Successful fetal sacrococcygeal teratoma resection in a hydropic fetus.

BACKGROUND/PURPOSE: The development of hydrops in a fetus with a sacroccocygeal teratoma (SCT) usually is a predictor of fetal demise; in utero resection may offer the only chance of survival. Although the authors had performed this procedure in 3 previous cases, they had no long-term patient survival. The authors report a successful case of in utero resection of a fetal sacrococcygeal teratoma. METHODS: The authors resected a fetal SCT from a 23-weeks-gestation hydropic fetus, using gradually tightening umbilical tapes at the tumor base, electrocautery, and careful sharp dissection. After a blood transfusion, the fetus suffered cardiac arrest but was resuscitated and returned to the uterus. RESULTS: Postoperatively, residual SCT growth ceased, and hydrops rapidly resolved. Five weeks after the procedure, the infant was delivered because of preterm labor, and, after resection of residual SCT, was discharged home at 3 months of age. She is now a healthy 3 year old. CONCLUSION: This case shows that successful fetal SCT resection and long-term patient survival is possible.

Successful resuscitation during fetal surgery.

After in utero resection of a sacrococcygeal teratoma coupled with a transfusion of packed red blood cells, a 23-week-gestation fetus had bradycardia. Chest compressions were begun and epinephrine, atropine, and sodium bicarbonate were given, while the fetus remained bathed in warm saline. After 3 rounds of drugs, and just before withdrawing support, the fetal heart resumed beating and normal cardiac function. Based on to this case, the authors developed a resuscitation protocol for fetal surgery.

Creation of myelomeningocele in the fetal rabbit.

OBJECTIVE: Myelomeningocele is a neural tube defect resulting in an exposed spinal cord, which leads to irreversible neurologic damage at birth. We proposed development of a fetal rabbit model of myelomeningocele to study in utero spinal cord injury and repair strategies. METHODS: New Zealand white rabbits (n = 10) at 22 days of gestation (term = 31 days) underwent laparotomy to expose the gravid uterus; a hysterotomy exposed the fetal hindlimbs and back. A three to four level lumbar laminectomy was performed, and the dura over the posterior spinal cord was removed. At 30 days of gestation, the does underwent C-section for fetal harvest, and total fetal number, length, weight, and the presence or absence of a spinal defect were recorded for all viable fetuses. RESULTS: All injured fetuses were smaller and weighed less than the nonoperated littermate controls, and histologic examination confirmed a spina bifida-like lesion of their spinal cords. CONCLUSIONS: We successfully created an exposed spinal cord defect in the fetal rabbit model similar to the lesion found in humans. Advantageous because of low animal cost, relatively large fetal size, multiple fetuses per pregnancy, and short total gestation, this model will allow us to study the mechanism of injury to the exposed spinal cord, and perhaps develop strategies to repair human myelomeningoceles.

Neurodevelopmental outcome after open fetal surgery.

PURPOSE: This study examined the neurological and age-appropriate developmental outcomes of 11 children who underwent open fetal surgery. RESULTS: Nine children have normal neurological outcomes, and nine have normal development. CONCLUSION: Intensive postnatal care, intracranial hemorrhage, and requirement for prolonged respiratory support were associated with a worse neurological and developmental prognosis.

The antegrade continence enema procedure: a review of the literature.

Since the antegrade continence enema (ACE) was first described in 1990 for fecal incontinence, more than 100 cases have been reported in the literature. This report reviews the indications, operative modifications, outcome, and complications of the procedure.

Successful treatment of liver hemorrhage in the premature infant.

Intraoperative neonatal liver hemorrhage usually results in exsanguination and death. The parenchyma of a neonatal liver is fragile, and the capsule is very thin, unlike that in the pediatric and adult patient. This renders the treatment of a neonatal liver fracture almost universally unsuccessful. The current report describes two cases of successful management of intraoperative neonatal liver hemorrhage during surgery for necrotizing enterocolitis (NEC).

A surprising histological evolution of preterm sacrococcygeal teratoma.

In fetuses with sacrococcygeal teratomas (SCT), the development of hydrops is a harbinger of imminent fetal demise. In such instances, tumor debulking, either at fetal surgery or postnatally after urgent cesarean section, may prove lifesaving. Definitive SCT resection is carried out in the newborn period when the infant has stabilized. In five such patients, retrospective comparison of the SCT histological findings from the initial debulking procedure and later definitive resection or, in one case, autopsy results, showed a surprising change. After the initial debulking procedure, SCT histology findings from three patients were grade 3 immature teratoma without malignant elements, and in two patients grade 3 immature teratoma with nests of malignant yolk sac elements. In the three patients having grade 3 immature tumor without malignant elements, histological examination after definitive resection (two cases) and autopsy (one case) showed only fibrosis; there was no evidence of the SCT despite the fact that residual tumor had clearly been left at the initial debulking. In the patients in whom the initial histology finding was grade 3 immature with malignant elements, SCT histology results from the definitive resection showed grade 3 immature teratoma without malignancy in one case, and mature, benign tissue in the other. Preterm SCT debulking may induce tumor maturation or, alternatively, maturation of SCT that occurs naturally during gestation may be observed.

Chorioamniotic membrane separation: a potentially lethal finding.

