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Outcome of gastroschisis: a 20-year case review of infants with gastroschisis born in Galveston, Texas.

BACKGROUND/PURPOSE: In the past decade, the preferred method of closure of gastroschisis at our institution has been staged reduction using a silo with repair on an elective basis (SR) rather than primary surgical closure (PC). We performed a 20-year case review of infants with gastroschisis at a university hospital to compare these shifts in management and to determine factors affecting outcome. METHODS: Seventy-two cases were reviewed from 1983 to 2003. Times to first and full feeds were outcome variables for statistical analysis. RESULTS: The prevalence of gastroschisis increased from 0.03% to 0.1% since 1983. Patients had low birth weights (mean = 2294 g) and were borderline premature (mean = 35.8 weeks). Only 3% of the infants were African American. There was a high rate of cesarean deliveries (57%). Ten patients (15%) had gastroschisis complicated by liver herniation, intestinal atresia(s), and/or necrosis/perforation. Most patients were managed by SR (67%). Eight percent of the infants died, 9% developed necrotizing enterocolitis, and 50% had other gastrointestinal complications. Twenty-seven percent of the infants managed with SR did not need initial mechanical ventilation. However, the patients who underwent SR were ventilated longer after birth as compared with those who underwent PC (P < .08). Infants with a complicated gastroschisis had significantly longer times to first and full feeds (P < .001). Patients managed with SR took significantly longer to reach full feeds (P = .001), and there was a trend of starting feeds later (P = .06). When patients with a complicated gastroschisis were excluded, the differences between the SR and PC groups were even greater (P = .01; P < .001). CONCLUSIONS: In our patient population, the prevalence of gastroschisis increased by more than 400% since 1983. The defect was rare in African-American infants. Management by SR was associated with longer ventilation times and longer times to first and full feeds for both uncomplicated and complicated gastroschisis cases.

Digestive System Surgical Procedures↗

Monozygotic twins discordant for gastroschisis: case report and review of the literature of twins and familial occurrence of gastroschisis.

We describe a pair of monozygotic (MZ) female twins discordant for gastrochisis. To our knowledge, this is the first such case reported. The zygosity was verified by DNA analysis using highly polymorphic microsatellites. There was no family history of gastroschisis. During pregnancy there was no suspicion of any exposure responsible for the malformation. The number of twin cases described so far does not allow any conclusion as to hereditary factors in the cause of gastroschisis, but the number of families reported with familial gastroschisis suggests that the recurrence risk is higher than previously thought.

Abdominal Muscles↗

Effects of amnio-allantoic fluid exchange on bowel contractility in chick embryos with gastroschisis.

BACKGROUND/PURPOSE: Intestinal damage in patients with gastroschisis is characterized by bowel wall thickening, intestinal dilatation, mesenteric shortening, and a fibrous peel. The prevention of intestinal damage in gastroschisis by amnio-allantoic fluid (AAF) exchange has been reported using histologic and macroscopic evaluation of intestines, but the effects of this treatment on bowel contractility have not been investigated. The current study was performed to determine the effect of AAF exchange on the intestinal contractility in chick embryos with gastroschisis. METHODS: Thirteen-day-old fertilized chick eggs were used. Gastroschisis was created through amnio-allantoic cavity. There were 3 study groups: control group, gastroschisis-only group, and gastroschisis-plus-exchange group. The bowels were evaluated by an in vitro muscle strip technique, and the response was expressed as a percentage of the maximum acetylcholine evoked contraction (E(max)) in each tissue obtained. Additionally, parasympathetic ganglion cells per 10 plexus at the intestinal wall were counted. Differences between groups were analyzed by analysis of variance (ANOVA) followed by Tukey-Kramer. Probabilities of less than 5% were considered significant. RESULTS: The intestines were thickened and covered by fibrous peel in the gastroschisis-only group when compared with the control group and the gastroschisis exchange group morphologically. There was a statistically significant decrease in contractility in the gastroschisis-only group compared with the control group (P <.05). It exerted 42.03 +/- 46.73% contraction of control group's E(max). This decrease in contractility was significantly reversed in the exchange group (P <.05; E(max) value of gastroschisis plus exchange group was 71.45 +/- 23.54% of control group's E(max)). Although the number of ganglia per 10 plexus was 76.7 +/- 4.3 in the control group, it was measured 28% less in the gastroschisis-only group (P <.05). There was no significant difference between the ganglion numbers of control and exchange groups. CONCLUSIONS: Prenatal AAF exchange treatment prevents decreased bowel contractility in gastroschisis. Gastroschisis does not affect intestinal ganglia morphology, but the number of ganglion cells decreases. AAF exchange prevents these functional and morphologic adverse effects of disease. By these findings the expectancy of a better clinical result in gastroschisis with intrauterine pretreatment by amniotic fluid exchange increases.

