Congenital Anterior Abdominal Wall Defects Research Articles

Early outcome analysis of Gastroschisis from a high-volume tertiary care institute in India: A prospective observational study

Background: Gastroschisis is one of the common congenital anterior abdominal wall defects with uncovered abdominal contents (usually intestines) protruding through it. Immediate reduction of the abdominal contents is crucial after birth because of the grave consequences of delayed management. The aim of our study was to evaluate the early outcomes of Gastroschisis at a tertiary care institute in India. Methods: This prospective observational study was undertaken over a one-year duration extending from January to December 2021 at our pediatric tertiary care teaching institute. Results: There were 30 male and 28 female patients, out of which 37 were preterm neonates. The mean birth weight was 2019±357g. Seven patients (12.07%) had major associated malformations. Fifty (86.21%) patients underwent primary skin flap closure. Staged reduction with silo was accomplished in five (8.62%) patients; three patients died during resuscitation before any therapeutic procedure could be undertaken. Among 55 (94.83%) patients with surgical procedures, only 26 (47.27%) could be salvaged with overall favorable (survival) outcomes in 44.83% of patients. Mortality was high (92.59%, 25/27) in the patients presenting with markedly edematous bowel with leathery peel. Seventeen (29.31%, 17/58) neonates died in the first 72 hours (postoperatively) due to complications of abdominal compartment syndrome, eight (13.79%) patients died due to postoperative sepsis with thrombocytopenia, and two (3.44%) had intestinal perforation. The duration of hospital stay in neonates who survived ranged from one to four weeks. Conclusion: Overall survival rates in our study were 44.83% markedly in contrast to the series published in the recent literature. The outcome of preterm (premature) patients, associated intestinal atresia, presence of edematous bowel with leathery peel, patients requiring silo due to viscero-abdominal disproportion, necrotizing enterocolitis, and associated malformations, was dismal.

Patterns and Short Term Outcome of Children with Major Congenital Anterior Abdominal Wall Defects Admitted At Mbarara Regional Referral Hospital, South Western Uganda, from September 2019 to June 2020.

Background: Omphalocele and gastroschisis are the most common major congenital abdominal wall defects (CAWDs) globally. Mortality among neonates major CAWDs is higher in low-income countries than in high-income countries. This study described the patterns and short-term clinical outcomes of infants with gastroschisis and omphalocele at a regional referral hospital in southwestern Uganda.
 Methods: A case series was conducted at Mbarara Regional Referral Hospital ten months. Children born with CAWD during the study period 54 samples size were consecutively recruited into the study. Data were entered, cleaned, and analyzed using Stata version 15. Descriptive statistics were performed where continuous variables were summarized using median and interquartile ranges, while categorical variables were summarized using frequencies and proportions. Time to mortality was assessed using Kaplan–Meier survival analysis.
 Results: A total of 54 newborns were recruited into the study, of which 40 (70.04%) had Gastroschisis and 14(25.93%) had omphalocele. The median age of the infants was 9 days with an interquartile range of 4 to 21days. Of the 54 newborns, 30(69.2%) were male within the age group of 1-10 days. Mortality was higher in babies with complex gastroschisis (22) than simple gastroschisis (10). Not administering antibiotics and low birth weight were the factors associated with mortality among infants diagnosed with gastroschisis and omphalocele in the neonatal period.
 Conclusion: The most common observed defect was gastroschisis, Mortality was generally high among children presenting with gastroschisis. Complex gastroschisis has a poorer prognosis than simple gastroschisis over 30 days follow up period. Non-use of antibiotics before referral to a health facility and low birth weight were the factors associated with mortality among infants diagnosed with gastroschisis and omphalocele in the neonatal period.
 Recommendation: The most common pattern observed was Gastroschisis. The majority of children presenting with Omphalocele were more likely to survive for 30 days. There is a need for timely referral for babies born with congenital anterior abdominal wall defects.

Acquired anterior abdominal wall defect in a neonate following umbilical catheterization: a case report

Congenital anterior abdominal wall defects are well known clinical entities. However, acquired abdominal defects are uncommon.

