Closure of a giant omphalocele can be challenging. Preservation of the amnion in staged closure is not commonly practiced. Here, we describe 2 cases of giant omphalocele treated with a modified amnion preservation, staged closure technique. This paper demonstrates the feasibility and safety of this technique, and the versatility of amnion to adapt to an escharization strategy if closure is not achievable.

The rupture of a huge omphalocele is an emergency that threatens the newborn baby's life. It constitutes a therapeutical concern in the absence of prosthesis especially in developing countries.

We are reporting herein the case of a newborn baby that we managed in emergency successfully thanks to a simple treatment.

It was a huge omphalocele, ruptured during delivery, in a male newborn baby. We conducted a simple and conservative surgical treatment without prosthesis, which consisted of reconstruction of the omphalocele's membrane by closing it with absorbable suture materials. The suture of the omphalocele's membrane was followed by treatment with the Grob's method. This treatment saved the newborn baby's life. The total skinning was obtained after 3 months.

In case of rupture of huge omphalocele in absence of prosthesis, it is better to suture the membrane, and continue the treatment according to the Grob's method; the residual disembowelment can be repaired later.

The advent of preformed silos has facilitated routine bedside placement often without any attempt of intestinal reduction. It is unclear whether a strategy of routine silo (RS) placement with delayed fascial repair is beneficial over attempted primary repair (aPR) and silo placement only for those patients who cannot be reduced. We retrospectively compared clinical outcomes of neonates having aPR to those having RS placement to determine the impact of routine silo use and silo duration on gastroschisis care.

Neonatal records from patients with gastroschisis at a single children's hospital between 1990 and 2008 were reviewed. Demographic and outcome data were recorded and subjected to statistical analyses. Documentation of attempted intestinal reduction was used as a surrogate marker for aPR. The remaining patients were placed in the RS group.

Two hundred forty-eight neonates with gastroschisis were identified. Thirteen were excluded for congenital or clinical issues which precluded aPR. Of the remaining 235 patients, neonates with RS had significantly more ventilator days (6.2 vs 4.4; P = .0011), more time of total parenteral nutrition (36.5 vs. 28.5; P = .0018), longer length of stay (LOS, 46.5 vs. 40.5; P = .0011), and greater hospital charges ($216,000 vs $172,000; P < .0001) than patients who had aPR. There was no significant difference observed in complications or survival. Linear regression modeling demonstrated that time to closure was significantly related to LOS as an independent variable. Each day to closure was associated with 2.2 extra days of hospitalization and approximately $9557 in hospital charges.

Although limited by retrospective biases, this study demonstrates that time to closure is the most significant variable related to LOS in gastroschisis. This relationship is intuitive since longer time to closure is probably determined by the severity of gastroschisis. The method of closure, by primary repair or silo, is of secondary importance. Conversely, unnecessarily increasing the time to closure may increase the LOS. The speed of reduction, whether through primary repair or by silo, should be guided by physiologic principles.

This study evaluates whether, on the long run, in patients born with a giant omphalocele, the liver and other solid organs reach their normal position, shape, and size. Seventeen former patients with a giant omphalocele, treated between 1970 and 2004, were included. Physical examination was supplemented with ultrasonography for ventral hernia and precise description of the liver, spleen, and kidneys. The findings were compared with 17 controls matched for age, gender, and body mass index. We found an abnormal position of the liver, spleen, left kidney, and right kidney in eight, six, five, and four patients, respectively. An unprotected liver was present in all 17 patients and in 11 controls, the difference being statistically significant (p = 0.04). In ten of the 11 patients with an incisional hernia, the liver was located underneath the abdominal defect.

In all former patients with a giant omphalocele, an abnormal position of the liver and in the majority of them, an incisional hernia was also found. The liver and sometimes also the spleen and the kidneys do not migrate to their normal position. Exact documentation and good information are important for both the patient and their caretakers in order to avoid liver trauma.

"Intra-abdominal hypertension", the presence of elevated intra-abdominal pressure, and "abdominal compartment syndrome", the development of pressure-induced organ-dysfunction and failure, have been increasingly recognized over the past decade as causes of significant morbidity and mortality among critically ill surgical and medical patients. Elevated intra-abdominal pressure can cause significant impairment of cardiac, pulmonary, renal, gastrointestinal, hepatic, and central nervous system function. The significant prognostic value of elevated intra-abdominal pressure has prompted many intensive care units to adopt measurement of this physiologic parameter as a routine vital sign in patients at risk. A thorough understanding of the pathophysiologic implications of elevated intra-abdominal pressure is fundamental to 1) recognizing the presence of intra-abdominal hypertension and abdominal compartment syndrome, 2) effectively resuscitating patients afflicted by these potentially life-threatening diseases, and 3) preventing the development of intra-abdominal pressure-induced end-organ dysfunction and failure. The currently accepted consensus definitions surrounding the diagnosis and treatment of intra-abdominal hypertension and abdominal compartment syndrome are presented.

Retrospective studies have suggested that routine use of a preformed silo for infants with gastroschisis may be associated with improved outcomes. We performed a prospective multicenter randomized controlled trial to test this hypothesis.

Eligible infants were randomized to (1) routine bedside placement of a preformed Silastic spring-loaded silo, with gradual reduction and elective abdominal wall closure, or (2) primary closure.

There were 27 infants in each group. There was no significant difference between groups with respect to age, weight, sex, Apgar scores, prenatal diagnosis, or mode of delivery. The total number of days on the ventilator was lower in the spring-loaded silo group, although it did not reach statistical significance (3.2 vs 5.3, P = .07). There was no significant difference between groups with respect to length of time on total parenteral nutrition, length of stay, or incidence of sepsis and necrotizing enterocolitis.

