Tandler’s paper “Developmental history of the human duodenum during early embryological stages” serves as an example of scientific networking at the begin- ning of the last century . Well established anatomists, including Zuckerkandl, Hochstetter, and Kollmann, contributed specimens to the study. Tandler ex- amined histological sections of 7 human embryos with a body length between 7 and 21.6 mm. Near total or total epithelial occlusion of the duodenal lumen was present in embryos of 12.5, 14.5, and 15 mm length. Then, epithelial occlusion recanalization occurred. Furthermore, Tandler described a cranio-caudal direc- tion of epithelial and mesenchymal proliferation and a delayed increase in the diameter of the duodenal tube during subsequent development. In addition, he reported closely comparable duodenal embryology in rats and guinea pigs. However, the latter observations are not reported in detail. Furthermore, Tand- ler compared his observations with earlier observations regarding the pathoge- nesis of jejunoileal atresia, and fetal intrauterine events, such as torsion of the bowel, embryonic ligamentous residuals, fetal peritonitis, and vascular thrombo- sis, seemed to be causative . Tandler concluded that a failure of physiologic recanalization of the duodenum might be the most likely mechanism for the pa- thogenesis of duodenal atresia.
ultrasonography, it is possible to establish the diagnosis among 52 % patients. Duodenal atresia is manifested by a“double-bubble” sign, in the way that the first bubble corresponds to the stomach, while the second one matches postpiloric prestenotic dilated duodenum . Half of the infants have birthweight below 2.5 kg, which correlates to hydramnion because fetus is not able to absorb nutrients from the amniotic fluid. Clin- ically, there is manifestation of symptoms and signs of high bowel obstruction and presence of bilious vomiting several hours after delivery. Among 15 %
DOI: 10.4236/ojog.2019.98115 1191 Open Journal of Obstetrics and Gynecology ment that has complete obliteration of the lumen. Duodenal atresia may occur as an isolated anomaly or in association with other anomalies. Approximately one-third of fetuses with duodenal atresia have trisomy 21 (30%), and with anatomic malformation in about 50% cases including cardiac defects, cleft lip and palate and other digestive system anomalies, defect of the urinary and mus- culoskeletal systems. Duodenal atresia is the most common, but not the only cause of duodenal obstruction  .
Case presentation: We present a case of a 30-year-old G1P0 white woman at 22-week gestation with a monochorionic-diamniotic twin pregnancy discordant for esophageal atresia, duodenal atresia with gastric perforation, hypoplastic left heart structures, and significant early gestation maternal polyhydramnios. In this case, fetal magnetic resonance imaging was able to depict additional findings including area of gastric wall rupture, hiatal hernia, dilation of the distal esophagus, and area of duodenal obstruction and thus facilitated the proper diagnosis. After extensive counseling at our multidisciplinary team meeting, the parents elected to proceed with radiofrequency ablation of the anomalous twin to maximize the survival of the normal co-twin. The procedure was performed successfully with complete cessation of flow in the umbilical artery and complete cardiac standstill in the anomalous twin with no detrimental effects on the healthy co-twin.
In 1954, Santulli reported a mortality rate of 44.8% 6 , compared with the mortality rate of 2.8% in 1989 5 . These data are consistent with other more recent studies that looked at lesions responsible for neonatal bowel obstruction. In three series that reviewed intestinal atresia, the operative mortality for jejunoileal atresia was 0.8% and increased to 4% for duodenal atresia when associated with a cardiac anomaly 7. The long-term survival ranged from 86% to 93% 8,9,10 .
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The newborn infant of a mother with polyhydramnios should always have a nasogastric tube passed soon after delivery to exclude oesophageal atresia. Infants with oesophageal atresia are unable to swallow saliva and are noted to have excessive salivation requiring repeated suc- tioning. At this stage, and certainly before the first feed, a stiff wide-bore (10–12 French gauge) catheter should be passed through the mouth into the oesophagus. In oesophageal atresia the catheter will not pass beyond 9– 10 cm from the lower alveolar ridge. A plain X-ray of the chest and abdomen will show the tip of the catheter arrested in the superior mediastinum (T 2–4) while gas in the stomach and intestine signifies the presence of a distal tracheooesophageal fistula (Fig 2). The absence of gas- trointestinal gas is indicative of an isolated atresia. A fine bore catheter may curl up in the upper pouch giving the false impression of an intact oesophagus or rarely it may pass through the trachea and proceed distally into the oesophagus through the fistula. The X-ray may reveal additional anomalies such as a "double bubble" appear- ance of duodenal atresia, vertebral or rib abnormalities. Associated anomalies
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Intestinal malrotation is a developmental anomaly affecting the position and peritoneal attachments of the small and large bowels during organogenesis in foetal life. It has been defined as absent or incomplete rotation and fixation of the embryonic gut around the superior mesenteric artery. In the present paper, we review the definition, history, embryology/aetiology, epidemiology, symp- toms and signs, diagnosis and treatment of intestinal malformations. Moreover, we report the records of 30 cases of malrotation admitted to our department over a period of five years. The final intraoperative diagnosis of the cases pre- sented was 53.3% pure malrotation, 33.3% malrotation with mid-gut volvulus, 6.7% malrotation with duodenal atresia, 3.3% malrotation with Meckel’s diver- ticulum and duodenal atresia, and 3.3% malrotation and biliary atresia. Preoper- ative imaging studies were performed for 27 cases and surgical management was successfully conducted without any mortality among the cases studied. This article provides an overview of basic and clinical aspects of intestinal malrota- tion. In addition, the signs and symptoms, imaging findings, and final intraoper- ative diagnoses presented by the subjects reported on are of potential use and clinical interest.
