Reversal of PSA progression on abiraterone acetate through the administration with food in men with metastatic castration-resistant prostate cancer.

BACKGROUND: Owing to efficacy and tolerability, abiraterone acetate (AA) is a leading treatment for men with metastatic castration-resistant prostate cancer. Increased serum concentrations of AA, such as by taking AA with food, may lead to the inhibition of additional enzymes in the androgen synthesis pathway implicated in castration-resistant prostate cancer progression. METHODS: Medical records of men with metastatic castration-resistant prostate cancer (mCRPC) who received AA between 1 April 2011 and 31 December 2013 were retrospectively reviewed. The primary outcome was the percent of men with a rising PSA on AA who experienced any PSA decline within 3 months after changing the administration of AA from without food to with food. Secondary outcomes were median time on AA therapy in men who received AA therapy without food versus those that switched administration from without food to with food at PSA progression, and the percent of men who experienced any decline in serum testosterone concentration, and the rate of adverse events observed while taking AA with food. RESULTS: Nineteen men who switched AA administration from without food to with food and 41 patients who administered AA without food only were included in the study. Of those patients who took AA with food at PSA progression, a PSA decline was observed in 3 of the 19 (16%) men, including 3 of the 14 men who had an initial response to AA (21%). Testosterone declined in five out of seven patients from pre-food levels. The median time on AA therapy was increased by nearly 100 days in patients who switched AA administration from without food to with food. No increases in toxicity were observed. CONCLUSION: Some men with mCRPC may benefit from taking AA with food. Further prospective comparative studies are needed to determine if changing AA administration is beneficial.

Fetal spina bifida repair--current trends and prospects of intrauterine neurosurgery.

Myelomeningocele is a common dysraphic defect leading to severe impairment throughout the patient's lifetime. Although surgical closure of this anomaly is usually performed in the early postnatal period, an estimated 330 cases of intrauterine repair have been performed in a few specialized centers worldwide. It was hoped prenatal intervention would improve the prognosis of affected patients, and preliminary findings suggest a reduced incidence of shunt-dependent hydrocephalus, as well as an improvement in hindbrain herniation. However, the expectations for improved neurological outcome have not been fulfilled and not all patients benefit from fetal surgery in the same way. Therefore, a multicenter randomized controlled trial was initiated in the USA to compare intrauterine with conventional postnatal care, in order to establish the procedure-related benefits and risks. The primary study endpoints include the need for shunt at 1 year of age, and fetal and infant mortality. No data from the trial will be published before the final analysis has been completed in 2008, and until then, the number of centers offering intrauterine MMC repair in the USA is limited to 3 in order to prevent the uncontrolled proliferation of new centers offering this procedure. In future, refined, risk-reduced surgical techniques and new treatment options for preterm labor and preterm rupture of the membranes are likely to reduce associated maternal and fetal risks and improve outcome, but further research will be needed.

Subcutaneous endoscopically assisted ligation (SEAL) of the internal ring for repair of inguinal hernias in children: report of a new technique and early results.

BACKGROUND: Open inguinal hernia repair with high ligation is an excellent method of repair in the pediatric population. Advantages of endoscopic repairs include the ability to evaluate the contralateral side, avoidance of access trauma to the vas deferens and gonadal vessels, and decreased operative time. We now report our experience with subcutaneous endoscopically assisted ligation (SEAL),: a novel technique that has proved to be a safe and effective in the treatment of inguinal hernia in the pediatric population. METHODS: The study is based on a retrospective review of 204 pediatric patients with 300 inguinal hernias treated with the SEAL technique from November 2001 to August 2003 at a tertiary referral center. Patient age ranged from 30 days to 16 years at the time of operation, with a mean follow-up of 235 days (median follow-up, 189 days). Statistical analysis was done with chi2 test, with the main outcome measures being intraoperative and postoperative complications including recurrence rate, suture abscesses, and postoperative hydroceles. RESULTS: There were 13 recurrences in 300 SEAL repairs, for a recurrence rate of 4.3% (95% C.I. 2.01%-6.65%), with only two recurrences in the last 100 repairs (2%). There were 10 suture abscesses or granulomas and 7 postoperative hydroceles. There was no statistically significant association between recurrence and gender, age at operation, history of prematurity, bilaterality, or kind of suture used. CONCLUSIONS: Our 4.3% (95% C.I. 2.01-6.65%) recurrence rate is comparable to prior series of laparoscopic repairs citing recurrence rates of 0%-5.7%. The majority of recurrences occurred within the first 4 months of developing this new procedure, with only two recurrences in the last 100 repairs. These pilot data suggest that SEAL is a safe and effective technique for inguinal hernia repair in the pediatric population. A prospective study is planned to compare this laparoscopic technique with open herniorrhaphy.

