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The prenatal diagnosis of laparoschisis may be established by ultrasound early in the end of the first pregnancy trimester. The visualisation of the abdominal wall defect may predict a good fetal prognosis in the absence of the other anomalies and may establish the most favorable moment of birth. Identification of several risk factors for this malformation could lead to the decrease of its incidence.

A 28-year-old woman was referred at 33 weeks of gestation with suspected fetal intestinal atresia. Sonography showed a large extra-abdominal mass on the right of the normal umbilical cord insertion. Following Cesarean section at 36 weeks and immediate surgical treatment, the malformation was not definable either as an omphalocele or as gastroschisis. This reported case involves a previously undocumented malformation of the fetal abdominal wall described as a 'hernia' of the fetal abdominal wall.

Tension and infection often cause wound dehiscence or incisional herniation after the fascial closure of congenital abdominal wall defects in neonates. To overcome these problems, a modification of the Boerema technique (a method for repairing large incisional hernia in adults) was applied to repair abdominal wall defects in 14 neonates. The efficacy of this technique is discussed in this report.

Gastroschisis is a congenital anomaly in which exposure of the intestines to amniotic fluid throughout fetal life results in nutrient malabsorption. To begin to understand the molecular basis underlying epithelial changes in this condition, we investigated enterocytic gene expression during fetal development. Gastroschisis was surgically created at 24 days gestation (term = 31 days) in fetal rabbits; sham-operated and unoperated fetuses served as controls. Bowel was harvested at 28 and 31 days gestation. Cellular lactase expression was detected using immunohistochemistry, and apolipoprotein A-I and cellular retinol binding protein II (CRBPII) mRNA levels were quantitated using Northern blot analysis. Despite absence of gross histological changes in the mucosa, lactase protein expression and apolipoprotein A-I and CRBPII mRNA expression were decreased in intestine from gastroschisis compared to sham-operated animals. Expression of GAPDH (a housekeeping gene) increased over the same period, suggesting that the changes in enterocytic absorptive gene expression associated with gastroschisis were relatively specific. In conclusion, a decrease in expression of a variety of genes involved in nutrient absorption and trafficking within the enterocyte may contribute to the absorptive defects seen in this gastroschisis.

Congenital spigelian hernia (SH) is a rarity. The authors present two cases of congenital SH with undescended testis, and examine the significance of the concurrence of these events.

Background: Gastroschisis is an uncommon condition in which viscera protrude at the base of the umbilical cord. To investigate the possible relationships between antenatal ultrasound findings, patient demographics, smoking, alcohol consumption and this condition, 21 causes are reviewed., Methods: The medical records and antenatal ultrasounds of 21 children diagnosed with gastroschisis at the Adelaide Children's Hospital between 1 January 1985 and 31 December 1992 were reviewed., Results: Antenatal ultrasound was employed in 15 cases, and the diagnosis was accurately made in 13 (86.7%) of these. There were 17 live births, two elective terminations and two pre-term abortions. Seven of the 21 cases had associated anomalies. The anomalies included five atresias, a ventricular septal defect (VSD), and a dislocated gall-bladder. Postoperative complications (which included one death) occurred in seven of the 17 patients. Bowel dilatation or thickening was first detected on five ultrasound examinations performed before 21 weeks' gestation, and four ultrasounds after 21 weeks. The nine cases with bowel changes on ultrasound were associated with a high atresia rate and a longer hospital stay, but not with an increased complication rate. Maternal race, parity, and alcohol consumption were not associated with increased risk of fetal gastroschisis. All mothers were under 27 years of age. There were nine mothers who smoked during pregnancy and a disproportionate number of mothers who lived outside the metropolitan area with gastroschisis-affected offspring., Conclusions: Bowel changes seen on antenatal ultrasound increase the chances of intestinal atresia and longer hospitalization. Smoking during pregnancy may be associated with an increased risk of gastroschisis.

