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Fetal aortic isthmus growth and morphology in late gestation.

OBJECTIVE: To establish normal values for fetal aortic isthmus diameter in late gestation and to identify any changes in aortic isthmus dimensions and morphology in pathological conditions. METHODS: In this prospective study, the fetal aortic isthmus was evaluated in 110 low-risk pregnant women at between 30 and 40 weeks of gestation and 42 pregnant women who were at high risk for congenital heart defects. From coronal echocardiographic images of the connection between the aorta and ductus arteriosus, the internal diameter of the aorta was measured at the middle of the isthmus, at the point of the isthmus just proximal to the entry of the ductus arteriosus and at the descending aorta below the entry of the ductus arteriosus. RESULTS: Correlation coefficients for the diameter of each aortic segment when related to gestational age varied from r = 0.60 to r = 0.80 (P < 0.001 for each), and growth curves were derived from the third and 97th percentiles about each linear regression analysis. The mean and the third percentile for the ratio of the isthmus just proximal to the entry of the ductus arteriosus to the middle of the isthmus were 1 and 0.81. In one fetus of the high-risk patients, a contraductal shelf and the accompanying area of tubular isthmic hypoplasia were suspected and a diagnosis of coarctation of the aorta was subsequently confirmed after birth. In two fetuses with growth restriction and one fetus with intestinal atresia, the isthmus diameters were below the third percentile but the ratios of the isthmus end to the middle of the aortic isthmus were all normal and no cardiac anomalies were detected after birth. CONCLUSIONS: We could establish normal values for aortic isthmus diameters in late gestation from a coronal view and identify even minimal changes in aortic isthmus dimensions and morphology in pathological conditions.

Aorta↗

Sonographic findings and fetal chromosomal studies undertaken prenatally by fetal blood sampling using cordocentesis.

Prenatal karyotyping was carried out on 206 pregnancies with sonographic abnormalities, including fetal malformations, growth retardation and disorders of the amniotic fluid volume. Overall, chromosomal aberrations were detected in 37 cases (18.0%), but the frequency of chromosomal aberrations was 8.2% in fetuses with a single anomaly and 52.7% in fetuses with multiple anomalies. The chromosomal risk was very high in malformations associated with growth retardation (81.8%) or with both growth retardation and disorders of the amniotic fluid volume (82.4%). High rates of chromosomal aberrations were observed in cases of heart anomalies, diaphragmatic hernia, urinary tract obstruction, hydrocephalus, intestinal atresia, abdominal wall defect and fetal effusion/hydrops. In fetuses at high risk of chromosomal aberrations, cordocentesis for rapid karyotyping is justified in the second or third trimester.

Journal Article↗

The role of intestinal transplantation in the management of babies with extensive gut resections.

BACKGROUND/PURPOSE: Modern neonatal care, surgical treatment, and total parenteral nutrition (TPN) have improved survival rate for babies with extensive gut resections. The authors examined the role of intestinal transplantation in the treatment of these patients. METHODS: The authors reviewed all pediatric intestinal transplants performed for short bowel syndrome at our center (70 transplants performed between Aug 1994 and Feb 2002). Factors affecting patient survival were analyzed. RESULTS: Older patient age at the time of transplant was a significant factor favorably affecting patient survival (P =.031). Trends toward better survival rates were observed in those transplants performed more recently (P =.063), in those patients with greater body weight (P =.084), in those not hospitalized at the time of transplant (P =.14), and in those without concomitant liver failure (P =.12). Three-year survival rate for patients greater than age 2 years and without liver failure was 90%. However, 32% of our recipients underwent transplant at age less than one year, and most in this group (75%) had concomitant liver failure. CONCLUSIONS: For babies with irreversible intestinal failure, intestinal transplantation is a life-saving option. Results, which have recently improved, are best when transplantation compliments more conservative surgical treatments and TPN. However, there is a subset of patients who have liver disease early requiring urgent transplant.

Age Factors↗

[Prenatal diagnosis: intestinal obstruction].