Ultrasonographic detection of gastroschisis in the first trimester: Actualities in pregnancy management and outcome

Gastroschisis is a congenital anterior abdominal wall defect that occurs in 4 to 5 in 10,000 births, consisting in the evisceration of bowel in the amniotic cavity without a membranous covering. Gastroschisis cases can be detected during the first trimester using ultrasonography or at the beginning of the second trimester with a detection rate of 100%. Although an isolated condition in most of the cases, further complications such as malrotation, atresia, stenosis, perforation or volvulus can be associated. Periodic monitoring is required to assess the progress of the pregnancy with measurements of the level of amniotic fluid, fetal biometry and bowel diameters at every ultrasound examination. Gastroschisis is most frequently associated with oligohydramnios, intrauterine fetal growth restriction, increased bowel wall thickness and bowel dilatation. The outcome of the disease is favorable with a survival rate of around 90% for born infants. The long time on ventilator and multiple intestinal surgeries that delay the moment of oral feeding with a long-term use of total parental nutrition and short bowel syndrome are the most important comorbidities. Delivery is recommended at 38 weeks of gestation, preserving caesarean delivery for complicated gastroschisis. Although a complex pathology, gastroschisis is associated with normal intellectual development and few to none long-term complications.

Gastroschisis with malrotation, gut duplication and Meckel's diverticulum; a rare association

Gastroschisis is defined as a congenital anterior abdominal wall defect which is rarely associated with other congenital anomalies. Gastrointestinal anomalies are the most common associations including malrotation. Other gut anomalies like intestinal duplication and Meckel's diverticulum are very rare. Our report a rare case of gastroschisis who is associated with malrotation, intestinal duplication and Meckel's diverticulum.

MAIN PRINCIPLES OF ETIOPATHOGENETIC TREATMENT OF NEWBORNS WITH GASTROSCHISIS

Introduction. The paper focuses on the challenging issue of treatment of newborns with gastroschisis (GS) and a high level of intraabdominal hypertension in case of severe viscero-abdominal disproportion. The aim of the paper is to present the current state of the problem at the regional level based on our own clinical experience in observation and treatment of newborns with GS. Material and methods. The study is based on the analysis of examination and treatment of 29 newbornswith GS. The distribution of boys and girls was nearly the same: 16 (55.17%) boys and 13 (44.83%) girls. The number of premature babies was 23 (79.31%). The complex of diagnostic procedures included clinical laboratory examination, radiologic investigation (using contrast agents when required), ultrasound investigation and Doppler sonography, intraabdominal pressure measurement, histologic study of surgical specimens.Results. Prenatal information on the congenital anterior abdominal wall defect was obtained with the help of prenatal ultrasound. In most cases, fetal GS is diagnosed before 20 weeks of pregnancy. Prenatal ultrasound was performed in 13 (44.83%) pregnant women out of the researched group. Two false test results were obtained.Radiologic investigation of a child with GS is necessary to detect intestinal obstruction, necrosis or bowel perforation. Among patients under our study, two cases of ileal atresia were registered. The analysis of intraabdominal pressure indices in patients with GS showed a high level of intraabdominal hypertension in approximately 70% of cases. It proves that patients with GS have a high level of surgical and anesthetic risk. The main therapy measures include the following steps: appropriate preoperative preparation, anesthetic management, and the choice of appropriate perioperative techniques taking into account the level of intraabdominal hypertension and viscero-abdominal disproportion.Depending on the child's condition, type of GS and level of intraabdominal hypertension, there were twosurgical options:1. Primary radical surgery.2. Staged surgical treatment.Analyzing the results of the study, it may be noted that 8 newborns in total died after the surgery, which is 27.59%(it used to be 56-60% till 2005). The main causes of death were respiratory failure, neonatal sepsis and unfavorable premorbid conditions (very low gestational age, severe comorbidity, multiple congenital malformations).Conclusions. 1. Successful treatment of newborns with GS depends on the early diagnosis of the pathology, which must be prenatal, and elimination of contradictions in treatment tactics. 2. Before suturing the abdominal wall defect in newborns with GS, it is necessary to measure intraabdominal pressure, since intraabdominal hypertension leads to a significant deterioration in the mechanical properties of the lungs, hemodynamic abnormalities,oliguria, intestinal ischemia, decrease in organ perfusion, which must be taken into account when carrying out preoperative preparation and aesthetic management. 3. The level of intraabdominal hypertension in patients with GS,which is high in 68.96% of cases, must be an indication for choosing the method of surgical correction of the defect i.e.the refusal of radical plasty of the abdominal wall and the resort to staged intervention or other surgical techniquesthat involve an increase in the abdominal cavity volume. 4. The reduction in mortality in newborns with GS to 27.59%is possible due to the introduction of etiopathogenetic approaches to early diagnosis, preoperative management, anesthetic management and surgical correction of this pathology into the practice of neonatal surgery.