Fifty cases (24 males, 26 female) of gastroschisis were admitted to the Regional Neonatal Surgical Unit at the Royal Hospital for Sick Children in Yorkhill, Glasgow, between January 1983 and October 1993. The mean birthweight was 2.17 kgs and mean gestation was 35.8 weeks. Prenatal diagnosis was made by ultrasound scan in 39 cases (78%) and 33 (66%) were delivered in the Regional Obstetric Unit and Fetal Medicine Centre at the Queen Mother's Hospital, Yorkhill, Glasgow, immediately adjacent to the neonatal surgical unit. Spontaneous vaginal delivery occurred in 23 (46%) but Caesarean section was performed in 27 (54%). Sixteen Caesarean sections were emergencies and 11 elective (5 on account of dilated bowel loops). Associated bowel problems were noted in 11 (22%)--atresia (5), stenosis (2), ischaemia (1), duodenal perforation (1), ileal perforation (1) and jejunal band obstruction (1). Primary closure was achieved in 42 (84%) and there were 5 deaths (10%). Prenatal diagnosis and mode of delivery showed no direct correlation with mortality (10%) in this series. Emergency Caesarean section was necessary for fetal indications in 28%. The prediction of compromised or damaged intestine by prenatal ultrasound was unreliable and the majority of cases of atresia and stenosis escaped detection. However, it is concluded that prenatal diagnosis and delivery in a regional centre offers the best outcome for the fetus. Further study is needed to define "at risk" pregnancies and fetuses and to determine the role of intervention.

The data on the first experience in the use of synthetic resolvable materials in plasty of the anterior abdominal wall in children are reported. Tissue glue compositions based on alpha-cyanoacrylates as well as connective biocompatible elements (CBE) developed at the SRIIMT were used. An aponeurotic suture-glue duplication was utilized for plastic enforcement of the anterior abdominal wall; individual methods were developed for each concrete localization of the defects. Operations were carried out on 201 children with congenital abnormality of the anterior abdominal wall: inguinal hernias, hernias of the linea alba, ventral hernias. Ninety-six (47.8%) children were examined in late postoperative periods of 2 to 9 years. Recurrences were not encountered. No inflammatory complications were found. It is concluded that the use of Russian synthetic resolvable materials for surgical treatment of pathological conditions of the abdominal wall in children is effective and safe.

Gastroschisis, an abdominal wall defect, most often occurs in infants of young mothers. To identify risk factors for gastroschisis, we conducted a case-control study in the population surveyed by the California Birth Defects Monitoring Program (CBDMP). From structured questionnaire data, we compared sociodemographic, reproductive, and lifestyle factors for 110 mothers of infants with gastroschisis with those for 220 age-matched mothers of normal infants. Univariate matched-pair analysis showed significant associations of gastroschisis with mother's education, yearly family income, marital status, a history of mother's mother smoking, mother's father's absence from home during the mother's youth, more than one elective abortion, a short interval between menarche and first pregnancy, siblings from different fathers, and use of either a recreational drug (either cocaine, amphetamine, marijuana, or LSD), alcohol, or tobacco during the trimester preceding pregnancy. For cocaine, amphetamine, and marijuana, use of more than one drug showed a stronger association than single drug use. The association was stronger if both parents used drugs. Although many variables were correlated, odds ratios (OR) were significant (95% confidence intervals) in multivariate conditional logistic analysis for: yearly family income < $10,000 [OR = 4.34 (1.54, 12.22)] or $10,000-$49,999 [OR = 3.93 (1.43, 10.80)]; mother's mother's smoking status not known [OR = 3.99 (1.66, 9.56)]; mother's father's absence from home during her youth [OR = 3.11 (1.14, 8.46)]; and drug use by mother [OR = 2.21 (1.21, 4.03)], father [OR = 1.66 (1.02, 2.69)], or both [OR = 3.05 (1.48, 6.28)]. The best predictive model explained 32% of the deviance. Young, socially disadvantaged women with a history of substance use were at highest risk for a child with a gastroschisis.

In recent years, we have treated two families that had successive siblings with gastroschisis. In the first family, the delivery of their second child, a boy with gastroschisis, was followed 11 months later by the delivery of a girl also with gastroschisis. The mother admitted to use of tobacco, alcohol, and marijuana during both pregnancies; the father was the same for both children. In the other family, the second child was a boy with gastroschisis, and the third, born 6 years later, was also a boy with gastroschisis. The mother denied any use of tobacco, alcohol, or recreational drugs; these two boys had different fathers. Gastroschisis in siblings has been reported only rarely, and has been noted in both identical and dizygotic twins. Our second set of siblings is the first documented case involving paternity by different fathers. Although a genetic factor cannot be excluded completely, teratogenic factors operative through the mother seem most probable. In conjunction with other reports of "epidemics" of gastroschisis, these two families amid our own recent outbreak of gastroschisis strongly suggest that the teratogenic factors may be environmental.

