[Total colon atresia in infants].
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The VACTERL complex comprises renal agenesis and atresias of the alimentary and respiratory tracts. We report on a case with this combination causing severe oligohydramnios but with normal lung development. The likely protective mechanism for pulmonary development was an increase in alveolar pressure and reduced alveolar fluid loss due to the esophageal-tracheal malformation. This suggests the possible treatment of oligohydramnios by tracheal occlusion.
The association of pure esophageal atresia, duodenal atresia, and unilateral lung agenesis has not been reported previously. Here the authors present a case of a newborn with this constellation of anomalies that underwent staged repair. The primary principle guiding treatment was the avoidance of iatrogenic injury to the single lung. Therefore, the order of operations proceeded as follows: (1) placement of a decompressing gastrostomy tube, (2) repair of the duodenal atresia, and (3) repair of the esophageal atresia. The congenital closed loop obstruction caused by the esophageal and duodenal atresias was beneficial in that it resulted in growth by stretching of the distal esophagus, allowing a tension-free primary repair of the esophageal atresia.
This report presents an unusual case of a congenital long-segment jejunoileo-colic obstruction without mesenteric or intestinal interruption. Histologically, the intestinal lumen was completely or partially obstructed by fibrous or granulation tissue, and the mucosa had disappeared.
An unusual case of biliary atresia with jejunal atresia is herein described. Only 12 cases demonstrating biliary atresia associated with a jejunal atresia have been previously reported in Japan. The pathogenesis of biliary atresia is thought to be secondary to the influence of jejunal atresia.
Ultrasound examination of a 22-week fetus demonstrated a grossly distended stomach and proximal duodenum, with a large cystic area in the chest. There was associated polyhydramnios. A presumptive diagnosis of duodenal atresia with a congenital diaphragmatic hernia was made. Analysis of a fetal blood sample showed that the fetus had Down's syndrome. Histological examination after termination of the pregnancy revealed the unusual combination of duodenal and esophageal atresia, with an intact diaphragm.
Rectal atresia is an extremely rare anorectal malformation. A unique case of rectal atresia presenting as an imperforate membrane associated with congenital cardiac malformations and unilateral choanal atresia in a newborn is reported. Ultrasound examination in the newborn infant located the presence of the rectal membrane which was surgically treated successfully.
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