Routine use of a preformed silo was associated with similar outcomes to primary closure for infants with gastroschisis but with a strong trend toward fewer days on the ventilator. Use of a preformed silo has the advantage of permitting definitive abdominal wall closure in a more elective setting.

Non-operative management of gastroschisis also known as plastic closure (PC) has been described as an alternative to conventional primary operative closure (POC) or staged silo closure (SSC). The aim of this study was to compare these techniques in neonates with gastroschisis. A retrospective review of neonates with gastroschisis who underwent PC was undertaken. The minimum follow-up was 1 year. Premature neonates (<35 weeks) and those with intestinal atresia or multiple congenital anomalies were excluded. Frequency matching of PC cases with two control groups with either POC or SSC was performed on 1:1 ratio, based on gestational age and birth weight. Statistical analysis using univariate analysis was performed. Three groups were assembled: PC, POC and SSC (n = 33). Median follow-up was 1,198 days. Groups were comparable with regard to: time to first feed, time to full enteral feeding, frequency of vascular access related infections, ventilation time, NICU LOS (length of stay) and hospital LOS. There was no difference observed in surgical complications. Almost all neonates in the PC group developed an umbilical hernia (83.8%). Umbilical hernias were highly associated with PC compared to the other two groups P = 0.001. To date only one patient has had to have an operative repair of the umbilical hernia in the PC group. Plastic closure is safe and comparable to conventional closure techniques. However, PC is associated with the development of more umbilical hernias. The natural history of these hernias remains to be defined.

The aim of this study was to develop a method of management of large omphalocele, with easily available inexpensive materials. The efficacy of using the plastic of urine collection bag and paper stapler, in creating the "silo" for the management of 3 newborns with such defects, were assessed.

All operations were done within 36 hours of birth. A silo was created with the plastic of a sterile urine collection bag, which was stapled with a paper stapler at its free margin. The omphalocele was gradually reduced every 24 to 48 hours, using the stapler, until the contents were reduced, when the abdominal wall was repaired.

The mean time taken to close the abdominal defect was 34 days. All patients could be breast-fed from 48 hours after the first stage is done. Rooming in was done by day 7. None of the babies required assisted ventilation.

This method is simple and cost-effective, using minimally expensive, easily available materials.

The purpose of this study was to assess the effectiveness of routine staged reduction and closure at the bedside, using preformed silos with no general anesthesia (PSnoGA), compared to emergency operative fascial closure (OFC) under general anesthesia for gastroschisis (GS).

A retrospective matched case-control analysis of neonates with GS was performed between 1990 and 2004 inclusively. Assessment included demographics, method of closure, days on ventilator, days to first enteral feed, days to full oral feeds, days on parenteral nutrition, length of hospital stay, and complications.

Sixty-five patients with GS were treated in our institution between 1990 and 2004. Thirty-five underwent OFC, 4 had Bianchi ward reduction, and 26 received PSnoGA. Seventeen patients with bowel perforation, atresia, ward reduction, medical complications necessitating ventilation, or any other condition requiring urgent surgical intervention were excluded from the analysis. Patients were well matched for gestation and birth weight. Forty-eight patients (OFC = 27 and PSnoGA = 21) were compared by using Mann-Whitney U test. Median days on ventilator (4 vs 0; P < or = .0001) was significantly reduced, but there was no difference for days to full oral feeds (26 vs 31; P = .26), days on parenteral nutrition (25 vs 30; P = .28), and length of stay (32 vs 36; P = .32), respectively. Complications were similar for both groups.

PSnoGA has outcomes statistically similar to OFC, although days on ventilator are significantly reduced. Slow reduction of the bowel avoids abdominal compartment syndrome and closure may be achieved without fascial sutures. PSnoGA is performed at the bedside and aims to avoid general anesthesia, a period of ventilation, and out-of-hours operating, thereby reducing costs. A prospective, multicenter, randomized control trial is needed to evaluate the effectiveness of PSnoGA.

The aim of this study was to analyze the outcome of giant omphalocele repaired in the neonatal period.

Twelve consecutive (1997-2004) neonates with giant omphalocele (defect >6 cm with liver herniation) were reviewed. A silo of Prolene mesh (Ethicon) was attached to the fascia and the defect was closed without opening the amniotic sac after sequential reduction. In 2 neonates with ruptured omphalocele a plastic sheet was inserted below the mesh. Data are reported as median and range.

Gestational age was 38 weeks (range, 32-40 weeks) and birth weight was 2.9 kg (range, 1.0-3.1 kg). The final closure was achieved at 26 days (range, 16-62 days). Three neonates (25%) died before final closure (causes: ruptured omphalocele, lung hypoplasia, cardiac anomalies, and intestinal failure). In the 9 surviving neonates, mechanical ventilation was required for 8 days (range, 2-20 days), hospital stay was 42 days (range, 23-73 days), and full enteral feeding was achieved on day 12 (range, 4-53 days). Complications included wound infection in 5 neonates and midgut volvulus in 1. Prophylactic Ladd's procedure was performed laparoscopically at a later stage in 4 children. At laparoscopy, intraperitoneal adhesions were minimal and the central liver did not preclude the operation. The 9 survivors are all well after 46 months (range, 12-67 months).

Giant omphalocele can be safely repaired in the neonatal period without opening the amniotic sac. Intestinal malrotation should be excluded and Ladd's procedure can be performed laparoscopically at a later stage.