Two infants with gastroschisis had most of the midgut avulsed during cesarean section. There was only 12 and 20 cm of small bowel remaining, with an intact ileocecal valve in each case. Cutaneous enterostomy followed by anastomosis was done in both patients. The first child is 4 years old and is now on total enteral feedings after prolonged intravenous support. The second infant is still on partial parenteral nutrition. The cases illustrate the vulnerability of the fetus with gastroschisis during extraction by cesarean section.

Gastroschisis is considered by the authors to be an embryopathy. The main principles of management of newborns with gastroschisis are: warming of the newborn, rehydration, measures against pseudoobstruction of the intestine. An effective method which could be recommended for all kinds of operations for defects of the anterior abdominal wall is a "manual enlargement of the abdominal cavity" during operation.

The short and thick bowel found in gastroschisis has been attributed to contact with amniotic fluid, among other factors. We designed an original animal model in order to isolate the effect of amniotic fluid on the fetal bowel. We created experimental gastroschisis in fetal rabbits. Extruded bowel was kept out of the amniotic sac in half of the operated fetuses. At term, seven days after surgery, 21 surviving fetuses--12 intra-amniotic (GIA) and nine extra-amniotic (GEA)--and 16 control littermates were harvested for study. Five parameters were measured in each animal: body weight, intestinal weight, intestinal length, bowel diameter and bowel wall thickness. We found no statistically significant difference in body weight, intestinal weight or bowel wall thickness. An enormous difference was found in intestinal length among the three groups. Bowel diameter was significantly different in the extreme groups (GIA and controls), with the intestine being more dilated in GIA than in GEA and controls. Gastroschisis in the absence of amniotic fluid has not been as damaging to the fetal intestine as "natural" intraamniotic gastroschisis.

There has been increasing interest within the European sports medicine community regarding the etiology and treatment of groin pain in the athlete. Groin pain is most commonly caused by musculotendinous strains of the adductors and other muscles crossing the hip joint, but may also be related to abdominal wall abnormalities. Cases may be termed "pubalgia" if physical examination does not reveal inguinal hernia and there is an absence of other etiology for groin pain. We present nine cases of patients who underwent herniorrhaphies for groin pain. Two patients had groin pain without evidence of a hernia preoperatively (pubalgia). In the remaining seven patients we determined the presence of a hernia by physical examination. At operation, eight patients were found to have inguinal hernias. One patient had no hernia but had partial avulsion of the internal oblique fibers from their insertion at the public tubercle. The average interval from operation to return to full activity was 11 weeks. All patients returned to full activity within 3 months of surgery. One patient had persistent symptoms of mild incisional tenderness, but otherwise there were no recurrences, complications, or persistence of symptoms. Abnormalities of the abdominal wall, including inguinal hernias and microscopic tears or avulsions of the internal oblique muscle, can be an overlooked source of groin pain in the athlete. Operative treatment of this condition with herniorrhaphy can return the athlete to his sport within 3 months.

A total of 48 infants with abdominal wall defects referred to the South West Regional Neonatal Surgical Centre over a period of six years were reviewed. There were 27 (56%) infants with gastroschisis and 21 (44%) infants with exomphalos. At the regional centre, four pregnancies with gastroschisis were terminated following a prenatal diagnosis, one child was stillborn, and one with exomphalos and trisomy 13 died soon after birth. Of the remaining 42 live births, the one year survival for babies born with a gastroschisis was 95% (21/22) and with an exomphalos was 81% (17/20). All deaths, except one, were due to factors other than the abdominal defect. The effect on outcome of prenatal diagnosis, parental counselling, in utero transfer, mode of delivery, and methods of surgical closure was reviewed.

Fifty cases of gastroschisis were treated at the Hôpital des Enfants Malades from 1960 to 1976. Up to 1973, 90% of the babies died. From 1974-1976, 17 infants were treated and 10 (59%) were cured. The authors emphasise the importance of intravenous feeding, the prevention of infections and intensive respiratory care. Complete intestinal ileus for more than one month is associated with a bad prognosis. The use of drugs and further surgery in this situation in discussed.

