Patch repair is an independent predictor of morbidity and mortality in congenital diaphragmatic hernia.

BACKGROUND: Infants with congenital diaphragmatic hernia (CDH) have variable outcomes. There is a considerable potential benefit in being able to predict perinatally, which infants have severe hypoplasia and are thus more likely to die or survive with significant morbidity. We examine the relationship between a need for patch repair of CDH (PR) and outcome, using a national database. METHODS: Baseline characteristics of patients undergoing PR or non-patch repair (NPR) were compared. Multivariate analysis was performed to determine the association of PR with mortality and morbidity independent of other known predictors. RESULTS: Baseline characteristics of PR and NPR infants were similar although those infants with PR had higher SNAP-II scores. PR was an independent predictor of mortality with an odds ratio of 17.1 (95%CI 2.0-149.2) and was independently associated with secondary outcome measures of morbidity, including the need for oxygen at discharge and the duration of ventilation. CONCLUSIONS: Infants requiring PR have significantly higher mortality and suffer greater morbidity than those undergoing NPR. This association is independent of other known predictors of mortality. Identifying prenatal features associated with this high risk group would be of great clinical value.

A current analysis of primary lymphoma of the thyroid.

Over a 9-year period ending in May 1990, 27 patients with histologically proved thyroid lymphoma were assessed and treated. There were 24 female and three male patients with a median age of 67 years at the time of diagnosis (age range, 39 to 85 years). The usual presentation was that of a rapidly enlarging neck mass. Incisional biopsy was the diagnostic procedure of choice; however, nine of 27 patients underwent diagnostic partial or total thyroidectomy based on a preoperative impression of thyroid carcinoma. All 27 patients had non-Hodgkin's lymphomas of intermediate (77%) or high (23%) grade. Detailed staging was carried out in 25 patients; seven patients (28%) had disease confined to the thyroid gland (stage I), while 18 (73%) had accompanying disease in cervical lymph nodes or the mediastinum (stage II). Combined multiagent chemotherapy and irradiation was given to 19 of 25 staged patients (76%). Actuarial, overall 5-year survival for all patients was 70% with 48 months being the median follow-up for living patients (follow-up range, 3 to 102 months). Of a number of factors evaluated using log-rank survival tests, only the absence of dysphagia at the time of hospital admission, a primary tumor mass not greater than 10 cm, restriction of disease to the thyroid gland, and the absence of mediastinal lymph node involvement were statistically significant predictors of improved survival. Surgery should usually be restricted to diagnostic biopsy, as there is infrequently a role for resection in the management of thyroid lymphoma, given the effectiveness of combined multiagent chemotherapy and radiotherapy.

Function and quality of life results after ileal pouch surgery for chronic ulcerative colitis and familial polyposis.

The aim of this study was to assess gastrointestinal function and quality of life, including occupational, social, and sexual function, in 75 patients who underwent pelvic pouch construction between November 1984 and May 1988 at our institution. Complications occurred in 45 percent of patients after pouch construction and in 17 percent after ileostomy closure. One patient died from sepsis caused by an anastomotic leak after ileostomy closure. The most common complication was a pouch-anal anastomotic stricture (22 percent), and the complication with the greatest potential morbidity was pouch-anal dehiscence (8 percent), which was highly predictive of pouch failure. Functional results were assessed by questionnaire during the 3-month period after ileostomy closure in all 58 patients who successfully attained intestinal continuity. A second assessment was performed at 15 +/- 11 months after ileostomy closure in 52 patients whose continuity had been restored for longer than 3 months. In an overall assessment, 94 percent of all patients with restored intestinal continuity (73 percent of entire patient group) rated the pouch as being superior to a permanent ileostomy and 92 percent (71 percent of entire group) would go through another pouch procedure. These results support the continued recommendation of this procedure as an acceptable alternative to proctocolectomy and permanent ileostomy in patients with ulcerative colitis or familial polyposis.

An effective strategy for decontamination, ex vivo expansion, and storage of human fetal liver hematopoietic stem cells.

