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Prognostic factors of prenatally diagnosed gastroschisis.

OBJECTIVE: To evaluate the prognosis of prenatally diagnosed gastroschisis. STUDY DESIGN: In a retrospective study, we analyzed the clinical and echographic data of gastroschisis. These data were correlated with fetal outcome including delivery, surgical procedure, follow-up in the neonatal intensive-case unit and in the gastropediatric unit. RESULT: Twenty cases were analyzed. The overall survival rate was 85%. Classical criteria were analyzed (maximal bowel dilatation, thickening of bowel wall). Fetuses with both severe perivisceritis and meconium-stained amniotic fluid were born earlier than fetuses with mild perivisceritis and normal amniotic fluid (p < 0.01). CONCLUSION: Our data suggest that an inflammatory response could follow bowel exposure to amniotic fluid. This response could lead to perivisceritis and premature birth. This hypothesis is currently under investigation.

Adolescent↗

[Surgery of congenital defects--present trends].

The objective of the present work is a review of contemporary therapeutic possibilities of neonates with serious developmental defects. Neonatal surgery of congenital developmental defects was in recent decades favourably influenced by the development of neonatology and prenatal diagnosis. Nowadays prenatally diagnosed congenital anomaly is not a primary indication for termination of pregnancy but for comprehensive examination and consultation in a specialized centre. The definite decision regarding continuation of pregnancy or its termination is up to fully informed parents. In the majority neonatal surgery is not surgery of urgent operations, adequately stabilized neonates are operated while ensuring comprehensive extrasurgical care. The prognosis as regards survival and quality of life is favourable for the majority of isolated congenital defects. During the period 1994-2001 at the authors' departments a 73% survival of neonates with an isolated diaphragmatic hernia was recorded, 87% with omphalocele, 93% with gastroschisis, 90% with oesophageal atresia, 97% with anorectal atresia and 100% with intestinal atresia. With declining mortality, in the foreground of interest are problems of early definitive correction of long-term results and quality of life. With regard to the low incidence of different congenital anomalies and the contemporary birth rate in the Czech Republic its is justified to concentrate selected defects in centres which are able to provide comprehensive prenatal and early postnatal treatment.

Digestive System Abnormalities↗

How much of a misnomer is "asymptomatic" intestinal malrotation?

BACKGROUND: Intestinal malrotation is usually observed in the neonatal period with signs of acute high intestinal obstruction due to midgut volvulus. However, malrotation presenting beyond the neonatal period and well into adult life is associated with a variety of atypical and frequently non-specific gastrointestinal symptoms that may often cause prolonged delay in diagnosis and appropriate treatment. OBJECTIVES: To emphasize the difficulty in predicting the risk of midgut volvulus based on age or symptoms, and to recommend surgery in all patients found to have intestinal malrotation even if they are considered asymptomatic. METHODS: We reviewed 41 patients with malrotation treated over a period of 24 years at the Soroka University Medical Center. RESULTS: In our series, 27 patients (66%) had acute midgut volvulus while 14 (34%) had malrotation found during investigation of various long-term gastrointestinal non-specific symptoms. Two patients died of total parenteral nutrition-related sepsis following extensive resection of small bowel. A total of 28 patients was available for long-term follow-up and are asymptomatic. CONCLUSIONS: We recommend elective laparotomy and Ladd procedure in all patients found to have intestinal malrotation. This will prevent the catastrophic results of midgut volvulus and a variety of gastrointestinal symptoms wrongly attributed to other conditions in the span of a lifetime.

Abdominal Pain↗

Gastrointestinal perforations in neonates with anorectal malformations.

We describe the presentation and management of gastrointestinal perforation in four neonates with anorectal malformations. Two neonates with high malformation had pneumoperitoneum on X-ray; surgery revealed sigmoid perforation in one patient and transverse colon perforation in the other. Colostomy was done, followed by posterior sagittal anorectoplasty at four months; both recovered satisfactorily. The third neonate had no radiological feature of gut perforation but cecal perforation was found at surgery; the neonate recovered following right hemicolectomy with stoma followed by anorectoplasty at five months. The fourth neonate presented with clinical and radiological features of perforation and recovered satisfactorily after anoplasty and colostomy.

Digestive System Abnormalities↗

[Current concepts on gastroschisis].

Present day concepts about history, etiology, embriology, and management of gastroschisis are reviewed and a case is reported; it was treated in two surgical steps. A silastic net was applied and the intestinal loops were gradually introduced until the abdominal defect closed completely. Parenteral feeding was also given.

Abdominal Muscles↗

[Duplication of the alimentary canal in infants and children].

This is a review of 30 duplications of the alimentary tract in 28 patients treated at the Surgical Unit of the Children's Department of the Medical University of Pécs, Hungary and at the Department of Pediatric Surgery of the Medical Academy of Dresden, Germany, from 1964 to 1989. The ages of patients ranged from 1 day to 13 years, 80 per cent were less than 2 years of age at initial presentation. There were 6 thoracic, 20 abdominal and 2 thoracoabdominal duplications. Distended abdomen, vomiting, bowel obstruction and palpable abdominal mass were most frequently encountered. Plain thoracic and abdominal X-rays, ultrasonography, barium esophagogram, barium meal and enema were the most common diagnostic procedures. Emergency operative intervention was required in 18 patients. One infant died of an unrelated disease. Twenty-three duplications were cystic and 3 tubular. One patient had an appendiceal duplication, and another patient a flat lumenless duplication located on the perineum close to the anal opening. The surgical procedure--removal of the duplication--should not be more radical than necessary to eliminate the potential complaints and prevent recurrence. During surgery the common blood supply shared between the duplication and the native bowel must be carefully protected to avoid undue sacrifice of normal bowel.

Child↗

[Digestive physiopathology of the fetus].

The evolution en enzymatic activity in the amniotic fluid follows the various stages of development of the gastrointestinal tract during pregnancy. Before 12 weeks of amenorrhea, no enzymatic activity can be detected, as this period corresponds to the persistence of the pharyngeal and anal membranes. At 13-14 weeks, a very high level of enzymatic activity is suddenly observed in the amniotic fluid, reaching its peak at 16-18 weeks. This phase corresponds to the opening of the pharyngeal membrane, the appearance of swallowing and the opening of the anal membrane. After 18 weeks, the digestive enzyme level progressively decreases until 22-24 weeks, after which date no gastrointestinal enzymatic activity can be evidenced in normal fetuses (probably because of functional anorectal obstruction). We have determined the enzymatic anomalies related to some gastrointestinal deformations (duodenal atresia, cystic fibrosis, atresia of the bile ducts, anorectal atresia).

Amniotic Fluid↗

[Duplication of the intestinal tract].

Twelve duplications of the alimentary tract in eleven children are reviewed. There was a predominance of the spheric type of duplication as well as the neonatal group of children with clinical sign of tumour in most cases. There were no major problems in resection of spheric duplications but tubular type cases afforded different procedure in each case. Three cases with associated atresias were noted. There was one death related to gastric duplication.

Colon↗