Based on 14 years' experience with the surgical repair of gastroschisis abdominal wall defects in 32 infants at the UCLA Hospital, certain aspects of care evolved which have served to reduce the overall long-term mortality to 6.2%. The severity of gastroschisis defects appears to be related to the length of time the eviscerated intestine has been exposed to amniotic fluid, and the degree of vascular obstruction to the viscera. In contrast to reports by previous authors recommending a specific operative technique for all infants with this malformation, we believe that choice of the optimal surgical repair depends on the degree of disproportion between the size of the eviscerated intestine and the size of the abdominal cavity. Three of the 32 patients with minimal disproportion underwent primary skin and muscle closure followed by early recovery. Twenty-seven who had primary skin flap closure later underwent secondary ventral hernia repair within six to 12 months. Two of the 32 infants had severe viscerobadominal disproportion and required temporary prosthesis coverage in addition to extensive skin flaps during the primary repair. The low morbidity and mortality following gastroschisis repair are apparently related to these factors: avoiding undue compression of the viscera; early coverage of the contaminated viscera with skin or muscle to minimize infection; careful supportive perioperative management to maintain body heat and provide adequate fluid repletion; and the infusion of intravenous hyperalimentation solutions during the lengthy period of post-operative ileus. Prosthetic materials should be reserved for more complex abdominal wall reconstruction in infants who have severe visceroabdominal disproportion.

Closure of gastroschisis can be achieved by primary adaptation of the abdominal wall, by implanting a dura patch or by covering the protruding gut with a silastic pouch. In the Pediatric Surgical Department of the University of Innsbruck this last method was used in 14 of 18 cases during the last 8 years. In three children multiple complications and infection resulted in the necessary removal of the silastic pouch from the still protruding gut. Lacking any other alternative, the defect was covered with mesh skin grafts, which took well and permanently closed the abdominal wall.

The question has been raised whether omphalocele and gastroschisis are fundamentally different pathologic entities, or whether they are only different manifestations of the same underlying cause. After studying the family history of 37 patients with omphalocele and 14 patients with gastroschisis, it seems probable that hereditary factors contribute to the origin of omphaloceles. Such factors were not shown in patients with gastroschisis. Therefore genetic evaluation and counseling is advocated for the parents of a child with omphalocele.

Twenty-three cases of omphalocele or gastroschisis managed at the Pediatric Surgical Department Juntendo University Medical School, Tokyo, Japan during the period 1973-78 are analysed. There were 5 cases with hernia into the cord, 13 with omphalocele proper and 6 with gastroschisis. The overall mortality was 26%. Both omphalocele proper and hernia into the cord had high incidence of concomitant malformation. Survival could be closely correlated with severity of associated congenital anomalies. Patient with birth weight lower than 2000gm and/or with defect larger than 8 cm was also at great risk. T.P.N. and assisted respiration evolved the management of omphalocele and gastroschisis. Significant differences in associated anomalies, birth order and birth weight between hernia into the cord and gastroschisis tends to rebut that both are the results of same congenital accident.

Gastroschisis is often complicated by damage to the herniated small bowel, resulting in motility and absorption disturbances and occasional intestinal necrosis and atresia. To study the pathophysiology of this process, a model of gastroschisis was developed in fetal lambs. At 80 days' gestation, the anterior abdominal wall was partially excised to create a small peritoneal cavity, and the small bowel was extruded through a Silastic ring to create a defect of uniform size. In one experimental group, a tie was placed around the herniated bowel at the level of the abdominal wall to provide gradual constriction as the fetus grew. In a second group, no tie was placed. Control animals had a simple laparotomy and no abdominal wall defect; some also had a constrictor placed around the base of the bowel. The animals were delivered near term, and the bowel was evaluated histologically and by an in-vitro bowel motility assay. Histologic examination showed normal ganglion cells in all groups and no evidence of ischemic injury. A fibrous peel was seen only in bowel exposed to amniotic fluid, with or without a constrictor. Lymphatic and venous dilation, smooth-muscle thickening, and focal mucosal blunting were seen in bowel subjected to chronic obstruction by a constrictor, regardless of whether it was exposed to amniotic fluid. Both constriction of the bowel and amniotic fluid exposure were associated with a decrease in motility; these two effects were independent and additive.(ABSTRACT TRUNCATED AT 250 WORDS)

An operation is described for repair of skin-covered large anterior abdominal wall defects, using the patient's own tissues, without synthetic materials, and its use in three patients is reported.