The transplantation of human fetal tissue has the potential to cure a variety of life-threatening diseases. The strategy for procurement, quality control, and functional assessment of human fetal liver HSC may prove useful for the transplantation of other fetal tissues. In addition to technical limitations, there are ethical and legal issues which need to be resolved before widespread use of fetal tissue. Further development of regulatory standards for the acquisition and distribution of fetal tissues will foster the application of this novel technology.

Use of Doppler ultrasonography in the evaluation of liver blood flow during silo reduction of a giant omphalocele.

Despite the utility of prosthetic silo reduction techniques, surgical treatment of giant omphaloceles containing herniated liver may result in acute compromise of hepatic vascular flow. The authors report a case of an infant with a giant omphalocele containing liver, in whom hepatic vascular compromise was suspected during the course of prosthetic visceral reduction. Doppler ultrasonography performed on the herniated liver through the prosthetic silo demonstrated triphasic vascular flow in the liver as well as normal hepatic venous flow and vena caval flow, and permitted continued gradual visceral reduction and a safe, delayed primary abdominal wall closure on the seventh day of life. Doppler ultrasonography performed through the silo may be an important diagnostic adjunct during the visceral reduction phase of staged abdominal wall closure in infants with giant omphaloceles.

Carpet bezoar obstruction of the small intestine.

Bezoars are food or fiber concretions of the alimentary tract, which infrequently cause intestinal obstruction. The authors describe a case of a 7-year-old child with pica in whom a synthetic fiber bezoar obstruction of the small intestine developed at the site of a previously stapled intestinal anastomosis.

Papillary cystic neoplasm of the pancreas: a report of three pediatric cases and literature review.

BACKGROUND/PURPOSE: Papillary cystic neoplasms are rare pancreatic tumors that typically present in women in their third decade of life. Few cases have been reported in children. METHODS/RESULTS: The authors report on three pediatric patients: a 10-year-old boy, an 11-year-old girl, and a 14-year-old girl. The authors have reviewed the existing literature on papillary cystic neoplasms of the pancreas and suggest that these tumors probably arise early in life, grow slowly, and metastasize infrequently. CONCLUSION: Even when these tumors metastasize, patients seldom die as a result of the malignancy.

Postpericardiotomy syndrome and chylopericardium: two unusual complications after aortopexy for tracheomalacia.

In two boys (aged 10 years and 7 months), large symptomatic pericardial effusions developed after aortopexy for tracheomalacia. Both patients underwent percutaneous pericardial drainage. The delayed presentation (3 weeks postoperatively) and associated findings in the 10 year old were typical of postpericardiotomy syndrome, and he had an uneventful recovery after a course of aspirin therapy. The 7 month old had chylopericardium and was treated with pericardial drainage and a low-fat, medium-chain triglyceride diet. Both cases represent previously unreported complications of aortopexy.

Congenital diaphragmatic hernia associated with aortic coarctation.

Congenital diaphragmatic hernia (CDH) may be associated with other anomalies, most frequently cardiovascular in nature. Despite fetal echocardiography, diagnosis of an accompanying cardiac malformation often is not made until after birth and sometimes not until after extracorporeal membrane oxygenation (ECMO) has been instituted. Aortic coarctation associated with CDH may occur as an isolated, surgically correctable malformation or it may be a component of the usually fatal left heart "hypoplasia" or "smallness" syndrome. The authors present two cases of aortic coarctation associated with CDH requiring ECMO that illustrate the management challenges of these coincident diagnosis. In one case, the accompanying coarctation was suspected and required precannulation angiography for confirmation, whereas in the other case, the diagnosis of coarctation was not made until after ECMO cannulation. Depending on its anatomic location and severity, an aortic coarctation associated with life-threatening CDH may limit the physiological efficacy of venoarterial ECMO. Furthermore, arterial cannulation for extracorporeal support requires that flow through the remaining carotid artery be maintained during aortic reconstruction, which may prove difficult for lesions best treated by subclavian flap angioplasty. When the diagnosis of coincident aortic coarctation and CDH is suspected or proven before institution of extracorporeal support, serious consideration should be given to venovenous bypass, because this may provide better postductal oxygenation and facilitate aortic repair with the option of left carotid artery inflow occlusion.

