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At least 343 records · Page 19Linked to original sources
PubMed2005

Colonic atresia without mesenteric vascular occlusion. The role of the fibroblast growth factor 10 signaling pathway.

BACKGROUND/PURPOSE: Colonic atresia occurs in 1:20,000 live births, offering a neonatal surgical challenge. Prenatal expression of fibroblast growth factor 10 (Fgf10), acting through fibroblast growth factor receptor 2b (Fgfr2b), is critical to the normal development of the colon. Invalidation of the Fgf10 pathway results in colonic atresia, inherited in an autosomal recessive pattern. Classically, disturbance of the mesenteric vasculature has been thought to cause many forms of intestinal atresia. The purpose of this study was to evaluate the role of vascular occlusion in the pathogenesis of colonic atresia. METHODS: Wild type (Wt), Fgf10(-/-), and Fgfr2b(-/-) mutant mouse embryos were harvested from timed pregnant mothers. Immediately following harvest, filtered India ink was infused via intracardiac microinjection. The gastrointestinal tract was dissected, and photomicrographs of the mesenteric arterial anatomy were taken at key developmental time points. RESULTS: Photomicrographs after India ink microinjections demonstrate normal, patent mesenteric cascades to the atretic colon at the time points corresponding to the failure of colonic development in the Fgf10(-/-) and Fgfr2b(-/-) mutants. The mesenteric arterial anatomy of the colon demonstrates no difference between the Wt and mutant colonic atresia. CONCLUSIONS: The absence of embryonic expression of Fgf10 or its receptor Fgfr2b results in colonic atresia in mice. India ink microinjection is a direct measure of mesenteric arterial patency. Colonic atresia in the Fgf10(-/-) and Fgfr2b(-/-) mutants occurs despite normal mesenteric vascular development. Thus the atresia is not the result of a mesenteric vascular occlusion. The patent colonic mesentery of the Fgf10(-/-) and Fgfr2b(-/-) mutants challenges an accepted pathogenesis of intestinal atresia. Although colonic atresia can occur as a result of vascular occlusion, new evidence exists to suggest that a genetic mechanism may play a role in the pathogenesis of this disease.

Animals↗
PubMed2004

A genetic mechanism for cecal atresia: the role of the Fgf10 signaling pathway.

BACKGROUND: Intestinal atresia represents a significant surgically correctable cause of intestinal obstruction in neonates. Intestinal development proceeds as a tube-like structure with differentiation along its axis. As the intestine differentiates, the cecum develops at the transition from small to large intestine. Fgf10 is known to serve a key role in budding morphogenesis; however, little is known about its role in the development of this transitional structure. Here we evaluate the effect of Fgf10/Fgfr2b invalidation on the developing cecum. MATERIALS AND METHODS: Wild-type C57Bl/6, Fgf10(-/-), and Fgfr2b(-/-) embryos harvested from timed pregnant mothers were analyzed for cecal phenotype, Fgf10 expression, and differentiation of smooth muscle actin. RESULTS: Wt cecal development is first evident at E11.5. FGF10 is discreetly expressed in the area of the developing cecum at early stages of development. One hundred percent of Fgf10(-/-) and Fgfr2b(-/-) mutant embryos demonstrate cecal atresia with absence of epithelial and muscular layers. The development of neighboring anatomical structures such as the ileocecal valve is not affected by Fgf10/Fgfr2b invalidation. CONCLUSIONS: FGF10 expression is localized to the cecum early in the normal development of the cecum. Fgf10(-/-) and Fgfr2b(-/-) mutant embryos demonstrate cecal atresia with complete penetrance. Epithelial and muscular layers of the cecum are not present in the atretic cecum. The Fgf10(-/-) and Fgfr2b(-/-) mutants represent a genetically reproducible animal model of autosomal recessive intestinal atresia.

Animals↗
PubMed2005

[New surgical strategy in gastroschisis: treatment simplification according to its physiopathology].