Giant exomphalos containing liver as its major component and with visceroabdominal disproportion presents difficult management options to a paediatric surgeon. At Starship Children's Hospital, we deal with these with primary skin closure, if possible, followed by staged repair of the ventral hernia beginning in the 2nd year of life. During the closure of a ventral hernia, we encountered major hepatic venous bleeding resulting from the inadvertent injury to the right hepatic vein, resulting in the death of the child. An autopsy report showed the position of the hepatic veins superficially just beneath the skin. Subsequently, we performed magnetic resonance imaging (MRI) of the abdomen to look at the hepatic venous and caval anatomy in two children before closure of the ventral hernia. This was of immense help in limiting the dissection in the area and thus avoiding catastrophe. We recommend routine imaging with MRI before closure of a ventral hernia in children with giant exomphalos.

Management of giant omphalocele in a full-term neonate is a challenging clinical situation. Even more challenging is giant omphalocele in a premature low-birth-weight infant. The authors describe a successful staged noninvasive technique for the management of giant omphalocele in a premature, low-birth-weight neonate without the use of prosthetic material until delayed primary closure could be attempted.

This retrospective study elicits information regarding the dependence of neonatal outcome in gastroschisis upon: (1) the mode of delivery, (2) place of birth, (3) time for birth to surgery, (4) method of closure, (5) time from operation to commencement of first enteral feeds. The neonatal intensive care database from five major tertiary centres was used to identify 181 neonates with gastroschisis from 1990 to 2000. There were 8 deaths. There were no significant differences in outcome for infants delivered vaginally (102) versus Caesarean section (79), those born near the tertiary centre (133) as compared to infants born away (48), ones operated within 7 hours (125) compared with those operated after 7 hours (56), with delayed closure (30) versus primary closure (151). Neonates fed within 10 days of operation (85) had significantly lower incidence of sepsis, duration of TPN and hospital stay when compared to those fed after 10 days (96). Early commencement of feeds decreases the incidence of sepsis, duration of total parenteral nutrition (TPN) and hospital stay. Place of delivery, mode of delivery, time to surgery and type of closure do not influence neonatal outcome.

The optimal surgical approach for gastroschisis remains controversial, although primary closure after vigorous stretching of the abdominal wall and decompression of the intestinal contents is currently preferred.

Between 1984 and 1997, 24 newborns with gastroschisis were treated at Saitama Children's Medical Center. The average gestational age was 37.3 weeks, and the average birth weight was 2,285 g. One patient had the associated anomaly of intestinal atresia and short bowel. Rupture of the intestines during delivery was noted in one patient. The authors applied their nonaggressive staged repair using a prosthetic silo with preservation of the umbilical cord in 20 of the 24 cases (83.3%). Primary closure with preservation of the umbilical cord was performed in the remaining four cases (16.7%). In these patients, the gastroschisis was mild.

In the 20 cases treated by staged repair, the average interval between the first and second operation was 9.8 days. Mechanical ventilation was not required in 16 of 20 (80%) patients treated by staged repair, or in two of four (50%) patients treated by primary repair. The number of days to the first feeding averaged 14.6 days in 23 cases, excluding the patient with short bowel syndrome who required continuous total parenteral nutrition (TPN). TPN through a central venous catheter was required in 3 of 23 patients (13.0%). The overall average hospital stay was 55.1 days. Survival was 24 of 24 or 100%. Complications included perforation of the intestines, gastric bleeding, ventral hernia, and wound infection. No infections were associated with the prosthetic silo. All of the patients had a satisfactory cosmetic outcome. Recent advances in neonatal intensive care, including antibiotic therapy, reduced the possibility of infection.

This staged repair of gastroschisis was simple and safe, neither requiring experienced surgical judgment nor complicated postoperative management, and achieved satisfactory results. Furthermore, preservation of the umbilical cord provided an improved cosmetic appearance.

Although the mortality associated with gastroschisis (GS) has fallen markedly over recent years, postoperative morbidity and the incidence of complications remain high. Many different factors may contribute to this morbidity; the aim of this study was to determine which factors contributed most. Measures of morbidity used were time to full oral feeding (FOF), time on parenteral nutrition (PN), age at discharge, and incidence of complications. Between 1969 and 1995, 44 neonates with GS were treated; there were 6 deaths. The average initial temperature of the patients who died was 34.6 degrees C compared with 36.0 degrees C for the rest of the group (P = 0.02). Staged repair and prematurity were associated with increased time to FOF, time on PN, and age at discharge (P < 0.001). When the corrected post-term age was used, the difference between preterm and term babies was no longer significant. Mode of delivery did not influence any measure of morbidity. Seventeen patients (46%) had complications related to PN administration and 18 (43%) developed complications related to their surgery. There were no significant differences in these measures of morbidity when comparing patients born in the first half of the study period with those born in the last half. Multivariate analysis revealed that time to FOF, time on PN, and age at discharge were all strongly independently associated with staged repair and with the presence of complications of PN (all F > 7.2 and P < 0.01). Mode of delivery, gestational age, admission temperature, the need for postoperative ventilation, and complications of surgery were not independently associated with any of the measures of morbidity examined. Our data suggest that term delivery and primary closure of the defect are likely to minimise the morbidity experienced by infants with GS.

