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At least 19 recordsLinked to original sources
PubMed2004

Fetal intestinal obstruction induces alteration of enteric nervous system development in human intestinal atresia.

Intestinal motility disorders are a major cause of morbidity after surgical repair of intestinal atresia of unknown mechanism. We hypothesized that interruption of antenatal peristalsis may disturb the normal development of the enteric nervous system. Using a series of neuronal (synaptophysin, neuronal nitric oxide synthase, neurofilaments) and nonneuronal markers (glial acidic fibrillary protein and c-Kit) and immunohistochemistry, we have defined developmental steps of the enteric nervous system in normal intestine (12 fetuses, 15 children, and 4 adults) and their alterations above and below the obstacle in 22 human intestinal atresia compared with age-matched controls. Antisynaptophysin antibody revealed the progressive conversion of the myenteric plexus from a continuous belt into regularly spaced ganglions during normal fetal gut development and, by contrast, the significantly delayed appearance of individual neuronal ganglions in the distal segments of atresia (p < 0.05). Staging using three other markers for neuronal (neurofilaments and neuronal nitric oxide synthase) and nonneuronal cells (glial acidic fibrillary protein) confirmed that maturation of the myenteric plexus was significantly delayed below atresia (p < 0.01). These results indicate that intestinal atresia impairs the development of the enteric nervous system and provide an anatomical substrate for the motility disorders observed after surgical repair. They point to the role of peristalsis in normal gut development and suggest that stimulation of peristalsis might be used to accelerate recovery.

Biomarkers↗
PubMed2000

Intestinal atresia.

Intestinal atresia accounts for about one third of all cases of neonatal intestinal obstruction. The survival rate has improved to 90% in most of the series with the operative mortality being < 1%. The survival rate improves with distal atresias. An increased mortality is observed in multiple atresias (57%), apple peel atresia (71%), and when atresia is associated with meconium ileus (65%), meconium peritonitis (50%) and gastroschisis (66%). Although appearance of echogenic bowel on prenatal ultrasonography is suggestive of GI, it is confirmed in only 27% cases. Prenatal ultrasonography is more reliable in detection of duodenal atresia than more distal lesions. Short bowel syndrome is the major impediment in the management of jejunoileal atresia. Although total parenteral nutrition (TPN) is the main adjunctive treatment, it delays intestinal adaptation and may cause cholestasia and subsequent liver damage. Graduated enteric feedings, use of growth hormone, glutamine and modified diets containing low fat, complex carbohydrates and protein supplements have been used in a adults with short bowel syndrome to successfully diminish TPN requirements and enhance nutrient absorption in nearly half of the patients. Utilization of growth factors to facilitate intestinal adaptation and advances in small bowel transplant may improve the long-term outcomes in future.

Diagnosis, Differential↗
PubMed2002

Use of intraluminal stents in multiple intestinal atresia.

Multiple intestinal atresia presents a difficult technical problem because of extreme loss of intestinal length, disparity of residual bowel wall size, and discontinuity of multiple short intestinal segments. The authors report on a 3,000-g infant with gastroschisis complicated by intrauterine volvulus and multiple intestinal atresias who was treated successfully with intraluminal stenting and sutureless anastomoses. A total of 25 cm of small bowel was salvaged including 13 segments each measuring 1 to 8 cm in length. Subsequent radiographic studies showed spontaneous anastomosis with a compartmental configuration of the residual bowel and decreased transit time. Five months postoperatively, the patient was weaned off total parenteral nutrition completely and one year later is growing and gaining weight with 4 to 6 bowel movements per day.

Abnormalities, Multiple↗
PubMed1997

[The importance of tapering the intestines in congenital intestinal atresia].