INTRODUCTION: Gastroschisis (GS) is a congenital abdominal wall defect that permits bowel exposure to amniotic fluid (AF). Intestinal damage is related to the chemical action of AF and constriction. After birth at term, a thickened intestinal wall with inflammation and, in some cases, intestinal atresias were observed. Surgical repair and intestinal reubication may be difficult, and thus staged silo repair could be necessary. These patients require a long hospital stay owing to bowel damage causing severe intestinal hypoperistalsis and poor absorptive capacity. Total parenteral nutrition (TPN) is required for a long period. OBJECTIVE: The aim of this prospective study is to evaluate the benefits of a preterm delivery to avoid bowel damage and its post-natal consequences. PATIENTS AND METHODS: Six cases of prenatally-diagnosed GS have been treated following a new strategy since July 2002. A preterm Cesarean section (c-section) delivery was programmed at 34-35 weeks of gestational age (GA). Some hours after birth, at bedside in the NICU, bowel reduction through the defect hole was performed under general anesthesia. This preterm group (PT) was compared the past 6 cases at term (AT) from January 1998 to July 2002. Macroscopic appearance, atresia existence, surgical technique, silo requirement, neonatal outcome, TPN and hospital stay were analyzed. RESULTS: All six cases AT (mean GA: 36.3 weeks) presented bowel inflammation and thickened wall. Only 2/6 cases allowed the intestine to be housed in a primary closure after laparotomy. 4/6 cases required staged silo repair. 1 patient presented intestinal atresia and other had perforations who died at 17 days of life from intestinal sepsis. Mean postoperative intubation period was 16.2 days. Mean TPN was 41.2 days and mean hospital stay 69.8 days. PT group was monitored by prenatal sonography seeking bowel sonolucency. After programmed PT c-section delivery (mean GA: 34.8 weeks) in all 6 cases, bowel loops presented normal appearance and intestinal thickening was absent, except in one case. No prematurity-related respiratory complications were observed. Easy bowel reduction without abdominal compression was performed in all cases. 1/6 cases required surgical release of occlusive intestinal adherence. Mean postoperative intubation period was 0.4 days (9.6 hours). Oral feeding was started at 6 days. Mean TPN was 13.4 days and mean hospital stay 28.6 days. CONCLUSIONS: The third trimester is a critical period for fetal bowel development. Intestinal damage rises with increasing exposure time to amniotic fluid. This strategy of preterm delivery for the treatment of GS avoids intestinal damage, prevents "peel" and intestinal atresia, renders surgical reduction easier, reduces the hypoperistalsis, need for TPN and hospital stay. Multidisciplinary coordination between obstetricians, neonatologists and pediatric surgeons is required.

Digestive System Surgical Procedures↗
PubMed1990

Placental emboli from a fetus papyraceous.

A syndrome in monozygotic twins that consists of a macerated twin fetus (fetus papyraceous) and a live-born twin with various anatomical defects has been described. The etiology is thought to be placental transfer of emboli or thromboplastic material through vascular shunts. Thromboplastic material precipitates disseminated intravascular coagulation (DIC) in the fetus, with a resultant hypercoagulable state due to relative fetal antithrombin III deficiency. Two cases of this syndrome will be discussed. The case of a live-born twin with intestinal atresia, who developed in utero with a fetus papyraceous, is reported. Emboli were demonstrated in vascular shunts of the diamniotic-monochorionic placenta. The hypothesis of intestinal atresia as a result of a vascular accident is reviewed. Another case involving a live-born twin with congenital skin defects, who developed in utero with a fetus papyraceous, is also reported. The skin defects were a congenital disruption from fetal DIC with resultant hypercoagulable state. Several other manifestations of the placental emboli syndrome will be discussed and the vascular etiology of the disruptions explained.

Adult↗
PubMed2002

Midgut atresias result from abnormal development of the notochord in an Adriamycin rat model.