Giant omphalocele: Delayed closure using the San Martin technique following epithelialization of the membrane

AimThe management of patients with congenital anterior abdominal wall defects remains challenging, particularly in cases of giant omphalocele. In 1948, San Martín described a surgical technique for the repair of large midline incisional hernias in adults without the need for a mesh. The purpose of this report is to describe our experience with this technique for the delayed closure of giant omphaloceles. MethodsWe retrospectively reviewed the outcomes of all patients with giant omphalocele managed with the San Martin technique between September 2013 and March 2019. Data collected included birth weight, gestational age, associated malformations, neonatal hospital stay, age at the time of the abdominal wall closure, days on mechanical ventilation (MV) after the closure, time to initiation of enteral feedings, intra- and postoperative complications, and postoperative hospital stay. ResultsA total of 8 patients were included in the study. The median birth weight was 3.190 (2.150 to 3.400) grams. The median gestational age was 35 (32 to 38) weeks. The median age at surgery was 6 (5 to 13) years. The median postoperative days on MV was 3 (3 to 11) days. Enteral feeding were initiated postoperatively at a median of 4 (2 to 4) days. There was one intraoperative complication (minor vascular injury). There were no short-term or long-term complications directly related to the surgical technique. The median postoperative hospital stay was 10 (6 to 16) days. The follow-up was 18 months to 8 years. ConclusionWe believe that the San Martín technique is a valid alternative for the delayed closure of giant omphaloceles. Level of evidenceAccording to the Journal of Pediatric Surgery this research corresponds to type of study level IV for case series with no comparison group.

Gastroschisis: five cases after primary closure at Sanglah General Hospital, Bali, Indonesia

Background: Gastroschisis is a congenital anterior abdominal wall defect characterized by intra-abdominal organs evisceration without covering the membrane. Several risk factors affect the outcome of a gastroschisis patient. Considering these risk factors might also increase the chance of better prognosis and survival. We report five neonates with gastroschisis after primary closure in Sanglah General Hospital, Denpasar, Bali, in 2017. This case series aims to describe the clinical characteristics and outcome of different babies with gastroschisis managed by primary closure surgery.Case Presentation: Four of our cases were detected during pregnancy with fetal gastroschisis at the age of 32, 19, 23, 25 weeks of gestation, respectively. We reserve cesarean delivery for the patient who did antenatal care in our hospital, but one of our patients was born by spontaneous labor due to lack of antenatal care. Four cases had the primary closure of less than 2 hours and one of them after 10 hours. Good outcome was found in three cases that started enteral feeding on the 7th day after post closure. Other cases started oral feeding on the 3rd day and 12th day—both of them shown poor outcomes. Three cases survive, but two others died because of sepsis.Conclusion: We found poor outcomes in both cases, which neonatal sepsis as a significant cause. One of our cases with poor outcome was referred from another hospital without antenatal care and had a long duration of entering the operating room. Diagnosing these babies in their early prenatal period and transferring them to an experienced medical centre containing multidisciplinary working facilities will contribute to both the mother and the baby's health.

Challenges and Outcome of Management of Gastroschisis at a Tertiary Institution in North-Eastern Nigeria.