Fetendo-clip: a fetal endoscopic tracheal clip procedure in a human fetus.

Fetal tracheal occlusion accelerates lung growth and corrects the often fatal pulmonary hypoplasia seen in fetuses with congenital diaphragmatic hernia. Fetoscopy presents a unique opportunity to glimpse into the world of the fetus, but its use, until recently, has been limited to diagnostic and simple procedures. Using fetoscopic techniques ("Fetendo"), we now report successful tracheal occlusion in a 30-week-old fetus for the treatment of congenital diaphragmatic hernia.

Fetal endoscopic ('Fetendo') surgery: the relationship between insufflating pressure and the fetoplacental circulation.

Application of video-endoscopic surgery to the gravid uterus provides a new treatment option for the fetus with a correctable congenital anomaly. "Fetendo" surgery requires temporary enlargement of the uterine cavity to create a working space. Volume expansion of the amniotic space raises intrauterine pressure, which could increase placental vascular resistance and thereby reduce placental blood flow. To test this hypothesis, the authors developed a fetal sheep model to examine the relationship between insufflating pressure and flow in the placental circulation. Fetoplacental blood flow was measured via ultrasonic flow probes placed around the fetal common umbilical artery and the maternal uterine artery in five anesthetized 120-day-gestation ewes. Invasive feto-maternal monitoring permitted synchronous measurement of fetal mean arterial pressure, fetal central venous pressure, maternal mean arterial pressure, amniotic pressure, and fetal oxygen saturation, with calculated values for fetal and maternal placental vascular resistance. Amniotic pressure was raised from 10 mm Hg to 40 mm Hg in 5-mm Hg increments by a combination of saline amnioinfusion and external uterine compression. At amniotic pressures of 20 mm Hg or less, placental blood flow was preserved; however, elevation of amniotic pressure above 20 mm Hg resulted in a significant decrease in placental flow, with concomitant fetal hypoxia. The authors conclude that the relationship between intrauterine pressure, flow in the placental circulation, and fetal oxygen delivery must be considered when selecting intrauterine insufflation pressures for hysteroscopic intervention.

The role of prospective randomized clinical trials in pediatric surgery: state of the art?

PURPOSE: This study sought to determine the role of randomized controlled trials (RCT) in the evolution of pediatric surgical practice. METHODS: The authors used a computer-assisted literature search to identify all clinical trials related to pediatric surgery published in the English-language literature from 1966 through 1999. Each article was reviewed in detail for purpose, content, conduct, and quality of the trial. The authors assessed quality with a previously validated instrument (Chalmers Qualitative Assessment). RESULTS: The authors identified 134 RCTs related to pediatric surgery over the past 33 years. This accounts for 0.17% of 80,377 articles published in the field. The areas of surgery studied were analgesia 65 (49%), antibiotics 17 (13%), extracorporeal membrane oxygenation (ECMO) 9 (7%), gastrointestinal, burns, oncology, minimally invasive surgery, vascular access, congenital anomalies, and trauma (each <5%). Only 16 (12%) trials compared 2 surgical therapies, 9 (7%) compared a medical versus a surgical therapy, and 109 (81%) compared 2 medical therapies in surgical patients. Fourteen (10%) RCTs were funded by peer-reviewed agencies. Only 17 (13%) RCTs included a biostatistician as an author or a consultant. Trial design included calculation of sample size and statistical power in 21 (16%) RCTs. Method of randomization was reported in only 51 (38%). The test statistic and observed probability value was reported in 15 (11%). CONCLUSIONS: Clinical trials are used infrequently to answer questions related to pediatric surgery. When RCTs are utilized, they often suffer from poor trial design, inadequate statistical analysis, and incomplete reporting. Pediatric surgery could benefit from increased expertise, funding, and participation in clinical trials.