Acetylcholine↗

Nitric oxide synthase inhibition prevents intestinal damage in gastroschisis: a morphological evaluation in chick embryos.

PURPOSE: Increased small bowel nitric oxide (NO) synthase activity accused for postnatal intestinal dysmotility in gastroschisis. The purpose of this study is to evaluate the effect of prenatal NO synthase inhibition on intestinal damage in gastroschisis. METHODS: Sixteen-day-old fertilized chick eggs were divided into 4 groups. In the control group, the allantoic and amniotic membranes were opened to create a common cavity. In the gastroschisis group, a defect in the abdominal wall was made, and intestinal loops were exteriorized. In the gastroschisis pretreated with L-NAME group, gastroschisis was created, and L-NAME was administered into the amnioallantoic cavity for 4 days. In the gastroschisis sham pretreated group, after the same surgical procedure as the previous group, same amount of saline was given beside L-NAME. At the end of 20th day of incubation, intestinal morphological changes were investigated macroscopically and microscopically. RESULTS: Macroscopic changes such as shortening, thickening, and fibrous adhesions were found in the exteriorized bowels of the just gastroschisis group and the gastroschisis pretreated saline group. However, there was only mild thickening in the gastroschisis pretreated with L-NAME group. Microscopically, compared with the gastroschisis group, serosal thickness, muscular thickness, and bowel wall thickness were found to be significantly lower in the gastroschisis pretreated with L-NAME group (128.0 +/- 19.3 microm and 239.5 +/- 3.0 microm v 57.0 +/- 8.2 microm and 145.0 +/- 9.7 microm). CONCLUSION: It is possible to decrease intrauterine intestinal morphological changes in gastroschisis by inhibiting NO synthase.

Animals↗

Alterations of enteric nerve plexus in experimental gastroschisis: is there a delay in the maturation?

BACKGROUND/PURPOSE: After surgical correction of gastroschisis, intestinal transitory hypoperistalsis usually occurs. Long-term parenteral nutrition often is necessary leading to a higher morbidity associated with this malformation. The etiology of this transitory intestinal hypomotility is unknown. It may be caused by a reversible inflammatory process in the intestinal wall or other causes, including an alteration of the maturation of intestinal neural plexus, because the disturbance disappears spontaneously after a variable period. The aim of this work was to study the neuronal cells of the myenteric plexus of the fetal intestine in experimental gastroschisis. The main hypothesis was that the transitory intestinal dismotility seen in gastroschisis could be secondary to alteration in the maturation of the enteric nervous plexus. METHODS: Twenty-seven time-mated rabbits, on gestational day 25, were submitted to a midline laparotomy; the gravid bicornuate uterus was exposed and opened, and the more distal fetuses relative to the vaginal opening had the abdominal wall opened by a small incision to produce gastroschisis (n = 29). The fetuses not submitted to gastroschisis were used as controls (n = 12). The amniotic fluid was carefully aspirated from the opened uterus and saved for later repositions. On gestational day 30, the does were again submitted to general anesthesia, and the fetuses were delivered by cesarean section. The fetal intestine was removed, the adjacent mesentery excised, and intestinal specimens were harvested for histologic studies. The specimens were stained for acetyl-cholinesterase activity (AChE) to assess the maturity of the nervous enteric cells and for lactate dehydrogenase (LDH) that identify specifically immature nervous cells. The histologic sections stained by LDH were submitted to histomorphometric analysis of the nervous cells through an image system analysis (Kontron 300). The results were submitted to statistical analyses (P <.05). RESULTS: Macroscopic alterations of the fetal gastroschisis intestine are similar to the human findings: shortening of the intestine, intestinal wall thickening, and a hypertrophied muscular layer. In the gastroschisis group, histologic AChE activity was decreased in comparison with control intestines. The histomorphometric assessment in slices stained with LDH, which identify immature nervous cells, showed that the neuronal intestinal cells of the gastroschisis group were significantly smaller and more numerous relative to the control group. CONCLUSIONS: There were significant differences in the nervous plexus of the intestine of fetuses with gastroschisis relative to the controls. The observed morphologic changes may be caused by alteration in the maturation of the intestinal neuronal in gastroschisis. This alteration may explain the transitory intestinal hypomotility observed in infants after surgical correction of gastroschisis.

