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Duodenojejunal atresia with apple peel configuration of the ileum and absent superior mesenteric artery: observations on pathogenesis.

A child with loss of the third and fourth part of the duodenum and of the proximal jejunum was found to have an apple peel configuration of the remaining small bowel. The complete absence of branches from the superior mesenteric artery impaired the blood supply of the distal duodenum. An annular pancreas was found in this patient with Down's syndrome. This anomaly may have impaired the flow through the pancreaticoduodenal arcade, which would normally compensate for the distal vascular occlusion. According to current understanding, duodenal atresia is a primary malformation. The current case suggests, however, that in rare circumstances vascular accidents may be the underlying cause for duodenal atresia.

Duodenal Obstruction↗

Gastrointestinal malformations in Funen county, Denmark--epidemiology, associated malformations, surgery and mortality.

AIM: To report the epidemiology, associated malformations, morbidity and mortality for the first 5 years of life for infants with gastrointestinal malformations (GIM). METHODS: Population-based study using data from a registry of congenital malformations (Eurocat) and follow-up data from hospital records. The study included livebirths, fetal deaths with a gestational age of 20 weeks and older and induced abortions after prenatal diagnosis of malformations born during the period 1980 - 1993. RESULTS: A total of 109 infants/fetuses with 118 GIM were included in the study giving a prevalence of 15.3 (12.6 - 18.5) cases per 10 000 births. Anal atresia was present in seven of the 9 cases with more than one GIM. There were 38 cases (35 %) with associated malformations and/or karyotype anomalies. Thirty-two of the 90 live-born infants died during the first 5 years of life with the majority of deaths during the first week of life. Mortality was significantly increased for infants with associated malformations or karyotype anomalies compared to infants with isolated GIM (p < 0.01). An uneventful surgical course was reported for 74 % of the 58 survivors. CONCLUSIONS: The prognosis for infants with GIM is highly dependent on the presence of associated malformations or karyotype anomalies. Surgery for GIM can be performed with low mortality. Morbidity is high for a small group of infants, but the majority of survivors have an uncomplicated surgical course.

Anal Canal↗