The 'PLUG' odyssey: adventures in experimental fetal tracheal occlusion.

In animal experiments, it has been shown that tracheal occlusion counteracts the pulmonary hypoplasia associated with congenital diaphragmatic hernia (CDH). Successful clinical implementation requires a reliable, reversible, and atraumatic technique of occluding the fetal trachea. With this clinical goal in mind, the authors evaluated the following three methods of tracheal occlusion in a fetal lamb CDH model: (1) an occluded foam-cuffed endotracheal tube, (2) a foam-cuffed endotracheal tube with a magnetically controlled flow valve, and (3) a tracheal insert constructed of a water-impermeable, expandable, polymeric foam, which is placed by a translaryngeal approach. The foam-cuffed endotracheal tube did not provide consistently reliable fetal tracheal occlusion. Although the magnetically triggered flow valve functioned well, it was not necessary to open the valve in utero (to prevent overdistension of the lungs), and the presence of the valve contributed to several occlusive failures. In contrast, the foam insert was easy to position and to remove from the trachea, while providing reliable tracheal occlusion for several weeks with consequent enlarged fetal lungs, increased lung fluid volumes, complete reduction of abdominal viscera, and improved pulmonary gas exchange after birth. Bronchoscopic evaluation of the foam-occluded neonatal tracheas showed little or no tracheal damage, which was confirmed during necropsy by gross and histological examination. Translaryngeal placement of a compressible, water-impermeable polymeric foam appears to be a simple and safe technique to achieve fetal tracheal occlusion.

Fetal endoscopic tracheal occlusion ('Fetendo-PLUG') for congenital diaphragmatic hernia.

Despite recent advances in surgical technique, posthysterotomy preterm labor remains a major determinant of postoperative fetal morbidity and mortality after in utero repair of congenital diaphragmatic hernia (CDH). Temporary fetal tracheal occlusion, or "PLUG" (Plug the Lung Until it Grows), reverses the pulmonary hypoplasia seen in experimental models of CDH and provides an alternative treatment strategy for some fetuses with CDH. Adaptation of current, minimally invasive surgical technology to the PLUG technique allows treatment of CDH without opening the uterus. In this report the authors describe a video-fetoscopic, intrauterine technique of tracheal occlusion (called Fetendo-PLUG) that could be used in human fetuses with CDH. The technique was developed in four fetal lambs that underwent video-fetoscopic intervention at 110 days' gestation (full term, 145 days), having undergone open creation of diaphragmatic hernias at 75 days. After maternal laparotomy and uterine exposure, the fetal head was located and a 5-mm curved, balloon-cuffed trocar was introduced through a uterine puncture directly into the fetal oral cavity. A steerable "bronchoscope" (with an instrument channel) was used to endoscopically intubate the trachea through the trocar, and the trocar was advanced over the bronchoscope and its balloon inflated to provide secure tracheal access below the vocal cords. Next, a 10-mm trocar was placed directly over the fetal neck, and the amniotic space was expanded with warm saline. A 5-mm laparoscope was introduced, and under simultaneous, dual video-fetoscopic (endotracheal and endoamniotic) visualization, a 1-mm nephrostomy puncture wire was advanced along the instrument channel of the bronchoscope, through the anterior wall of the trachea and fetal neck, into the amniotic space, then through the uterine wall to the outside. Withdrawal of the bronchoscope over the wire left a 5-mm endotracheal "trocar channel" along which a compressed, gelatin-encapsulated, polymeric foam insert (outer diameter, 4.8 mm) could be delivered by suture attachment to the guide wire. Once the foam was in its final endotracheal position, dissolution of the gelatin membrane allowed expansion of the foam to produce a water impervious tracheal occlusion. This two-trocar video-fetoscopic PLUG technique was performed successfully in all four fetuses, with a sequential decrease in operating time (median, 3.5 hours). Although two fetuses aborted postoperatively, the other two were carried successfully to term and demonstrated the anticipated physiological effects of adequate tracheal occlusion at the time of delivery.