Recognition of the disruptive vascular nature of the structural defects associated with gastroschisis and an appreciation of the embryology of the umbilical region suggest that gastroschisis results from an intrauterine interruption of the omphalomesenteric artery. This mechanism accounts for the usual location of gastroschisis to the right of the umbilical cord, the integrity of the rectus muscles in affected children, and many of the clinically observed differences between gastroschisis and omphalocele. The vascular basis of this defect explains its negligible recurrence risk and should alert the clinician to the possibility of concomitant structural defects of a similar pathogenesis.

We report on 6 laparoschisis treated in the last 5 years. Five patients survived whereas one died at the age of 41 days. We review clinical aspects of this malformation and point out the need for a prompt operation and an adequate nutritional management.

Five littermate Cocker Spaniels were born with concomitant pericardial, diaphragmatic, caudal sternal, and cranioventral abdominal wall defects. Three of the 5 pups had ventricular septal defects. A sixth pup with a cranioventral abdominal wall defect died 4 days after birth. No history of teratogen exposure was reported, and none of the pups from previous litters of the dam was affected. Each pup underwent successful surgical correction (without median sternotomy) of the diaphragmatic and cranial abdominal wall defects when they were 10 to 12 weeks old. The ventricular septal defects were not corrected. In 2 of 3 pups with ventricular septal defects, thoracic radiography 6 months after surgery revealed moderate generalized cardiomegaly. All pups were healthy one year after surgery.

Twenty-three patients with gastroschisis or omphalocele undergoing delayed ventral herniorrhaphy were reviewed. The ventral defects resulted from elective skin flap coverage in 15 patients, prosthetic silo failure in five, and nonoperative management using escharotic agents in three. Herniorrhaphy was performed at 2 months to 15 years of age. Fascial closure was achieved in a single procedure in 15 patients (65%) and with multiple operations involving prosthetic material in five patients (22%). Three patients (13%) had prosthetic material left in their defects without sequelae. There was no operative mortality from interval herniorrhaphy, and complications were minor. We conclude that conversion of a gastroschisis or an omphalocele to a ventral hernia is strategically useful in an infant with a giant defect, prosthetic silo failure, or when associated anomalies preclude early definitive surgery.

During the past 11 years, 18 infants with gastroschisis abdominal wall defects have undergone surgical repair at the UCLA Hospital. Sixteen infants had skin flap closure in infancy. A gastrostomy was performed on all infants, and peripheral intravenous hyperalimentation was used in 14 of the 18 infants. Sixteen of the 18 infants (89%) lived more than one year after surgical repair. Of these, 12 have undergone second-stage closure of the ventral hernia. Operative repair was greatly facilitated by forceful stretching of the abdominal musculature and milking of the bowel contents proximally into the stomach and distally out through the anus. The low morbidity and mortality of gastroschisis repair by primary skin closure, supplemented by intravensou hyperalimentation with late secondary ventral hernia repair, appear to justify continued use of this technique. Prosthetic materials probably should be reserved for reconstructing more complex abdominal wall defects.

The development of gastroschisis was studied experimentally as well as clinically, particularly concerning the characteristic fibrous coating of the protruding bowel loops, associated intestinal atresia, and postoperative hypoperistalsis without intestinal obstruction. Experimental investigation was carried out in the chicken embryo. The clinical study was a joint one, involving a total of 50 patients with gastroschisis seen at the Sophia Children's Hospital, Rotterdam, and the Royal Hospital for Sick Children, Glasgow, between 1972 and 1985. Some patients were followed antenatally. The results, correlating with previous experiments, were compared with data from the literature. The fibrous coating of the protruding bowel loops appeared to be a late occurrence and directly related to changes of the amniotic fluid secondary to the onset of renal function. Associated intestinal atresia and postoperative hypoperistalsis in the absence of an obstruction both appeared to be due to another late gestational event, consisting of ischemic changes of the bowel wall secondary to the compression of bowel loops and mesentery in the small abdominal wall defect.

