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We report on prenatal diagnosis of gastroschisis at 20th gestational week. In addition to gastroschisis intraabdominal intestinal stenosis was detected. As a consequence cesarean section was planned close to term. Control examinations in the last trimester didn't confirm initial diagnosis so that the recommended mode of delivery was changed. However postpartum diagnosis again confirmed the early diagnosis of gastroschisis including spontaneous necrosis of dislocated intraamniotic intestinal parts. The course demonstrates that even at unambiguous prenatal diagnosis control examinations are recommended as dynamic changes may occur in the second and third trimester. Thus mode of delivery may have to be adapted close to term to reduce maternal risk.

47 children with gastroschisis were operated at the Hannover Medical School between 1980 and 1995. The average gestation period was the 36th week of pregnancy with an average birthweight of 2370 gr. A primary layered closure of the abdominal wall was performed on 46 children. A multi-sided closure of the abdominal wall defect using Gore-Tex was necessary in only one case. Post-operative intubation lasted for an average of 56 hours. The children were fed by parenteral nutrition for an average of 32 days, with oral feeding starting on the 15th post-operative day. The average weight on discharge was 3035 gr. 18% of the children had post-operative problems such as sepsis, necrotic enterocolitis and ileus. A death rate of only 2% is proof of the progress that has been made in prenatal care of the newborn child suffering from gastroschisis and suggests that primary closure of the abdomen is the operative method to be aimed for.

Objective: Our purpose was to investigate the influence of the mode of delivery on the outcome of children with gastroschisis., Methods: 38 children were born and treated at the University Hospital of Graz between 1977 and 1995. The perinatal and surgical data were analyzed retrospectively. Furthermore, we compared the prevalence of gastroschisis in Styria to international data., Results: The mode of delivery did not influence the fetal outcome. The higher rate of acidosis and shorter interval from rupture of the membranes to delivery in cases of cesarean section may be attributable to the obstetric decision for an operative delivery. The prevalence of gastroschisis is high as compared to European data., Conclusions: Our data and the majority of publications do not show a benefit of cesarean section for children with gastroschisis. A randomized prospective trial is still lacking to end controversies.

The association of increased maternal serum alpha-fetoprotein levels (MS-AFP) with certain morphologic anomalies of the fetus is fully established. These anomalies are abdominal wall defects (e.g. omphalocele, gastroschisis, complete eventration), neural tube defects (anencephalus, spina bifida, encepalocele) and other malformations (e.g. coccygeal teratoma). The present study compares MS-AFP levels with amniotic fluid alpha-fetoprotein (AF-AFP) and acetylcholinesterase activity, the results of ultrasound, genetic and morphologic examination. Between April 1992 and November 1995 60 patients were referred to our clinic for further diagnosis after detection of elevated MS-AFP. After an ultrasound examination was carried out amniocentesis (AC) was performed in 54 cases, AC + placentesis (PC) in 3 patients and PC only in 3 cases with anhydramnion. Mean maternal age was 29 years (range 20-37 years). Punction was performed at a median of 19 + 1 weeks of gestation (15 + 2-23 + 2 weeks of gestation). MS-AFP, AF-AFP and acetylcholinesterase activity was measured. MS-AFP and AF-AFP were given as multiples of the median (MoM). Values < or = 1-2.0 MoM were considered normal. Elevated levels were defined to be greater than 2 MoM. In all cases the chromosomal finding was normal or norm variant. Sonographical anomalies were detected in 7 fetuses (3 cases with spina bifida, 1 case with omphalocele and 3 cases with abdominal wall defects in connection with other malformations). All sonographic diagnoses were confirmed post partum. The MS-AFP varied between 0.9-6.0 MoM. In 7 cases MS-AFP previously (referring center) evaluated as increased was found to be within normal range in our department. This cases included normal values were found in 30 cases and 25 findings were pathological. 2 out of 7 structurally abnormal fetuses had normal values, 5 of them had elevated MS-AFP. In the group of normal fetuses the highest MS-AFP we found was 6.6 MoM. In the AC-group the AF-AFP ranged between 0.7-8.7 MoM, it was within normal range in 50 and pathological in 8 cases. In the group of fetuses without structural anomalies the highest AF-AFP was 7.5 MoM. In the group of fetuses with structural anomalies the AF-AFP values were 1x normal and 3x pathological (> 3.5 MoM). Acetylcholinesterase activity in the amniotic fluid was negative in 51 cases, positive in 4 cases and faint positive in 2 cases. In all fetuses with structural anomalies in which an AC could be performed, acetylcholinesterase activity was positive. On the other hand we found only 1 of the structurally normal fetuses with positive acetylcholinesterase activity. Elevated MS-AFP and AF-AFP may be an indicator for morphological anomalies. Yet there were many positive results without any sonographical findings. The determination of a positive acetylcholinesterase activity in the amniotic fluid is more specific. All malformations have been detected by ultrasound examination before the results of the AF-AFP were available.