Extensive intestinal resections in inborn intestinal atresias are the second most frequent cause of the short gut syndrome. Because treatment of this condition is so far minimal, prevention is of fundamental importance. One possible approach is tapering of the gut, i.e. longitudinal antimesenterial resection of the gut. At the Clinic of Paediatric Surgery in 1991-1995 30 patients with inborn atresias of the gut were operated (17 atresias of the duodenum, 11 atresias of the small intestine, 2 atresias of the large intestine). Six patients (20%) died. In 2 patients (one girl with atresia of the colon and one boy with atresia of the jejunum) developed dilatation of the gut orally from the site of resection of the atresia and a chronic subileous condition. Instead of resection of the dilated portion the gut was modelled by tapering. In both children the passage improved and the children thrive. Based on data in the literature and their own experience the authors assume that tapering of the gut should supplement primarily high jejunal atresia, apple peel syndrome and extensive dilatation of the jejunum. Tapering cannot be used above the aganglionic portion of the intestine.

Female↗
PubMed1995

A syndrome involving immunodeficiency and multiple intestinal atresias.

Multiple intestinal atresias (MIA) is a severe form of intestinal atresias throughout the gastrointestinal tract. In two reports, MIA have been associated with severe immunodeficiency. We report a newborn girl who had profound humoral and B cell immunodeficiency and impaired T cell function. The patient had agammaglobulinemia and decreased blood lymphocytes, with virtually no B cells in the blood or in intestinal lymph nodes. T cells were reduced in number and weakly proliferated to mitogens. These data suggest that a syndrome involving the development of MIA is associated with various forms of severe immunodeficiency, and therefore newborns with MIA should be examined for immunodeficiency.

Agammaglobulinemia↗
PubMed2003

The occurrence of unusual smooth muscle bundles expressing alpha-smooth muscle actin in human intestinal atresia.

BACKGROUND/PURPOSE: Intestinal dysmotility is an important problem in the postoperative management of patients with intestinal atresia (IA). Changes in the intramural components have so far been histochemically and immunohistochemically examined in both the proximal and distal segments of IA, but no detailed analysis of the muscular elements has been performed. The aim of this study was to carefully examine any alterations in the muscular elements in the intestines from patients with IA. METHODS: Resected intestines were obtained from 6 patients with ileal atresia, 4 patients with jejunal atresia, and 3 controls without gastrointestinal diseases obtained by autopsy (congenital diaphragmatic hernia). All specimens were immunochemically stained with a monoclonal antibody to alpha-smooth muscle actin (alpha-SMA) as a smooth muscle marker. RESULTS: In the normal small intestine, almost all the enteric smooth musculature were positive for alpha-SMA antiserum, except for the bulk of the circular musculature. In the proximal segments of all cases with IA, a reduced staining intensity for alpha-SMA was observed mainly in the severely hypertrophic muscle layers. In addition, some bundles of smooth muscle also were located in the submucosal connective tissue near the border of the innermost layer of the circular musculature, in which large amounts of smooth muscle fibers extended occasionally from the innermost layer of the circular musculature to the muscularis mucosae in the proximal segments of 4 cases. In the distal segments of IA, the distribution of alpha-SMA-positive smooth muscle fibers was similar to that in the control intestines, excluding mild to moderate hypertrophy of the muscular layers. CONCLUSIONS: Both severe hypertrophy and a reduced immunoreactivity for alpha-SMA were observed in the circular muscle layer of the proximal segments. In addition, the occurrence of alpha-SMA-positive abnormal smooth muscle fibers was recognized in the submucosal layers of the proximal segments, thus, suggesting a delay in the intestinal muscular formation or a regressive reaction secondary to dilatation. These muscular alterations in the proximal segments might be considered to contribute to the postoperative intestinal dysmotility in IA cases.

Actins↗
PubMed1999

Abnormalities of enteric neurons, intestinal pacemaker cells, and smooth muscle in human intestinal atresia.