Animals↗

Comparison of trends in gastroschisis and prenatal illicit drug use rates.

Gastroschisis rates have been increasing over time, and gastroschisis risk is elevated in women of young maternal age. Similar patterns were reported for prenatal illicit drug use in Hawaii. Cases of gastroschisis and prenatal use of methamphetamine, cocaine, or marijuana among deliveries in Hawaii during 1986-2002 were identified from a common registry. The pattern of gastroschisis rates by maternal age and time period was compared to the pattern of prenatal illicit drug use rates. The prenatal marijuana use rate among gastroschisis cases was significantly greater than among the total population. Prenatal methamphetamine and cocaine rates were not significantly elevated among gastroschisis cases. The gastroschisis rate was highest among the youngest maternal age group, then declined with increasing maternal age. Only prenatal marijuana use demonstrated a similar pattern, but the ratio of the gastroschisis rate to the prenatal marijuana use rate declined with increasing maternal age. The gastroschisis rate increased during the 17-yr period. Both methamphetamine and marijuana demonstrated similar annual trends; however, the ratio of the gastroschisis rates to the illicit drug use rates varied greatly for the 2 drugs over time. The results of this investigation suggest that prenatal illicit drug use of methamphetamine, cocaine, and marijuana may not play a major role in explaining the increased risk of gastroschisis among younger mothers and the annual increases that have been reported.

Age Factors↗

[An epidemiological investigation on gastroschisis in China during 1996 to 2000].

OBJECTIVE: To study the epidemiological characteristics of gastroschisis in China from 1996 to 2000. METHODS: The birth defects monitoring program was undertaken in 450-466 hospitals from 31 provinces, cities and autonomous regions of China. Data about births including intrauterine death and stillbirth of 28 weeks of gestation was collected to report through the medical records over the time period 1996 to 2000. The maximal diagnosis time was up to 1 week after the delivery. The international Classification of Diseases of Ninth Revision with some modifications was used to code the birth defects. RESULTS: A total of 569 cases with gastroschisis was found in 2,218,616 births monitored. The overall birth incidence of gastroschisis was 2.56 per 10,000 births during the monitored period. The increasing trend of incidence of gastroschisis hadn't been found (P > 0.05). The birth incidence of gastroschisis in rural areas was significant higher than that in urban areas (3.93 vs.1.98 per 10,000, P < 0.01). There was also significantly difference on birth incidence between female and male (2.64 vs. 2.15 per 10,000 births, P < 0.05). The birth incidence of gastroschisis in the mothers less than 20 years old was the highest among all mothers age, which were at 5.4 times increased risk relative to that of mother age of 25-29 years old. The overall perinatal mortality rate of gastroschisis was 74.69 percent, and 41.46 percent of gastroschisis were diagnosed before birth. 27.44 percent of cases were accompanied with other major defects. CONCLUSIONS: There was no increasing trend on the incidence of gastroschisis to be found in China during 1996 to 2000. The mother age less than 20 years old was at an increasing risk of fetal gastroschisis. The cases with gastroschisis had a high perinatal mortality rate.

Adult↗

Omphalocele and gastroschisis in Taiwan.

