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A twin is described who was born with ileal atresia and hydranencephaly, the co-twin having died at about 24 weeks' gestation. If a macerated or autolysed twin is found at birth, the paediatrician should be alerted to the possibility of serious and not immediately obvious defects in the surviving infant.
The problems of surgical treatment of the low fistulous forms of anal atresia in children with the use of postero-sagittal approach are considered. A modification of this method is described. The postoperative results are presented, and the possible complications as well. A conclusion about the advantages of the operative approach described is made.
Rectourethral and vesical fistulas in patients with high anorectal atresia can be demonstrated by using a pressure Gastrografin enema through the distal colostomy opening. A technique is described where contrast medium is forced by gentle pressure, filling the fistula and the urethra. This picture is of great value during the repair of these anomalies.
We report 2 cases of umbilical cord ulcer associated with fetal jejunal atresia. Both of them developed a severe intrauterine hemorrhage, followed by fetal heart rate decelerations, and underwent emergency cesarean section. Bloody amniotic fluid and umbilical cord ulcers were observed in both cases. Although both cases were successfully resuscitated, neurological impairment and renal failure developed in 1 case due to prolonged asphyxia. In a review of the literature, umbilical cord ulcer was associated only with congenital duodenal atresia or jejunal atresia, but not with ileal atresia. Although the prenatal diagnosis of duodenal or upper jejunal atresia has been established, the prenatal diagnosis of this complication has not been reported. In such cases, detailed examination of the umbilical cord by ultrasonography may be useful for the prenatal diagnosis of this disease.
Two children with cholelithiasis several years after relief of a duodenal atresia are reported. A survey of the literature on duodenal atresia suggests that there may be associated abnormalities in the biliary tract in this condition, and raises the possibility of cholelithiasis developing in these patients many years after successful relief of the duodenal obstruction by some form of by-pass procedure.
Associated anomalies are often found in newborn with duodenal obstruction. Anomalies of the biliary tract are of special interest. Malformations of the terminal common bile duct and the papilla of Vater are the most common ones. The recognition of the possible anatomical variations is most important to avoid inadvertent injuries in the correction of duodenal obstruction.
Atresia of the colon was first described in the literature. The authors present 4 cases seen at the paediatric surgery division of the Regina Margherita Children's Hospital in Turin. After a brief discussion of the embryology, attention is focused on the treatment of this malformation.
Duodenal atresia can be fatal unless promptly diagnosed and treated surgically. Death occurs in the newborn secondary to emesis, aspiration, and electrolyte imbalance. Serial ultrasound scans were obtained for 2 patients, but duodenal atresia was not detected until 29 and 32 weeks' gestation, respectively. With prior knowledge of an infant with Down syndrome and duodenal atresia, management of fetal distress with subsequent operative delivery can be altered. Early prenatal diagnosis by ultrasonography and subsequent amniocentesis plays an important role in the antenatal and postpartum counseling and management of these patients and neonates.
Bezoar formation is a rare but well-recognised complication of previous gastric surgery and persimmon ingestion. We present a case of bezoar formation in the duodenum of a child with a grossly dilated, dysmotile duodenum following surgery for jejunal atresia. The presenting symptoms were vomiting, diarrhoea, and growth failure, with evidence of malabsorption secondary to bacterial overgrowth in the bezoar. Plication of the duodenum to reduce its size and improve its motility may reduce the likelihood of future bezoar formation.
Colonic atresia (CA) has been reported in association with Hirschsprung's Disease (HSCR) on 11 previous occasions. In most reported cases the atresia has involved the right side of the colon; but in this, the twelfth case report, the atresia involved the left side of the colon, with aganglionosis of the entire distal gut. In addition, the child had an absent hand. The authors believe that this triad has not been reported previously. The literature is reviewed, and possible mechanisms are discussed.
A newborn infant with spontaneous oesophageal rupture associated with duodenal atresia is described. The diagnosis was established by the pattern of hydropneumothorax on the chest x-ray film and by the aspiration of gastric juice by thoracocentesis. Necropsy showed a tear, which extended from the oesophagogastric junction to the upper portion of the stomach.
We report the case of a neonate with an ileal duplication cyst and type-3 ileal atresia. Although various pathogenetic mechanisms have been suggested to explain the genesis of these separate entities, this case and others previously described lend support to the theory of "vascular pathogenesis" for both of these lesions. The relevant literature pertaining to the causation of these lesions is discussed.
A new case of duodenal atresia with gas below the obstruction is reported. This apparently contradictory feature is due to a bifid termination of the common bile duct in the duodenum, air by-passing the atretic area. The anomaly was proven by operative cholangiography.
Congenital duodenal obstruction, a common complication of Down syndrome, most often presents as neonatal bilious vomiting. We report the first premature infant with Down syndrome whose duodenal atresia presented with a massive hematemesis secondary to duodenitis. Since a plain radiograph of the abdomen is diagnostic in the case of duodenal obstruction, it should be included in the diagnostic workup of hematemesis in newborn infants.
Correction of incontinence as the sequel of a pull-through operation for anorectal atresia, leaving aside cases due to a mistake of the surgical technique, is based on the construction of a new sphincter. This can be done in several ways, using striped, pedicled, denervated, free, or, more recently, smooth muscle. The techniques, objectives, and results are described with reference to the latest data in the literature.
Pyloric atresia occurs in one in a million births and usually occurs sporadically. We report a family with nine affected members and review the literature for all reported familial cases. The article stresses the possibility of recurrence of pyloric atresia in the progeny of consanguinous parents, with an emphasis on prenatal diagnosis and counseling, diagnosis of the entity in neonates, associated conditions, and prognosis in sporadic and familial cases. A high index of suspicion in affected families contributes to the early diagnosis and treatment with a great impact on outcome.
Intrauterine intussusception, an uncommon cause of bowel obstruction, has rarely been detected by prenatal ultrasonography. We report two cases of intrauterine intussusception after gestation, which presented as isolated fetal ascites at 30 weeks of gestation by ultrasonography. In case 1, on the follow-up ultrasonography at 32 weeks of gestation, the previously observed ascites had disappeared, whereas the echogenicity of the bowel was increased without any sign of dilation, suggesting the presence of meconium peritonitis. The fetus was delivered at 39 weeks. In case 2, however, the amount of fetal ascites became increased, and the fetus was delivered at 34 weeks of gestation. After delivery, both infants were surgically explored with resection of the ileum with end-to-end anastomosis because of intrauterine intussusception and ileal atresia. From the experience of these cases, we suggest that the ultrasonographic finding of isolated or transient fetal ascites might contribute to the early diagnosis and management of intrauterine intussusception.
Eighty-four patients with congenital jejuno-ileal atresias and stenoses admitted over a 20-year period (1959-1978) are reviewed. The overall survival rate of 88% is a reflection of the improvement in diagnosis and supportive care and refinements in technique. Our current ideas on this subject are expressed and a detailed account of the presentation, diagnosis and management is given.