BACKGROUND/PURPOSE: Intestinal dysmotility, which usually has been encountered in the severely dilated proximal segment, is an important problem in postoperative management of patients with intestinal atresia (IA). Changes of enteric nerves had been histochemically examined in both the proximal and distal segments of IA, but a systemic immunohistochemical analysis is still lacking. The aim of this study was to examine precisely alterations of neuronal and muscular elements and pacemaker cells in intestines from patients with IA. METHODS: Resected intestines were obtained from 5 patients with ileal atresia, 3 patients with jejunal atresia, and 3 controls without gastrointestinal diseases (congenital diaphragmatic hernia). All specimens were immunochemically stained with a monoclonal antibody to alpha-smooth muscle actin (SMA) as a smooth muscle marker, polyclonal antibodies to protein gene product (PGP) 9.5 as a general neuronal marker, and to c-kit protein as a maker of intestinal pacemaker cells. In addition, all specimens also were stained by NADPH-diaphorase (NADPH-d) to know the distribution of inhibitory nitrergic nerves. RESULTS: A hypoplasia of the myenteric ganglia and a marked reduction of intramuscular nerve fibers, including nitrergic neurons, were observed in the dilated proximal segment of IA. C-kit-positive cells were localized around the myenteric plexus, but rarely found within the muscularis propria in the proximal segment. The distribution of nerves and c-kit-positive cells in the distal segment was comparable with that seen in controls. A reduced staining intensity for alpha-SMA was mainly observed in the hypertrophic circular muscle layer of the proximal segment. CONCLUSIONS: A hypoplasia of intramural nerves and pacemaker cells was seen predominantly in the proximal segments of IA. Hypertrophy and reduced immunoreactivity for alpha-SMA also were observed in the circular muscle layer of the proximal segment. These alterations of the proximal segment may thus contribute to the postoperative intestinal dysmotility in IA cases.

Actins↗
PubMed1984

[Ultrasonic diagnosis of fetal ascites with polyhydramnios resulting from partial small intestine atresia and secondary intestinal wall perforation].

A primigravida was admitted to hospital in the 31st week of pregnancy after normal pregnancy course, with premature rupture of the amnion. Ultrasonography revealed a marked fetal ascites as well as polyhydramnion. Pregnancy was terminated via Caesarean section because of threatening intrauterine asphyxia. The asphyctic, hypotonic infant, which did not show any visible malformation, was intubated and the ascites was punctured. After a brief recovery, the infant died 24 hours post partum. Autopsy revealed partial atresia of the small intestine with secondary perforation of the intestinal wall, as well as a fibrinous-purulent peritonitis. Pathogenesis of the foetal ascites, as well as the possibilities of diagnosis via sonography in case of fetal malformations, are discussed on the basis of the present case and previous literature.

Adult↗
PubMed2002

In utero sonographic findings in a fetus with a hereditary multiple intestinal atresia.

Hereditary multiple intestinal atresia is a rare syndrome with an autosomal recessive pattern of inheritance. We described the antenatal sonographic appearance of a case of hereditary multiple intestinal atresia. A markedly dilated cystic mass noncommunicating with the stomach was observed in the right upper quadrant of the fetal abdomen by an ultrasound scan at 30 weeks of gestation. Real-time sonographic evidence of strong peristaltic activity was not demonstrated. The infant weighing 2315 g was delivered at 35 week's gestation with Apgar scores of 7 and 9 at 1 and 5 minutes, respectively. At laparotomy, a prepyloric septal atresia, multiple-level small intestinal atresias and a rectal diaphragm were discovered. The infant died 52 days after the operation. The sonographer should be aware that a proximal bowel distention can be associated with hereditary multiple intestinal atresia in patients with a family history of an affected sibling.

Adult↗
PubMed1994

Mesenteric defects as a cause of intestinal volvulus without malrotation and as the possible primary etiology of intestinal atresia.