[Fetal surgery: for selected patients. The experiences of the Fetal Treatment Center in San Francisco]. / Foetale chirurgie: voor geselecteerde patiënten. De ervaringen van het Fetal Treatment Center in San Francisco.

Foetal surgery, i.e. invasive treatment of the unborn child, has pros and cons. Foetal surgery is the therapy of choice in a selected group of patients. For obstructive uropathy, intrauterine urinary drainage is seldom indicated due to disappointing results. For congenital diaphragmatic hernia, the pressure in the respiratory tract is increased by tracheal occlusion therapy and so pulmonary hypoplasia is prevented. This probably has a beneficial effect for children with a poor prognosis. For congenital cystic adenomatoid malformation of the lung and foetal hydrops, resection of the lesion gives a survival rate of 70%. For sacrococcygeal teratoma and foetal hydrops, intrauterine resection of the tumour might save the life of the foetus. For high myelomeningocele, intrauterine covering of the defect has until now given no improvement in the neurological outcome. For twin transfusion syndrome, laser coagulation of the placental anastomoses is probably superior to serial amnioreduction. For women the mortality and morbidity associated with foetal surgery are low.

Reversed latissimus dorsi muscle flap for repair of recurrent congenital diaphragmatic hernia.

BACKGROUND/PURPOSE: Neonates with large congenital diaphragmatic hernias (CDH) require prosthetic patch closure of the defect because of the paucity of native diaphragmatic tissue. As the child grows, patch separation can occur necessitating reoperation. Use of vascularized autologous tissue may decrease the incidence of reherniation as tissue incorporation and growth may be improved. The authors report our early experience using a local muscle advancement flap with microneural anastomosis for those children in whom reherniation develops after prosthetic patch placement. METHODS: Seven patients with CDH (6 left and 1 right) whose synthetic diaphragmatic patch separated from the chest wall resulting in a clinically significant recurrent hernia were followed up with prospectively. After dissecting the ipsilateral latissimus dorsi off the chest wall and dividing the thoracodorsal neurovascular bundle (based on its lumbar blood supply), the synthetic patch was removed via an eighth intercostal incision. The muscle flap was placed into the hemithorax through the bed of the tenth rib and sutured in place over a Vicryl mesh scaffold. The thoracodorsal nerve was anastomosed to the phrenic nerve. Functional analysis of the flap was performed in 4 patients. RESULTS: Age at placement of the muscle graft ranged from 2 months to 48 months (median, 24 months). There has been no evidence of reherniation after placement of the muscle graft. Long-term outcome and functional analysis of the flap was available in 4 patients (mean, 19 months). Two infants had fluoroscopic and sonographic evidence of nonparadoxical neodiaphragmatic motion. In one of these, electromyographic evidence of function was documented with a phrenic nerve conduction velocity of 22 meters per second. The third infant showed no evidence of neodiaphragmatic motion, and the fourth infant had paradoxical motion. CONCLUSIONS: This is the first direct documentation of phrenic nerve function in an infant with CDH. An innervated reversed latissimus dorsi (RLD) flap reconstruction for recurrent CDH provides an alternative to prosthetic patch repair. This technique offers the advantages of autologous vascularized tissue with potential phrenic nerve innervation and physiologic neodiaphragmatic motion.

Gastroschisis: small hole, big cost.

