Pregastroschisis.

In this report, a patient with an interesting umbilical anomaly has been presented. There was a skin projection on the right side of the normal umbilical cord and a sac was extending from there. The sac contained some intestine and abdominal musculature was found normal. These findings revealed us to define the anomaly as "pregastroschisis".

Parasitic conjoined twins with omphalocele and tetralogy of Fallot.

Human parasitic twins are very rare. Here we report a unique case of a partial twin attached to the host in the midline at the forehead, chin, chest, and epigastrium. The parasite lacked thoracic organs and major neural tube derivatives. However, it had small peripheral nerves and ganglia within perirenal and pericolonic connective tissue. Also present were a well-developed small intestine, colon, and appendix with normal submucosal and myenteric plexuses. These findings may represent either the initial presence of a neural tube that later regressed or migration of autosite neural crest cells. The parasite had a mature, functioning kidney with its ureter opening to skin and complete absence of urinary bladder or genital organs. This raises questions about the embryological development of the ureteric bud, which is an outgrowth of the mesonephric duct. The host had tetralogy of Fallot and omphalocele containing the parasitic kidney and bowel. Parasitic twinning occurs at 3 weeks of gestation, tetralogy of Fallot at 3-7 weeks, and omphalocele at 6-10 weeks. A single noxa acting at 3 weeks could have caused sequential malformations that initially seem unrelated.

[Prenatal sonographic diagnosis of abdominal wall defects and stenoses of the intestinal tract]. / Pränatale sonographische Diagnose von Bauchwand-defekten und Stenosen des Intestinaltraktes.

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.

[Laparoschisis. Hypotrophy and intestinal lesions]. / Laparoschisis. Hypotrophie et lésions intestinales.

37 cases of gastroschisis were operated on at Saint-Vincent-de-Paul's Hospital from january 1983 to july 1987. They were more frequently premature by birth weights (70% below the 10 th percentile), than by period of gestation (40%). The comparison of their weights plotted on the centile charts with 28 neonates with omphaloceles and 25 neonates with upper intestinal atresia (esophageal atresia without tracheoesophageal fistula and complete duodenal and proximal jejunal atresia) shows that the 2 groups of laparoschisis and anomalies of the alimentary tract are quite similar. The authors suggest that the amniotic fluid may play a role in the fetal growth so that the alteration of the herniated bowel is responsible of the fetal growth retardation observed in gastroschisis.

[193 cases of gastroschisis and omphalocele--postoperative results]. / 193 Fälle von Gastroschisis und Omphalozele--Postoperative Ergebnisse.

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.

Cloacal exstrophy: a report of 34 cases.

A clinical review is presented on 34 patients with cloacal exstrophy who were seen between 1963 and 1986. The patients were separated into 2 main groups: classical cloacal exstrophy (type I) and variant cloacal exstrophy (type II). In the classical cases 3 surface patterns were recognized: A-hemibladders confluent cranial to the bowel, B-hemibladders lateral to the bowel and C-hemibladders confluent caudal to the bowel. Surgical reconstruction was performed in 24 patients, with a 50 per cent survival rate. However, there was marked improvement in survival from 22 per cent between 1963 and 1978 to 90 per cent between 1979 and 1986.

Cloacal exstrophy and cloacal exstrophy variants: a proposed system of classification.

A coding system that documents the abnormalities within the cloacal exstrophy complex is presented. Analysis allows the classification of these abnormalities into classical and variant series in a logical manner. The application of the coding system to selected reported material is described and it demonstrates the similarities between apparently divergent cases.

[Intestinal malformations and congenital heart diseases]. / Malformations intestinales et cardiopathies congénitales.