Salvage laparotomy for failure of peritoneal drainage in necrotizing enterocolitis in infants with extremely low birth weight.

BACKGROUND/PURPOSE: Peritoneal drainage is a temporizing procedure for infants with extremely low birth weight (ELBW) who have perforated necrotizing enterocolitis (NEC). "Salvage" laparotomy is advocated when patients worsen after drainage. Some patients have survived with intact gastrointestinal functional after drainage alone. The purpose of this study is to determine if these salvage laparotomies are beneficial. METHODS: The authors reviewed the records of ELBW infants treated at Stanford University with perforated NEC from 1993 through 1998. Data collected included demographic makeup, type of operation, survival rate, postoperative complications, length of stay (LOS), and cost. RESULTS: The authors treated 26 patients, 9 with laparotomy and 17 with peritoneal drainage. The peritoneal drainage group had lower birth weight and more comorbid conditions. Survival rate was similar between laparotomy and drainage: 55.6% versus 41.2%. Four patients in the drainage group underwent salvage laparotomy for perceived clinical deterioration. All of these patients died. The clinical status of patients who had salvage laparotomy and died was similar to those who did not and lived. Seven of 13 patients treated with drainage followed only by supportive care and antibiotics survived. Cost and LOS for patients undergoing salvage laparotomy were much greater than for nonsurviving patients undergoing only peritoneal drainage: 84 +/- 20 days and $660,000 compared with 34 +/- 11 days and $306,000. CONCLUSIONS: Both primary peritoneal drainage and laparotomy should be considered primary therapy for perforated NEC. Patients undergoing peritoneal drainage typically experience clinical deterioration after operation. In this limited experience, salvage laparotomy did not appear beneficial.

The incidence and spectrum of neurological injury after open fetal surgery.

A preterm infant's immature brain is susceptible to both anoxic and hemorrhagic injury during periods of physiological stress. The advent of in utero surgery has created a new population of premature patients at risk for central nervous system (CNS) injury. The aim of this study was to evaluate the frequency and nature of CNS injuries in fetal surgical patients. Of 33 fetuses with known neurological outcome after fetal surgery, CNS injuries were identified in seven (21%). Of the seven, four had significant episodes of fetal bradycardia (3) or neonatal hypotension (1), which suggests that asphyxia contributed to the neurological injury. The CNS injuries in the other three patients occurred unexpectedly and without associated signs of fetal distress. The authors speculate that these injuries may have been caused by sudden fluxes in cerebral blood flow, induced by maternal hypoxia (1) or by maternally administered tocolytic drugs (2) used to treat postoperative preterm labor.

The OOPS procedure (operation on placental support): in utero airway management of the fetus with prenatally diagnosed tracheal obstruction.

Tracheal obstruction of the newborn caused by cervical masses such as teratomas and cystic hygromas can result in a profound hypoxic insult and even death, owing to an inability to establish an adequate airway after birth. Prenatal sonographic diagnosis of these congenital anomalies permits (1) anticipation of an airway problem at the time of delivery and (2) formulation of an algorithm for airway management while oxygen delivery to the baby is maintained through the placental circulation. This is the report of a fetus in whom a large anterior cervical cystic hygroma was detected by prenatal ultrasonography. A multidisciplinary management team was assembled, and an algorithm for airway management was developed. Elective cesarean delivery of the fetal head and thorax, under conditions of uterine tocolysis, permitted a controlled evaluation of the airway and endotracheal intubation while oxygen supply to the infant was maintained through the placenta. The baby remained intubated, and 2 days later underwent subtotal excision of the cervical cystic hygroma. Pharmacological maintenance of the feto-placental circulation after hysterotomy is an invaluable adjunct to airway management of the neonate with prenatally diagnosed tracheal obstruction.

Does pre-biopsy contrast enema delay the diagnosis of long segment Hirschsprung's disease?

