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Umbilical cord ulceration and intestinal atresia: a new association?

In three fetuses, congenital intestinal atresia was associated with linear ulcerations of the umbilical cord. In two cases, hemorrhage was seen from the cord ulcer. Both fetuses required emergency cesarean section for fetal distress and were born anemic. The third fetus was mildly hydropic, attributed to hemorrhage, and was stillborn. The mechanism of the association could not be determined. These cases suggest a risk of prenatal umbilical cord hemorrhage in infants with intestinal atresia.

Adult↗

Donor immune reconstitution after liver-small bowel transplantation for multiple intestinal atresia with immunodeficiency.

The syndrome of multiple intestinal atresia with immunodeficiency is a rare, invariably fatal congenital disorder. At 16 months of age, a child with this syndrome underwent liver-small bowel transplantation from a 1-of-6 HLA-matched donor. He acquired full enteral tolerance and normal liver function and has never shown evidence of allograft rejection. After mild graft-versus-host disease developed, studies revealed that more than 99% of his CD3(+) lymphocytes and 50% of his CD19(+) lymphocytes were of donor origin, whereas granulocytes and monocytes remained of recipient origin. He synthesizes polyclonal immunoglobulin G (IgG), IgA, and IgM and has developed antibodies to cytomegalovirus (CMV) and parainfluenza 3. His T lymphocytes are predominately CD3(+)CD4(-)CD8(-) with T-cell receptor gammadelta heterodimers and CD3(+)CD4(-)CD8(+) with CD8alphaalpha homodimers, populations consistent with an intraepithelial lymphocyte phenotypic profile. We postulate that he has engrafted a donor intestine-derived immune system and is incapable of rejecting his engrafted organs.

Cytomegalovirus Infections↗

Hereditary multiple intestinal atresia--ultrasound findings and outcome of pregnancy in an affected case.

A case of multiple intestinal atresia is described. Dilatation of the bowel was observed at 17 weeks' gestation during routine ultrasound scan of a healthy Caucasian primigravida from a non-consanguineous marriage. Amniocentesis was performed. The karyotype was normal male and cystic fibrosis screening was negative. Regular scans were performed throughout the pregnancy and a simple bowel obstruction was suspected. The baby was delivered at 37 weeks' gestation in good condition. Initial clinical examination was normal but abdominal distension developed during the first day. At laparotomy, prepyloric septal atresia, a distal duodenal membrane, and multiple intestinal atresia were found. The baby died aged 4 days. Post-mortem examination of the abdomen confirmed the absence of lumen from long segments of the small intestine together with areas of colonic atresia. Histology and distribution were consistent with those reported in familial multiple intestinal atresia. The pitfalls in the interpretation of prenatal ultrasound scans and the possibility of prenatal diagnosis in future pregnancies are discussed.

Fatal Outcome↗

Intestinal atresia and stenosis as seen and treated at Kenyatta National Hospital, Nairobi.

In this retrospective study carried out covering the period, 1978-1991, 62 neonates were seen, diagnosed and treated for intestinal atresia which included: duodenal atresia and stenosis, small bowel atresia and atresia of large bowel. Locations of obstruction were duodenal in 17 patients, jejunal in 25 patients, jejuno-ileal in 5 and colon in two. Duodenal atresia was noted in 9 infants and duodenal stenosis due to annular pancreas, Ladd's bands with malrotation of bowel in 8. Associated anomalies which were observed were anorectal malformations in 2 and malrotation in 2 infants. Birth weights ranged from 1450 gm to 3000 gm. Prematurity was recorded in 11 infants. Diagnosis of intestinal atresia in our patients was made clinically and radiologically. Intestinal atresia in neonates was differentiated from other causes of obstruction such as Meconium Ileus, Hirschsprung's disease, neonatal volvulus, rectal atresia in anorectal malformations. Treatment of infants with intestinal atresia was surgical. Surgical techniques used depended on pathological findings. In 36 patients, complications such as functional obstructions with vomiting and failure to thrive, malabsorption, aspiration, bronchopneumonia, sepsis were observed. Overall mortality rate in our cases was 25 (41.9%) out of 62 patients.

Birth Weight↗

[Epidemiological study of intestinal atresias: central-eastern France Registry 1976-1992].