A series of 21 patients with both congenital heart disease and intestinal malformation seen over a 12-year period is reported. The intestinal malformations were: anorectal malformations (11 cases), duodenal atresia (5), omphalocele (4) and common mesentery (1). Congenital heart diseases consisted of: atrial septal defect (ASD) (10 cases), ventricular septal defect (VSD) (2), tetralogy of Fallot (2) and miscellaneous cardiopathies. In patients with anorectal malformations ASD and VSD predominated (6/11 cases) and multiple malformations syndromes were present in 8 cases, including trisomy 13, Vater syndrome, skeletal (4), neurological (3) and renal abnormalities (3); 3 children died. Duodenal atresia was always associated with left-to-right shunt: VSD (3), ductus arteriosus (2), complete atrioventricular canal (1) and trisomy 21 (2); one child died. Omphalocele coexisted with VSD (2), tetralogy of Fallot (1), dual outlet right ventricle (1), trisomy 21 (1) and multiple malformations syndromes (3); 2 children died. The patient with common mesentery had left-to-right shunt. Comparison of this series with data from the literature showed that children with congenital heart disease have a much higher incidence of intestinal malformations than those with normal heart and that they frequently present with multiple malformations (chromosome aberrations or multiple organ lesions). This multiple malformation complex is particularly common in anorectal malformations where the incidence of congenital heart diseases is 9 to 14%, with predominance of VSD and tetralogy of Fallot. In patients with duodenal atresia trisomy 21 is extremely frequent, and the incidence of cardiopathies is 18%; in the absence of trisomy 21 the cardiopathy is complex.(ABSTRACT TRUNCATED AT 250 WORDS)

Prune belly syndrome associated with exomphalos and anorectal agenesis.

We have recently treated a child with prune belly syndrome in association with exomphalos and anorectal agenesis. This case raises the total incidence of exomphalos and anorectal agenesis to four and five, respectively, in our series of 13 cases of prune belly syndrome. Our case report lays emphasis on the severe respiratory problems that can complicate surgery in prune belly patients. We also attempt to relate the pattern of anomalies in our series to the controversy surrounding the embryogenesis of prune belly syndrome and suggest that our case material lends support to the concept of prune belly syndrome being due to an early disturbance of mesodermal development in both the abdominal wall and the urinary tract. We also suggest that the reported occurrence of gastroschisis in association with prune belly syndrome adds weight to the unifying theory of the embryogenesis of gastroschisis and exomphalos.

Nonsurgical (conservative) treatment of giant omphalocele. A report of 10 cases.

Between January 1970 and December 1984, 65 patients with a congenital anterior abdominal wall defect were admitted to the Pediatric Surgical Center of the St. Radbound Hospital, Nijmegen, The Netherlands. There were 39 cases of omphalocele. Ten had giant omphalocele, defined as omphalocele that could not be closed primarily. In these cases, a conservative method of treatment was used. The results are reported of these 10 cases of giant omphalocele with special emphasis on mortality, complications, and length of hospital stay. Mean hospital stay was 95 days. Local infection did not present serious problems. Sepsis occurred in half the patients but was managed with antibiotics. There was one case of late volvulus of the stomach. All local applications, if used regularly, gave rise to complications. Two cases of hypothyroidism caused by the application of povidon-iodine and one case of alcohol intoxication were observed. In early years, one child was lost due to mercury poisoning. There was one early and one late death unrelated to the procedure.

[Clinico-genetic analysis of omphalocele]. / Kliniko-geneticheskii analiz omfalotsele.

The study of 302 cases of omphalocele in foetus and newborns revealed genetic heterogeneity of this congenital malformation. Multiple birth defects were found in 54.3% of probands, including 11% of cases (Wiedemann-Beckwith syndrome being neglected), diagnosed as syndromes with hereditary etiology. The spectrum of anomalies associated with omphalocele in non-classified complexes corresponds to that for caudal regression and cloaca extrophy syndromes. A positive syntropy index for these anomalies shows that these combinations are not coincidental. The increase of omphalocele incidence among relatives of probands with descending gradient, depending on relationship, is demonstrated, which testifies to multifactorial determination of some omphalocele cases. Empirical risk for sibs was 0.6 +/- 0.3% and the index of inheritance was 50.7 +/- 11.4%.