The genesis of intestinal atresia.
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Intestinal Atresia↗
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Small intestinal atresia and segmental absence of muscle coats.
The case of a newborn infant with small-intestine atresia coexisting with segmental absence of muscle coats leading to perforation and meconium peritonitis is presented. The theories of pathogenesis of the simultaneous occurrence of segmental absence of gut musculature and atresia are briefly reviewed. A possible etiologic role of vascular accident in utero for both conditions is discussed.
Female↗
[Intestinal atresia].
The authors reviewed their experience with 20 cases of intestinal atresia (jejunum, ileum and colon) admitted between January 1972 and January 1977. International literature shows 80% mortality rate for this malformation before 1940 and 10-25% in current reports. In Mexico, before 1959, the mortality rate was 80%, but the most recent reports showed 28% mortality rate for atresia and 33% in cases of stenosis. The group of patient studied included twelve cases with a weight over 2,500 g. and eight patients under 2,500 g. Four patients showed multiple associated malformations (incompatible with life in one case) and in 17, there were no associated malformations. Nineteen cases were operated, the dilated and atresic segments were resected and anastomosis was performed. It was termino-terminal in 13 cases and termino-lateral in 6. One case was explored surgically and no attempt to repair the atresias was made, because there were malformations incompatible with life. Four patients died. Even if this series is very small, the associated malformations, low weight and techniques used do not seem to influence the prognosis of these patients.
Female↗
[Surgical management of intestinal atresia].
The study included 33 newborns with the diagnosis of intestinal atresia after discarding those of the duodenum. According to the clinical conditions of the patient and of the malformation, surgery was carried out by derivative ileostomy or primary anastomosis. Survivorship reaches 67% for ileostomy and 65% for anastomosis which are much higher figures than those obtained before.
Female↗
ACQUIRED INTESTINAL ATRESIA.
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Colonic Diseases↗
Congenital multiple intestinal atresia successfully treated with multiple anastomoses in a premature neonate: report of a case.
We report herein the case of a 2080-g premature male infant born at 34 weeks' gestation with multiple intestinal atresia, for whom multiple anastomoses were successfully performed. A total of 11 atresias were found in the small bowel, and five anastomoses were performed to preserve 59 cm of small bowel and the ileocecal valve. Postoperatively, he developed several episodes of sepsis caused by persistent enterostasis, but was able to be weaned from total parenteral nutrition (TPN) by postoperative day (POD) 106. Thus, multiple anastomoses may be the appropriate procedure to prevent short-gut syndrome for congenital multiple intestinal atresia, even in premature infants.
Abnormalities, Multiple↗
Mucosal morphology in experimental intestinal atresia: studies in the chick embryo.
Lesions identical to those observed in human intestinal atresia (IA) have been experimentally reproduced in several mammal models by either mesenteric vessels or intestinal wall injury. The recent availability of an avian model led us to investigate whether the same lesions could be studied at less expense. An intestinal loop within the body stalk hernia was coagulated on the 12th incubation day in 427 chick embryos (group 3), the lesions were studied in survivors 4 days later under light and scanning electronmicroscopy (EM), and the findings were compared with those in 12 control embryos (group 1) and 14 sham-operated ones (group 2). Types I or II atresias were obtained in 61 (73.4%) of the 83 survivors in group 3. Seromuscular layers were normal at both ends of the lesion; there was some flattening of villi at the proximal, dilated end, and hyperplasia with apparently lengthened, branched villi at the distal, unused one. Mucosal pattern under light microscopy was strikingly close to that observed in human IA and in the fetal lamb experimental model. On the other hand, scanning EM showed that in this model there were no villi as such, but rather prominent mucosal folds that were regularly arranged in a tire-rubber pattern in the normal intestine, flattened and distended in the dilated one, and again roughly normal in the distal unused gut. High-power magnification scanning EM showed that enterocytes were normal at all levels but, in agreement with previous findings in the fetal lamb model, that intercellular spaces were widened in the dilated portion adjacent to the obstruction.(ABSTRACT TRUNCATED AT 250 WORDS)
Animals↗
Multiple intestinal atresia and amyoplasia congenita in four unrelated infants: a new association.
Four unrelated infants with multiple segments of nonduodenal intestinal atresia and amyoplasia congenita are described. The extreme rarity of each of these conditions as a single entity indicates that their concurrent incidence in four separate unrelated patients is of significance with respect to pathogenesis. The nature of the relationship between the two conditions is unknown.
Arthrogryposis↗
Intestinal atresia or stenosis in the newborn associated with fibrocystic disease of the pancreas.
