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PubMed1987

A retrospective study of 91 cases with gastroschisis or omphalocele 1956-1985.

The experiences of treatment of 91 infants with abdominal wall defects during thirty years are presented. The occurrence of gastroschisis increased during the last ten years. The results of treatment have improved markedly during the period of study but there is still a high mortality rate in children with omphalocele and multiple concomitant malformations and in children with gastroschisis and coexisting intestinal atresia. In cases with large omphalocele defects, if primary closure is not possible, an implant is recommended instead of silastic silo, skin flap closure or mercurochrome application. The clinical course in thirty-six patients during the last ten years is reported.

Abdominal Muscles↗
PubMed1984

[Current aspects of laparoschisis. Apropos of 22 cases].

Study about 22 gastroschisis observed 17 years along allows to the authors to point out the elements of amelioration of prognostic and decrease of mortality. Antenatal diagnosis allows the hysterotomy at the end of gestation avoiding traumatic or infectious risks. Reanimation and transport are very important to bring the neonate in good conditions to the pediatric surgeon. The choice of surgical technics is only between primitive parietal closure or progressive reintegration (Schuster). Intestinal atresia must be cured in the same time, if possible. Finally, perisurgical nursing, antibiotherapy and recent parenteral nutrition method improved the prognosis. During the last 15 years, mortality has fallen from 80% to 10%.

Abdominal Muscles↗
PubMed1980

Gastroschisis update.

Sixty-four infants with gastroschisis have been managed in the 9-yr period, 1970-1979, with four postoperative deaths. The silo technique has been the standard method of management in this series, permitting initial expansion of the abdominal cavity without increased abdominal pressure and respiratory embarassment. Removal of the silo and complete closure of the abdominal wall deficit were possible 5-12 days later. A high proportion of the infants were below 2500 g in weight at birth (61%); and although 3 of the 4 postoperative deaths occurred in the low birth weight group, this did not appear to be a factor in mortality. Associated anomalies occurred in 25 of the 64, but only the coincident intestinal atresias (6) were of major significance. These additional anomalies were not responsible for deaths. Two deaths occurred from problems dating from birth, one from aspiration and the second from sepsis. The other two resulted from postoperative complications resulting in infarction of the midgut. Both were caused in part by failure to adequately enlarge the abdominal wall defect at the time of the initial procedure. Other postoperative complications were relatively few. Although all required intravenous nutritional support, the long term results in terms of growth and development and of intestinal function were quite satisfactory.

Abdominal Muscles↗
PubMed1988

[Laparoschisis. Hypotrophy and intestinal lesions].

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.

Amniotic Fluid↗
PubMed2004

Intussusception in neonates: analysis of 14 Japanese patients.

OBJECTIVE: To clarify the clinical features and pathogenesis of intussusception in neonates. METHODS: Fourteen neonates were diagnosed with intussusception between June 1974 and January 2001. Patients were divided into two groups according to whether or not signs were present on the first day of life. The clinical features were interrelated with the pathological findings. RESULTS: All six patients in the group whose signs were present on the first day of life also had intestinal atresia or malrotation. Among patients whose signs began less than 24 h after birth, five of eight patients suffered hypoxia. Moreover, it was very difficult to establish the diagnosis of intussusception, particularly in the group of late-onset type intussusception. CONCLUSIONS: Hypoxic events may play a crucial aetiologic role in the pathogenesis of late-onset neonatal intussusception.

Female↗
PubMed1999

Congenital hernia of the abdominal wall: a differential diagnosis of fetal abdominal wall defects.

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.

Abdominal Muscles↗
PubMed2006

Mothers' adaptation to antenatal diagnosis of surgically correctable anomalies.

OBJECTIVE: To assess the applicability of Drotar model when the diagnosis of congenital malformation is made antenatally. METHODS: In a 3-year period (2000-2003) fifty mothers, counselled for fetal malformations amenable to surgical correction at birth, were interviewed. Statistical associations were sought between each stage of the adaptation process and type of anomaly, gestational age at diagnosis, maternal age, educational background and previous miscarriage. Emotional experience at each stage was studied as dichotomous variables. RESULTS: Fetuses were affected by the following anomalies: abdominal wall defects (11), intestinal atresia (14) and diaphragmatic hernia (25). All mothers experienced stage one, two and three. No association was found between anger, type of anomaly, maternal age, educational background, and age at diagnosis. On the contrary, such association was statistically significant for previous miscarriage. Thirty-nine mothers reached the adaptation stage while none of them experienced that of reorganization. CONCLUSION: Despite significant differences, the adaptation process proposed by Drotar to describe parents reactions is applicable in prenatal age.

Abdominal Wall↗
PubMed2006

Multidisciplinary surgical approach to a surviving infant with sirenomelia.

Sirenomelia is an extremely complex and rare malformation with different degrees of lower-extremities fusion associated with gastrointestinal, musculoskeletal, vascular, cardiopulmonary, and central nervous system malformations. In the English literature, there are only 5 reports of infants surviving with this condition. In our case, a 2540-g female infant was born with normal vital signs, no facial dysmorphism, and a complete soft tissue fusion of the lower limbs, from perineum to ankles. Radiologic examinations revealed an intestinal atresia and a single pelvic kidney, with a unique ureter, 2 femurs, 2 tibias, 2 fibulas, and 2 feet (simpus dipus). At 7 months of age, a multidisciplinary surgical team achieved complete separation of the lower limbs, with independent vascular and nerve supplies. At the time of this writing, the infant was 28 months old and had a regular growth curve. Many future reconstructive surgeries have been planned to achieve an acceptable quality of life for this infant.

Abnormalities, Multiple↗
PubMed1998

Is a normally functioning gastrointestinal tract necessary for normal growth in late gestation?

