreis[66] pointed out, this description is not sufficiently complete to make a definite diagnosis of Hirschsprung's disease. Parry[67] in 1825 described an adult with a megacolon proximal to a sigmoid loop of normal diameter and no findings of obstruction at autopsy. Similar autopsy findings were reported by Ebers in 1836,[68] Barth in 1870,[69] Peacock in 1872,[70] Gee in 1884,[71] and Bristowe in 1885.[72] But it was not until Hirschsprung presented his first two cases in 1886[73] that this entity became widely recognized. In 1946, Ehrenpreis[74] first diagnosed this lesion in neonates. The pathophysiology was disputed until Swenson and Bill clarified the basic defect in the late 1940's and designed an operation that excised the pathological bowel.[75,76] Duhamel[77] and Soave[78] also introduced operations which have successfully stood the test of time.
Imperforate Anus. It was not until the group of investigators in Melbourne, Australia, headed by Stephens,[79] published their findings in a composite volume (which contains an excellent review of the history of this lesion) that the disordered anatomy of the levator ani and anal sphincter complex in anorectal malformations was widely recognized. Stephens,[80] in 1953, pioneered a sacrococcygeal approach to define and preserve the leading edge of the levator mechanism, the puborectalis. Romauldi,[81] Soave,[82] Rehbein,[83] and Kisewetter[84] have developed alternative approaches designed to preserve what sphincters are present. The radical new posterior sagittal anorectal approach developed by DeVries and Pena[85] may prove to be a dramatic advance in the treatment of the difficult, high anorectal malformation.
Necrotizing Enterocolitis. This troublesome problem continues to plague both neonatologists and neonatal surgeons alike. Early recognition and aggressive nonoperative management have markedly decreased mortality from this lesion. Ein and his group[86,87] have improved results with minimal surgery (the placing of Penrose drains under local anesthesia) in very small (less than 1 kg) moribund infants. Controversy still persists as to the etiology and pathophysiology of this lesion, as well as the best management.
Biliary Atresia. Although this is not strictly a disease associated with neonates, the diagnosis should be considered towards the end of the neonatal period in babies with persistent jaundice. Hays and Kimura[88] have recently documented the history and the current understanding of the pathophysiology of this lesion. The first descriptions of biliary atresia are attributed to John Thompson[89,90,91] in 1892 and to an initial case report by Hirota[92] in 1893 (Japan). Holmes[93] in 1916 made an extensive review of autopsied children dying of biliary atresia and predicted that some 16% of these patients could be saved by an anastomosis between the intestine and biliary tree. This paper laid the foundation for subsequent classification of biliary atresia into "correctable" and "noncorrectable" categories. Ladd,[94,95] and later Ladd and Gross,[96] achieved some success with surgery for infants with "correctable" biliary artresia, but their early optimism was not warranted and later reports by Kiesewetter[97] et al., and Clatworthy and McDonald[98] highlighted the poor results obtained in this disease. A survey by the Surgical Section, American Academy of Pediatrics,[99] showed that. from 1954-64 something less than 5% of children with biliary atresia survived beyond childhood. Similar results were published from Europe.
Attempts at external drainage of the lymphatic system of the liver via the thoracic duct enjoyed a rather short lived but widespread popularity. This popularity died when the limited success achieved with this technique became obvious. It was not until Kasai[100] and later Oh-i[101,102] and Chiba[103] carried out detailed histological examination of the porta hepatis that it was realized that even the "uncorrectable" lesions might respond to surgery. In 1957, Kasailoo published his first series detailing a precise anastomosis of a loop of intestine to the remnant of the hepatic ducts at the level of the hepatic capsule. Numerous variations on this approach have been proposed, but the "best" surgical procedure has not yet been found.
The problems of diagnosing this lesion and differentiating it from a lesion that is probably at the other end of the same spectrum, neonatal hepatitis, still remains. Cholangitis still kills appreciable numbers of these children following apparently successful surgical correction, and the problems of progressive biliary cirrhosis and portal hypertension still haunt all of the long-term survivors.