INTRODUCTION: The diagnosis of long segment Hirschsprung's disease (LSHD) is frequently delayed. Our purpose was to: 1) summarize contrast enema (CE) findings in patients with LSHD, and 2) evaluate the utility of CE by comparing LSHD patients managed with/without pre-biopsy CE. METHODS: All LSHD cases (transition zone [TZ] proximal to the splenic flexure) treated between 1984 and 2009 were stratified according to whether a pre-biopsy CE was done (Group 1) or not (Group 2). CE were reviewed by a single pediatric radiologist, and the original reports were categorized as "helpful", "inconclusive" or "misleading". Group comparisons included elapsed days from admission to diagnostic rectal biopsy/first operation and initial hospitalization length of stay (LOS). RESULTS: 29 patients (16 in Group 1; 13 in Group 2) were identified. CE review revealed TZ in 7/16 (44%); and of these, 6 (86%) underestimated the actual aganglionic segment length. 6/16 (38%) original CE reports were "misleading". Overall, Group 1 patients experienced a significant delay in time to biopsy (p=0.047), first operation (p=0.005), and showed a trend towards prolonged LOS. CONCLUSIONS: Pre-biopsy CE offers little to the diagnosis of LSHD and may contribute to diagnosis/treatment delays. Even if a TZ is recognized in biopsy proven HD, the predicted aganglionic segment length should not guide the operative planning.

Perinatal predictors of outcome in gastroschisis.

OBJECTIVE: To identify perinatal risk variables predictive of outcome in gastroschisis. STUDY DESIGN: Gastroschisis cases were collected over a 3-year period from a national database. Risk variables evaluated included gestational age (GA), birth weight, time of birth, admission illness severity (score for neonatal acute physiology-II, SNAP-II) score, and abdominal closure type. Mortality and survival outcomes were analyzed. Multivariate analyses were performed. RESULT: In all, 239 infants were survived (96%). SNAP-II score predicted mortality (relative risk (RR)=1.07, 95% confidence interval (CI)=1.0 to 1.1). Length of hospital stay (LOS) and ventilation days were predicted by GA and by SNAP-II score. SNAP-II score predicted total parenteral nutrition (TPN) days (P=0.006). Severe cholestasis (conjugated bilirubin of >10 mg per 100 ml) was inversely related to GA (RR=0.77, 95% CI=0.61 to 0.97) and directly to categorical SNAP-II score (RR=3.4, 95% CI=1.2 to 10.1). Urgent closure predicted fewer TPN days (P=0.003) and shorter LOS (P=0.0002). CONCLUSION: SNAP-II scores significantly predict mortality and survival outcomes. Urgent closure favors fewer TPN days and shorter LOS. Our data refute routine preterm delivery in gastroschisis.

Impact of target blood gases on outcome in congenital diaphragmatic hernia (CDH).

INTRODUCTION: Neonatal intensive care unit (NICU) stabilization strategies which normalize physiology according to predetermined blood gas targets may contribute to observed improved survival rates of patients with CDH. The purpose of our study was to compare risk-adjusted outcomes of CDH patients managed with or without blood gas targets established at NICU admission. METHODS: Cases were collected from a national CDH network between May 2005 and November 2007. On NICU admission, the responsible neonatologist was asked to establish target ranges for pH, pCO (2), pO (2), and pre/post-ductal O (2) saturation. The outcomes analyzed were mortality, need for ECMO, days of mechanical ventilation/supplemental oxygen, and length of stay. RESULTS: Of 147 CDH infants, 63 had admission blood gas targets. Severity of illness and gestational age in both groups were comparable (SNAP-II score). Infants with blood gas targets had a significantly lower mortality than those without (Hazard ratio 0.27, p=0.006). CONCLUSIONS: Blood gas targets for the management of infants with CDH are associated with improved survival. Although the willingness to create and use stabilization targets to guide early NICU care may be a surrogate for other factors (experience, staffing, lack of interest), it is clearly associated with improved survival in CDH.