BACKGROUND/PURPOSE: Prenatal exposure to Adriamycin in a rat model (ARM) has been reported to lead to a spectrum of tracheoesophageal and associated malformations of the gastrointestinal tract, including multiple intestinal atresias. An abnormal relationship of the notochord with the foregut has been implicated in the formation of esophageal atresias. The authors hypothesised that midgut atresias arise from abnormal notochord development in the region of the midgut. This study was designed to examine the gut-notochord relationship during early embryonic development. METHODS: Timed pregnant Wistar rats were given 1.75 mg/kg of Adriamycin intraperitoneally on days 7, 8, and 9 of gestation. Embryos were recovered at 12-hour intervals from days 9.5 to 14, and at term. A control group was given saline instead of Adriamycin. Embryos were embedded in resin or wax, sectioned, and studied using light microscopy, paying particular attention to the notochord and surrounding structures. RESULTS: The notochord appeared identical in controls and experimental embryos on day 9.5. However, on day 10.5 the notochord was diffusely abnormal in ARM, distorted, and tethered to foregut as well as midgut compared with controls. This abnormality was not seen in control embryos. On day 12 the notochord abnormalities were more exaggerated in the region of the midgut in ARM embryos. Full-term ARM animals had esophageal and multiple intestinal atresias. CONCLUSIONS: The notochord is abnormal in the region of the developing midgut, and this may account for the occurrence of atresias found in this region.

Animals↗
PubMed1992

Further evidence that arthrogryposis multiplex congenita in the human sometimes is caused by an intrauterine vascular accident.

A 7 1/2-year-old girl with arthrogryposis multiplex congenita of the amyoplasia type in association with intestinal atresias, gastroschisis, Möbius anomaly, and hypoplasia of the pectoral, biceps, and deltoid muscles is described. Several combinations of these birth defects have been previously described. There is considerable evidence that gastroschisis, intestinal atresia, Poland sequence, and Möbius anomaly each has a vascular pathogenesis. Based on the associations seen in this child and past reports of more limited, similar cooccurrences, we suggest that arthrogryposis multiplex congenita may sometimes be caused by an intrauterine vascular catastrophe.

Abdominal Muscles↗
PubMed2002

Unusual presentations of gastroschisis.

Gastroschisis is a defect of the ventral abdominal wall nearly always located to the right of the umbilicus. A relatively low incidence of associated anomalies exists with the most common being nonduodenal intestinal atresia occurring in 10 per cent of patients. Although multiple hypotheses have been proposed the pathogenesis of gastroschisis is unproven and controversial. Herein we describe four infants with rare presentations of gastroschisis: one infant with true left-sided gastroschisis and three infants with unusual patterns of intestinal atresia. At present there is a female preponderance of left-sided gastroschisis as reported in the world literature (five female:two male). These cases illustrate the anatomic spectrum of gastroschisis as well as support intrauterine spontaneous closure of the abdominal wall defect as an etiology for midgut atresia.

Female↗
PubMed2005

Intercellular adhesion molecule (ICAM-1) response after major neonatal surgery.

Surgical stress induces systemic endocrine-metabolic responses that influence the function of endothelial cells (EC) to cause various systemic reactions. Intercellular adhesion molecule (ICAM)-1 is an adhesion molecule that plays an important role in inflammation, and increased expression of ICAM-1 on EC is a reflection of EC activation. In this study, we investigated the ICAM-1 response to surgical stress in neonates undergoing major surgery. Fifteen neonates (mean age at surgery: 3.5 +/- 1.2 days) were divided into two groups according to indications for surgery: Group I: Congenital diaphragmatic hernia without persistent pulmonary hypertension (n = 5); Group II: Gastrointestinal surgery [n = 10: duodenal atresia (n = 3), intestinal atresia (n = 6), and esophageal atresia (n = 1)]. Serum samples were obtained preoperatively, immediately after completion of surgery (time zero), and 24, 48, 72, 96, and 120 h after surgery to measure ICAM-1 levels using an enzyme-linked immunosorbent assay, C-reactive protein (CRP), and white blood cell count (WBC). Postoperative recovery was uneventful in all cases. ICAM-1 levels in both groups increased significantly within 24 h of surgery (Group I: P = 0.0038, Group II: P = 0.0320). In Group I, ICAM-1 peaked 72 h postoperatively while in Group II it continued to rise until 96 h postoperatively. The difference between peak levels reached was not significant. CRP was first detected 24 h postoperatively in both groups and continued to increase until 48 h postoperatively. Again, the difference between peak levels reached was not significant. No significant changes in WBC were observed in either group. We found that ICAM-1 increases in response to surgical stress in neonates, although there was no significant difference in levels. However, surgical stress as represented by serum ICAM-1 would appear to last longer with intestinal surgery than with non-intestinal surgery. Further research is required to establish the usefulness of ICAM-1 as an easily detectable substance associated with endothelial damage that reflects the host's response to major surgical stress.