BACKGROUND/PURPOSE: This study was designed to assess the outcome and financial costs incurred for the treatment of gastroschisis. METHODS: A retrospective analysis was conducted of all patients with gastroschisis at a single institution over the past decade (n = 69). Hospital costs were determined and standardized to December 2001 dollars. RESULTS: Of the 69 patients, average gestational age at delivery was 35.9 weeks. Thirty-six patients had a primary fascial closure; 33 had a silo placed. The mean time to first feeding was 22 days and full feeding, 33 days. Average length of stay was 47 days. There were 3 deaths (2 shortly after birth, and one 131 days later owing to sepsis). The average cost of hospitalization and physician fees for patients with gastroschisis was $123,200. Using multivariate regression analysis, significant variables (P <.05) associated with cost of hospitalization were number of operative procedures, ventilatory days, male gender, and length of stay. Room expenses (43%), physician fees (15%), respiratory and pulmonary care (10%), and supply and devices (10%) made up the majority of costs. CONCLUSIONS: Cost of care associated with treatment for gastroschisis is high. Strategies designed to reduce cost must limit gastrointestinal, respiratory, and operative complications and reduce length of stay.

Congenital diaphragmatic hernia and hydrops: a lethal association?

BACKGROUND/PURPOSE: Nonimmune hydrops in the fetus is a finding that often portends death. The association and prognosis of fetuses with congenital diaphragmatic hernia (CDH) and hydrops is not known. METHODS: A retrospective review of all prenatally diagnosed cases and referrals of CDH was performed. Variables analyzed included gestational age at diagnosis and delivery, side of hernia, presence of associated anomalies and hydrops, and neonatal outcome. RESULTS: Since 1993, 474 prenatal referrals for CDH have been made. One hundred seventy-five were evaluated; 15 fetuses had hydrops (9%). Five patients had CDH, hydrops, and associated lethal anomalies. In the remaining 10 patients, 6 of the diaphragmatic defects were right-sided and 4 were left-sided. All except one had a major portion of the liver herniated into the chest. Six fetuses had prenatal intervention. Five neonates died shortly after birth. There were 5 long-term survivors; all received prenatal intervention. CONCLUSIONS: The association of CDH and hydrops is rare but often results in fatality. Hydrops appears to be associated with liver in the hernia, right-sided lesions, and lethal anomalies. Fetal intervention can be performed successfully in patients with CDH and hydrops, and may improve long-term survival rate in this group.

Fetoscopic treatment for discordant twins.

BACKGROUND/PURPOSE: In rare instances in monochorionic twin pregnancies, one twin can have a discordant anomaly (eg, cystic hygroma). If this twin dies in utero, neurologic injury and death can occur in the surviving cotwin. To protect the normal twin, the authors developed an approach to separate the circulations and ablate the umbilical cord of the abnormal twin. METHODS: From September 1998 to February 2001, 6 cases of discordant anomalous twins were diagnosed by prenatal ultrasound scan in which the anomaly was lethal or parents desired prenatal termination for this abnormal twin. All underwent surgical intervention with gestational ages varying from 19 to 24 weeks. RESULTS: Depending on cord insertion site and placental anatomy, blood flow was interrupted to the anomalous fetus by either radiofrequency ablation (RFA; 2 cases), cord transection (1 case), or cord transection after laser ablation of communicating vessels (3 cases). Fetal death occurred in one normal twin 4 days postoperatively. Average age at delivery for the 5 surviving fetuses was 34.5 weeks' gestation. On follow-up, all surviving infants are neurologically intact. CONCLUSION: An otherwise normal monochorionic twin threatened by an anomalous cotwin can be salvaged successfully with a strategy tailored to interrupt the vascular connections between the 2 twins.

[Fetal surgery for severe congenital abnormalities]. / Fetale Chirurgie für schwere kongenitale Fehlbildungen.