Sonographic detection of chorioamniotic membrane separation (CMS) has been considered a benign incidental finding. We now report 6 cases of CMS identified by prenatal ultrasound; 1 in an otherwise normal pregnancy and 5 following fetal surgery. Following membrane separation, amniotic bands formed and compromised the umbilical cord in 4 cases leading to 2 fetal deaths. In the first case, CMS was detected by ultrasound at 22 weeks' gestation in an otherwise uncomplicated pregnancy. Because CMS was considered benign and umbilical cord blood flow was ample, the mother was followed by intermittent sonographic examinations. Fetal demise occurred 2 weeks later, clearly due to umbilical cord strangulation by an amniotic band. Surprised by this unexpected outcome, we reviewed our experience with CMS after hysterotomy for fetal surgery. Out of more than 40 fetal surgical cases, we have 5 cases in which CMS was recognized after hysterotomy. Three of these fetuses had umbilical cord compromise by a band of amniotic membrane leading to 1 fetal death. This experience demonstrates that membrane separation may be associated with amniotic band formation which can lead to cord strangulation and fetal compromise. Following fetal surgery, serial ultrasound evaluation and close fetal monitoring are indicated. In otherwise unremarkable pregnancies, clinician awareness of the possibility of amniotic band formation following CMS should be heightened. In either situation, knowledge of this potential life-threatening complication may identify cases in which cord compromise requires emergent delivery or fetoscopic release of the strangulating amniotic band.

Fetal hydrops after in utero tracheal occlusion.

In utero tracheal occlusion accelerates fetal lung growth in experimental animals. Following tracheal occlusion, animals with an intact diaphragm can develop hydrops; presumably the enlarged lungs increase intrathoracic pressure, compress the fetal heart, and occlude venous return. In contrast, in animals with a diaphragmatic hernia, even excessive lung expansion has not led to hydrops because the lung can expand through the diaphragmatic defect into the abdomen. The authors had assumed that the diaphragmatic defect in human fetuses with congenital diaphragmatic hernia would provide a similar "release valve" if excessive lung growth occurred. A recent case proved this assumption wrong. At 26 weeks' gestation, an in utero fetal tracheal occlusion was performed on a human fetus with a diaphragmatic hernia. Over the next 9 days there was rapid lung expansion and overdistention, compression of the fetal heart, and hydrops. These findings mimic those seen in fetuses with congenital high airway obstruction syndrome and has implications for in utero treatment of congenital diaphragmatic hernia.

Operating on placental support: the ex utero intrapartum treatment procedure.

While treating eight fetuses with predictable airway obstruction, the authors developed a systematic approach, the ex utero intrapartum treatment procedure, to secure the airway during delivery. Six patients had their trachea plugged or clipped in utero for treatment of congenital diaphragmatic hernia, and two patients had prenatally diagnosed cystic hygroma of the neck and oropharynx. The ex utero intrapartum treatment procedure was performed by using high doses of inhaled halogenated agents to facilitate uterine relaxation during cesarean section, securing the fetal airway while feto-placental circulation remained intact, and then dividing the umbilical cord. A variety of procedures were performed during the ex utero intrapartum treatment procedure including bronchoscopy, orotracheal intubation, tracheostomy, tracheostomy with retrograde orotracheal intubation, tracheoplasty, removal of internal tracheal plug, removal of external tracheal clip, central line placement, and instillation of surfactant. There were minimal maternal or fetal complications during the procedure. This approach requires the coordinated efforts of pediatric surgeons, obstetricians, anesthesiologists, sonographers, and neonatologists. The combination of intensive maternal-fetal monitoring, cesarean section with maximal uterine relaxation, and maintenance of intact feto-placental circulation provides a controlled environment for securing the airway in babies with prenatally diagnosed airway obstruction.

Nitroglycerin attenuates the bowel damage of necrotizing enterocolitis in a rabbit model.

Endogenous enteric nitric oxide has multiple functions. Enteric nitric oxide may be diminished in the premature infant and may therefore predispose the immature intestine to injury. The aim of this study was to determine if the infusion of a nitric oxide donor (nitroglycerin) would attenuate intestinal damage in a rabbit model of necrotizing enterocolitis. Transmural injection of rabbit intestinal loops with an acidified solution of casein and calcium gluconate simulates certain aspects of necrotizing enterocolitis. After injection of acidified casein solution into rabbit intestinal loops, twelve rabbits were randomly divided into two groups: six received maintenance fluids only and six received maintenance fluids and a nitroglycerin infusion adjusted to maintain mean arterial pressure 10 mm Hg below baseline (range, 2 to 12 micrograms/kg/min). After 3 hours, the rabbits were killed, and the intestinal tissue graded histologically. Intestinal damage in the nitroglycerin-treated rabbits was significantly less than that of untreated controls (mean histological grade of 0.39 v 1.48, P < .001). In this rabbit model of necrotizing enterocolitis, infusion of the nitric oxide donor nitroglycerin significantly attenuates intestinal damage. We speculate that enteric nitric oxide deficiency, as may exist in the preterm infant, predisposes the intestine to necrotizing enterocolitis.

The effects of intratracheally administered coarse mode particles on respiratory tract infection in mice.

Because coarse mode particles are rarely studied in their existing size ranges (greatest mass about 5-7 microns, aerodynamic diameter), we investigated the effects of four such particles, quartz, ferric oxide, calcium carbonate, and sodium feldspar, on host defenses against bacterial pulmonary infection. Mice which received intratracheal instillations of 10, 33, and 100 micrograms/mouse were exposed within an hour to aerosols of viable Streptococcus, and pneumonia-induced mortality was measured. At 33 and 100 micrograms/mouse, all particles significantly increased mortality. At the lower dose, only Fe2O3 caused a significant increase in mortality. To evaluate potential delayed effects, mice were challenged with the bacteria 24 h after exposure to 100 micrograms particles/mouse. Delaying the challenge did not significantly alter the response, except for the sodium feldspar group for which a partial recovery was observed. When mice exposed to 100 micrograms particles/mouse received aerosols of Klebsiella pneumoniae 24 h later, there was no significant effect on pulmonary bactericidal activity. For the model system used, it appears that Fe2O3, CaCO3, and sodium feldspar have effects roughly equivalent to quartz.