UNLABELLED: The purpose of this study was to analyse the clinical differences between omphalocele and gastroschisis in Taiwan, with special reference to associated anomalies and outcomes. A retrospective review was conducted of 115 cases seen between January 1990 and June 2000 at two tertiary medical centres in Taiwan. Data included perinatal events and associated anomalies. Of 115 patients, 65 were classified as having gastroschisis and 50 as having omphalocele. Other anomalies were found in 24 omphalocele cases, compared with 23 gastroschisis cases. The range of anomalies associated with omphalocele varied more widely than in the gastroschisis cohort. Of patients with omphalocele and associated anomalies, six had chromosomal abnormalities compared with none of the patients with gastroschisis. In patients with gastroschisis and additional malformations, 17 had gastrointestinal anomalies, the most common of which was intestinal malrotation. A comparison of perinatal data revealed that infants with gastroschisis were more likely to be small for gestational age. Gastroschisis was associated with a younger overall maternal age than omphalocele and a lower birth weight. There was a male predominance among omphalocele patients, but this did not reach statistical significance. CONCLUSION: Gastroschisis was frequently associated with intestinal anomalies and transient dysfunction, and outcomes were related to postoperative complications. Infants with gastroschisis more frequently required prolonged parenteral nutrition supplement, resulting in longer hospital stay. Omphalocele was often accompanied by chromosomal disorders leading to early neonatal death, so we recommend that amniocentesis should be indicated if omphalocele is suspected on fetal ultrasonography.

Abdominal Wall↗

The changing face of gastroschisis and omphalocele in southeast Georgia.

OBJECTIVE: To document trends in the clinical characteristics of gastroschisis and omphalocele in southeast Georgia, USA, from 1994 to 2002. METHODS: All babies with an abdominal wall defect in a 19-county region were referred to one Perinatal Center for genetic counseling, level II ultrasound scans, pregnancy follow-up and delivery. Karyotyping was offered for omphalocele, advanced maternal age, family history predisposing to aneuploidy, and gastroschisis with an additional anomaly. RESULTS: There were 64 patients, 34 with gastroschisis and 30 with omphalocele. From 1994 to 2002, the birth prevalence of gastroschisis was 1:3600 and omphalocele 1:3400, but from 2000 to 2002, gastroschisis increased to 1:1667, while omphalocele increased to only 1:2709. Gender distribution was different: for gastroschisis the M:F ratio was 1:2.1; for omphalocele the ratio was 1.7:1. In the patients with omphalocele, 90% had an amniocentesis and 9/27 were aneuploid: five had trisomy 18, three had trisomy 13 and one had trisomy 21. Seventy-six per cent of the patients with omphalocele had associated anomalies, but only 17.6% of those with gastroschisis. Mothers whose babies had gastroschisis showed a trend to progressively younger age, while no such trend was observed among mothers whose babies had omphalocele. CONCLUSION: The birth prevalence of abdominal wall defects in general is increasing, but more notably for gastroschisis. Maternal age continues to decrease for gastroschisis. In the study population, gender distribution showed a statistically significant variation between the defects.

Adult↗

Epidemiology of gastroschisis in metropolitan Atlanta, 1968 through 2000.

BACKGROUND: An increase in the rate of gastroschisis has been documented by birth defects surveillance systems in the United States and in other countries. This study sought to evaluate historical trends in the rate of gastroschisis in Atlanta, Georgia, and to describe the epidemiology of gastroschisis over 33 years. METHODS: Gastroschisis cases were identified through the Metropolitan Atlanta Congenital Defects Program (MACDP) from 1968 through 2000. Poisson regression techniques were used to evaluate trends over time. Data on covariates were compared for three maternal age groups (< or =19, 20-24, and > or =25 years). RESULTS: From 1968 through 1975, the rate of gastroschisis was stable at 0.8 per 10,000 births. After 1975, the rate of gastroschisis was 2.3 per 10,000 births with no significant increase observed from 1976 through 2000. The rate of gastroschisis was six times higher among teenage mothers compared with mothers > or =25 years of age. Affected infants born to teenage mothers were less likely to be born to Black mothers compared to White mothers (rate ratio [RR], 0.4; 95% confidence interval [CI], 0.2-0.6). This was also true for mothers 20-24 years of age (RR, 0.5; 95% CI, 0.3-0.8) but not for mothers 25 years of age or older (RR, 1.6; 95% CI, 0.9-2.7). CONCLUSIONS: An increase in the rate of gastroschisis was observed in the mid-1970s, but no temporal trend has been observed since that time. In light of recent reports of an increasing prevalence of gastroschisis in the United States, continued monitoring of this birth defect is warranted.