A retrospective analysis of a series of 63 cases of gastroschisis managed over an 11-year period distinguished a single statistically significant prognostic factor. There were 6 (9.5%) deaths, of which 4 occurred in the 8 infants with small-bowel atresia/stenosis (P < 0.005, Fisher's exact test). One died at 48 h and the remaining 3 of liver disease related to total parenteral nutrition. Of the 4 survivors, 1 developed a late biliary stricture necessitating hepaticoenterostomy but is alive and well aged 4 years. The remaining 3, following initially prolonged hospitalisations and multiple operations, are alive and well after 2, 4 and 7 years. In 3 patients the atresia was not detected at the primary operation. The small number of cases of gastroschisis-associated small-bowel atresia seen in any one unit may conceal the importance of the problem, and limits experience in the approach to management.

A method is described permitting rapid and safe tucks and adjustments to be made to a silo in staged reduction of an anterior abdominal wall defect.

This article describes the use of amniotic grafts (AG) in the repair of large abdominal wall defects in newborns with gastroschisis. From 1988 to 1995, 22 newborns with gastroschisis underwent surgical repair. In 12 primary closure (PC) was performed; in 10 the abdominal wall defect was covered with an AG. A double layer of AG was used and the graft was additionally covered with a silastic silo in 8 cases. The overall mortality was 14%; 3 children died from necrotizing enterocolitis or sepsis after AG. However, the difference in mortality of newborns with PC versus AG was not statistically significant. Patient characteristics, the postoperative courses, nd the frequency of complications were similar after PC and AG. It is concluded that AG has no negative impact on the postoperative course and yields a low overall mortality. In our opinion there are several advantages in using the AG technique: it is an autoplastic material that is readily available without costs, reefing and removal is unnecessary, and there is a potentially low rate of adhesions.

A single neonatal surgical unit treated 42 cases of gastroschisis over a 12-year period (1981-1993). The surgical management of each case was individualised, but every attempt was made to perform a primary repair when possible, based on the premise that this strategy gave the best outcome. The eviscerated intestine was evaluated with the patient under general anaesthesia. Serosal peel was not removed and intestinal atresias were not repaired. Gangrenous intestine was resected. The contents of the bowel were emptied proximally via a large naso-gastric tube and distally via the anus with warm saline lavage. The anterior abdominal wall was stretched and then reduction of the prolapse attempted. Following maximal enlargement of the peritoneal cavity, it was left to the operator to decide whether primary repair was possible and, indeed, permissible in each instance. Staged repairs necessitated the use of silastic pouches. Respiratory and intestinal insufficiency were managed by intermittent positive-pressure ventilation and total parenteral nutrition (TPN). Over one-half of the cases (24 of 42) were under 2.5 kg at birth. Intra-uterine growth retardation was unusual. Ten babies were delivered for obstetrical indications by Caesarean section: 50% were pre-term and in 4 pre-natal diagnosis of a ventral abdominal wall anomaly had been made. The transmural defects were all sited at the umbilicus and were to the right of a consolidated cord in 41 instances. Midgut necrosis due to torsion was encountered in 1 case; 3 further cases with intestinal atresia occurred. Primary closure was obtained in 30 (71%) of the cases reviewed. A prosthetic pouch was used in 12 patients for on everage 10 days in 10 uncomplicated cases. The average length of time in days of tertiary care given to 25 uncomplicated cases treated by primary fascial closure was: ventilatory support 4; intensive care treatment 8; and nutritional source TPN 20. There were 5 deaths (12%): 1 was unpreventable due to prenatal intestinal infarction; 2 were due to abdominal compartment syndrome with renal failure, and, intestinal ischaemia complicating primary and planned staged repairs; 1 caused by intestinal infarction due to torsion of bowel in a pouch; and 1 due to invasive infection. The role played by the strategy taken by the surgeon in the management of gastroschisis is crucial to the outcome. The creation of a compartment-like syndrome produced uncorrectable complications in this series of cases in both primary and staged abdominal wall closures. Minor degrees of this complication proved to be reversible in some patients, which was the reason for the wait-and-see attitude adopted in the management of this problem, often with fatal outcome. Where intra-peritoneal pressure monitoring is not used, the operating surgeon relies on unscientific observations for decision-making at the operating table. The time from birth to operation in 25 of the reviewed cases was on average 5 1/2 h. Of this group, 20 were outborn babies. This is unsatisfactory, but as shown by this review, even in the absence of prenatal management, which should ensure prompt repair, satisfactory results are still possible.

Pediatric anesthesia and intensive care management has improved dramatically over the past two decades. Improved understanding of the pathophysiology underlying newborn surgical emergencies, new medications and new modes of ventilatory support have all contributed to better patient outcome. The authors have reviewed the anatomy and physiology of the infant airway, indications for and principles of endotracheal intubation, the management of newborn surgical emergencies, indications for post-operative ventilatory support, different modes of mechanical ventilation available, complications of mechanical ventilation with weaning parameters and extubation criteria. The introduction of nitric oxide and the implications of extracorpreal membrane oxygenation in the management of newborn emergency refractory to conventional ventilation are discussed.