BACKGROUND: Over the past two decades the diagnosis of life threatening congenital malformations has evolved rapidly. Sophisticated and powerful new imaging and sampling techniques have stripped the veil of mystery from the once secretive fetus. Early detection and close follow-up of the fetus with congenital malformations have allowed us to define their natural history, determine the clinical features that affect clinical outcome, and plan management approaches to improve prognosis. Fetal surgical intervention is the logical culmination of the progress in fetal diagnosis. The purpose of this article is to describe the current techniques and recent advances in prenatal diagnosis and fetal intervention of severe congenital malformation. MATERIAL AND METHODS: A complete review of the literature and our own experience concerning fetal surgery was performed. RESULTS: Although most prenatally diagnosed malformations are best managed by appropriate medical and surgical therapy after maternal transport and planned delivery at a tertiary care center, an expanding number of simple anatomical abnormalities with predictable, lethal consequences have been successfully corrected before birth. A malformation amenable to prenatal surgical intervention must fulfill a number of conditions. It must be severe enough to warrant the risks associated with in utero treatment and must be reliably detectable before birth. Additionally, the pathophysiology must be reversible by fetal surgery, significantly improving the prognosis over post-natal treatment. Many technical intricacies of open fetal surgery have been solved, but pre-term labor and premature rupture of membranes remain a omnipresent risks to both the mother and the fetus. To reduce maternal morbidity and the risk of prematurity we developed minimally invasive techniques to treat the fetus prenatally. Current indications of fetal surgery include the treatment of congenital diaphragmatic hernia, cystic adenomatoid malformation of the lung, sacrococcygeal teratoma, obstructive uropathy, twin-to-twin-transfusion-syndrome and myelomeningocele. Minimally invasive surgical techniques (FETENDO) have significantly lessened the incidence of preterm labor and promise to extend the indications for fetal surgical intervention. CONCLUSIONS: Fetal surgical therapy for severe congenital malformations may improve the outcome of selected patients. The development of FETENDO will in all probability reduce the importance of open fetal surgery in the future.

Fetal intervention for myelomeningocele: effect on postnatal bladder function.

PURPOSE: Myelomeningocele is the most common congenital malformation of the central nervous system noted on prenatal ultrasound. Due to its significant postnatal sequelae, treatment in utero could potentially have a profound impact on the newborn. Others have reported fetal surgical techniques for in utero repair of myelomeningocele and its potential benefits on motor and neurological function. We report our urodynamic findings in the newborn after in utero repair of spina bifida in an effort to characterize postnatal bladder function. MATERIALS AND METHODS: A retrospective review of the fetal surgery database at University of California San Francisco was performed identifying patients with a diagnosis of myelomeningocele. Prenatal surgical repair of myelomeningocele was considered if a normal karyotype was present, no other significant congenital anomalies were evident and gestational age was less than 24 weeks. The spinal defects were in the lumbar or lumbosacral region. All surgery was performed before 24 weeks of gestations. RESULTS: Fetal surgery to correct myelomeningocele was performed in 6 patients. All patients were born premature at 32 weeks of gestation or less. Videourodynamics performed at age 1 month in 4 patients indicated decreased bladder capacity for weight, increased detrusor storage pressures and significant post-void residual. Hydronephrosis was demonstrated in 4 patients on renal/bladder ultrasound, and moderate vesicoureteral reflux was seen in 3. CONCLUSIONS: Patients with spinal bifida treated in utero appear to have the same changes in urodynamic parameters and anatomical abnormalities in the urinary tract as other children with spinal defects who have undergone standard postnatal care. In utero treatment of spinal bifida may expose the newborn to the effects of prematurity. The long-term effects on bladder function in the fetus after in utero repair of myelomeningocele remain unknown. A randomized controlled trial is necessary to evaluate long-term bladder function as well as other outcome variables in this experimental approach to patients with myelomeningocele.

Fetoscopic temporary tracheal occlusion by means of detachable balloon for congenital diaphragmatic hernia.

Occlusion of the fetal trachea blocks the egress of fetal lung fluid and stimulates the growth of hypoplastic lungs in fetuses with diaphragmatic hernia. Accomplishing temporary and reversible occlusion of the fetal trachea has proven difficult without invasive fetal surgery. Using simultaneous real-time ultrasonography and fetal bronchoscopy through a single uterine port, we placed a detachable balloon in the trachea of 2 fetuses with severe diaphragmatic hernia. In both fetuses the fetal lung subsequently enlarged, allowing survival after birth.

High-efficiency retroviral transduction of fetal liver CD38-CD34++ cells: implications for in utero and ex utero gene therapy.