OBJECTIVE: Our purpose was to describe the epidemiology of small intestinal atresia. STUDY DESIGN: We used data collected by the Central-East France Congenital Malformations Registry from 1976 through 1992 to evaluate the prevalence of different types of intestinal atresia in liveborn and stillborn infants and to study some demographic and clinical features such as sex ratio, multiple births, gestational age, birth weight, maternal age, maternal disease, associated malformations. RESULTS: Through surveillance of more than 1.5 million births, we identified 344 liveborn and 14 stillborn infants with intestinal atresia (1A). The prevalence of 1A was 2.25 per 10,000 livebirths. Fifty percent of the liveborn infants had duodenal atresia, 36% had jejunoileal atresia, 7% had colic atresia, 3% had intestinal duplication and 5% had multiple atresia. The twinning rate was 4.4% which is significantly higher than in the non malformed population. Gestational age was less than 37 weeks in 35.4% of the cases. Birth weight was less than 2,500 g in 52% of the cases. For those two variables we observed significant differences among the different types of malformations. Study of 1A rates by maternal age showed an increased risk below the age of 20 (p < 10(-5)). We didn't find significant differences compared to the population for ovulation induction and maternal diabetes. Study of associated malformations demonstrated significant differences in rates and types of associated malformations in the different groups of 1A which suggests heterogenous embryological mechanisms. CONCLUSION: These findings confirm the literature data for most of the epidemiological characteristics. Only the association of an increased risk in the teenage mothers group was not previously described. This finding has to be confirmed by others studies.

Abortion, Therapeutic↗

Intestinal atresia: management problems in a developing country.

Over a 13-year period, 24 children with intestinal atresia were managed at the Jos University Teaching Hospital, Jos, Nigeria. Intestinal atresia ranks as the second most common cause of neonatal intestinal obstruction (after anorectal malformation) in our hospital. Five children had duodenal obstruction (two atresia, two duodenal webs, one annular pancreas), 17 had jejunoileal atresia, and two had colonic atresia. Fourteen were boys, and 10 were girls (M:F: 1.17:1). The median age at presentation to the surgeon was 6 days (range: 1 day-12 years). The most common presenting features were bilious vomiting and abdominal distension. Six patients did not pass meconium within the first 24 h of birth. The median weight at presentation was 2.6 kg (range: 1.1 kg-5.0 kg). Seven patients (four with jejunoileal atresia and three with duodenal obstruction) had associated congenital anomalies. Diagnostic investigation was plain abdominal x-ray, showing double-bubble gas shadows in duodenal atresia and varying degrees of air-fluid levels in jejunoileal and colonic atresias. An upper gastrointestinal series was done in three patients and a barium enema in one. Retrocolic duodenojejunostomy was done for all patients with duodenal atresia and annular pancreas, duodenotomy and web excision for those with duodenal webs, and resection with end-to-end anastomosis for those with jejunoileal atresia. One child with atresia involving the whole ileum and the colon had a jejunorectal anastomosis, while the other child with colonic atresia had caecostomy followed later by ileorectal anastomosis. Ten neonates died, giving a mortality rate of 41.7%. Mortality from intestinal atresia is still high in our environment, due mainly to lack of neonatal intensive care facilities.

Colonic Diseases↗

Surgical management of multiple intestinal atresias.

The unusual problem of multiple intestinal atresias associated with jejunal atresia has been reviewed and seven cases have been summarized. Maintaining maximal bowel length without jeopardizing the patient is of utmost importance. The shish kebob technique has been illustrated in a patient with 15 obstructions. The other cases include one patient with the apple peel deformity and two with atresias associated with gastroschisis. Although these children are small in general, they are bright and otherwise healthy. The 90 percent mortality reported previously has been reversed by a combination of techniques and lessons learned from the leaders in pediatric surgery.

Humans↗

Intestinal atresia.

Forty-five patients with intestinal atresia, including 20 with duodenal, 21 with jejunoileal, and 3 with colonic obstruction, were encountered at the Yale-New Haven Hospital between 1970 and 1976. The overall survival rate in the 43 operated cases was 93 per cent. The major reasons for the excellent operative survival with this malformation include: (1) the care received in a regional neonatal center; (2) the early recognition and appropriate case selection, denying operation unless mandated in babies with duodenal atresia and trisomy 21; (3) primary repair using modern surgical techniques which minimize anastomotic complications and the "blind-loop" and "short gut syndrome"; and (4) the use of uncomplicated long-term total parenteral nutrition in approximately one-half of operated cases.

Abnormalities, Multiple↗

[Has prenatal ultrasonic diagnosis modified the prognosis of intestinal atresia? Results of a multicenter study].

A multicentric investigation concerning intestinal atresia diagnosed in french university hospitals from 1979 to 1983, has been done. Out of 96 exploitable cases coming from 8 centers, 28 correspond to intestinal atresias which had subject to prenatal echographic diagnosis: that is 29%. 15 of the 96 atresias were affected with trisomy. 21 which had not been recognised in utero. Concerning the isolated duodenal atresia, not accompanied by any other malformation, the post surgical evolution has been appreciated during the 12 months following the surgical operation. There was no statistically significant difference, in favour of the group of children whose malformation has been recognised in utero, concerning the number of post surgical complications. This result, apparently deceitful, can be explained by the early management of these malformations in specialised centers which have participated in this study.