Giant omphalocele is a major clinical challenge for pediatric surgeons. Whereas small- to medium-sized defects can be repaired primarily, larger omphaloceles cannot be closed at birth because the liver and small bowel have lost the right of domain to the abdomen. Two divergent strategies have evolved for treating these giant defects: (1) use of a silon chimney with gradual reduction of the contents of the sac, and (2) initial nonoperative management (epithelialization) of the omphalocele followed by repair of the residual ventral hernia. In an 18-year retrospective study, we have reviewed our experience with these treatment methods. Ninety-four infants underwent treatment for omphalocele between 1975 and 1993. Primary closure (PC) was possible in 55 patients, silon chimney (SC) was used in 15, and 7 had nonoperative management (NM) with epithelialization. In the remaining 17 infants, surgery was believed to be inappropriate because of the lethality of their associated anomalies. Major (but potentially survivable) anomalies were present in 26% of PC, 13% of SC, and 71% of the NM group patients. The majority of the liver was present in 73% of SC- and 86% of NM-treated omphaloceles. There was a decrease in length of stay, time to enteral feeding, and mortality over the 18-year period. However, those patients whose defects could not be closed primarily had consistently longer hospital stays. This was particularly true for the SC patients. The decreased use of total parenteral nutrition seems to reflect a shift from SC to NM rather than a decrease in the interval to full enteral feeding in any given treatment group over time.(ABSTRACT TRUNCATED AT 250 WORDS)

The authors study reviewed patients who underwent operations for omphalocele and gastroschisis to determine survival, morbidity, and long-term quality of life.

Clinical follow-up of 94 patients cared for with omphalocele and gastroschisis during a 10- to 20-year period after birth.

Eighty-three patients survived initial treatment. Sixty-one had long-term follow-up. Mean follow-up in the group was 14.2 years. Survival was favorable in the absence of lethal or co-existing major congenital anomalies. Nineteen patients required 31 reoperations, most for abdominal wall hernias and the sequelae of intestinal atresia. Current quality of life was described as favorable (good) in 80% of patients.

Survival rate in patients with abdominal wall defects is favorable and deaths occur substantially in patients with co-existing lethal, or multiple, congenital anomalies. Reoperative surgery is necessary principally in those patients who have postclosure abdominal wall hernias, and in those with bowel atresia at birth. Reoperations are not likely to be necessary after school age. Quality of life in survivors is patient-perceived as entirely satisfactory.

Spinal anesthesia has been described for infants and premature babies undergoing minor operative procedures. The advantages of shorter operating time, avoidance of intubation, and shorter hospital stay have made this the gold standard for premature and other high-risk infants requiring minor procedures. However, little is known about this technique for major interventions in newborns and preterm infants. Recently, four infants born with gastroschisis underwent repair under spinal anesthesia. Two had accompanying intestinal atresia (one with a prenatal perforation and pan-hypopituitarism), and two had intact gastrointestinal systems. The gestational ages were 39, 33, 36, and 36 weeks, respectively. All had primary closure of the defect; one had no repair of the atresia because the bowel was thick and matted with a significant peel, and the defect was not identified. In the second case with atresia, necrosis and perforation of a localized segment of intestine was identified proximal to the intestinal atresia, and was exteriorized with the primary repair. When they arrived in the operating room, all four infants were breathing spontaneously, on room air, after appropriate fluid resuscitation. All underwent spinal anesthesia, which was the only agent used for the operation. The operative time was 45, 25, 30, and 25 minutes, respectively (mean, 31.25 minutes). The duration of anesthesia was 170 to 230 minutes (mean, 205 minutes). All infants were returned to the neonatal intensive care unit on room air and breathing spontaneously. One was given morphine postoperatively and suffered significant respiratory depression, requiring intubation. It appears that spinal anesthesia is safe and effective for major operative procedures in high-risk infants. (ABSTRACT TRUNCATED AT 250 WORDS)

Infants with gastroschisis (GS) commonly require total parenteral nutrition and prolonged hospitalization because of intestinal dysfunction resulting from dysmotility and/or malabsorption. To investigate prepartum small intestinal (SI) nutrient absorption in GS, a fetal rabbit model was surgically created on gestational day 24 (term, 31 to 33 days) in 11 time-mated New Zealand White does in each left ovarian-end fetus. Each right ovarian-end fetus served as a control (C) and was manipulated only. All does, 10 of 11 GS fetuses (91%), and 8 of 11 C fetuses (73%) survived to gestational day 30. GS fetuses had significantly reduced total body weights, SI weights, and SI lengths compared with C fetuses. Using the everted mucosal sleeve technique, the uptakes of an amino acid (proline) and a sugar (glucose) were determined. The uptakes of proline per milligram SI, proline per centimeter SI, and glucose per milligram SI were significantly impaired in GS fetuses compared with C fetuses (P < .04 by Student's paired t test). The uptake of glucose per centimeter SI was also reduced in GS fetuses, but not significantly. Uptake capacities (a measure of the entire SI's ability to absorb a given nutrient) were significantly reduced in GS fetuses compared with C fetuses (proline, 2,670 +/- 612 nmol/min/entire SI v 6,842 +/- 399 nmol/min/entire SI, P < .008 by Student's paired t test; glucose, 402 +/- 69 nmol/min/entire SI v 950 +/- 103, P < .008 by Student's paired t test).

When primary abdominal wall closure in a newborn with gastroschisis cannot be accomplished safely, placement of a reinforced Silastic silo facilitates delayed primary closure (DPC). In this report we describe our experience with the gastroschisis wringer clamp (GWC). The GWC is an autoclavable, 140-g, aluminum alloy device reminiscent of an old wringer washing machine. It consists of two apposing serrated rollers that pull the Silastic silo through a slotted base plate. This protects the intestine and converts the circular defect into a vertical slit to ease DPC. The GWC is adjusted daily on the awake newborn in the nursery and the magnitude of each adjustment is gauged by the infant's cardiac and pulmonary status. For the past 10 years we have cared for 116 newborns with gastroschisis. The average birth weight was 2,530 g (range, 1,380 to 3,300 g). Eighty-six infants (74.1%) have undergone primary closure. The remaining 30 infants (25.9%) were treated by placement of a Silastic silo and application of the GWC, forming the basis of this report. The DPC operation was performed an average of 6.7 days (range, 3 to 23 days) following the application of the silo. Extubation was usually possible prior to the DPC, with the mean length of mechanical ventilation being 3.8 days. Three patients developed serious complications including two dehiscences of the silo-fascia interface. There were no deaths in this group of 30 patients. The GWC offers many technical advantages and can be easily reversed when the infant's cardiopulmonary status deteriorates. We advocate its adoption as a method of choice in the newborn with gastroschisis who requires DPC.