Introduction: Gastroschisis is a congenital anterior abdominal wall defect characterized by herniation of abdominal contents through a defect usually located to the right side of the umbilical cord. It occurs in about 1 in 2,000–4,000 live births and is slightly commoner in males. Management has remained challenging in the low and middle-income countries, with high mortality rates. This study highlights the clinical presentation, treatment, outcomes, and challenges in the management of gastroschisis at a tertiary healthcare center in a resource-limited setting.Methods: This was a retrospective review of the records of all patients with gastroschisis managed over a period of 30 months (January 2016–June 2018). Data on patients' demographics, age, birth weight, clinical presentation, method of gastroschisis reduction and closure, complications, and outcomes were collated. Statistical analysis was performed using SPSS version 20. A p < 0.05 was considered significant.Results: Twenty-four patients with gastroschisis were managed. Of these, 18 patients had data available for analysis. There were 14 males, with a male-female ratio of 3.5:1. The median age at presentation was 11.0 h (range 1–36 h). Ten patients (55.6%) were delivered in a medical facility. One patient had type II jejunal atresia and transverse colonic atresia as associated anomalies. Improvised silos were applied by the bedside in 15 (83.3%) patients, while two patients (11.1%) had primary closure under general anesthesia. One patient died before definitive treatment could be done. Sterile urobags and female condoms were used for constructing improvised silos in 9 (60%) and 6 (40%) patients, respectively. Eight patients who had initial silo application had complete bowel reduction over a median time of 8.0 days (mean 10.0 ± 6.5days, range 2–23 days). Total parenteral nutrition was not available. The average time to commencement of feeding was 8.0 days ± 6.6 (median 6.0 days, range 2–22 days). Full feeding was achieved in five patients (two patients in the primary closure group and three from the silo group) over a mean time of 16.8 days ± 10.4 (median 14.0 days). Sepsis was the commonest complication. Four patients (22.2%) survived.Conclusion: Management of gastroschisis remains challenging in resource-limited regions.

Outcome of congenital anterior abdominal wall defects in Tasmania: 1996–2015

Outcome of congenital anterior abdominal wall defects in Tasmania: 1996–2015

Burden of congenital inguinal hernia and hydrocele in northern and southern Nigeria: an opportunity for awareness creation.

Nigeria, with a population of >150 million people in which half of the population are children encounters challenges in paediatric surgery practice in rural areas. There are paediatric surgeons in Nigeria, but majority practice in tertiary health facilities in cities. The poor rural dwellers have little or no access to such highly trained specialists. Hence, children with congenital and acquired paediatric surgical pathologies including anterior abdominal wall defects not only grow up with these diseases to adulthood, they are also exposed to various health hazards posed by unqualified personnel. Therefore, we are evaluating the burden of congenital inguinal hernia/hydrocele in northern and southern Nigeria for awareness creation and the way forward. Data obtained from organised free hernia missions to the rural populace from northern and southern Nigeria by the West African Collage of Surgeons in 2010 and Kano State Government in 2013 was analysed. A total of 811 patients aged from 3 months (0.25 years) to 35 years was screened and found to have congenital hernia and/or hydrocele from the two centres. 171 (21.1%) were successfully operated, while the remaining 640 (78.9%) could not benefit from a surgical procedure during the missions. There were n = 46 (26.9%) patients with various forms of genital mutilations/and or surgical mismanagements among the operated patients. The burden of congenital anterior abdominal wall defects among Nigerian children is high. A little effort could bring succor and create awareness among this group of people.

Look twice before you clamp the cord: iatrogenic ileal transection.

Anterior abdominal wall defects at the time of birth can present either as gastroschisis or omphalocele. Omphalocele is a congenital anterior abdominal wall defect at the base of an umbilical cord with herniation of the abdominal contents covered by the parietal peritoneum, the amnion and Wharton’s jelly. However, mild forms of omphalocele can present as congenital hernia of the umbilical cord with herniation of either the solitary intestinal loop or persistent omphaloenteric duct (POD). The risk factors in omphalocele as well as mild forms such as congenital hernia of the umbilical cord are still not completely known and their association with other birth defects has not always been established [1]. Traumatic injury to intestinal loops in congenital hernia of the umbilical cord has also been reported and emphasized in order to prevent this type of injury at the time of birth [2]. The case is reported because of its great rarity, and as a reminder to all that such an accident may take place. In tying or clamping the cord, especially one that is unduly large, it should be tied well away from the abdomen.