Down Syndrome↗

Complicated intestinal atresias.

In this group of 45 intestinal atresia patients (duodenum, 16; jejunum, 24; ileum five) at the University of Mississippi Medical Center, individual hospitalizations ranged up to 245 days. Twelve patients required multiple operations, and the overall mortality rate was 22% (ten patients). While the patients with duodenal atresia had the greatest incidence of other congenital anomalies, including Down's syndrome, the patients with jejunal atresia presented with the most challenging surgical problems. Of the 24 jejunal atresia patients, only three had a single, simple area of obstruction. The remainder were complicated by other gastrointestinal lesions (five patients), by multiple areas of atresia (seven patients) including those in one surviving patient with 22 separate atretic segments, and by the Christmas tree deformity (nine patients). Intraoperative management of the complicated atresia should include: 1) grouping of multiple atresias during resection, 2) adequate resection of the dilated proximal atonic loop, 3) end-to-end anastomoses, 4) avoidance of intraluminal catheters, 5) additional resection of a segment of the distal loop in the Christmas tree deformity and 6) consideration of the shish kebab technique for multiple atretic webs. Postoperative management should involve early intravenous nutrition and repeated exploration for continued obstruction.

Abnormalities, Multiple↗

Histochemical changes in intestinal atresia and its implications on surgical management: a preliminary report.

Histochemical studies of the intestine were performed on five neonates, three with intestinal atresia and two as normal controls. In this preliminary report, changes secondary to ischemia and obstruction were defined. It was shown that the ischemic changes were limited in extent both proximally and distally, and the obstructive changes were reversible. A conservative approach to resection in the management of intestinal atresia is suggested. Limited resection of the dilated proximal bowel together with the use of total parenteral nutrition will allow for a safe waiting period for the pathological changes to reverse themselves and effective peristalsis to return.

Acetylcholinesterase↗

Notochord-gut failure of detachment and intestinal atresia.

A spectrum of congenital anomalies have been described in an adriamycin-treated model with common features to the human pattern. Multiple intestinal atresias was part of this spectrum occurring in 25% of full-term experimental rat fetuses. The aim of this study was to examine the underlying developmental mechanism that results in intestinal atresia. Virgin timed-pregnant Sprague-Dawley rats were injected with Adriamycin i.p. at a dose of 2 mg/kg on days 6-9 of gestation. Embryos were removed on different gestational days during organogenesis and serial transverse histologic sections were examined and compared with control specimens. In experimental embryos, hindgut atresia was seen in day 12 embryos. Attachment of the intestine with the notochord was obvious observation resulting in abnormal position of the intestine. In some specimens the atretic intestine was splitting the dorsal aorta or even located behind the dorsal aorta. It is concluded that in the adriamycin-animal model, notochord-intestinal failure of detachment resulted in intestinal atresia during the beginning of organogenesis period. The possible underlying mechanisms are pinching of some endodermal cells as well as interference with normal intestinal circulation resulting in ischemic necrosis.

Animals↗

Surgical aspects of intestinal atresia in 58 calves.

Fifty-eight calves with intestinal atresia at different sites were treated surgically. In only four of the animals the anal opening was reconstructed directly in the perineal region. In the remaining cases the final extent of the atresia was confirmed by a right flank laparotomy and in seven calves an anal opening was made in the perineal region and in seven calves the opening was made in the right flank. The remaining 40 calves were euthanased for different reasons. Seven of the 18 surgically corrected animals made a satisfactory recovery.

Animals↗

Gastroschisis and intestinal atresia.

Controversy exists over the best method of treating gastroschisis with concomitant intestinal atresia because the mortality in such patients is still high. We present our experience of 74 neonates with gastroschisis seen over a 17-year period. Four patients (5.5%) had intestinal atresia; 1 had only one small bowel atresia, 2 had double areas of atresia (one in the mid small bowel and the other in the proximal colon), and the remaining 1 had multiple areas of atresia. Atresia with gastroschisis conventionally has been treated by either primary anastmosis or by exteriorization. Three of the patients were treated by replacing the atretic bowel into the abdomen initially and exploring later (1 to 3 weeks) when the bowel appeared much more healthy, making the anastomosis easier and more secure. One patient with extreme jejunal dilatation was treated by creation of a Mikulicz fistula. All 4 of the patients survived the newborn period, but one of them died at 1 year of age due to total parenteral nutrition-induced chronic liver failure. Our experience is presented to emphasize this uncommonly used approach to this complex problem because it seems to be associated with a lower morbidity and mortality than other conventional approaches.

Abdominal Muscles↗

Multiple intestinal atresias in two brothers.

Two brothers with multiple intestinal atresias, from jejunum to rectum, are reported. This syndrome with multiple atresias in small and large bowel and intestinal calcification is an entity of probably autosomal recessive inheritance.

Colon↗