In a review of cases of intestinal atresia or stenosis in the newborn at Children's Hospital of Los Angeles it was noted that in approximately 10 per cent there was clinical or anatomic evidence of fibrocystic disease of the pancreas. Histologic sections of the bowel in all these cases showed the alterations of the mucosa commonly found in fibrocystic disease. Extensive ulceration, foreign body reaction and calcium deposition in the bowel wall were observed in five cases. Sections through the site of obstruction in one patient showed narrowing of the diameter of the bowel with preservation of muscular layers, but replacement of the central portion by a vascular, fibrous diaphragm in which giant cells, hemosiderin and calcium were prominent. This suggested that in certain instances obstruction of the intestine may be caused by fibrosis secondary to injury of the mucosa by abnormal meconium. Awareness of the common association of intestinal obstruction and fibrocystic disease of the pancreas and recognition of the histologic change in the bowel in fibrocystic disease may lead to early diagnosis of this disorder in some infants with intestinal obstruction.
Child↗
Umbilical cord ulceration in association with intestinal atresia in a child with deletion 13q and Hirschsprung's disease.
The case is reported of a baby boy with an interstitial deletion of the long arm of chromosome 13 who, in addition to the described associations of Hirschsprung's disease and intestinal atresia, had umbilical cord ulceration resulting in massive intrapartum haemorrhage. This case provides support for the existence of a previously reported association between umbilical cord ulceration and intestinal atresia, and suggests that it is aetiologically heterogeneous.
Chromosome Aberrations↗
Duodenojejunal atresia with "apple-peel" small bowel. A distinct form of intestinal atresia.
Prenatal occlusion of the superior mesenteric artery results in a distinct type of distal duodenal or proximal jejunal atresia in which the dorsal mesentery is absent and the distal small bowel assumes a spiral configuration around its vascular stalk, strongly resembling an apple peel. In some instances the condition is transmitted genetically as an autosomal recessive disorder. The mortality rate is much higher than in either simple duodenal or jejunal atresia because of deficient collateral circulation to the small intestine. The authors tell of 2 patients who died of necrotizing enterocolitis, a disease related to vascular insufficiency.
Duodenum↗
Intestinal atresia and stenosis: a retrospective analysis of presentation, morbidity and mortality in Zaria, Nigeria.
Over a period of 19 years 22 children with intestinal atresia and stenosis were managed at the Ahmadu Bello University Teaching Hospital Zaria. The condition ranks as the fourth most common cause of neonatal intestinal obstruction after anorectal malformation, Hirschsprung's disease and strangulated inguinal hernia at the hospital. Three patients had duodenal atresia, 18 had jejunoileal involvement (atresia 15, stenosis 3) and one colonic atresia. The median age at presentation to the surgeon was 6 days (range 1 day-2 years). The common features were bilious vomiting and abdominal distension, the latter being more common in patients with lower atresia. Non-passage of meconium in the first 24 hours of birth occurred in 11 patients; mostly in those with lower jejunal, ileal and colonic involvement. Eight patients had associated anomalies, 5 of them with ileal atresia or stenosis. Diagnostic investigation was mainly plain abdominal radiography showing double-bubble shadow in duodenal atresia and varying degrees of air-fluid levels in other atresias. Contrast studies were not employed for diagnosis in any patient. The patients with duodenal atresia and had duodenoduodenostomy and duodenojejunostomy respectively while the jejunoileal atresia and stenosis were treated by resection and anastomosis. One patient with ileal atresia associated with total colonic Hirschsprung's disease and extensive atresia from terminal ileum down to descending colon had ileostomy. Postoperatively, 7 patients had infections of varying degrees and 3 anastomotic dehiscence. Mortality was 9 (41%) due mostly to septic complications and prematurity. While the survival of children with intestinal atresia and stenosis has improved over the years in developed countries, ours is still low (59%) due to late presentation and lack of neonatal intensive care facilities.
Child, Preschool↗
Histological study of intestinal atresia due to intrauterine intussusception.
Histological studies were conducted on 4 patients with intestinal atresia due to intrauterine intussusception. This atresia is characterized by a polypoid lesion as the remnant of the invaginated and necrotized intestine in the distal intestinal lumen. As the result of the histological survey in the affected intestine, we tentatively classified this atresia into two types, type A and B. In type A, the polypoid lesion is observed at the blind end, with relatively good preservation of the structure of the intestine. In type B, the polypoid lesion is located apart from the blind end, and the lesion is markedly necrotic. The interruption of intestinal blood flow seemed to be more severe in type B than in type A.
Female↗
Intestinal atresia and stenosis: analysis of survival in 120 cases.