A case of pentalogy of Cantrell associated with exencephaly and amniotic band syndrome, detected by ultrasound and then confirmed by pathological findings, is reported. Both conditions are very rare, usually occurring as a single anomaly; their association has not been reported in the literature. One should consider in differential diagnosis of pentalogy of Cantrell the omphalocele and the body stalk syndrome, and of exencephaly the osteogenesis imperfecta together with disorders of mineralisation. A viral infection could play a part in the aetiology of this condition.

Abnormal position of the gallbladder seems to be rare, but knowledge about the varieties helps to avoid intraoperative complications regarding the spreading procedure of laparoscopic cholecystectomy. In the reported case the gallbladder was found in the lower right abdomen fixed by adhesions to the jejunum. Embryological aspects are discussed and a literature review is given.

The Syndrome of Missing Cord has been diagnosed in a case of early pregnancy by means of transvaginal ultrasound. The sonographic criteria of this syndrome are described and correlated with the anatomical and pathological findings.

From 1986 to 1989 46 newborn were examined after correction of oesophageal atresia, defects of diaphragm or abdominal wall, to record postoperative cardial insufficiency. In 15 of 40 long-time pH metry analysis (37.5%) and in 11 of 22 x-ray studies (50%) a pathological result was found; in 8 times these findings were followed by operation and in 8 times by conservative therapy. pH metry is suitable even in very small newborn for postoperative screening of cardial function; if the results are positive, x-ray examination is supplementary to diagnosis and therapeutical planning.

Based on twelve cases of gastroschisis in newborn babys it has been possible to demonstrate that both, the time elapsed between birth and surgery, and adequate protection against heat loss, have a profound influence on postoperative recovery. To ensure a planned birth the cesarean section is to be preferred to vaginal delivery. The importance of a primarily correct post natal care is discussed.

On the basis of a specific case and of references, the problems concerning the symptoms, diagnoses and the therapy of the Prune-Belly syndrome are presented. The importance of a urodynamic check-up prior to surgical measures is underlined. Due to the many combinations of malfunctions and the lack of sexual specification, it seems preferable to consider mesenchymal dysplasia as the focal point of interest rather than the abdominal muscle defecite--uromalformation--kryptorchism--triad. In view of the high chances of infection, a conservative approach seems advisable, although the possibilities of active measures with due consideration of certain urodynamic parameters are also presented.

Reference is made to a case history of prenatal use of ultrasonography, in 34th week of pregnancy by which formation of a ventral cleft in conjunction with cephalocele was diagnosed. Additional methods to secure diagnosis are described and compared, including alpha-foetal protein evaluation and amniofoetography. Also discussed are obstetric consequences likely to result from possible prepartum diagnosis of such malformations.

The main function of paediatric surgery is devoted to the correction of congenital malformations especially those incompatible with further life. A survey of the actual situation is given.

The abdominal muscle deficiency syndrome ("prune belly syndrome"(, a symptom triad of aplasia of the abdominal wall musculature, malformations of the urinary tract, and cryptorchism, is described from the urologist's point of view, citing nine cases. With modern diagnostic procedures the early detection of the correct diagnosis in the usually very young patients is possible. While the urologic symptomatology is due to the malformations in the urinary tract, the life expectancy depends mainly on the degree of renal function impairment. Combat of infection together with proper operative repairs are the treatments of choice to date. Instrumental procedures should be limited as much as possible. The survival changes following kidney transplantation appear promising.

As yet, it has not been sufficiently investigated whether the pathogenesis of omphalocele and gastroschisis can be attributed to the same cause or to different processes. Therapeutical principles, however, are the same in omphalocele and gastroschisis: the very soon closure of the abdominal wall defect. Our own procedure strives for primary fascial closure. If this is not possible, we prefer skin closure above the silastic cylinder according to Schuster, 72 patients with omphalocele and 62 patients with gastroschisis were Schuster, 72 patients with omphalocele and 62 patients with gastroschisis were analysed. Lethality was 32% in omphalocele and 22% in gastroschisis during the last 6 years. The predominant cause of death was postoperative infection. It is therefore necessary to improve infection therapy in order to reduce lethality.