This is a report of our experience with 22 cases of large unruptured omphaloceles treated by amnion inversion during the period 1973 through 1990. The method is characterized by three stages: (1) a silastic sheet is sutured directly to the skin around the amniotic membrane, under local anaesthesia, without dissection between the skin and the amnion; (2) the reduction of herniated viscera into the abdominal cavity is achieved by squeezing the sheeting using a specially modified stapler; and (3) the amniotic membrane is preserved intact, and inverted into the abdominal cavity at the time of abdominal wall closure. Of the 22 infants, 19 survived with satisfactory results. Two patients died of multiple associated anomalies, and the remaining patient died of sepsis arising at the time of the final abdominal closure. This procedure has proved to be effective and safe for high-risk patients with congenital heart diseases, anal atresia, tracheoesophageal fistula, or bronchial stenosis and prematurity. The practical aspects of the procedure, as well as its advantages and pitfalls, are illustrated.

To determine the extent of pulmonary dysfunction following primary closure of an abdominal wall defect, we obtained pulmonary function tests (PFT) in 11 newborn infants with gastroschisis and 6 with large omphaloceles admitted to a newborn ICU in a children's hospital. Patients were 1 to 30 days of age at the time of the PFT; all required endotracheal intubation and mechanical ventilation for operative procedures or for postoperative ventilatory support. Full-term infants (n = 21) undergoing minor surgical procedures provided comparative measurements. Flow-volume curves were obtained with manual inflation of the lungs followed by forced deflation using negative pressure, or by passive expiration, under sedation and pharmacologic paralysis. Deflation flow-volume curves gave measurements of forced vital capacity (FVC) and maximal expiratory flow at 25% of vital capacity from residual volume (MEF25). Modified passive mechanics technique gave passive expiratory curves that provided measurements of respiratory system compliance (Crs) and resistance (Rrs). Tests were done: within 48 h (period A), 3-7 days (period B), and 8-30 days after surgical repair (period C). Pulmonary function testing after nebulized 0.1% isoetharine (a bronchodilator), to test for bronchial reactivity, began midway during the study period in 15 patients. Preoperative and postoperative tests were obtained in 5 patients. Closure of an abdominal wall defect decreased FVC, Crs, and MEF25 by up to 50% of normal, reference values after surgery (P less than 0.05). FVC and MEF25 approached values of normal infants by 4 weeks, whereas Crs remained 50% lower.(ABSTRACT TRUNCATED AT 250 WORDS)

Forty infants with gastroschisis were referred to two paediatric surgeons during a 13 year period. Overall survival was 90%. Nine patients were transferred in utero and 31 were referred postnatally. Birth weights, gestational ages, and Apgar scores were similar for both groups. Primary closure of the defect was successfully achieved in seven (78%) patients in the prenatally transferred group compared with 17 (55%) in the postnatal group. Significantly less postoperative assisted ventilation, and a trend in favour of early discharge home, were noted after prenatal transfer. Problems arising during postnatal transfer may have contributed to these differences. No major differences resulting from the mode of delivery were identified. Patients treated by primary closure fared significantly better than those undergoing staged repairs with prosthetic material. Prospective randomised studies are required to confirm these findings.

In 1970, this patient was first reported after her massive ventral hernia was repaired. Her large omphalocele was initially covered with skin flaps as a newborn, and at 3 years of age the resulting ventral hernia was completely repaired using the staging technique described by Schuster, and modified by Plzak and Gross. Silon sheeting was used as a temporary prosthesis. She remained well until 1988 (age 23 years) when she became pregnant with the expected date of confinement January 10, 1989. The first two trimesters were uneventful with sonograms showing a normal male fetus who was gaining weight appropriately. By the third trimester the abdominal girth did not increase coincident with the baby's size, vomiting prevented adequate caloric intake, and pelvic pressure from the baby's head caused increasing discomfort. She spent 7 weeks in the hospital on intravenous therapy, including peripheral total parenteral nutrition, and delivered vaginally, with the aid of forceps, a 2.9-kg normal boy 5 weeks prematurely. This is the first reported case of a large omphalocele patient conceiving and delivering a normal fetus. It also demonstrates the potential problems related to multiple surgical procedures to close a large congenital abdominal wall defect.

A 93% survival rate was achieved in 80 neonates treated for gastroschisis between 1979 and 1986. Uncomplicated gastroschisis occurred in 70 infants (88%); 51% underwent staged silo reduction and 49% had primary fascial closure. Gastroschisis associated with intestinal atresia or volvulus was present in 10 neonates (12%), half of whom had a residual jejunoileum between 10 and 55 cm. Major postoperative complications included gastrointestinal problems (infarction, obstruction, and prolonged dysfunction), wound infection, and catheter-associated difficulties (sepsis, infiltration, and malposition). Three of the six deaths were related to associated conditions (extreme prematurity, trisomy 13, and multiple anomalies) and three were caused by intraoperative hemorrhage, necrotizing enterocolitis, and extensive short-bowel syndrome. No statistical difference in morbidity, mortality, and length of hospitalization was demonstrated between infants treated by silo reduction and primary closure. Safe management of gastroschisis should include an individualized assessment of visceroabdominal disproportion and degree of intraabdominal tension. Vigilant expectation of potentially life-threatening complications is required to decrease postoperative morbidity, irrespective of the technique of abdominal wall closure.