Biomarkers↗
PubMed2002

Associated anomalies in intestinal neuronal dysplasia.

BACKGROUND/PURPOSE: Intestinal neuronal dysplasia (IND) is a complex alteration of the enteric nervous system (ENS) that may involve rectum, colon, or the whole intestine. This disorder is a frequent cause of intestinal dysmotility and pseudo-obstruction in the first 3 years of life. The aim of this study was to identify possible associations and correlations of IND with other gastrointestinal and nongastrointestinal anomalies. METHODS: From 1986 to 2000, 95 cases of IND type B without aganglionosis were diagnosed. Fifteen cases were diffuse IND, whereas the remaining 80 were rectocolonic neuronal dysplasia. The diagnosis was performed on rectal suction biopsy specimens taken 2 to 10 cm above the pectinate line. Acetylcholinesterase (AChE), lactic dehydrogenase (LDH), and NADPH-diaphorase (NADPH-d) histochemical techniques were performed on serial cryostatic sections. We used Schärli and Meier-Ruge criteria (1981) for the diagnosis of IND until 1992, when we adopted Borchard et al criteria (1991). A retrospective analysis of the clinical data was performed to identify IND-associated anomalies. RESULTS: These anomalies included anorectal malformations (9 cases), intestinal malrotation (8), megacystis (5), congenital short small bowel (4), hypertrophic pyloric stenosis (3), necrotizing enterocolitis (2), mental retardation (2), short stature (2), facial dysmorphism (2), Down syndrome (1), intestinal atresia (1), diffuse intestinal angiomatosis (1), histiocytosis (1), microvillus agenesia (1), and hearing loss (1). Overall, 43 associated anomalies were found in 29 IND cases (30.5%). Gastrointestinal anomalies accounted for 67.4% (29 of 43 anomalies) of associated disorders. The incidence of associated anomalies was higher in diffuse IND (80% of cases, 12 of 15) than in rectocolonic forms (21.2%, 17 of 80). CONCLUSIONS: Unlike Hirschsprung's disease, which is determined genetically, IND pathogenesis is unknown. The analysis of associated anomalies in IND population is an important clinical approach to investigate possible pathogenetic correlations. Two recessive syndromes were identified (3 families). The first was characterized by IND, intestinal malrotation, and congenital short bowel, the second by IND, short stature, mental retardation, and facial dysmorphism. In this study, gastrointestinal anomalies accounted for 67.4% of all associated disorders. These data suggest a strong correlation between IND and intestinal development. Abnormalities of the fetal ENS could determine the IND phenotype, which is likely to contribute to the pathogenesis of different intestinal malformations and in particular of anorectal and "rotation" anomalies.

Abnormalities, Multiple↗
PubMed1988

[Long term prognosis after massive small bowel resection in children].

Eight survivors after massive small bowel resection were reviewed for a period of 2 to 19 years to assess the long term prognosis of patients. The primary diseases of short bowel syndrome were congenital intestinal atresia (6) and midgut volvulus (2). The length of the residual small intestine ranged between 27 and 75 cm and ileocecal valve was also resected in 3 cases. All cases had been already weaned from parenteral nutrition and six children tolerate normal meals and two have still enteral formula at home now. Near normal somatic growth was achieved in cases which received intensive nutritional supports after operation. The D-Xylose absorption test revealed gradual improvement except one with the shortest intestine (27 cm) and fat absorption was disturbed in patients who had less than 45 cm residual intestine. Late metabolic complications, such as renal calculus, cholelithiasis and pathologic fractures were encountered in three cases.