Endoscopy. The problems of looking down a tube designed to be passed through any neonatal orifice are immense. Neonatal bronchoscopy was a hit or miss affair until Stortz introduced the scaled-down versions of a glass rod telescope designed by Hopkins. This same system was used to develop a cystoscope that can be safely passed in neonatal boys.
Cardiac Surgery. Pediatric cardiac surgery has closely paralleled the development of adult cardiac surgery. Gross was one of the early pioneers in correcting congenital cardiac defects. He was the first (in 1938) to successfully ligate a patent ductus arteriosus[104] and later he developed procedures to correct many other anomalies. Successful neonatal cardiac surgery had to await the development of oxygenators and pumps that did not destroy most of the red cells pumped through them. The membrane oxygenator has improved the ability to oxygenate the neonate.
Neonatal cardiac surgery has developed in two different, but not mutually exclusive, directions. The first is that of early palliation such as pulmonary artery banding developed by Muller and Dammann[105] and pulmonary-systemic shunts epitomized by the Pott's shunt,[106] the BlalcockTaussig shunt,[107] the Waterston shunt,[108] and the Blalcock-Hanlon procedure.[109] A modern addition has been balloon septostomy performed during diagnostic (and now therapeutic) cardiac catheterization. This was first described by Rashkind and Miller in 1966.[110] These palliative procedures are followed by definitive repair after the child has grown.
The second approach has utilized deep hypothermia and a one-stage definitive correction of the lesion. The use of hypothermia in cardiac surgery was initially suggested by Bigelow et al."' and introduced into clinical use by Lewis and Taufic.[112] The technique of deep hypothermia introduced by Horiuchi et a1.[113] and expanded upon by the addition of pump bypass by many others, including Barratt-Boyes[114] and his group from New Zealand, allows early complete correction of many anomalies. However, the relatively simple ligation of a patent ductus arteriosus remains a commonly performed procedure. This is often required in pre-term infants.
Urology. The technical advances in endoscopy have already been mentioned. This improved equipment is directly responsible for the current management of urethral valves by their early definitive endoscopic destruction, resulting in neonatal cure for many infants with this lesion.
Exstrophy of the bladder has been known since 2000 B.C.[115]MacKay and Syme provided the first medical description in 1849.[116] Hall et al.[117] quote a description in Aldovrandus' Historia Monstrorum, published in 1646, in which a probable exstrophy of the bladder, attributed to Schenke in 1595, is described. Simon"e attempted the first ureterosigmoidostomy in 1852. This patient died of pyelonephritis nine months later. In 1894[115] Maydel successfully transplanted the trigone into the rectum. In 1911 Coffey[120] developed the nonrefluxing ureterosigmoidostomy.
Jeffs[121] has championed primary closure of the exstrophied bladder early in the neonatal period. The long-term results of this approach are still awaited. Others prefer either later primary closure[122] or excision of the bladder, especially if it is small, and urinary diversion either with a ureterosigmoidostomy[123] or with a colon conduit which can later be anastomosed to the sigmoid colon.[124]
The prune-belly syndrome was first described by Frolich in 1839.[125] Parker[126]first described the full triad of abdominal wall muscle defect, urinary tract abnormality and undescended testes. Osler[127] underlined this triad in 1901. Many of the severely affected babies die within the first few days of life with hypoplastic lungs. Recommended therapy for the survivors varies between early aggressive one- or two-stage correction in the neonatal period, recommended by Hendren,[128] Woodard[129] and Randolph,[130] and a selective, generally nonoperative approach adopted by Duckett[131] and Williams.[132]
Neurosurgery. Nulsen and Spitz[133] were the first to develop a valve which enabled the ventricular system to be drained into the internal jugular vein. J. Holter, in 1956, modified the valve, introducing silicone rubber and thus revolutionized the treatment of hydrocephalus. Pudenz[134] introduced a different valve, and yet other surgeons have utilized the ventriculoperitoneal shunt.
The computerized axial tomographic scan (CAT scan) has, in the very recent past, dramatically altered the approach to neurosurgical diagnosis. Further detailed information is now available with real-time ultrasonography utilizing the anterior fontanel as a sonic window. This allows hydrocephalus to be recognized early, and ultrasound now detects minimal germinal plate hemorrhage with accurate delineation of hydrocephalus.