OBJECTIVES: Defining methods for the efficient transduction of fetal stem cells could lead to novel fetal therapies for blood cell disorders and other birth defects. In this study, we analyzed the effects of various parameters on the retroviral transduction of primitive hematopoietic progenitors/stem cells isolated from fetal liver. METHODS: Candidate stem cells were isolated by fluorescence-activated cell sorting from midtrimester human livers based on the phenotype CD38-CD34++lineage- (lineage = glycophorin A, CD3, CD14, CD19, CD20 and CD56). A murine retroviral vector with a truncated human low-affinity nerve growth factor receptor (Delta NGFR) gene was used to transduce the candidate stem cells. Marker gene expression was monitored by flow cytometry using an anti-NGFR mAb. Candidate stem cells were transduced immediately after isolation or after up to 4 days of culture in serum-deprived medium containing the growth factors kit ligand and granulocyte-macrophage colony-stimulating factor. The effects on transduction efficiency of the addition of 4 microg/ml protamine sulfate and/or centrifugation to concentrate the candidate stem cells and virus were tested. After transduction, the cells were expanded for 10-21 days before determining the frequency of NGFR+ cells among the different hematopoietic progeny. RESULTS: Efficient transduction of candidate stem cells, at an average rate of 46%, was achieved after 3 days of culture with a single exposure to virus. Longer than 3 days of culture or repeated exposure to viral supernatant did not significantly improve the rate of transduction. The use of centrifugation at 1,200 g for 1 h and the addition of protamine sulfate during the transduction procedure were critical to achieving a high rate of transduction. Marker gene expression was observed on the progeny of the transduced cells in conjunction with CD34 (progenitors), glycophorin A (erythrocytes), CD14 (monocytes), CD15 (granulocytes) and CD41 (megakaryocytes). CONCLUSIONS: This study demonstrates that the efficient transduction of fetal candidate stem cells can be achieved under defined culture conditions using a retroviral vector. These results encourage further examination of in utero and ex utero gene therapy as a means of treating birth defects.

Pylephlebitis, portal-mesenteric thrombosis, and multiple liver abscesses owing to perforated appendicitis.

The authors report a case of a ruptured retrocecal appendix presenting with pylephlebitis, portal-mesenteric thrombosis, and multiple liver abscesses in an 8-year-old boy. A right hemicolectomy was performed, and liver abscesses were drained. The patient was treated with long-term antibiotics and anticoagulation. This the only case of perforated appendicitis associated with these 3 morbid complications in the modern literature.

Outcome of prenatally diagnosed solid fetal tumors.

BACKGROUND/PURPOSE: In the last 10 years, the ability to diagnose fetal tumors in the prenatal period has improved greatly because of technical advances in imaging. Early diagnosis and determination of tumor may affect prognosis. METHODS: The authors retrospectively reviewed the records of 1316 fetuses who underwent sonographic evaluation for congenital defects at University of California-San Francisco over a 6-year period. Of these, 16 had fetal tumors and were followed up at our institution. There were solid or predominantely solid with small cystic component masses in one of 3 locations: cervical, mediastinal, or abdominal. Excluded from our study were those fetuses with either sacrococcygeal teratoma, congenital cystic adenomatoid malformation of the lung, or ovarian cyst, because these defects have been extensively reviewed elsewhere. In addition, masses that were primarily cystic also were excluded. Data collected included diagnosis, gestational age at diagnosis and at delivery, mode of delivery, fetal and neonatal survival, and disease confirmation. RESULTS: Of the 16 fetuses, 4 had mediastinal tumors: 2 with pericardial teratomas (both of whom died in utero) and 2 with cardiac rhabdomyomas (1 died; the other presented tuberous sclerosis and is alive at 2 years of age). Four patients had cervical tumors (3 died; 1 survived and is alive and well), and 8 had abdominal tumors (3 with liver tumors, 4 with a left adrenal mass, and 1 with retroperitoneal teratoma). All eight patients with an abdominal tumor are alive and well. CONCLUSIONS: Fetal tumors are rare, and the prognosis seems to depend on their location and size. Although easier to detect, cervical and mediastinal tumors have a worse prognosis. Abdominal masses are more difficult to detect but have a better prognosis.

Fetal surgery for posterior urethral valves: long-term postnatal outcomes.