Presentation And Management Of Gastroschisis: Experience In 8 Years In Benin-City, Nigeria

Congenital anterior abdominal wall defect occurs commonly and requires specialized management. Thirteen cases of gastroschisis treated at the University of Benin Teaching Hospital, Benin City over an 8-year period is presented. There were nine males and four females (male/female ratio 2.3:1). Time lag before presentation ranged between 5 and 36 hours (mean 16.8 ± 9.9 hours). Only six (46.15%) presented in the unit within 12 hours of birth, while seven (53.85%) presented after 12 hours with varying degrees of complications. Of the six that presented within 12 hours, one death (16.65%) was recorded whereas six deaths (85.71%) were recorded among those that presented after 12 hours (P = 0.0291, Fisher\'s exact). The causes of deaths were fluid and electrolytes derangement, hypothermia, overwhelming sepsis, respiratory failure and inanition. Two were too ill for surgical intervention and died during resuscitation. Eleven were operated between 4 and 8 hours on presentation. Of the Six that had digital stretching of abdominal cavity, gastric suctioning/rectal irrigation and primary abdominal closure, three died and three survived. The two who had bowel gangrene for which resection/anastomosis and primary abdominal closure were done, survived. Of the three that had improvised prosthetic silo closure, two died and one survived. Overall, six (46.2%) survived while mortality of seven (53.8%) was recorded. This study shows that delay in presentation resulted in high morbidity and mortality rates. Keywords: Gastroschisis, Congenital, Abdominal wall defect)Sahel Medical Journal Vol. 10 (4) 2007: pp. 115-118

Management of omphalocele major

Congenital anterior abdominal wall defect occurs commonly and requires specialized management. The commonest form is the Exomphalos. Fourteen cases of large exomphalos which have been treated at the University of Benin Teaching Hospital, Benin City are presented. Three were minor Omphalocelem and 11 major omphalocele. All minor cases were treated by primary repair, with good results. Of the major cases 8 could not be treated by primary repair: two had torn sac and were treated with application of prosthetic bags and the remaining 6 were treated conservately by sterile dressing method. Results show that supervised conservative dressing is convenient and achieves good results. Nigerian Journal of Surgical Sciences Vol. 15(2) 2005: 71-73

Gastroschisis

After completing this article, readers should be able to: 1. Describe normal embryology and various theories contributing to derangements in development leading to gastroschisis. 2. Delineate several theories regarding the pathogenesis of gastroschisis. 3. Explain the environmental and other risk factors linked to gastroschisis. 4. Describe the prevalence of gastroschisis in developed countries and various theories explaining it. Gastroschisis is a congenital anterior abdominal wall defect, adjacent and usually to the right of the umbilical cord insertion. It occurs as a small, full-thickness periumbilical cleft either immediately adjacent to the umbilicus or separated from it by a strip of skin. This results in herniation of the abdominal contents into the amniotic sac, usually just the small intestine, but sometimes also the stomach, colon, and ovaries (Figure⇓). The abdominal wall defect is relatively small compared with the size of the eviscerated bowel, which often develops walls that are matted and thickened with a fibrous peel. Gastroschisis has no covering sac and no associated syndromes. This differentiates it from an omphalocele, which usually is covered by a membranous sac and more frequently is associated with other structural and chromosomal anomalies (Table 1). In addition, although gastroschisis may be associated with gastrointestinal anomalies such as intestinal atresia, stenosis, and malrotation, it has a much better prognosis than omphalocele. Figure. Gastroschisis, resulting in herniation of the abdominal contents into the amniotic sac. | | Gastroschisis | Omphalocele | |:---------------------------:| ----------------------------- | ---------------------------------------------------------- | | Incidence | 1 in 10,000 (now increasing) | 1 in 5,000 | | Defect Location | Right paraumbilical | Central | | Covering Sac | Absent | Present (unless sac ruptured) | | Description | Free intestinal loops | Firm mass including bowel, liver, etc | | Associated With Prematurity | 50% to 60% | 10% to 20% | | Necrotizing Enterocolitis | Common (18%) | Uncommon | | Common Associated Anomalies | Gastrointestinal (10% to 25%) | Trisomy syndromes (30%) | | | Cardiac defects (20%) | | | Beckwith-Weidemann syndrome | | | Cryptorchidism (31%) | Bladder extrophy | | Prognosis | Excellent for small defect | Varies with associated anomalies | | Mortality | 5% to 10% | Varies with associated anomalies (80% with cardiac defect) | Table 1. Differences Between Gastroschisis and Omphalocele The term gastroschisis is derived from the Greek word laproschisis , meaning “bellycleft.” It was used in the 19th and early 20th centuries by teratologists to designate all abdominal wall defects. No clear distinctions were made between abdominal wall defects until 1953, when Moore and Stokes classified them based on their appearance at birth. They suggested that the term gastroschisis be reserved for those cases in which the …