Adult↗

The effect on the intestines of continuous release of methylene blue from a drug delivery system: an experimental study in a chick embryo gastroschisis model.

Increased small bowel nitric oxide synthase (NOS) activity has been suspected as a cause of postnatal intestinal dysmotility in gastroschisis. The effect of continuous delivery of methylene blue loaded polymer (MBLP) hydroxy-propyl methyl cellulose-ethyl cellulose (HPEC-MC) and daily injection of methylene blue (MB) on the intestinal damage (ID) was evaluated using a chick embryo gastroschisis model. Fourteen-day-old fertilized chick eggs were divided into five groups. In the control (C) group, no intervention was performed. In the sham (S) group, the allantoic and amniotic membranes were opened to create a common cavity that resembles the amniotic cavity in human. In the gastroschisis only (GO) group, a defect in the abdominal wall of the embryo was made, and intestinal loops were exteriorized following connection of amniotic and allantoic cavities. In the gastroschisis plus methylene blue (G+MB) group, gastroschisis was created and MB administered into the amnioallantoic cavity (AAC) by daily injections for 5 days. In the gastroschisis plus methylene blue loaded polymer (G+MBLP) group, MBLP was placed into AAC after gastroschisis was created. At the end of the 19th day of incubation, intestinal morphological changes were investigated macroscopically and microscopically. Although the survival rates were decreased in the chick embryos with creation of gastroschisis compared with C and S groups ( p<0.001), the survival rates were increased in G+MBLP group (76.92%) when compared with the GO group (41%) ( p<0.001). Because of multiple intervention of embryos, higher mortality was observed in the G-MB group (75.61%). Macroscopic and microscopic scores of ID and mean intestinal wall thickness were significantly higher in the GO group when compared with C, S, G+MB, and G+MBLP groups ( p<0.001). The mean score of intestinal ganglia morphology was significantly increased and the total number of ganglion cells was significantly decreased in the GO group when compared with C, S, G+MB, and G+MBLP groups ( p<0.001). It is possible to decrease intrauterine intestinal morphological changes in gastroschisis by inhibiting NOS. As a first preliminary study, we believe that use of MBLP may be an alternative for fetal treatment by eliminating the harmful effects of multiple interventions or amniotic fluid exchanges.

Administration, Topical↗

Risk factors in congenital abdominal wall defects (omphalocele and gastroschisi): a study in a series of 265,858 consecutive births.

The aim of this study was to describe the prevalence at birth of two abdominal wall defects (AWD), omphalocela and gastroschisis and to identify possible etiologic factors. The AWD came from 265,858 consecutive births of known ouome registered in the registry of congenital malformations of Strasbourg for the period 1979 to 1998. Request information on the child, the pregnancy, the parents and the family was obtained for cases and for controls. Hundred five cases with AWD were analysed, 55.2 % were omphalocele and 44.8 % were gastroschisis. The mean prevalence rate for omphalocele was 2.18 per 10,000 and for gastroschisis 1.76 per 10,000. Associated malformations were found in 74.1 % of omphalocele compared with 53.2 % of gastroschisis; 29.3 % of fetuses with omphalocele had an abnormal karyotype, 44,8 % had a recognizable syndrome, association or an unspecified malformation pattern; 51.0 % of fetuses with gastroschisis had additional malformations that were not of chromosomal origin, but 1 case. Antenatal ultrasound examination was able to detect 39 (67.2 %) cases of omphaloceles and 27 (57.4 %) cases of gastroschisis. In 30 (51.7 %) cases of omphalocele and in 7 (14.9 %) cases of gastroschisis parents opted for termination of pregnancy. The overall survival rate was 14 (24.1 %) for omphalocele and 30 (63.8 %) for gastroschisis. Weight, length and head circumference at birth of infants with AWD were less than those of controls. The weight of placenta of infants with AWD was not different from the weight of placenta of controls. Gastroschisis was associated with significantly younger maternal age than omphalocele. Pregnancies with AWD were more often complicated by threatened abortion, oligohydramnios and polyhydramnios. Mothers of children with AWD took more often medication during pregnancy than mothers of controls.

