[PTHOGENESIS OF CERTAIN SURGICAL FORMS OF INTESTINAL ATRESIA].
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Two siblings who presented shortly after birth with signs of upper intestinal obstruction were successfully operated for apple peel jejunal atresia. In addition to intestinal malformations, both siblings exhibited severe microcephaly and ocular abnormalities not previously reported in this condition.
BACKGROUND: Fetal alcohol syndrome is quite common in our region (incidence: 1/700 live births). It usually associates facial dysmorphism, intra-uterine growth retardation and mental delay. CASE REPORT: Jonathan, born to an alcoholic mother, presented a typical dysmorphy of fetal alcohol syndrome and a low inserted umbilicus. A small bowel atresia was discovered at the third day of life and operated on. CONCLUSION: Although never been previously described, this association suggests a common embryological origin between fetal alcohol syndrome, small bowel atresia, and umbilical abnormality.
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A simple technique was used successfully for retraining maximum bowel length in a premature baby born with type 3A jejunal atresia. Primary end-to-end anastomosis of the tip of the dilated proximal segment to the remaining viable distal 5 cm of ileum was performed. A tube passed via the cecum proximally into the small bowel acted as a stent for the anastomosis and decompression of the bowel contents in the proximal dilated segment. This simple method may be a viable option to avoid resection of the dilated segment when the bowel length is marginal.
Eighty-four patients with congenital jejuno-ileal or colonic atresia treated at this hospital during the years 1961-86 were studied. There were 42 cases of jejunal atresia, seven cases of jejuno-ileal atresia, 33 cases of ileal atresia and two cases of colonic atresia. Multiple atresias occurred in 19 patients. During the first 15 years of the study the mortality rate was 56%. In the last 10 years it was 22%. Multivariate linear modelling techniques were used to determine the factors associated with a fatal outcome. In order of magnitude associations were found between death and year of treatment (P less than 0.01), the type of anastomosis performed to correct the atresis (P less than 0.05), the presence of other congenital abnormalities (P less than 0.01) and presence of a malrotation (P less than 0.02). The anastomoses associated with the highest mortality were those with a stoma. The mean length of bowel resected proximal to the anastomosis was 15 cm and the mean length resected distally 5 cm. Eleven patients had 60 cm or less of small bowel remaining after surgery and only three of these survived. The highest mortality rate can be expected in patients with other congenital abnormalities and those with a malrotation. A primary anastomosis without a stoma is recommended.
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In children with biliary atresia, defective intestinal absorption of vitamin D and impaired hepatic uptake and 25-hydroxylation of vitamin D lead to a deficiency of vitamin D and rickets. We recently observed severe rickets in a 3-year-old boy with corrected biliary atresia resulting in jaundice, despite oral treatment with 1 alpha-hydroxyvitamin D3 (1 alpha-OHD3) or 1,25-dihydroxyvitamin D3 [1,25-(OH)2D3]. He had low 25-hydroxyvitamin D (25-OHD) and high 1,25-(OH)2D serum levels. Intramuscular vitamin D2 administration produced radiological and biochemical evidence of recovery. Oral 1,25-(OH)2D3 (0.1 microgram/kg) and 25-OHD3 (10 micrograms/kg) tolerance tests were done to assess the ability to absorb vitamin D and the effectiveness of using these drugs orally. Eleven children with corrected biliary atresia, aged 9 months to 7 years, were studied. In oral 1,25-(OH)2D3 tolerance tests, the increments above the baseline serum levels of 1,25-(OH)2D were 140.7 +/- 27.4 pg/ml in nonjaundiced patients (n = 5). In jaundiced patients (n = 3), 1,25-(OH)2D3 absorption in two patients with high basal 1,25-(OH)2D values was lower than that of nonjaundiced patients; however, the absorption in the third patient with a low basal value was similar to that of nonjaundiced patients. In oral 25-OHD3 tolerance tests, the mean increase of serum 25-OHD was 48.9 +/- 30.6 ng/ml in nonjaundiced patients (n = 5) and 23.7 +/- 9.5 ng/ml in jaundiced patients (n = 4), the peak serum 25-OHD levels being reached 6-12 h after 25-OHD3 loading.(ABSTRACT TRUNCATED AT 250 WORDS)
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CASE REPORT: A case of intrauterine intussusception causing ileal atresia is reported. The neonate presented at the age of one day with bilious vomiting, failure to pass meconium and progressive abdominal distension. A clinical diagnosis of intestinal obstruction was made, and was confirmed by plain abdominal radiographs. At laparotomy, atresia of the terminal ileum was found. The lumen of the resected distal segment contained an intussusceptum. The blind ends were resected and an anastomosis was performed. Recovery was uneventful. COMMENTS: Intrauterine bowel intussusception is a rare clinical entity. When intussusception occurs in the foetus and the time elapsed is enough to allow for gangrene and resorption of the intussuscepted portions of the bowel, intestinal atresia may result. CONCLUSIONS: The careful examination of the distal blind end of the ileum aids to obtain a correct classification and etiologic diagnosis of the atresia.
Information on oesophageal, duodenal and rectal atresias was collected from the hospitals, malformation register and death certificates in Finland from 1970-79. The respective prevalence rates were 4.1, 1.4 and 3.6 per 10,000 births, which are among the highest rates cited in the literature. Mothers of children with oesophageal and duodenal atresias were older than the average for child-bearing women in Finland. The sex ratio (male/female) of rectal atresia patients was 2.4. About 50% of the atresia patients had associated malformations. The data indicated time-spaced clustering for atresias. Furthermore, the cases of oesophageal and duodenal atresias appeared to spread within a time period of 30 days from a province to its neighbouring provinces to the north. Spreading was not observed for rectal atresias but clustering was noted when smaller geographic areas and individual communities were analysed. Occupations involving animal contacts were overpresented among the fathers of atresia patients. The data suggest infective aetiology, probably with no known clinical symptoms and no seasonal patterns.