This report reviews the clinical presentation, operative management, and survival in 120 infants with intestinal atresia and stenosis treated from 1972 to 1984. Duodenal atresia occurred in 39 neonates and duodenal stenosis in 19. Thirty-two infants had severe associated anomalies. Operative management included duodenoduodenostomy in 47 infants, duodenotomy and web excision in four, and duodenojejunostomy in seven. Jejunoileal atresia occurred in 49 infants and stenosis in three. Six infants had cystic fibrosis and nine had gastroschisis. Operative therapy included wide proximal resection and end-to-end anastomosis in 18 infants, minimal resection with antimesenteric tapering enteroplasty and anastomosis in 14 neonates, and resection with temporary enterostomies in 20 infants. Twenty-nine infants (56%) required total parenteral nutrition. Colon atresia occurred in 11 infants and stenosis in one. Initial end-colostomy with subsequent resection and anastomosis was performed in 11 infants while one underwent a primary resection. The survival rate was 91% for duodenal defects, 87% for jejunoileal cases, and 100% for colonic anomalies. Death is currently caused by severe associated anomalies in infants with duodenal atresia and sepsis and total parenteral nutrition-related cholestasis with progressive liver failure in instances of jejunoileal atresia.
Colon↗
Gastroschisis complicated by intestinal atresia.
Five of 22 infants operated upon in the last 4 yr for gastroschisis had an associated loss of intestinal continuity. Four of the infants had an intestinal atresia and one had a gangrenous segment of ileum. Each had primary closure of the gastroschisis and decompression of the proximal intestine. Four had cutaneous enterostomy and one had long tube intubation. Four infants were returned to the operating room between 2 and 4 wk of age and their intestinal continuity was established. At the second operation the intestine, which had presented as a matted mass at birth, appeared normal with resolution of the serosal edema and few adhesions. The length of the intestine that initially had appeared shortened may be more accurately evaluated and is usually considerably greater than was appreciated at birth. If distal atresias are present, they will become apparent. A primary anastomosis can be carried out using the principles of repair for an isolated intestinal atresia in the newborn. All five of these infants have survived.
Abdominal Muscles↗
Isolated small intestinal atresias in Latin America and Spain: epidemiological analysis.
Stenosis, atresia, or absence of part of the duodenum, jejunum, or ileum are generally considered small intestinal atresias (SIAs). SIAs occur as isolated defects, in combination with other unrelated congenital anomalies, or as part of syndromes. We performed an epidemiological study of infants with isolated SIAs using data from two large congenital defects registries, one from Latin America (ECLAMC) and the other from Spain (ECEMC). The overall prevalence of SIAs is similar in both programs, being 1.32 per 10,000 livebirths in Spain and 1.29 per 10.000 livebirths in Latin America. Our results suggest that infants with isolated SIAs are characterized epidemiologically on the basis of shorter gestational age and low birthweight, an association with twinning, the parents are more frequently consanguineous, and their pregnancies are more frequently complicated by vaginal bleeding. The results also suggest an association between some maternal infections and ileal atresia. The fact that these characteristics have been observed in children with these types of anomalies occurring in different geographical areas and populations supports the conclusion that these characteristics are causally related to these defects.
Adult↗
Umbilical cord ulcer: a serious in utero complication of intestinal atresia.
We have experienced six cases of umbilical cord ulcer that resulted in life-threatening antenatal fetal haemorrhage. Fetal distress and bloody amniotic fluid that led to intrauterine and neonatal death in four patients complicated the pregnancy courses. The remaining two infants are still alive. All the infants had upper intestinal atresia below the papilla of Vater. Pathologically, umbilical arteries and surrounding Wharton's jelly were necrotic in all cases. Activated macrophages containing pigment granules were present within the ulcer bed, necrotic areas of Wharton's jelly and fetal membranes. During the same period, we encountered 38 other cases of upper intestinal atresia and the placentae were examined in 23 cases. Microscopic degeneration of Wharton's jelly was seen in three out of 23 cases. Total bile acids content in amniotic fluid was elevated in one case of umbilical cord ulcer. We speculate that the umbilical cord ulcer is closely related to in utero regurgitation of the bile. We also think that the incidence of this serious association is higher than has been reported previously.
Adult↗
Fusion of intussusceptum and intussuscipiens in intrauterine intussusception: a rare type of intestinal atresia.
Intrauterine intussusception is well known as one of the rare causes of intestinal atresia. Although the polypoid intussusceptum is usually observed at the obstructed end on the distal side, a few cases with the polypoid lesion located apart from the blind end have been reported. To elucidate the etiology of separated polypoid intussusceptum, we reviewed 42 surgical cases of jejunal or ileal atresia over the last 12 years at Kobe Children's Hospital, Kobe, Japan. Of the 42 cases, 11 were intrauterine intussusception. Two of the 11 cases were associated with polypoid intussusceptums separated from the obstructed ends; the intestinal portion between the polypoid intussusceptum and the obstructed end showed a partial two-fold proper muscle wall and a mesenteric structure invaginated between the two walls. Another case showed linear ulcers facing each other on both the intussusceptum and intussuscipiens. Linear ulceration and subsequent fusion of the intussusceptum and intussuscipiens are suggested to be the pathogenesis of the first two cases.
Age of Onset↗