Abdominal Muscles↗

Esophageal ligature in experimental gastroschisis.

BACKGROUND/PURPOSE: Recently, the authors have shown that in human fetuses suffering from gastroschisis, there is an amniotic fluid inflammatory response and that amniotic fluid exchange designed to disrupt the inflammatory loop seems to have a favorable impact on outcome. The authors, therefore, designed in the fetal sheep a model of gastroschisis in which amnioinfusion significantly improved the deleterious process. They hypothesized that regurgitation and presence of digestive enzyme in the amniotic fluid triggers and maintains the process of inflammation. METHODS: To test this hypothesis, the authors used their model of gastroschisis in the fetal lamb combined with esophageal ligation and compared it with gastroschisis with or without amnioinfusion. RESULTS: Of 34 fetuses operated on at midgestation (days 70 through 80), 11 died in utero or were stillborn, 8 had gastroschisis and amnioinfusion, 8 had gastroschisis and no amnioinfusion, and 7 had gastroschisis and esophageal ligation. There were 9 control fetuses. Fetuses were killed at day 145 by cesarean section. Extraabdominal bowels with fibrous peel were processed for histologic examination. Thickness of bowel muscularis (micrometers) was 82.7 +/- 19 for controls, 159 +/- 56 for the nonamnioinfused fetuses, 126 +/- 21 for the amnioinfused fetuses (P =.001), and 240 +/- 225.8 for fetuses with esophageal ligature combined with gastroschisis. The same results were obtained for thickness of serous fibrosis and plasma cell infiltration. Assay of amniotic fluid ferritin, lipase, and protein showed that only amnioinfusion lowered ferritin and protein to levels similar to those of controls, thus, illustrating its preventive effect on inflammation and that esophageal ligature did not prevent digestive enzyme presence in the amniotic fluid. CONCLUSION: In this model of gastroschisis in the fetal sheep, ligature of the esophagus, which was supposed to protect the extruded bowel by preventing oral regurgitation of digestive enzymes and by creating a relative hydramnios, did not improve the inflammatory and deleterious process, which is best prevented by amnioinfusion.

Amniotic Fluid↗

Omphalocele and gastroschisis: an 18-year review study.

PURPOSE: Using data from a single University Genetics Division practice, the authors sought to determine if gastroschisis is increasing in occurrence compared to omphalocele. Associated abnormalities were also examined. In addition, prenatal exposure to teratogens and other parameters were compared. METHODS: All fetal and infant cases of omphalocele and gastroschisis seen in the University of South Florida Division of Genetics between January 2, 1982 and December 31, 1999 were retrieved through the database and analyzed through chart review. RESULTS: There were 127 cases of omphalocele and 121 cases of gastroschisis. Ninety-three of the 127 probands with omphalocele had karyotype determinations and 20% were abnormal; for gastroschisis, 37 had cytogenetic studies and none had chromosomal anomalies. Seventy-six percent of the probands with omphalocele had associated abnormalities; twenty-three percent of the probands with gastroschisis, none of which were syndromic, had associated anomalies. The prematurity rate for omphalocele was 42% and mortality was 22%; for gastroschisis, the figures were 57% and 9%, respectively. There were no significant differences in teratogen exposure between the two groups. Familial cases of both defects occurred in < 4%, and the prevalence of dizygous twinning was increased in both groups. Mean maternal age was 21.8 years for the gastroschisis group, 27.2 years for the omphalocele group. CONCLUSIONS: In this study, the number of cases of omphalocele and gastroschisis were similar, compared to the expected 3:2 ratio, suggesting an increase in the occurrence of gastroschisis. Cases with omphalocele had more syndromic and nonsyndromic anomalies, more chromosomal anomalies, a higher mortality rate, and older mothers.