Ectopia cordis is defined as a congenital malposition of the heart partially or completely outside the thorax. Severe structural malformations of the heart are very frequently associated and are responsible for the extremely poor prognosis. Only 16 out of 219 cases reported in the literature up to now have survived. We report on a case of Ectopia cordis thoraco-abdominalis associated with an univentricular heart and a hypoplastic pulmonary vascular bed detected prenatally in the 32nd week of gestation. The male newborn died 23 h after a spontaneous and uneventful delivery at term. Considering the poor prognosis no surgical attempts had been undertaken to correct the malformation.

The gastroschisis is a congenital defect of the abdominal wall without other abnormalities and can be diagnosed by ultrasonography before 20 weeks of gestation. The management of these cases requires the cooperation of the obstetric and pediatric clinician in respect of an elective abortion. As a result of the advances in pediatric surgery and neonatal intensive care the treatment of the gastroschisis is possible and the prognosis of these children is very good. In the years 1978-1983 the primary closure of the abdominal wall defect was performed in 13 newborns. Whereas one child died due to intrauterin complications (volvolus and intestinal gangren) the 12 remaining patients could be operated successfully. We are of opinion that termination of pregnancy is indicated no longer in cases of gastroschisis.

Treatment was applied to 97 cases of omphalocele and 96 cases of gastroschisis at the Dortmund Department of Paediatric Surgery over the past 20 years. The survival rate was 122. Follow-up checks were recently applied to 56 of those former patients, after nearly ten years had elapsed from surgery. Thirty-eight of these patients were clinically examined, while questionnaires were completed for the rest. Primary closures had been performed on 50 per cent of the cases, while the defects in the other children were closed in two stages, using dura implantation or silastic pouches, or were conservatively treated. Accompanying malformations were recorded from 21 per cent of the gastroschisis cases and from 28 per cent of those with omphalocele. Overall mortality accounted for 37 per cent, with mortality in the wake of receptive operations being as high as 40 to 50 per cent, the latter rate not depending on the primary approach. One and the same risk was found to exist for conservative treatment (applicable only to closed omphalocele) and primary surgical closure, as may be seen from statistical evaluation. The highest rate of relaparotomy occurred in the wake of dura implantation and use of silastic pouches.

Today prenatal sonographic diagnosis of abdominal defects and stenoses of the intestine is possible without few exceptions during general screening in the 20th week of gestation. In a retrospective study, 56 malformations in this area were investigated, which had been diagnosed at the University Hospital Freiburg during 1973 and 1985. Besides the criteria of sonographic diagnosis the etiology, incidence, prognostic factors, the course of pregnancy and delivery and the postpartal management are described. The rate of direct sonographic diagnosis in this area has increased from 26% up to 91%. The worst prognosis was found in infants with diaphragmatic hernias and exomphalos, whereas infants with stenosis of duodenum and small intestine had the best prognosis.

An extensive muscle-fascia-peritoneum defect of the abdominal wall was closed by means of an implant of preserved human dura mater in rats. The behaviour of the implant was tested by macroscopic, microscopic, and mechanical examination until the 60th week after the operation. Microscopic investigations showed a predominance of absorption to the 12th week later the ingrowing of collagenous fibres. Mechanical investigations demonstrated a considerable reduction of the tensile strength until the 12th week and a slight increase afterwards. An advanced protrusion of the abdominal wall formed in reach of the implant after the 30th week. The implant transformed itself to a scarlike connective-tissue membrane of poor quality in the end. The mechanical function as abdominal wall was not accomplished effectively.

Pulse oximetry has been recently introduced into anesthetic practice as an additional monitoring technique. In contrast to other methods (ECG, inspection, auscultation, blood gas analysis), it immediately detects an impending lack of oxygen, whatever its cause. Therefore, especially in pediatric risk patients, precious time can be saved. This is illustrated during the perioperative period for correction of gastroschisis. Even with a critical look at the mishaps of this method - overestimation of true O2 saturation (sO2) and partial O2 saturation (psO2) and overestimation of hemoglobin derivatives--pulse oximetry seems superior to the established monitoring techniques in the perioperative period. Therefore, we believe pulse oximetry to be an useful tool for additional monitoring, especially in pediatric risk patients. It is a suitable continuous and noninvasive in vivo technique for the early detection of hypooxygenation. Due to the special disadvantages of the method, we consider it advisable to perform control measurements with a CO-oximeter from time to time.