We present 4 cases of fetal intestinal obstruction and their ultrasonographical findings. With regard to the reported cases we discuss the diagnostic and differential-diagnostic considerations of this rare but not uncommon fetal malformation.

Adolescent↗

Multidisciplinary management of fetal surgical anomalies: the impact on maternal anxiety.

AIM: To assess the impact on maternal anxiety of a multidisciplinary approach in prenatal management of fetal surgical anomalies. DESIGN: A case-control study was undertaken: Group A (cases) consisted of 16 couples receiving a prenatal multidisciplinary counselling, Group B (controls) was represented by 16 couples, who received diagnosis of the fetal anomaly and the relevant counselling by an obstetrician only. The fetuses were affected by the following gastrointestinal anomalies: gastroschisis, omphalocele, intestinal atresia. METHODS: The Italian version of the Spielberger State-Trait Anxiety Inventory was utilized to assess maternal anxiety. In Group A maternal anxiety level was assessed after the first antenatal counselling and at birth, whereas in Group B only at birth. RESULTS: At birth, Group A presented STAI-S scores significantly lower than after the first antenatal consultation with the team (Mean +/- SD = 39.87 +/- 6.46 versus 68.93 +/- 5.81; p < 0.01). At the end of the first day spent with the baby in the Neonatal Surgery Unit, Group A presented STAI-S scores significantly lower than Group B (Mean +/- SD = 39.87 +/- 6.46 versus 70.62 +/- 4.12; p < 0.01). CONCLUSIONS: This study provides evidence of the positive impact on maternal anxiety of a multidisciplinary approach in prenatal management of fetal surgical anomalies.

Adult↗

[Intrauterine meconium peritonitis. A rare cause of non-immunologic hydrops fetalis].

A case of foetal non-immune hydrops fetalis is presented. A second gravida was referred to our department at 31 weeks' gestation after normal pregnancy course. Sonography revealed marked foetal ascites as well as a polyhydramnion. Prenatal examinations did not yield a safe diagnosis. The foetal ascites was punctured via a pigtail-catheter that was left in the foetal abdomen. This procedure was supposed to facilitate the development of foetal lungs. At 33 weeks' gestation, after spontaneous rupture of membranes, a boy was spontaneously delivered. Postpartal radiography pointed to bowel perforation. Surgery on the first day of life showed a severe meconium peritonitis following a perforation of the ileum. 22 months after this operation, the boy is physically and mentally well developed. Until now, there is no sign of mucoviscidosis. We believe that early diagnosis, the possibility of prenatal therapy and the close collaboration of disciplines made the survival of this child possible.

Colon↗

[Sonography of congenital abnormalities of the gastrointestinal tract].

In 12 children aged from 1 day to 15 years with various kinds of congenital gastrointestinal anomaly (atresia/stenosis in the duodenum, jejunum, or ileum; duplication cyst in the duodenum/Bauhin's valve; ectopic pancreas antropyloric) the almost invariably present disturbance of passage was sonographically documented and located and, in the cases with concomitant processes of the bowel wall, the causative lesion demonstrated. In 3 neonates, the diagnosis including complications (meconium peritonitis with calcifications secondary to small bowel perforation) had already been accurately made prenatally. Despite the always unequivocal ultrasound findings, additional x-ray examinations (only plain in 5, only with contrast medium in 1, plain and with contrast medium in 6 cases) were performed in all patients to confirm the diagnosis and reassure the operator. Comparison of the sonographic with the radiological and, later, with the surgical results showed that, in all cases, ultrasound had already provided the information essential to the surgical intervention. The results indicate that, in future, a substantial reduction of additive x-ray examinations is possible in such diseases and that their early intrauterine sonographic documentation should be attempted much more frequently.

Digestive System↗

[Prenatal sonographic diagnosis of abdominal wall defects and stenoses of the intestinal tract].

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.