Adolescent↗

[Clinical analysis of congenital abdominal wall defects--omphalocele and gastroschisis].

INTRODUCTION: This is a retrospective analysis of therapeutic approaches and treatment outcomes of congenital abdominal wall defects (omphalocele and gastroschisis) in a five year period. The aim of this study was to identify factors which can affect the prognosis of future therapeutic procedures. MATERIAL AND METHODS: We evaluated 13 children, 7 with omphalocele (2 female/5 male; mean birth weight of 2862 g; mean gestational age of 37 weeks), and 6 patients with gastroschisis (2 female/4 male; mean birth weight of 2640 g; mean gestational age of 36/2 weeks). All patients were treated at the Clinic of Pediatric Surgery from 1999 to 2003. RESULTS: In this study, thirteen cases of congenital abdominal wall defects (omphalocele and gastroschisis) were retrospectively investigated. All patients underwent prenatal ultrasound. Omphalocele was prenatally detected in 42.8% of fetuses, and gastroschisis in 16.7%. Coexisting anomalies were present in 57.1% of patients with omphalocele and in 16.7% of newborns with gastroschisis. Three patients with omphalocele were treated operatively, and four only conservatively. The abdominal wall of patients with gastroschisis was primarily closed in three patients. Two patients required a staged abdominal wall closure. One patient with gastroschisis and intestinal atresia underwent primary closure after partial intestinal resection and enterostomy. Based on these responses, a management protocol (algorithm) was recommended. The most common postoperative complication, in 7 cases of omphalocele, was mechanical ileus (n = 1), whereas among patients with gastroschisis the commonest were mechanical ileus (n = 1) and intestinal perforation (n = 1). The mortality of patients with omphalocele was 52% and with gastroschisis 66.7%. CONCLUSION: A strategy designed to optimize antenatal and neonatal factors is expected to increase the survival rate of patients with abdominal wall defects.

Abnormalities, Multiple↗

Is the incidence of gastroschisis rising in South Africa in accordance with international trends? A retrospective analysis at Pretoria Academic and Kalafong Hospitals, 1981-2001.

OBJECTIVES: Analysis of the incidence of gastroschisis compared with the incidence of exomphalos as a percentage of total paediatric surgical admissions. DESIGN: Retrospective observational analysis using data from the ward admissions registers of the paediatric surgical wards of Pretoria Academic and Kalafong hospitals and from the weekly statistics sheets and audits thereof of the Paediatric Surgery Department at Pretoria Academic Hospital. Patient files from Pretoria Academic were used where available to confirm data. SETTING: Pretoria Academic Hospital (PAH) and Kalafong Hospital (KH), Pretoria, from March 1981 to December 2001, excluding Kalafong Hospital data from February 1984 to 12 June 1984 and for 2001. RESULTS: Forty-eight cases of gastroschisis and 139 cases of exomphalos were seen at PAH and KH out of 21 495 total paediatric surgery ward admissions. The average incidence of gastroschisis increased 35-fold from the 7-year period 1981 - 1987 to the 7-year period 1995 - 2001, while the average incidence of exomphalos compared across the same periods only showed a 1.82-fold increase. The incidence of gastroschisis rose above that of exomphalos in 1999 and remained so through to 2001. The incidence of exomphalos showed a general rising trend from 1981 to 2001 except for a sudden unexplained increase in 1995 and 1996. Gastroschisis presenting at PAH was associated with a mortality rate of at least 38.7%. Reasons for this high mortality rate cannot be adequately evaluated owing to poor record keeping. CONCLUSIONS: The incidence of gastroschisis presenting at PAH and KH has risen dramatically relative to the incidence of the macroscopically similar exomphalos over the period 1981 - 2001, with the incidence of gastroschisis rising above the incidence of exomphalos. A larger scale study looking at birth prevalence of gastroschisis and exomphalos in South Africa is necessary to determine whether this represents a true differential change in the incidence of gastroschisis, reflecting internationally observed increases in the birth prevalence of this defect, or whether it is merely due to logistical changes within the administration of the Department of Health serving the area.