OBJECTIVE: Fetal intervention for obstructive uropathy was first performed at the University of California, San Francisco in 1981. Indications for treatment were bilateral hydronephrosis with oligohydramnios. Preintervention criteria included fetal urinary electrolytes with beta-microglobulin levels, karyotyping, and detailed sonography specifically looking for renal cortical cysts. We reviewed the outcomes of children who underwent fetal intervention with specific long-term follow-up in patients who were found postnatally to have posterior urethral valves. METHODS: A retrospective review of the University of California, San Francisco fetal surgery database was performed for patients with a prenatal diagnosis of obstructive uropathy. Medical records from 1981 to 1999 were reviewed. Long-term follow-up was documented if the cause of the urinary tract obstruction was posterior urethral valves. We collected data points, focusing on time and type of intervention, fetal urinary electrolytes, appearance of fetal kidneys, present renal function, length of follow-up, and present status of the urinary tract. RESULTS: Forty patients were evaluated for fetal intervention; 36 fetuses underwent surgery during this time period. Postnatal confirmation of posterior urethral valves was demonstrated in 14 patients. All patients had favorable fetal urinary electrolytes. Mean gestational age at intervention was 22.5 weeks. The procedures performed included creation of cutaneous ureterostomies in 1, fetal bladder marsupialization in 2, in utero ablation of valves in 2, and placement of vesicoamniotic catheter in 9. Six deaths occurred before term delivery with premature labor and the newborns succumbing to respiratory failure. One pregnancy was terminated electively because of shunt failure and declining appearance of fetal lungs and kidney. The remaining 8 living patients had a mean follow-up of 11.6 years. Chronic renal disease with abnormal serum creatinine was present in 5 patients. Two patients have undergone renal transplantation, and 1 is awaiting organ donation. Five of the 8 living patients have had urinary diversion with vesicostomy, cutaneous ureterostomy, or augmentation cystoplasty with later reconstruction. CONCLUSIONS: Fetal intervention for posterior urethral valves carries a considerable risk to the fetus with fetal mortality rate of 43%. The long-term outcomes indicate that intervention may not change the prognosis of renal function or be a predictor for possible urinary diversion. Despite all of these patients' having favorable urinary electrolytes, this did not seem to have any implication postnatally. When counseling families about fetal intervention, efforts should be focused on that intervention may assist in delivering the fetus to term and that the sequelae of posterior urethral valves may not be preventable. Fetal surgery for obstructive uropathy should be performed only for the carefully selected patient who has severe oligohydramnios and "normal"-appearing kidneys.

Transplantation of a fetus with paternal Thy-1(+)CD34(+)cells for chronic granulomatous disease.

A fetus diagnosed with X-linked chronic granulomatous disease was transplanted with Thy-1(+)CD34(+) cells of paternal origin. The transplant was performed at 14 weeks gestation by ultrasound guided injection into the peritoneal cavity. The fetus was delivered at 38 weeks gestation after an otherwise uneventful pregnancy. Umbilical cord blood was collected and used to determine the level of peripheral blood chimerism as well as levels of functional engrafted cells. Flow cytometry was used to detect donor leukocytes identified as HLA-A2(-)B7(+) cells, whereas recipient cells were identified as HLA-A2(+)B7(-) cells. No evidence of donor cell engraftment above a level of 0.01% was found. PCR was used to detect HLA-DRB1*15(+) donor cells among the recipient's HLA-DRB1*15(-) cells, but no engraftment was seen with a sensitivity of 1:1000. The presence of functional, donor-derived neutrophils was assessed by flow cytometry using two different fluorescent dyes that measure reactive oxygen species generated by the phagocyte NADPH oxidase. No evidence of paternal-derived functional neutrophils above a level of 0.15% was observed. Peripheral blood and bone marrow samples were collected at 6 months of age. Neither sample showed engraftment by HLA typing using both flow cytometry and PCR. Functional phagocytes were also not observed. Furthermore, no indication of immunological tolerance specific for the donor cells was indicated by a mixed lymphocyte reaction assay performed at 6 months of age. While there appears to be no engraftment of the donor stem cells, the transplant caused no harm to the fetus and the child was healthy at 6 months of age. Analyses of fetal tissues, obtained from elective abortions, revealed that CD3(+) T cells and CD56(+)CD3(-) NK cells are present in the liver at 8 weeks gestation and in the blood by 9 weeks gestation. The presence of these lymphocytes may contribute to the lack of donor cell engraftment in the human fetus.