To determine whether intragastric pressure (IGP) and central venous pressure (CVP) would reliably predict successful primary closure of congenital abdominal wall defects (omphalocele/gastroschisis) in newborn infants, we developed the following prospective intraoperative management protocol. Following a temporary trial of fascial closure, infants who had an IGP less than 20 mm Hg or an increase in CVP of less than 4 mm Hg were primarily closed. If IGP was greater than 20 mm Hg or if CVP increased by more than 4 mm Hg, the temporary closure of the abdomen was reopened and a prosthetic silo was placed. Ten infants who were less than 24 hours old and averaged 2.7 kg (range, 1.4 to 4.2 kg) and 37-weeks gestation (range, 32 to 41 weeks) were studied. Eight infants met criteria for primary closure. Their IGP averaged 14 +/- 4 mm Hg (+/- SD) (range, 8 to 19 mm Hg), and their increase in CVP averaged 1 +/- 2 mm Hg (range, -2 to 3 mm Hg). In the two infants who required staged repair, IGP averaged 25 +/- 1 mm Hg (+/- SD) (range, 24 to 25 mm Hg), and the increase in CVP averaged 7 +/- 1 mm Hg (range, 6 to 8 mm Hg). All patients were anesthetized with fentanyl (12.5 micrograms/kg) and paralyzed with metocurine (0.3 mg/kg) intraoperatively. There were no postoperative complications in either group of patients related to increased intraabdominal pressure, and all patients were extubated within 48 hours of the initial surgery. We conclude that the intraoperative measurement of changes in IGP and CVP can serve as a guide to the operative management of congenital abdominal wall defects and can reliably predict successful outcome following repair.

From 1970 to 1987, 23 neonates with gastroschisis were treated at Kobe Children's Hospital. One patient died and 22 survived (96%). Seven patients were hypothermic upon arrival at the hospital, with body temperatures of 31.8 to 35.4 degrees C. Six patients were successfully managed by warm saline bathing (40 to 42 degrees C for one minute) to improve hypothermia. One patient who did not receive this rewarming procedure (body temperature, 31.8 degrees C) died of intractable metabolic disorders related to hypothermia. Ten patients were treated by primary fascial closure of the abdominal wall defect, eight by the skin flap method with secondary closure of the deliberately created abdominal hernia, and five by the silastic sac technique. Two patients required creation of intestinal stomas for ileal atresia. After surgery, 19 patients required ventilatory care with total paralysis (average 6.0 days). Nine patients required total parenteral nutrition (TPN), while 13 tolerated enteral feeding on days 3 to 11 (average, 6.0 days). In the latter 13 patients, intravenous fluid therapy was discontinued on day 11. Our experience suggests that (1) rewarming in a saline bath is effective treatment for hypothermia, (2) ventilatory assistance with total paralysis is mandatory, and (3) TPN can be avoided in 60% of the patients.

We recently observed the development of necrotizing enterocolitis (NEC) in two consecutive newborn infants after gastroschisis repair. Because this association was unexpected, a retrospective review of our 11-year experience was performed using a multivariant computer analysis. The cohort consisted of 54 newborns with gastroschisis. All infants with omphalocele were excluded. Ten of 54 infants (18.5%) developed NEC as defined by classical clinical findings and pneumatosis intestinalis. Twenty-one distinct episodes of NEC occurred with up to three episodes (mean, 2.1) per patient. Twenty of the 21 episodes were successfully treated nonoperatively. Two infants developed pneumoperitoneum, one of whom underwent laparotomy upon which no perforation or intestinal infarction was found. Eight of the ten patients survived--a survival rate no different than for the remainder of the study group. Neither of the two deaths was attributable to NEC. The NEC was atypical in that no significant relationship was established with known predisposing conditions such as prematurity or low Apgar scores. Suspected risk factors such as time of feeding, type of closure, type of formula, total parenteral nutrition (TPN), and composition of TPN were not statistically related. Significant associations included concurrent TPN associated cholestatic liver disease in nine of the ten infants, antecedent intestinal surgery other than abdominal wall closure in five of the ten infants, and delay in initiation of enteral feedings (greater than 30 days) in eight of ten infants. In addition, the NEC occurred significantly later (range, 32 to 79 days; mean, 52 days) in the clinical course than usual; in fact, three of ten infants were rehospitalized with NEC following discharge. We conclude that a relationship exists between NEC and gastroschisis.(ABSTRACT TRUNCATED AT 250 WORDS)

Between 1983 and 1986, four newborns who had primary closure of gastroschisis had postoperative ischemic bowel. Suspicion was raised almost immediately after closure that something was wrong inside the abdomen when there was persistent acidosis, sepsis, abdominal wall redness, and a generalized worsening condition. All four neonates were re-explored. Necrotic bowel was found, and three required silon pouch closure. The two survivors were left with a temporary short gut. Whether the cause of the bowel ischemia in the four babies was due to excessive intraabdominal pressure, volvulus, or the intestines being too vigorously manipulated, is speculative. Therefore, excessive manipulation and compression of gastroschisis contents seem unwise; if such a newborn has persistence of the above signs and symptoms, immediate reoperation and decompression are warranted.