It is known that neonates with congenital abnormalities of the intestine tend to be growth-retarded. We wished to explore the hypothesis that normal fetal gut function is needed for normal growth in late gestation. If this is true, then different populations of babies with different congenital gut abnormalities would be expected to have similar impairments of growth and be small at birth. This growth retardation would be more marked in term than in preterm babies and would be independent of other congenital anomalies. To test these hypotheses, we examined 43 babies born with gastroschisis (GS) in Auckland, New Zealand; 69 babies born with GS in Birmingham, England; and 60 babies born with intestinal atresia (IA) in Auckland. For Auckland babies with GS, the mean weight standard deviation score (WSDS) (i.e., birth weight relative to the mean birth weight for gestation) for term babies was lower than that for preterm babies (-0.932+/-0.180 vs -0.064+/-0.237, P=0.014). This was also true for Birmingham babies with GS (-0.991+/-0.193 vs -0.36 +/-0.153, P=0.028). For babies with IA, the mean WSDS for term babies was lower than that for preterm babies (-0.627+/-0.266 vs 0. 057+/-0.211, P=0.034). There was no significant difference between the mean WSDS of babies with and without major congenital abnormalities (-0.402+/-0.201 vs -0.271, P=0.70). Our results demonstrate that term babies born with GS are significantly growth-retarded compared with premature babies born with GS. Term babies born with a proximal IA are also growth-retarded. This strongly suggests that in late gestation, the normal growth is dependent on a normally functioning gastrointestinal tract that allows exposure of the proximal intestinal mucosa to ingested amniotic fluid.

Abdominal Muscles↗
PubMed1999

Intestinal lengthening and growth hormone in extreme short bowel syndrome: a case report.

Recombinant growth hormone (GH) has been used successfully to promote mucosal intestinal adaptation in adults with short bowel syndrome. Here the authors report the case of an infant with extreme short bowel syndrome successfully treated with intestinal lengthening and GH. The patient was born with intestinal atresia leaving only 12 cm of jejunum and 2 cm of ileum with ileocecal valve. A Bianchi procedure extended the length to 25 cm, but 6 months later she still received 50% of her nutritional requirements by parenteral route. A 4-week course of 0.5 U/kg of GH dramatically changed the situation allowing weaning of total parenteral nutrition (TPN) 2 weeks after the end of the treatment with striking nutritional improvement, decrease in the number of stools from 7 to 2 per day, and no secondary effects. The authors believe that GH deserves clinical trial in cases of extreme short bowel.

Enteral Nutrition↗
PubMed1998

Study of Down syndrome in 238,942 consecutive births.

The genetics and the epidemiology of Down syndrome (DS) was studied in the area which is covered by our registry of congenital malformations. For each of the 398 new DS cases which were ascertained during the period 1979 to 1996 more than 50 factors were studied and compared to those from control infants. The prevalence of DS was 1.66 per 1000; 2.2% of the DS cases were stillbirths and 29.4% were induced abortions. Karyotypes were obtained in 391 cases of which all but 23 were 47,+21;9 were mosaics (2.3%), and 14 had translocations (3.6%). Interchromosomal effect was a question in 7 cases. The most common types of associated malformations were cardiac anomalies (46.2%) and intestinal atresias (6.0%). Seasonality or time/space clusters were not observed in spite of the Chernobyl nuclear accident. No paternal age effect was demonstrated; 5.3% of the mothers of DS had 2 previous spontaneous abortions (controls 3.7% p < 0.05). At birth, the DS infants measured and weighted less and their head circumference was lower than in control infants. Weight of placenta was also lower than in control infants. In this material there were 4.5% of consanguineous marriages (P < 0.01). The pregnancies of the DS children were more often complicated by threatened abortions than in the controls, 3.2% of the mothers of the DS children were diabetic controls (1.7%), although the difference was not statistically significant. For all other factors studied no statistically significant difference with respect to controls could be demonstrated.

Abnormalities, Multiple↗
PubMed2003

Is adrenomedullin involved in the pathophysiology of persistent pulmonary hypertension of the newborn?

Although adrenomedullin (ADM) is a potent vasodilating peptide reported to play a possible role in the mechanisms of fetal lung differentiation and maturation, the ADM blood level in fetuses and in neonates with persistent pulmonary hypertension (PPHN) and pulmonary hypoplasia is not known. Therefore, we examined 15 patients with PPHN: 10 with congenital diaphragmatic hernia, four with congenital cystic adenomatoid malformation of the lung, and one with misalignment of pulmonary vessels with alveolar capillary dysplasia. Eight surgical patients with neonatal conditions such as intestinal atresia served as controls. Blood samples were drawn from the umbilical artery and vein at birth, and arterial blood was drawn from patients with PPHN on the 3rd and 6th days after birth. Plasma levels of ADM were measured by radiometric assay. Plasma levels of ADM in the umbilical artery and vein were elevated in patients with PPHN compared with controls, and in all groups the levels in the umbilical vein were higher than those in the umbilical artery. The arterial levels in patients with poor prognoses were elevated on the 3rd and 6th days after birth compared with those in survivors. These results indicate that ADM may be involved in the pathophysiology of PPHN and in the mechanisms of lung differentiation and/or maturation.

Adrenomedullin↗
PubMed1978

Intraluminal calcifications in the small bowel of newborn infants with total colonic aganglionosis.

Intraluminal calcifications were found in the small bowel of 4 newborns with total colonic aganglionosis. Abdominal radiography demonstrated circular aggregations of small punctate calcific densities in the right lower quadrant and evidence of bowel obstruction. There was a microcolon in each case. The calcifications, which resemble those seen in small intestinal atresia and stenosis, are probably related to fetal intestinal stasis, and may be differentiated from those due to meconium peritonitis.

Calcinosis↗