Child, Preschool↗
PubMed1995

Familial intestinal polyatresia syndrome.

Familial multiple-level intestinal atresia is a rare syndrome with autosomal recessive inheritance. Only a few well-documented families have been reported in the medical literature. In this article two new cases from a consanguineous couple are presented. These are the first reported cases of Familial Intestinal Polyatresia Syndrome from Turkey. The importance of this report is that the recognition of multiple intestinal atresias as an invariably fatal syndrome will be helpful for genetic counselling and attempts at early prenatal diagnosis for successive pregnancies in these couples.

Female↗
PubMed2003

Single umbilical artery and the VATER-animal model.

BACKGROUND/PURPOSE: Vascular disruption is recognized as a cause of congenital malformations. The authors analyzed the significance of single umbilical artery (SUA) in the Adriamycin-animal model to find out if it was associated with organ malformations. METHODS: Pregnant SD rats were injected with Adriamycin intraperitoneally at a dose of 2 mg/kg on days 6 through 9 of gestation. Serial transverse sections of full-term fetuses were analyzed by light microscopy. Embryos also were removed on different gestational days during organogenesis, and serial transverse histologic sections were examined and compared with suitable controls. RESULTS: In experimental embryos (n = 47), presence of a SUA resulted from either persistence of the primitive umbilical arteries that joined each other ventral to the hind gut to give rise to one umbilical artery or from secondary atrophy of one of the definitive umbilical arteries. Malformations such as intestinal atresia were associated with anomalous fusion between the dorsal aorta and the persistent primitive umbilical arteries. CONCLUSIONS: SUA is a prominent feature of the Adriamycin-animal model. No obvious association was found between malformations and SUA that resulted from atrophy of one of the definitive umbilical arteries; however, it was associated with anomalies such as intestinal atresia when it resulted from persistence of the primitive umbilical arteries.

Abnormalities, Drug-Induced↗
PubMed2005

Evaluation of intestinal absorption after longitudinal intestinal lengthening for short bowel syndrome.

PURPOSE: The aim of this study was to answer if the longitudinal intestinal lengthening and tailoring (LILT) by Bianchi, modified by Aigrain, can allow the child to be weaned from parenteral nutrition (PN) and if the length of the bowel after the procedure can influence the results of the absorption test such as Schilling or D-xylose test. PATIENTS AND METHODS: We reviewed the files of 7 children who have had LILT from 1980 to 2003. We performed to explore 2 intestinal function tests: the D-xylose and the Schilling tests. Both were performed early (during the first year after the procedure) and late (during the second year) after the LILT. We used the chi2 and Bartlett's correlation tests for statistical analysis. RESULTS: There were 6 boys and 1 girl. The surgical indication was short bowel syndrome with parenteral nutrition owing to multiple intestinal atresia (2 cases), severe necrotizing enterocolitis with volvulus (1 case), necrotizing enterocolitis (1 case), intestinal atresia with gastroschisis (2 cases), and volvulus owing to malrotation (1 case). The length of the bowel was significantly different before and after LILT (P < .0001). After LILT, the length of the bowel was significantly correlated with the percentage of PN on energy at 6 months (P = .02) and at 12 months (P = .001). Moreover, the length of the bowel after the procedure was significantly correlated with the results of the D-xylose test during the first year (P = .002) but not with the results after the second year. The length after lengthening influenced neither the results of the Schilling test during the first nor those of the second year after. Four patients were weaned from the PN 21 months in average after the LILT (57%); 1 was not because we had only a 2-month follow-up. The average follow-up was 111 (5 months; range, 4-206). CONCLUSION: Longitudinal intestinal lengthening and tailoring for short bowel syndrome is a good option to allow children to be weaned from the PN. The length of the bowel after the procedure can influence the absorption test such as D-xylose during the first postoperative year but not during the second and does not influence the Schilling test. We think it is not necessary to perform these tests during the follow-up of these patients.