THE FUTURE
Pediatric surgeons today owe a tremendous debt to all of those who led the way in developing the field of neonatal surgery. Major problems yet remain, however, with necrotizing enterocolitis and biliary atresia being only two of them. There are still some congenital heart lesions which have defied all attempts to devise a good surgical correction which achieves long-term survival. Hirschsprung's enterocolitis still claims lives, both in the neonatal period and after apparently successful definitive correction. The problems of salvaging the infant with short intestine due to atresia, necrotizing enterocolitis or midgut volvulus, are still immense. For those with less than 30 cm of upper small bowel and no ileocecal valve, the prognosis is still poor and long-term parenteral nutrition has tended to delay, rather than prevent, death. Research into the best management of these babies continues to receive a high priority.
However, further challenges await the neonatal surgeon. Improved technology now allows antenatal diagnosis of many lesions, and this raises the distinct possibility that the neonatal surgeon of today may well become the perinatal surgeon of the future.
ANTENATAL SURGERY: A GLIMPSE OF THE FUTURE
The problems of premature delivery and the high mortality of certain major congenital anomalies have forced us to consider the possibility of antenatal surgery. Techniques for safe antenatal operations have been developed in a host of experimental animals. Many different types of congenital defects have been created and studied. The emphasis in the experimental field is now shifting to their antenatal repair. With increasing use (relatively early in pregnancy) of maternal ultrasound, more and more congenital anomalies are being diagnosed in utero, allowing their immediate correction after delivery. However, if immediate neonatal correction does not improve mortality and morbidity for a given congenital defect, then antenatal repair is one possibility for improving fetal salvage.
Mandatory Prerequisites. One of the most potent stimuli to premature labor is surgical intervention of any type, late in gestation. At the present time, surgical procedures involving the human uterus (and especially fetal surgery) will almost certainly trigger premature labor and delivery of the fetus. Labor occurring within the first week after a uterine procedure may well result in uterine rupture, with severe maternal morbidity and possible mortality. Accordingly, we must be able to either, a) prevent premature labor in the face of the surgical stimulus, or b) support the fetus adequately outside the uterus in spite of fetal immaturity which would, at present, preclude extrauterine survival. Before intrauterine surgery can even be considered, one or both of these prerequisites must be met.
Development of an artificial placenta. This is a concept that is relatively easy to put forward, but extremely difficult to accomplish. First, we need far more information as to just what the placenta does. As with any given subject, the more one learns about the subject the more one realizes how little is really known. Initially, the placenta was thought to be little more than a membrane oxygenator and renal dialysis machine combined. It is now apparent that the placenta is much more than these, but just how much more is as yet unknown. Critical to developing an artificial placenta is accurate knowledge of placental function during the last trimester of pregnancy. Subprimate investigations will probably have minimal relevance; primate investigations will be difficult but will have some relevance, and studies on the human placenta will almost certainly be next to impossible. We do know that the placenta passively transports oxygen and urea, and also actively transports a wide variety of substances including amino acids, calcium, glucose and possibly even maternal antibodies. The placenta also functions as an endocrine organ, secreting hormones that affect both mother and fetus. Again, we know few details of placental endocrine function. It follows that developing an artificial placenta, while initially attractive, will prove to be extremely difficult.
The other major problem associated with the development of an artificial placenta is that of vascular access. Gaining sufficient vascular access to place a 3 kg baby on a membrane oxygenator is a major problem, and achieving the same type of access in a 600 gm fetus is next to impossible. The logical access site is the umbilical vessels. At present, any manipulation of the umbilical cord is likely to precipitate intense spasm of the umbilical vessels. While this is teleologically attractive for the term baby undergoing a normal delivery, it is disastrous if these vessels need to be utilized for vascular access to an artificial placenta. Little is known of the mechanisms that regulate umbilical vessel spasm, but if these mechanisms can be elucidated and umbilical vasospasm can be prevented, then in utero antenatal surgery will be rendered much less risky, and the problem of vascular access to an artificial placenta will be solved.