Visceral ischemia secondary to increased intraabdominal pressure (IAP) following closure of abdominal wall defects presents a serious postoperative problem. Currently, the method of closure and postoperative management are determined by clinical impressions rather than measurement of IAP. In this study various methods of indirectly measuring IAP were compared in 17 rabbits in which IAP was sequentially increased with an intraabdominal balloon. Vesical and inferior vena caval (IVC) pressures were found to have good statistical correlation with IAP. Other methods tested were gastric, rectal, superior vena caval, femoral and brachial artery, and rectus compartment pressures. All were found to be poor indicators of actual IAP. In nine of the rabbits, radiolabeled microspheres were used to assess cardiac output and visceral blood flow. Renal blood flow was very sensitive to increased IAP with dramatic impairment at IAP above 10 to 15 mmHg. Small intestinal flow was less sensitive and did not become significantly diminished until IAP exceeded 25 to 30 mmHg. Our studies suggest that vesical and IVC pressure monitoring should be used to evaluate IAP in the clinical setting. If IAP is in excess of 10 to 15 mmHg surgical intervention is indicated to prevent the development of renal ischemia.

Despite prenatal diagnosis, maternal transport and early perinatal treatment in specialized hospitals, improvement in overall mortality has not been observed among newborns with abdominal wall defects (omphalocele and gastroschisis). A comparison has been made, for both anomalies, between 55 cases treated in the periods 1967-1979 and 30 treated in the periods 1980-1985. In this last group 14 prenatally detected cases were separated from cases discovered at birth. Recent advances in treatment have reduced mortality rates for both of these anomalies. This was especially true for gastroschisis; in omphalocele cases, associated abnormalities appeared to be an unavoidable limiting factor in survival. With prenatal diagnosis a surprising increase in mortality rate was observed among omphalocele cases detected before birth. A possible reason for this is the total number of antenatally diagnosed cases that, with maternal transport, are concentrated in centers where surgical facilities are available. Before introduction of antenatal diagnosis, most of these cases would never have been observed by the pediatric surgeon because of death prior to referral or treatment. Gastroschisis cases may benefit more from antenatal recognition and early treatment because multiple abnormalities or immaturity are not so important limiting factors in survival as in omphalocele cases. Reduced trauma and contamination of exposed viscera, immediate treatment and adequate supportive measures may significantly reduce mortality rate in gastroschisis cases. Operative delivery of fetuses with an abdominal wall defect is controversial. The risk of vaginal route delivery has been over emphasized. Intrapartum rupture of an omphalocelic sac was an extremely rare event before introduction of antenatal diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)

The ideal surgical closure for large omphaloceles remains controversial. Most techniques described advocate removal of the amniotic sac prior to repair of the abdominal wall defect. Herein, the authors describe a surgical technique preserving the amniotic sac, which has distinct advantages in achieving safe and rapid closure of the omphalocele by primary or secondary means in selected patients.

Long-term follow-up of children with gastroschisis has been made possible by the increased survival of these infants over the past 20 years. We have observed that children with isolated gastroschisis defects exhibit normal growth and development beyond 5 years of age without significant bowel sequelae. Infants with gastroschisis with concomitant bowel atresia or complications who require small bowel resection are at a greatly increased risk for long-term bowel problems and abdominal complaints.

In 1975, a new Silastic device was developed by the authors to be used in the staged reduction of viscera in newborns with giant gastroschisis or omphalocele. That device and their early experience were described in 1977. This article reports the results of use of this device over the past 10 years. Primary closure of gastroschisis and omphalocele is accomplished, if possible. If tension of the closure is too great, the pneumatic bag is attached and staged reduction accomplished over 7-8 days. The advantages of this device are: it is preformed and easily sterilized by autoclaving; it is strong enough to elevate the abdominal wall; the pneumatic sac eliminates bulky ligatures; when it is pulled through a slotted rod, the edges of the defect are opposed; and it is available in two sizes.

Mortality of patients with gastroschisis has decreased from nearly 90% to 13% (14 of 106) during the period from 1967 to 1984. Coincident with advances in perioperative management, including parenteral nutrition and mechanical ventilation, has been the introduction of staged reduction of the viscera using prosthetic material. To assess the relative merits of primary closure, skin flap coverage, and silo reduction, operative treatment of 106 consecutive infants with gastroschisis was reviewed. Primary fascial closure was accomplished in 54 patients (52%). When fascial approximation resulted in excessive intra-abdominal pressure, the viscera were covered with lateral skin flaps in 10 infants (10%), or the defect was closed after staged reduction with a prosthetic silo in 40 infants (38%). Detailed analysis of the hospital records revealed no significant differences between the primary closure, skin flap, and silo groups with regard to duration of ileus (22 +/- 25, 30 +/- 27, 31 +/- 30 days), length of hospitalization (39 +/- 36, 54 +/- 37, 53 +/- 39 days), or mortality (6, 20, 18%). Respiratory, septic, hemorrhagic, renal, and wound complications occurred in significantly fewer patients with primary closure (36%) and skin flap coverage (30%) than in those with silos (68%) (p less than 0.05). Postoperative mortality was 12% (12/104) and was most often due to respiratory insufficiency (35%) or nonviable small bowel (19%). Primary fascial closure may be accomplished safely in a majority of patients with gastroschisis. However, no single operative strategy is ideal for all patients with gastroschisis, and initial treatment of individual defects should be tailored to the degree of visceroabdominal disproportion.