Child, Preschool↗
PubMed1994

Proton magnetic resonance spectroscopy of human amniotic fluids sampled at 17-18 weeks of pregnancy in cases of decreased digestive enzyme activities and detected cystic fibrosis.

Low digestive enzyme activities in human amniotic fluid can be observed in normal and disease-affected pregnancies: cystic fibrosis, trisomy 21, intestinal atresia. Amniotic fluids were analyzed by proton nuclear magnetic resonance (NMR) spectroscopy in order to specify prenatally the etiology of low digestive enzyme activities observed at 17-18 weeks of amenorrhea. A total of 114 amniotic fluid samples were collected at 17-18 weeks of amenorrhea. Karyotyping and assays of digestive enzyme activities were performed in all cases. Samples were divided into six groups according to enzyme activities and pathology. Proton spectra were retrospectively recorded. Many compounds, such as amino acids and carboxylic acids, were detected by NMR. The same resonance intensities (normalized to creatinine) were observed in the six groups. Nevertheless, an unidentified resonance at 1.05 ppm was detected in seven out of 13 cases of cystic fibrosis affected fetuses. The NMR spectra demonstrated the stability of the amniotic fluid composition at 17-18 weeks of amenorrhea, even when the fetus was affected by a disease such as trisomy 21 or intestinal atresia. The resonance associated with most cases of cystic fibrosis should be further investigated.

Alkaline Phosphatase↗
PubMed1992

Massive small bowel resection in neonates--is weaning from parenteral nutrition the final goal?

Eight surviving cases from an original 15 pediatric patients who underwent massive small bowel resection during their neonatal period were reviewed for a period of 2-19 years to assess long-term prognosis. the primary diseases were congenital intestinal atresia in 6 cases and midgut volvulus in 2. The length of the residual small intestine ranged from 27-75 cm and the ileocaecal valve had been resected in 3 cases. All cases were able to be weaned from parenteral nutrition and at present, 6 of the children can tolerate normal meals but 2 are still receiving enteral nutrition, in the form of a low residue diet at home. Near normal somatic growth was achieved in the most recent 5 cases who received intensive nutritional treatment in the immediate postoperative period, whereas 3 who did not receive nutritional management exhibited growth retardation. The D-Xylose absorption test revealed gradual improvement and finally normal absorption in all except one case with the shortest remaining intestine (27 cm). However, the fat absorption test revealed abnormal absorption in cases with a residual intestine of less than 45 cm. Late metabolic complications such as renal calculi, cholelithiasis and pathologic fractures were encountered in 3 cases.

Child↗
PubMed1990

[Inter-intestinal compression anastomosis in children].

Enterostomy of the afferent intestine with preliminary formation of an intestinal anastomosis with the efferent loop was carried out in 11 children. The anastomosis was created by means of a compression device of an alloy "with shape memory". Among the children who underwent operation 7 were newborns with intestinal atresia and 4 were children of an older age with peritonitis and perforation of the intestine. No operative complications were encountered. An intestinal anastomosis with a fistula-bearing intestine allows multiple complications of routine enterostomy to be avoided.

Anastomosis, Surgical↗
PubMed1982

Fetal jejunal atresia and intrauterine volvulus--a case report.

Ultrasonic B-scan not only confirms the clinical suspicion of polyhydramniosis, but will also in many cases diagnose the etiology. In case of a high intestinal atresia, two or more fluid-filled structures are seen in the fetal abdomen. In case of a congenital defect combination, intestinal atresia is generally associated with malrotation. This combination is frequently complicated by a midgut volvulus. The report describes a case of intrauterine volvulus with a surviving child. Repeat CTG-recordings in this case led to an acute caesarean section, when the CTG turned from normal to silent pattern.

Adult↗