Abdominal Muscles↗

Megacystis-microcolon-intestinal hypoperistalsis syndrome: a functional intestinal obstruction in the female newborn.

A fatal case of the megacystis-microcolon syndrome in a newborn girl is presented and two previously unreported findings are discussed. The umbilical hernia is thought to represent a mechanically induced embryological defect, secondary to the expanding nonfunctional megacystis which appears to be the dominant component from an early stage. Hypertrophic changes of the large intestinal wall musculature, adjacent to dilated segments, may indicate an impaired propulsive activity in utero, as a result of defective neurotransmission. The latter is proposed as the common pathophysiological mechanism for both urinary and intestinal malfunction.

Colon↗

The development of the characteristic anomalies found in gastroschisis--experimental and clinical data.

Gastroschisis was investigated experimentally as well as clinically. An experimental model developed in the chick embryo demonstrated that the characteristic picture of gastroschisis evolved only if the herniated bowel was exposed to urine components in the allantoic fluid. Remarkable similarities were revealed on comparing changes in human amniotic fluid composition (from 30th week) with changes in chicken allantoic fluid composition (from 15th day of incubation). These changes correlate with progressive fibrotic coating of the exposed bowel loops leading to the characteristic picture of gastroschisis. No fibrous coating was found in human foetuses with a gestational age under 30 weeks, while investigation of these foetuses did confirm that gastroschisis itself occurs at an early developmental stage (6th-8th week). No primary structural defects were found in the nervous system of the bowel wall neither in the experimental gastroschisis model nor in the human cases investigated. The postoperative delay in intestinal motility affecting some gastroschisis patients was found to be secondary to multifocal ischaemic damage of the bowel wall.

Abdominal Muscles↗

[Prenatal ultrasonic diagnosis of malformations and their surgical consequences--a new chapter in pediatric surgery].

The importance of antenatal diagnosis of malformations for the paediatric surgeon is highlighted on the basis of the authors' own studies. Among 34000 examinations of pregnant women we found 40 malformations (0.1%), 12 of which were surgically corrected postnatally: 6 malformations of the urinary tract, 3 intestinal atresias, 2 ovarian cysts and 1 hydrocephalus. 6 cases are described in greater detail in consideration of the special problems involved. It is stated that today practically all gross malformations can be recognised prenatally. There are three groups as follows, with their pertaining consequences: non-viable, immediate surgical correction, and postponed need for immediate intervention. The necessity of close co-operation between gynaecologist, neonatologist and paediatric surgeon is stressed, as well as the need for including the parents in the consultations. Prognosis of correctable malformations can be improved by termination of birth and immediate interdisciplinary care of the newborn.

Central Nervous System↗

The effect of intrauterine vascular compromise on further development of corpus spongiosum and urethra.

An in-utero experimental study was performed to evaluate the effects of intrauterine vascular compromise on further development of corpus spongiosum and male urethra. Thirty time-mated pregnant New Zealand white rabbits on their twenty-third day of gestation were used. Deterioration of the blood supply of the corpus spongiosum and urethra was attempted by electrocauterizing the perineum adjacent to the root of the phallus without damaging the corpus spongiosum and urethra, under the operating microscope. A bipolar cautery was carried out using a specially designed forceps having 100 microns interspaced fixed tips. Five experimental and seven control live male litters were delivered at term by Cesarean sections. Their anogenital regions were examined histopathologically. While the corpus spongiosum extended as long as the corpus cavernosum to the tip of phallus in control litters, the corpus spongiosum ended under the skin in one of the experimental group and in the other four was shorter than the corpus cavernosum. In the latter group, no histopathological evidence of tissue destruction which might be related to electrocauterization, was found. Similar to the pathogenesis encountered in intestinal atresia, a vascular insult which might occur even after the completion of organogenesis, may affect the fate of the corpus spongiosum and urethra. Localized ischemia resulting from local vascular insults may explain, at least in theory, the pathogenesis of some congenital anomalies of corpus spongiosum and urethra such as hypospadias.

Animals↗