The results of operative treatment of gastroschisis have been improving in recent years. The survival rate is now between 50 and 60 p.c. It is very important to perform primary repair as soon as possible after birth. According to our own experiences resection of the bowel should be avoided as it gives a poor prognosis. Combined malformations of gastroschisis are very rare and their treatment should be tired since there is no other alternative. An own successfully treated case of combined malformations of gastroschisis with atresia of the small bowel is presented. Stenosis of the duodenum and tubular duplication are described

The advent of new and improved diagnostic methods has had favourable bearings on therapeutic achievements in paediatric surgery. Ultrasonography, computer tomography, manometry, and endoscopy are major examples of relevance. Prenatal diagnostic ultrasonography has been assuming a growing role in prenatal detection of malformations. Postnatal corrective surgery can thus be properly planned and performed under conditions which are optimum for mother and child. Many examples are given to demonstrate that in today's paediatric surgery possibilities for diagnosis and therapy have substantially improved, last but not least, owing to efficient interdisciplinary cooperation.

Since 1971, 35 children with prenatally ruptured omphalocele and paraumbilical abdominal wall defects were operated on in the surgical clinic of the Cologne Children's Hospital. 45.7% of them survived compared with 29.2% in earlier years. This improved chance of survival is attributed the use of the child's own fetal membranes.

Omphaloceles are congenital malformations with herniation of the abdominal viscera into the umbilical cord. The incidence in newborns is 1:4300, the sex ratio 1.24:1 in favour to boys. Children with this malformation are neither more frequent at the beginning nor at the end of the sibship; the average maternal age is not increased. In about 50% other different malformations are found. All those omphaloceles, which are found as an obligatory symptom in the EMG syndrome (Wiedemann-Beckwith syndrome) to be probably in most cases autosomal recessively inherited or which are seen within a chromosomal malformation syndrome are of etiologically known origin. Omphaloceles without other malformations are mainly sporadic, less frequent in sibs, possibly due to a polygenic or multifactorial mode of inheritance. Findings in population genetics and animal experiments suggest that exogenous factors, too, can be the cause of malformation syndromes with omphaloceles.

Whereas the omphalocele develops from a malformation of the mesoderm during the embryonal period, the gastroschisis appears to be the result of an intrauterine vascular occlusion; defined as disruption. Definite signs of malformations are seen in omphalocele. In our patient population observed during a three year period, eight cases of abdominal wall defects were seen, four of which with Wiedemann-Beckwith syndrome and one with trisomy 18, two with an isolated omphalocele and one with a gastroschisis. The concomitance of genetic malformation syndromes implies the necessity of intrauterine diagnosis evaluation. In proven cases of chromosomopathies and signs of omphalocele in ultrasound an interruption of pregnancy may be indicated. Similarly with very unfavourable prognosis such as Edwards syndrome, a post partum operative intervention is not necessary. This decision however can only be reached through interdisciplinary consultation between the pediatrician, pediatric surgeon, midwife and parents.

Judging according to the frequent description of the syndrome of aplasia of the abdominal muscle above all in paediatric literature (up to now more than 250 cases), the knowledge of the symptomocomplex (aplasia of the abdominal muscle, malformations of the kidneys and the urinary tracts, cryptorchism) might be of particular interest for the urologist, since the urologic malformations decisively determine the prognosis quoad vitam of these patients. On the basis of three own cases the importance of the removal of infravesical obstruction is emphasized, whereas the reconstructive intervention for dilatation of upper urinary tracts is seldom mandatory.

24 newborns with omphalocele and 23 with gastroschisis were operated upon in the 10-year period from 1971 to 1981. Mortality was 41% in omphaloceles and 61% in gastroschisis. The mortality of all cases dropped from 77% in the first 5-year period to 28% in the second half of the series. While the operative techniques remained unchanged, the better prognosis of the latter group is associated with better parenteral nutrition and fewer relaparotomies.

Reported in this paper are twelve cases of ventral schistasis transferred for cooperative prenatal assessment, diagnosis, and therapy to the Berlin Charité Hospital of Gynaecology and Obstetrics in 1985 and 1986. Concomitant malformations were recorded from eight in ten omphaloceles. Three of these children were delivered on full term by spontaneous parturition or caesarean section. They were surgically treated immediately after birth. Gastroschisis was recorded from two pregnancies with ventral schistasis. No generally valid recommendation can be given for approach to ventral schistasis. The approach should rather be interdisciplinary and should be preceded by careful diagnosis. Parents should be involved in decision-making.