Ten fetuses with gastroschisis on whom a prenatal sonographic diagnosis and evaluation were available and who delivered at the University of California, San Francisco, were evaluated retrospectively. Six fetuses were delivered vaginally without mortality and minimal or absent morbidity. Four were delivered abdominally, 2 for unrelated obstetrical indications. A 3rd fetus had a suspected prenatal bowel perforation and was the sole mortality. Only 1 mother underwent primary cesarean section, specifically due to the fetal gastroschisis. Umbilical cord pH values were normal in those delivered vaginally. With careful ultrasound examination and immediate neonatal surgical capability in a level III perinatal center, vaginal delivery of fetuses with gastroschisis can be associated with excellent outcome. A multinational randomized study of the delivery mode of fetuses with gastroschisis is necessary and appropriate at this time.

The antenatal sonographic appearance of gastroschisis is described. The findings consist of numerous extra-abdominal "cystic masses" respresenting loops of fluid-filled bowel eviscerated through the abdominal defect. This patient also had associated polyhydramnios, possibly on the basis of a functional bowel abnormality.

Porcine skin grafts and human amniotic membranes have been used as biological dressings for the treatment of gastroschisis or omphalocele in 16 newborn infants. Eleven infants with gastroschisis were managed initially by the Silastic pouch technique but delayed healing resulted in separation of the Silastic sheeting from the wound margins and required use of biologic dressings to achieve complete closure. Biological dressings were used in five patients with intact omphaloceles for all or part of their course. Average time to achieve complete closure was 55 days. Three patients died of associated anomalies. One patient developed an enteric fistula. Biologic dressings appear to be useful adjuncts in the management of some patients with gastroschisis or omphalocele.

We reviewed a 22 year experience with 138 newborn infants with congenital evisceration through defects of the abdominal wall. Omphalocele is a large defect which always has a sac, in which the rectus muscles insert laterally on the costal margins and which usually has associated anomalies. Cord hernia is a small defect which always has a sac, in which the rectus muscles insert at the xiphoid and which commonly has associated anomalies. Gastroschisis is a small defect which never has a sac, in which the rectus muscles insert at the xiphoid and has few associated anomalies, though prematurity is frequent. We hypothesize that gastroschisis develops because the umbilical coelom fails to form, which forces the elongating midgut to rupture into the amniotic cavity. This differs from the embryogenesis of omphalocele, which is failure of closure of a primary body fold, and from that of cord hernia, which is failure of the midgut to return from the umbilical coelom. The number of infants in this series who survived after surgical repair of an omphalocele was 31 of 51 patients; of a cord hernia, 22 of 28 patients, and of gastroschisis, 40 of 59 patients. Factors contributing to mortality were associated anomalies, low birthweight and surgical closure under excessive tension.

The experience of 73 consecutive infants with gastroschisis and omphalocele is reported. The overall survival rate was 80 percent; however, since 1973 the survival rate for ruptured and intact omphaloceles has been 87 percent and 93 percent for gastroschisis. This remarkable reduction in mortality has been attributed primarily to the advent of total parenteral hyperalimentation, but mortality has also decreased due to the use of pediatric respirators which overcome the effects of increased intraabdominal pressure, and the creation of the neonatal intensive care unit where monitoring of these often fragile infants and the presence of specialty personnel assist in their care.

The management of 7 children with massive abdominal wall hernias is reviewed, utilizing a technique of stabilizing the defect by insertion of a Teflon mesh prosthesis followed by pneumoperitoneum and staged reduction. Teflon mesh has proven ideally suited for this purpose because of its flexibility, elasticity, and relative nonreactivity, allowing it to be applied directly over the surface of exposed bowel without inducing fistula formation. Although the mesh is securely incorporated into the fascial perimeter of the abdominal wall, a pseudomembrane is formed at the point of contact with the bowel surface which allows subsequent dissection and removal of the prosthesis with relative ease. Having limited the size of the defect by insertion of the mesh, an ideal situation is created for use of pneumoperitoneum to expand the peritoneal cavity and stretch the normal tissues of the abdominal wall, thus facilitating subsequent operative reduction of the ventral hernia. Utilizing this approach, excellent cosmetic and functional results have been achieved in all 7 patients.