Ward reduction without general anaesthesia versus reduction and repair under general anaesthesia for gastroschisis in newborn infants.

Gastroschisis is a congenital anterior abdominal wall defect with the abdominal contents protruding through the defect. Reduction of the abdominal contents is required within hours after birth as the infant is at risk not only of water and heat loss from the exposed bowel but also of compromised gut circulation with ischaemia and infarction. To avoid the complications of general anaesthetic and mechanical ventilation it has been proposed that the reduction of abdominal contents can be achieved without endotracheal intubation or anaesthesia. To determine which approach to the immediate surgical treatment of gastroschisis has the better outcomes: ward reduction without general anaesthetic or reduction and repair of the abdominal wall defect under general anaesthesia. Searches were made of MEDLINE from 1966 to March 2002, CINAHL from 1982 to March 2002, and the Cochrane Controlled Trials Register (The Cochrane Library, Issue 1, 2002). Previous reviews including cross references, abstracts, and conference and symposia proceedings published in Pediatric Research (from 1990 to 1994) were also searched, especially for any unpublished data. Randomised, controlled trials (RCT) comparing ward reduction with reduction under general anaesthesia, for neonates with gastroschisis. Outcomes considered were: mortality, duration of total parenteral nutrition, time to full enteral feeds, need for a silo, infection, gastro-intestinal tract perforation, length of bowel lost/resected, need for a general anaesthesia, need for and duration of mechanical ventilation and respiratory support, duration of oxygen therapy, need for further operative procedure after initial reduction, duration of hospital stay, cosmetic outcome, nutritional status, and neurodevelopmental outcome. No studies were found meeting the criteria for inclusion in this review. No studies were found meeting the criteria for inclusion in this review. There is no evidence from RCTs to support or refute the practice of ward reduction for the immediate management of gastroschisis. There is an urgent need for RCTs to compare ward reduction versus reduction under general anaesthesia in infants with gastroschisis. Initial trials would best be limited to those infants with uncomplicated gastroschisis (using pre-defined selection criteria excluding infants that are unstable, have gut perforation, necrosis or atresia, have other organs requiring reduction besides bowel, or are considered to need a silo prior to any reduction. Trials should use adequate pain relief and specify a pre-defined time period after which manual reduction is abandoned.

Congenital anterior abdominal wall defects in the north of England, 1986-1996: occurrence and outcome.

The aim was to describe trends in prevalence, maternal age-specific prevalence, associated anomalies, clinical outcomes and the sensitivity of antenatal diagnosis of congenital anterior abdominal wall defects (in particular gastroschisis and exomphalos). Data were identified from a population-based register of major congenital abnormalities in the Northern health region of England, the Northern Congenital Abnormality Survey (NorCAS), between 1986 and 1996. 296 cases were notified; there were 133 cases of gastroschisis, 98 exomphalos, 30 limb-body wall defects and 23 other anterior abdominal wall defects. 12 cases could not be classified. In 19 (6 per cent) the initial diagnosis was changed following case review. 30 (30.6 per cent) cases of exomphalos were associated with a chromosomal anomaly compared with 1 (0.8 per cent) case of gastroschisis. The total prevalence for the 11 years was 6.33 (95 per cent CI=5.57-7.08) per 10 000 live births, still births and terminations of pregnancy, and the overall birth prevalence was 4.30 (95 per cent CI=3.68-4.93) per 10 000 live births and still births. For gastroschisis, there was a significant increase over the study period in both the total prevalence (1.48 in 1986 to 5.29 per 10 000 in 1996; chi(2)=8.41, p=0.00433) and the birth prevalence (1.48 in 1986 to 4.72 per 10 000 in 1996; chi(2)=7.42, p=0.00644), but there was no such significant increase for exomphalos (total prevalence chi(2)=2.29, p=0.13055; birth prevalence chi(2)=0.16, p=0.69348). The maternal age-specific prevalence was highest in the 11-19 year age group for gastroschisis but in the 35-39 year age group for exomphalos. Fewer pregnancies with gastroschisis resulted in a termination and a greater proportion of cases were alive at one year compared with exomphalos. The sensitivity of abnormality detection by ultrasonography was 75 per cent and 77.3 per cent for gastroschisis and exomphalos, respectively. Antenatal diagnosis improved from 47.4 per cent during 1986-91 to 80 per cent between 1992-96 for gastroschisis (chi(2)=5.7, p=0.00169), and from 55.6 per cent to 68.8 per cent for isolated exomphalos, although this increase was not significant. Total and birth prevalence of gastroschisis increased in the Northern region between 1986 and 1996. For exomphalos, there was a trend towards an increase in total prevalence and towards a decrease in birth prevalence. This decreasing trend has been accompanied by improvements in antenatal detection and subsequent termination of cases of exomphalos associated with other anomalies.