Prevention of Premature Labor. Little is known at present of the factors involved in initiating either normal or premature labor. Until this basic knowledge has been accumulated, it will be difficult to develop mechanisms to prevent premature labor. Active research is currently in progress on a variety of factors and drugs that are thought to influence and possibly prevent premature labor. Unfortunately, many of these drugs affect the normal development of the fetal lung, or other major organs. Before considering fetal surgery, one must be able to prevent premature labor following surgical insult to the uterus, without impairing normal fetal development.
Ethics. Fetal surgery commits the mother to the risk of considerable morbidity and possible mortality. Any operation on the uterus necessarily implies an operation on the mother. There will not only be the initial operation to repair the defect, but there will almost certainly be a second operation (an elective cesarean section) at delivery. There may be continuing late maternal morbidity as well, in the form of repeated cesarean sections for subsequent deliveries.
These problems of maternal morbidity will almost certainly restrict antenatal surgery to the correction of major congenital anomalies that carry an extremely high mortality and possibly those that are also associated with major and prolonged morbidity when repair is carried out after delivery. If the concept of fetal surgery is accepted, then it may be feasible to develop techniques to surgically repair major congenital cardiac defects. Possibly the milieu of the fetal circulation, with its low peripheral resistance associated with placental circulation, may allow sound healing after cardiac surgery and currently uncorrectable anomalies may possibly lend themselves to in utero correction. Indications for fetal surgery may well be broadened considerably if it proves possible to develop an artificial placenta.
Antenatal Diagnosis. Various ultrasonic techniques have revolutionalized antenatal diagnosis of many structural congenital anomalies. Several large studies have confirmed that the antenatal yield of congenital anomalies is approximately equal to the numbers diagnosed during a prospective survey of all live born children. Hinselmann[135] successfully diagnosed 23 major anomalies while screening 10,000 high risk pregnancies. He missed 15 anomalies, but 11 of these were congenital heart lesions or chromosomal abnormalities. Sabbagha and Shkolnik,[136] and Dunne and Johnson[137] have stressed the importance of a systematic examination, with meticulous attention to detail and real-time confirmation of the appearances demonstrated on gray scale ultrasound in making accurate antenatal diagnoses. These authors and Hobbins[138] and his group all stress how important it is to establish a group of highly skilled, experienced physician-sonologists, if the rate of antenatal detection of anomalies is going to approximate the true rate. Hackeloer,[139] Allan et al.,[140] and Kleinman et al.[161] have reported successful antenatal diagnoses of major cardiac lesions using a combination of real time, gray scale imaging and M-mode ultrasonography. A question yet to be addressed is whether routine antenatal diagnosis with appropriate referral to a combined pediatric surgical center and high risk obstetrical center will improve morbidity and mortality for any given defect. What is not certain is whether delivery under optimal conditions with a pediatric surgery team standing by will make any difference to the prognosis.
If the fetus is shown to have a major structural defect, then amniocentesis should be carried out to detect major chromosomal anomalies. If major chromosomal anomalies are detected, it will certainly influence the obstetrician's handling of the remainder of the pregnancy.
A new diagnostic technique may well revolutionize the field of antenatal diagnosis. Active studies are now being carried out to utilize computerized axial tomography (CAT) scan type-technology to create images using the phenomenon of nuclear magnetic resonance. If an alternating magnetic field is applied to a tissue, the hydrogen nuclei within that tissue will resonate at a specific frequency. When the magnetic field is turned off, then these resonating hydrogen nuclei emanate specific radiofrequency waves. Detection of these waves can allow the production of CAT scan-like images of sections of the body. The technical details of this have been published in two easy to read articles by Hounsfield[142] and by Marx.[143] Hendren[144] has shown no ill effects yet in the neonates with esophageal atresia and imperforate anus that he has placed in powerful electromagnetic fields. However, the clinical application of this technique of nuclear-magnetic resonance is still some time off. There are still many technical "bugs" to be ironed out before this new diagnostic modality can be applied to antenatal diagnosis.