Mesenteric defects can lead to intestinal volvulus even when the midgut is normally rotated. There are two types of mesenteric defects: basilar, in which the entire base of the mesentery is involved, and segmental, in which only an isolated portion of the mesentery is affected. These defects can present at any age, and the clinical symptoms depend on the extent of the disease and the amount of intestine involved in the volvulus. In the newborn, the basilar defects have clinical signs and symptoms similar to those of midgut volvulus secondary to malrotation. Similar to midgut volvulus secondary to malrotation, this is a surgical emergency. In older patients, basilar defects can be misdiagnosed because of the normal placement of the ligament of Treitz and because of failure to consider mesenteric defects as a possible cause. The treatment for basilar mesenteric defects is intestinal fixation. Intestinal volvulus secondary to segmental defects always presents as intestinal obstruction. In the newborn, these lesions may be indistinguishable from intestinal atresia. Older children present with intestinal obstruction of an unknown cause. Resection of the affected intestine is the treatment for segmental mesenteric defects. Intestinal mesenteric abnormalities as a cause of intestinal atresia unifies under one etiology all the lesions observed in intestinal atresia. Although this theory does not rule out other causes of intestinal atresia, intestinal mesenteric defects may be the primary condition under which intestinal atresia occurs.

Child↗
PubMed2001

Management of intestinal atresia in patients with gastroschisis.

BACKGROUND/PURPOSE: Intestinal atresia occurs in approximately 10% to 20% of children with gastroschisis and may be missed at the initial closure if a thick peel obscures the bowel. Some investigators have identified intestinal atresia as a significant contributor to morbidity and mortality. The authors reviewed their experience with gastroschisis and intestinal atresia in an attempt to answer the following questions. What is the incidence of this association? How often is the intestinal atresia unrecognized as a result of the peel? What is the optimal management for infants with atresia and gastroschisis, and does the atresia affect morbidity or mortality? METHODS: The hospital charts and medical records of all patients with gastroschisis treated at our institution from 1969 to present were reviewed thoroughly. Parameters analyzed included gestational age (GA), birth weight (BW), antenatal diagnosis, mode of delivery, type of closure, era of repair, presence of other major anomalies, and development of necrotizing enterocolitis. Morbidity and mortality rates were examined. Characteristics of patients with and without atresia were compared. Chi-squared was used for crosstabular analysis. Sample parameters were compared with Student's t test. P values of less than.05 were considered significant. RESULTS: A total of 199 babies had gastroschisis and 25 (12.6%) had intestinal atresia. Intestinal atresia was initially unrecognized in 3 patients. Most patients (80%) underwent primary closure of the abdominal wall. Initial stoma formation and delayed anastomosis was performed in 12 (48%) patients, none of whom required prosthetic material for abdominal wall closure. Initial stomas were avoided in 5 patients who required SILASTIC (Dow Corning, Midland, MI) silos. Skin closure alone was used in 2 babies. The level of the atresia was most commonly jejunoileal (20 of 25, 80%). Mean hospital stay was increased in babies with intestinal atresia, 36.2 versus 63.1 days (P <.001). CONCLUSIONS: Although patients with intestinal atresia did have feeding delays, an increased incidence of adhesive intestinal obstruction, and prolonged hospitalization, neither chi(2) nor logistic regression analysis showed any correlation with mortality. Intestinal repair at the first operation is sometimes possible and depends on the severity of the peel. Delayed repair of the atresia after a period of bowel decompression and parenteral nutrition is preferred, but in certain situations (colonic atresia, necrotic intestine, complicated atresia) may not be possible. The combination of stomas and prosthetic material can be avoided in almost all patients. A management algorithm for patients with atresia and gastroschisis is discussed.

Female↗
PubMed2003

Association of vasoconstrictive exposures with risks of gastroschisis and small intestinal atresia.