Prevalence of congenital anterior abdominal wall defects in the United Kingdom: comparison of regional registers

Recent reports from England and Wales1 and Scotland2 imply that a gradient of increasing risk of congenital abdominalwall defects may exist from the south to the north of the United...

Congenital anterior abdominal wall defects in England and Wales 1987-93: retrospective analysis of OPCS data.

Analysis of incidence and characteristics of congenital abdominal wall defects, with special reference to the differences between the incidence of gastroschisis and exomphalos (omphalocele). Retrospective analysis using data from the Office of Population Censuses and Surveys (recoded to differentiate exomphalos and gastroschisis) and the National Congenital Malformation Notification Scheme. England and Wales, 1987 to 1993. 1043 congenital anterior abdominal wall defects were notified within the seven year study period. Of these, 539 were classified as gastroschisis, 448 as exomphalos, 19 as "prune belly syndrome," and 37 as "unclassified." Gastroschisis doubled in incidence from 0.65 in 1987 to 1.35 per 10,000 total births in 1991, with little further change; the incidence of exomphalos decreased from 1.13 to 0.77 per 10000 births. The overall incidence of notified congenital abdominal wall defects was 2.15 per 10000 total births. Gastroschisis was associated with a lower overall maternal age than exomphalos and with a significantly lower proportion of additional reported congenital malformations (5.0%) than in the cohort with exomphalos (27.4%) (odds ratio 0.14, 95% confidence interval 0.09 to 0.22; P < 0.001). The sex ratio of the two cohorts was the same. The incidence of gastroschisis and exomphalos was higher in the northern regions of England than in the south east of the country. The national congenital malformation notification system showed an increasing trend in the incidence of fetuses born with gastroschisis and a progressive decreasing incidence of exomphalos in England and Wales between 1987 and 1993. Although the reasons for this are likely to be multifactorial, a true differential change seems likely. The observed increase in incidence of gastroschisis relative to exomphalos and the differentiation in maternal age have implications for resource management within the NHS and warrant further epidemiological monitoring. Regional differences may be due to a dietary or environmental factor, which requires further study.

Current embryology and treatment of gastroschisis and omphalocele.

Oftentimes in children newly borne, the naval swelleth as bigge as an egg...—Ambroise Pare, 16th Century THE MEDICAL literature, since this first description of the omphalocele, has been vague in distinguishing this from other defects of the anterior abdominal wall. Nomenclature has been inconsistent and indefinite, as is evident in the Standard Nomenclature of Diseases, where omphalocele is classified with umbilical hernia and gastroschisis is not included. Gastroschisis has also been labeled paraomphalocele, and omphalocele called exomphalos. Omphalocele has further been subdivided into embryonic and fetal types—the latter a synonym for hernia into the cord. This has led to confusion in interpreting the results of treatment and the survival statistics reported. It seems, therefore, important to clarify the existing knowledge about congenital anterior abdominal wall defects. A study of the normal embryology of this area (Duhamel¹) provides an insight into the various deviations from normal and forms a