Congenital diaphragmatic hernia associated with a gastroesophageal duplication cyst: a case report.

Severe left congenital diaphragmatic hernia was diagnosed in a baby prenatally, and she underwent hernia repair on the sixth postnatal day of life. She was found to have a huge symptomatic gastroesophageal duplication cyst on day 24 of life. A thoracoabdominal dissection allowed successful cyst excision. J Pediatr Surg 36:626-628.

Radiofrequency ablation of human fetal sacrococcygeal teratoma.

OBJECTIVE: Fetuses with solid, highly vascularized sacrococcygeal teratomas can die as a result of the vascular steal syndrome. This is the first report in which a percutaneous technique, radiofrequency ablation, was used to interrupt blood flow to a sacrococcygeal teratoma in 4 human fetuses. STUDY DESIGN: A radiofrequency ablation probe was percutaneously inserted into the fetal tumor under ultrasonographic guidance. In 2 fetuses a significant portion of the tumor mass was ablated, whereas in the other 2 fetuses only the major feeding vessels were targeted. RESULTS: Two infants were delivered at 28 and 31 weeks' gestation, respectively, and are doing well. In 2 other cases hemorrhage into the tumor led to an unfavorable fetal outcome. CONCLUSION: Ablation of a majority of the tumor tissue in sacrococcygeal teratoma is not necessary and proved fatal in two instances. Targeted ablation of the feeding tumor vessels diminishes blood flow sufficiently to reverse high-output fetal heart failure.

Prosthetic patch durability in congenital diaphragmatic hernia: a long-term follow-up study.

BACKGROUND/PURPOSE: The purpose of this study is to assess the durability of prosthetic patch repair of congenital diaphragmatic hernia (CDH) over time. METHODS: The authors retrospectively reviewed the records of 109 children who had surgical repair of CDH between January 1989 and December 1996. Prosthetic patches were used for diaphragmatic agenesis or when the diaphragmatic defect was too large for primary closure after mobilizing the posterior leaf. Forty-five (41%) children required a prosthetic patch to repair the diaphragmatic defect, and 29 (64%) of these children survived. RESULTS: Twelve (41%) of the 29 survivors required 1 or more operations for diaphragmatic reherniation. First-time reherniations occurred between 1 and 36 months postoperatively, median, 12 months. There was a bimodal distribution of first-time reherniations, with 4 children experiencing reherniation between 1 and 3 months, median age, 2 months and 8 children experiencing reherniation between 10 and 36 months, median age, 20 months. Three children returned for surgical repair of second reherniation between 21 and 39 months. Patients who had reherniation presented with bowel obstruction (n = 7), respiratory distress (n = 2), or were asymptomatic (n = 3). Revision of the reherniated patch consisted of placing an additional domed patch to fill the defect. CONCLUSIONS: About half of all prosthetic patches used to repair CDH show evidence of reherniation and require revision within 3 years. Prosthetic patch repair is not a long-term solution for the child with CDH.

Tracheal stenosis: the long and the short of it.

PURPOSE: The aim of this study was to present slide tracheoplasty as the procedure of choice for tracheal stenosis. From 1990 through 1997, patients referred to University of California, San Francisco for tracheal stenosis were managed by resection and anastomosis. During this period, other centers reported successful use of slide tracheoplasty and, from 1998 to 1999, we performed 3 slide tracheoplasties in addition to 3 more resection and anastomosis procedures. METHODS: Between 1990 and 1999, 9 patients with tracheal stenosis of varying lengths underwent surgery at the University of California, San Francisco. They were treated surgically with either resection and anastomosis or with slide tracheoplasty. RESULTS: Two of the 6 patients treated by resection and anastomosis had an anastomotic breakdown; all 3 patients undergoing slide tracheoplasty did not have anastomotic problems. Technically, a slide tracheoplasty has only one half the tension distributed over an oblique anastomosis that is more than the circumferential length of a resection and anastomosis approach. CONCLUSION: Based on these results, a slide tracheoplasty may be the procedure of choice for tracheal stenosis, whether long or short.