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One major problem for patients with myelomeningocele (MMC) is fecal incontinence. To prevent this problem, fetal surgery for repair of MMC has been recently undertaken. The strategy behind this surgery is to allow normal development of anal sphincter muscles. The purpose of this study was to determine whether fetal surgery for repair of MMC allows normal development of anal sphincter muscles. Myelomeningocele was surgically created in fetal sheep at 75 days of gestation. At 100 days of gestation, fetal surgery for repair of the MMC lesion was performed. Three repair methods were used: standard neurosurgical repair (4 fetal sheep), covering the MMC lesion with Alloderm (2 fetal sheep), and covering the MMC lesion with Gore-Tex (2 fetal sheep). After the sheep were delivered (140 days of gestation), external and internal anal sphincter muscles were analyzed histopathologically. In control fetal sheep (not repaired) anal sphincter muscles did not develop normally. In contrast, in fetal sheep that underwent repair of the MMC, the external and internal anal sphincter muscles developed normally. Histopathologically, in the external sphincter muscles, muscle fibers were dense. In the internal sphincter muscles, endomysial spaces were small, myofibrils were numerous, and fascicular units were larger than those in unrepaired fetal sheep. There was no difference in muscle development for the repair methods. Fetal surgery for repair of MMC allows normal development of anal sphincter muscles.

The development of fetal surgery has led to promising therapeutic options for a number of congenital malformations. However, preterm labor (PTL) and premature rupture of membranes continue to be ubiquitous risks for both mother and fetus. To reduce maternal morbidity and the risk of prematurity, minimal access surgical techniques were developed and are increasingly employed. Congenital diaphragmatic hernia (CDH), obstructive uropathy, twin-to-twin transfusion syndrome (TTTS), and sacrococcygeal teratoma have already been successfully treated using minimal access fetal surgical procedures. Other life-threatening diseases as well as severely disabling but not life-threatening conditions are potentially amenable to treatment. The wider application of minimal access fetal surgery depends on a continued improvement in technology and a better understanding of complications associated with fetal intervention.

Background/purpose: The management of the fetus with a large neck mass that obstructs the airway remains a clinical challenge. The authors review their experience with giant fetal cervical teratoma and discuss options for management., Methods: A retrospective review of all patients referred since 1994 for prenatal management of a fetal neck mass was performed. Variables examined included gestational age at diagnosis and delivery, size and location of the neck mass, presence of fetal hydrops, associated anomalies, management methods, operating time, and outcome., Results: Seven patients were identified with a prenatal diagnosis of giant cervical teratoma. Four patients had fetal hydrops; of these, 2 died in utero of hydrops, and a third fetus underwent elective termination. The remaining hydropic and previable fetus underwent fetal surgery for resection of the mass. The 3 nonhydropic patients underwent ex utero intrapartum treatment (EXIT) procedures for airway control. Endotracheal intubation was possible in one patient, and one received a tracheostomy. In the third fetus, neither intubation nor tracheostomy were possible, and resection of the neck mass was performed on placental support. There were no deaths in the surgical group., Conclusions: The management of fetal giant cervical teratoma includes a spectrum of options. For the rare previable fetus with hydrops, fetal resection may be indicated. In patients with airway obstruction, EXIT procedure provides the luxury of time to obtain airway control either by intubation, tracheostomy, or, if necessary, tumor resection on placental support., (Copyright 2003, Elsevier Science (USA). All rights reserved.)

Congenital diaphragmatic hernia (CDH) is one of the most common causes of neonatal morbidity and mortality. The clinical spectrum of CDH ranges from minimally affected infants who do well with modern neonatal care to severely affected infants who die despite all interventions. Two decades of research have led to advances in the prenatal diagnosis of CDH and have better defined the natural history of CDH. Fetuses with CDH now can be stratified into "low" and "high" risk groups based on sonographic parameters. "Low risk" fetuses have an excellent chance of survival with postnatal therapy. Prenatal intervention is reserved for "high risk" fetuses. Ongoing research is focused on improving both prenatal and postnatal treatment of these severely affected infants.

There are a growing number and variety of fetal disorders that may benefit from intervention prior to birth. Despite the diversity, there are common pathophysiologic denominators or patterns that tie together many seemingly disparate disorders. The purpose of this article was not to review disorders presented in other, accompanying articles but, rather, to present pathophysiologic patterns that common influence fetal surgery within a cohesive framework.

The impetus for the development of minimal access techniques for fetal surgery was the unusual occurrence with open hysterotomy of preterm labor, premature rupture of membranes, and the maternal complications resulting from tocolytic therapy. This strategy involves a constellation of techniques that allow surgical procedures to be performed inside the uterus without a hysterotomy. The unique requirements of this approach necessitated modifications of existing endoscopic techniques, development of novel fetoscopic instruments, and utilization of the expertise of a wide variety of specialists. Technical expertise in the field and a natural evolution of techniques have given rise to innovative repairs previously not envisioned. Severe congenital diaphragmatic hernia, diseases of monochorionic twins, and obstructive uropathy have already been successfully treated using fetoscopic surgical techniques. Fetoscopic correction of many other non-life-threatening anomalies continues to evolve. The future of fetoscopic surgical intervention depends on the continual evolution of novel techniques, the elucidation of the pathophysiology and treatment of other fetal disorders, and a better understanding of treatment of complications of intervention.

Objective: Pericardial teratomas are rare congenital tumors which invade the developing mediastinum, compressing the venous return to the heart, leading to hydrops. Tumors, with large cystic components, have been treated previously with in utero pericardiocentesis with some success. We present the first reported case of in utero open resection of a fetal pericardial teratoma., Methods: A 31-year-old G1P0 woman was found to have a fetus with a pericardial teratoma. Hydrops developed at 24 weeks' gestation. After counseling, open fetal resection was performed via a fetal median sternotomy., Results: Although the tumor was successfully removed, the hydrops did not resolve. In addition, over the course of 3 weeks, the mother developed maternal mirror syndrome which prompted an emergent cesarean section. Neonatal death ensued shortly after birth., Conclusions: The fetus with a pericardial teratoma complicated by hydrops is compromised. Treatment options include early delivery, aspiration of the pericardial effusion, and in utero operative resection., (Copyright 2002 S. Karger AG, Basel)

Objective: As the volume of fetal surgery cases has steadily increased, an increasing incidence of chorioamniotic membrane separation (CMS) has been noted. Due to the potential adverse consequences from this abnormality, we reviewed the last decade of experience with fetal intervention at our institution and examined the incidence and outcomes of fetuses given this diagnosis., Study Design: A retrospective chart review of 75 fetal surgery cases at our institution was performed. Variables analyzed included preoperative, operative, and outcome data. Postoperative ultrasounds were evaluated for the presence of CMS., Results: Excluding operative deaths, the incidence of CMS was 47%. There were significant differences (p<0.05) in time to delivery (7 vs 5 weeks), cases using a perfusion pump (80% vs 60%), and number of trocars (2.13 vs 1.54) in cases of CMS versus those without. Ultrasounds showed normal to high levels of amniotic fluid in 97% of cases. There was an increased incidence of premature rupture of membranes (63% vs 45%), preterm labor (57% vs 38%), and chorioamnionitis (29% vs 15%) with CMS, but no difference in mortality rate., Conclusion: CMS is a frequent finding following fetal surgery. It is associated with significant morbidity but is manageable with close follow-up in a hospital setting. Following fetal surgery, the finding of CMS can be a life-threatening complication that warrants further study to understand its etiology and prevention.