Gastroschisis↗

Gastroschisis increases small bowel nitric oxide synthase activity.

In gastroschisis, the eviscerated fetal bowel frequently is damaged and this results in hypoperistalsis and malabsorption. The mechanistic link that ties gastroschisis-induced intestinal damage to dysfunction may be nitric oxide (NO) and the enzyme responsible for producing it, NO synthase. Using a fetal rabbit model, the authors investigated the hypothesis that the hypoperistalsis and malabsorption associated with gastroschisis may be attributable to abnormal small bowel NO synthase activity. Using the 3H-arginine-to-3H-citrulline conversion assay, they measured NO synthase activity in the small bowel of full-term fetal rabbits with and without gastroschisis. The mean total small bowel NO synthase activity of fetal rabbits with gastroschisis was 2.5 times greater than that of control littermates without gastroschisis (n = 6; 5,726 +/- 834 v 2,208 +/- 537 mean pmol/mg protein/min; P = .004). This increased NO synthase activity also was studied by measuring the individual isoforms of NO synthase, and the site of increased NO synthase activity was localized to the small bowel epithelium and neurons. After detecting and localizing the gastroschisis-induced increase in NO synthase activity, the authors explored the mechanism of this increase using NADPH-diaphorase staining. With this histological staining technique, no quantitative increase was found in the small bowel NO synthase of the rabbits with gastroschisis. This suggests that the increased NO synthase activity found in these rabbits is the result of accelerated enzyme kinetics. These findings suggest that the increased NO synthase activity caused by gastroschisis may contribute to the common clinical sequelae of malabsorption and intestinal dysmotility.

Animals↗

Association between mothers' nutrient intake and their offspring's risk of gastroschisis.

The young age of mothers of infants with gastroschisis, a congenital defect of the abdominal wall, suggested that deficient nutrition, with maternal-fetal competition for nutrients, could be a risk factor for gastroschisis. This population-based hypothesis-generating study consisted of 55 cases of gastroschisis and 182 matched controls. We assessed maternal nutrient intake during the trimester before conception with a self-reported food-frequency questionnaire and screened 38 nutrients to identify those most likely to be associated with gastroschisis. We used statistical classification trees to empirically generate cutpoints that determined the low and high levels of nutrient intakes corresponding to the risk of gastroschisis; cutpoints for most nutrients were similar to the corresponding recommended daily dietary allowances (RDAs). In univariate analysis, low intake of several nutrients emerged as the leading risk factors: carotenoids, e.g., alpha-carotene (odds ratio (OR) = 4.6; 95% confidence interval (CI) = 2.2-9.5), beta-carotene (OR = 3.1; 95% CI = 1.6-6.0); amino-acid compounds, e.g., total glutathione (OR = 3.5; 95% CI = 1.7-7.2); vitamin C (OR = 2.2; 95% CI = 1.5-7.8); vitamin E (OR = 2.3; 95% CI = 1.2-4.4); and minerals, fiber, and the fruit-and-vegetable group (OR = 3.1; 95% CI = 1.5-6.2). High intake of nitrosamines (OR = 2.4; 95% CI = 1.3-4.5) was also a good candidate. Many nutrient values were correlated and, in multivariate analysis, those most associated with gastroschisis were low alpha-carotene (OR = 4.3; 95% CI = 1.9-9.8), low total glutathione (OR = 3.3; 95% CI = 1.4-7.6), and high nitrosamines (OR = 2.6; 95% CI = 1.3-5.4). Adjusting for variables associated with gastroschisis in previous analyses of this population did not substantially alter those risks. These results suggest that maternal dietary inadequacy may be a risk factor for gastroschisis, and the three nutrients that emerged from the nutrient screening appear to be the best candidates to examine in further epidemiological analyses or biological studies.

Carotenoids↗