Combined alpha-fetoprotein screening in serum and specified sonographic examination has proved useful in prenatal diagnosis of neural tube and abdominal wall malformations. 207 amniocenteses were performed within a period of 3 years due to pathologic serum alpha-fetoprotein values and/or abnormal sonographic findings. 101 amniotic fluid specimens showed an increase of alpha-fetoprotein of more than 2.5 times the average value. 60 malformed neural tubes, 17 foetuses with abdominal wall defects, as well as 2 other malformations, were diagnosed. "Open" defects, as well as 17 false positive AFP values, were discovered through the electrophoretic presence of ZNS-specific acetylcholinesterase in the amniotic fluid.

A case is presented of complete aplasia of the umbilical cord with concomitant severe anomalies of the fetus at 19 week's gestation. The sonographic findings are demonstrated and the pathogenesis of this rare malformation is discussed.

5 Male children exhibiting the typical features of the Prune-Belly syndrome (malformations of the urogenital tract, aplasia of abdominal muscles) are described. Two of these are deaf. This syndrome has also been observed in a few females. Final interpretation of the cause and pathogenesis cannot yet be given. In two siblings with defects of the abdominal muscles an contractures of the joints, but without renal anomalies, a new and hitherto undescribed hereditary syndrome seems to be present.

A case of dysplasia umbilico-fetalis is demonstrated. The malformation comprises a rare and strange reduction anomaly of the feto-umbilical unit, which is caused during early embryonic life (around the 7 mm stage, corresponding to the end of the third week of gestation). The cause of the damage is not known. The folding off of the embryo from the yolk sac and the development of the abdominal stalk are impaired. No abdominal wall is formed, and the umbilicus and umbilical cord are not developed. The abdominal organs are enclosed in a short amnion-mesoderm tube, which is bordered by the retroperitoneum at the fetal side and by the chorionic plate at the placenta side. The umbilical vessels are only a few centimeters long and traverse in the mesodermal layer of the amnion-mesoderm tube. The thin wall of the tube usually ruptures before birth, thus causing an abacterial fibrinoid peritonitis by the chemical irritation of the peritoneum through the constituents of the amniotic fluid. The lower extremities of the fetus reveal varying degrees of reduction deformities. One leg and large parts of the pelvis may be entirely missing, if the side of origin of the extremity is included in the amnion-mesoderm tube. Severe kyphoscoliosis is probably a secondary phenomenon. In addition, malformations of the inner organs occur such as caudal displacement and hernia of the diaphragma, hypoplasia of the lungs, dysplasia of the genito-urinary tract and non-rotation of the gut. The pathophysiology of the rare developmental defect and its secondary implications are discussed with special reference to related malformations such as omphalocele and infraumbilical defect of the abdominal wall.

31 infants with anterior abdominal wall defects were treated over the past 10 years. Despite the falling birth rate gastroschisis occurred almost twice as often as omphalocele (19 and 12 cases, respectively). The mortality rate of 71% in omphalocele were related to large defects, rupture of the sac and associated cardiac malformations. The 73% incidence of prematurity, additional malformations and sepsis contributed to the mortality rate of 58% in gastroschisis.

Congenital diaphragmatic hernia is associated with a high mortality, especially when combined with other defects. The unusual repeated combination of the same congenital abnormalities by a number of patients and the fact that during embryonic life these organs develop from the mesoderm implies the existence of a specific syndrome. This article summarises the available clinical data and reports on three cases with Cantrell's syndrome, showing different pattern of congenital defects such as: 1) supraumbilical abdominal wall defects; 2) a defect of the lower sternum; 3) a deficiency of the anterior diaphragm; 4) a defect in the diaphragmatic pericardium; 5) congenital intracardiac defects.

Between 1979 and 1985 plastic closures with lyophilised softened dura mater were performed in 45 children with the following indications: gastrochisis: 8; omphaloceles: 2; meningomyeloceles: 29; meningoceles: 3; diaphragmatic aplasia: 1; oesophageal atresia with tracheo-oesophageal fistula: 2. Infection as well as rejection of the implants were never observed. Mortality came up to 11%. Complications and causes of death are described. A follow-up was carried out postoperatively at semi-annual intervals. The scars showed good functional and cosmetic results.