This study aimed to investigate the frequency of congenital foregut anomalies among neonates referred to pediatric surgery ward of Ahvaz Imam Hospital during 2007-2010. This was a retrospective study conducted on the medical records of all of patients with foregut anomalies. Sex, family history, associated anomalies, types of foregut anomalies including esophageal atresia/tracheoesophagel fistula, pyloric atresia, duodenal atresia, hypertrophic pyloric stenosis, gasteroesophageal reflux disease, and foregut duplication among neonates were investigated. Statistical analyses of the data were performed with SPSS. In this study, amount of esophageal atresia/ tracheoesophageal fistula is equal with hypertrophic pyloric stenosis and is higher than amount of duodenal atresia. Pyloric atresia is rare (2 cases). No patient has been admitted with diagnosis of Gastroesophegeal reflux disease and foregut duplication. There is relative increase in male to female. The amount of family history is similar to other investigations. The frequency of associated anomalies is less than other studies, which probably because of failure of diagnosis.
DESIGN AND RESULTS. Twenty-three fetuses diagnosed with pulmonary atresia with intact ventricular septum between 1990 and 2004 were studied. Of 13 fetuses with a midgestation fetal tricuspid valve z score ⱕ⫺ 3, 1 achieved biventricular repair, compared with 5 of 5 with a tricuspid valve z score ⬎⫺ 3. Of 13 fetuses with a midgestation fetal tricuspid valve z score ⱕ⫺ 3, 8 were diagnosed postnatally with a right ventricular dependent coronary circulation, compared with none with a tricuspid valve z score ⬎⫺ 3. Midgestation and late gestation fetal tricuspid valve z scores correlated with neonatal tricuspid valve z score. The average rate of tricuspid valve growth between mid- and late fetal echocardiograms was significantly lower in patients who did not achieve biventricular repair than in those who did (0.012 ⫾ 0.008 cm per week vs 0.028 ⫾ 0.014 cm per week).
Until now unknown what teratogen substances that can cause abnormalities of eso- phageal atresia, recurrent case reported only about 2% if one of the twin sibling is af- fected . Esophageal atresia was associated with trisomy syndrome 21.13 and 18 with suspected genetic cause. But this time, the theory about the occurrence of esophageal atresia according to most experts no longer associated with a genetic disorder .
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In our institution, preoperative tracheo-bronchoscopy (TrSc) is routinely employed to determine the presence, site, and number of TEFs. However, according to a recent international survey on the management of esopha- geal atresia, the use of perioperative endoscopy remains controversial  . The aim of the present study was to evaluate the primary management of TEF ± EA in a medium-sized center with 2200 operations per year. TrSc and surgical repair were routinely performed within the same operation. As a follow-up, we assessed surgically relevant airway morbidity over the first 3 months postoperatively.
There has been no improvement in the age at diagnosis of BA over the past 15 years. Indeed, there is a concern- ing trend toward an increase, with many patients still being diagnosed far too late. The overall mean age at diagnosis of 65 days, ranging from 21 to 152 days, is similar to that reported by the Biliary Atresia Research Consortium (BARC) of 9 referral centers in the United States, 5 where, between 1999 and 2003, the mean age at
Case presentation: Aplasia of the optic chiasm was identified in a Caucasian Italian 8-month-old boy with esophageal atresia. An ultrasound examination carried out at 21 weeks ’ gestation revealed polyhydramnios. Intrauterine growth retardation, esophageal atresia and a small atrial-septal defect were subsequently detected at 28 weeks’ gestation. Repair of the esophageal atresia was carried out shortly after birth. A jejunostomy was carried out at four months to facilitate enteral feeding. The child was subsequently noted to be visually inattentive and to be neurodevelopmentally delayed. Magnetic resonance imaging revealed chiasmal aplasia. No other midline brain defects were found. His karyotype was normal.
Post-operatively, different drugs have been proposed either for reducing the inflammatory process at the liver hilum, which might lead to granulation and fibrous scar obstructing the biliary ductules, or for increasing the bil- iary flow. Although recommended by several surgeons [72-74], the use of corticosteroids remains controversial since their long-term benefit has not been proven; in addi- tion there is a theoretical risk of exacerbating cholangitis. During the evaluation phase of biliary atresia, the infant's diet typically is not changed; post-operative breastfeeding is encouraged when possible, but an energetic supplemen- tation may be required to obtain a 150 to 180 Kcal/kg/day intake. As long as cholestasis persists, supplementation in fat-soluble vitamins (ADEK) is needed.