The Need For Prospective Studies. At the present time, there are few (if any) prospective studies being carried out to determine whether antenatal diagnosis of congenital anomalies will influence their outcome. It is difficult to assess just how much suboptimal management at peripheral hospitals, or the stress of transporting sick neonates to a pediatric surgical center, contributes to mortality for certain lesions. We badly need studies to determine which babies need to be transported to a pediatric surgery center in utero, and which babies can safely be delivered at a peripheral center and then transported postnatally. We urgently need to establish objective criteria by which we can assess the prognosis for a given lesion before the child is born. Some lesions are going to lend themselves to this approach, and others are not. We need to firmly establish indications for antenatal ultrasonic screening. We must answer the question, "Is pregnancy alone sufficient indication for an ultrasonic examination, or should more selective criteria be developed?"
Studies need to be commenced now to answer this and other questions so that by the time the "mandatory prerequisites" have been met, some clear idea of which lesions will lend themselves to intrauterine repair will have emerged.
A good case in point is the diaphragmatic hernia of the Bochdalek type. In 1853 Bowditch[12] reported an accumulated series of congenital diaphragmatic hernias (not all of the Bochdalek type). Forty-two percent died in the first two hours after birth. By two years of age, a total of 65% had died. Harrison et al.[13] in 1975, reported a 66% mortality among patients born alive with diaphragmatic hernia. Nearly three-quarters of these babies died before treatment could be instituted. To date, two cases have been reported[145,146] in which a diaphragmatic hernia was diagnosed antenatally, but delivery with a pediatric surgery team standing by failed to save either baby. For this lesion, we need to develop ultrasonic criteria which will accurately predict prognosis. When techniques for in utero repair of this lesion become available, this option can then be offered to a mother whose baby will have little or no chance of survival if intrauterine repair is not attempted. Unfortunately, diaphragmatic hernia is one lesion that will probably only be detected by routine ultrasonic screening of all pregnancies.
Techniques For Antenatal Surgery. Techniques for certain antenatal surgical procedures are being developed. There is active work going on in two centers in the United States to develop a model in the fetal lamb for in utero creation and repair of diaphragmatic hernia.
The equipment is already available for treating some obstructive urinary anomalies. A child with antenatal diagnosis of posterior urethral valves or hydronephrosis could have a Lily catheter or some self-retaining catheter placed under ultrasonic guidance with the urine drained into the amniotic cavity. Current techniques allow this to be done with minimal risk and an acceptable chance of success. The major obstacle at this time is knowing which fetus should be treated. Techniques for the repair of other anomalies must await the proof that these techniques are required and will result in minimal maternal and fetal morbidity.
CONCLUSION
The idea of fetal surgery is not new. During the 1960's, a surprising number of exchange transfusions were performed on fetuses after operative venous or arterial cutdown on the fetal foot. The first was attempted by Drs. Freda and Adamsons[147] in 1963. A similar procedure was also carried out on several occasions by Asensio et al.[148] in Puerto Rico. Adamsons[149] in 1966 and Jackson[150] in 1968 both suggested that fetal surgery would have a place in the future management of such fetal anomalies as hydrocephalus, fetal neoplasms, diaphragmatic hernias leading to hypoplasia of the lung and certain cardiovascular anomalies. Neither of these visionaries predicted antenatal surgery as a possible means of palliating severe obstructive uropathy.
Intrauterine surgery will have a definite, but limited, place in the future for repairing or palliating major congenital anomalies that are currently associated with a high mortality. There would be much more enthusiasm to pursue antenatal repair of a major anomaly if we had objective criteria demonstrating that a baby with a given anomaly has little or no chance of survival if delivery is accomplished without intrauterine repair. Such anomalies are few and far between. Further development of these techniques must necessarily wait until the risk of maternal morbidity and the risk of premature labor and delivery of the baby have been reduced to acceptable levels, or until a safe, adequate artificial placenta has been developed.
It is interesting to reflect upon the Neonatal Intensive Care Unit in the year 2000. If an artificial placenta has been developed by then, the rows of open radiant-heater beds and ventilators may well be replaced by rows of plastic bags filled with fluid. In these bags one may see floating fetuses connected via their umbilical cords to artificial placentas. The problems of respiratory distress syndrome and bronchopulmonary dysplasia will be solved. They will, however, be replaced by other problems which will develop to challenge the perinatologist of the day, most of whom are still in grade school today.
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