BACKGROUND: Gastroschisis and small intestinal atresia are congenital anomalies that may arise from vascular disruption. It is hypothesized that maternal exposure to cocaine, amphetamines, decongestants and nicotine, all of which have vasoconstrictive actions, can contribute to these defects. The present study examined risks of gastroschisis and small intestinal atresia associated with combined exposure to vasoconstrictive drugs and cigarette smoking. METHODS: This was a retrospective study conducted from 1995 to 1999 in 15 cities across the United States and Canada. Mothers of 205 gastroschisis cases, 127 small intestinal atresia cases, 381 malformed controls and 416 nonmalformed controls were interviewed within 6 months of delivery. RESULTS: Reported vasoconstrictive drugs included pseudoephedrine, phenylpropanolamine, ephedrine and methylenedioxymethamphetamine. Combined exposure to vasoconstrictive drugs and cigarette smoking in the first 2.5 months of pregnancy was reported by 9% of gastroschisis cases, 9% of small intestinal atresia cases and 4% of controls. Multivariate-adjusted odds ratios, controlling for the effects of age, education, income, other drug use and alcohol intake, were 2.1 (95% confidence interval = 1.0-4.4) for gastroschisis and 2.8 (1.1-6.9) for small intestinal atresia. Risks of each defect increased with increasing level of cigarettes (P for trend = 0.019 and 0.012, respectively). Vasoconstrictive drug use among smokers of 20 or more cigarettes a day increased gastroschisis risk 3.6-fold (1.3-10.3) and small intestinal atresia risk 4.2-fold (1.1-16.2). CONCLUSIONS: These findings provide further evidence of vascular disruption as an etiology for gastroschisis and small intestinal atresia.

Adult↗
PubMed2005

Serial transverse enteroplasty in intestinal atresia management.

In intestinal atresia, resection of the proximal dilated bowel segment, if long, might lead to bowel length compromise. Although tapering enteroplasty and plication are well described to avoid such a resection, the use of serial transverse enteroplasty is proposed here as an alternative in this situation.

Anastomosis, Surgical↗
PubMed1995

An association of multiple intestinal atresia and biliary atresia: a case report.

This is the first report of a 2-day-old girl having an association of biliary atresia and multiple intestinal atresia. She developed intractable vomiting in the first hours of life. An upper gastrointestinal series and barium enema showed jejunal atresia and rectal atresia. A laparotomy was performed on the fourth day of life. The operative findings were multiple sites of atresia of the intestine, 10 atretic sites in the small bowel, and 3 in the colon. The gall bladder was normal in size and position. After the operation, the patient's stool remained acholic and her jaundice did not resolve. On the 58th day of life, another laparotomy was performed with the presumptive diagnosis of biliary atresia. The operative finding was biliary atresia, type 3; the Kasai operation with external jejunostomy was performed. The patient suffered from postoperative cholangitis and enterocolitis because of her short bowel and subsequently died at 9 months of age. This is the first case of biliary atresia associated with multiple intestinal atresia reported in the literature.

Barium Sulfate↗
PubMed1988

New observations on the pathogenesis of multiple intestinal atresias.

It has been suggested that multiple intestinal atresias result from multiple ischemic infarctions of the intestinal tract. We have studied surgical material from 59 neonates with intestinal atresias seen at our hospital between 1975 and 1986. Forty (68%) patients had single intestinal atresias and 19 (32%) had multiple atresias. There were seven cases of hereditary multiple atresias seen in three families and 12 cases of nonhereditary multiple atresias. All hereditary cases had numerous type I or type II gastrointestinal atresias but none had type IIIa atresia. Six of the seven hereditary cases had multiple atresias in the small as well as large bowel. The 12 patients with nonhereditary atresias had various types of atresias but mesenteric or intestinal interruption was observed in only two patients. All patients with hereditary multiple intestinal atresias showed identical microscopic appearances in the small and large intestine, consisting of sieve-like multiple lumina, each surrounded by its own mucosa and muscularis mucosae but sharing a common muscle coat. There was no evidence of lanugo, bile pigments, or squames within the lumen distal to atretic segments in any of these patients. Six nonhereditary cases who had multiple septal atresias affecting only the small bowel demonstrated essentially similar lesions on microscopic examination as seen in hereditary cases. There was no evidence of arterial occlusion in the mesentery and lanugo, bile pigments, and squames could not be found distally in the intestinal contents in any of these cases. These pathologic findings suggest that all cases of hereditary multiple intestinal atresias and some cases of nonhereditary multiple intestinal atresias are a consequence of a malformative process of the gastrointestinal tract rather than an ischemic process.

Abnormalities, Multiple↗