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Introduction Duodenal atresia (DA) is the most common atresia of the small bowel. This study aims to assess the prevalence, mortality, and associated anomalies related to DA in Finland from 2004 to 2017. Material and Methods A nationwide study based on registers maintained by the Finnish Institute for Health and Welfare and Statistics Finland containing data on all live births and stillbirths and terminations of pregnancy. The cases were identified based on the ICD-9 and 10 (International Classification of Diseases revisions 9 and 10) codes. Associated anomalies were classified based on the EUROCAT criteria; minor anomalies were excluded. Results There were 249 DA cases including 222 (89.2%) live births, 16 (6.4%) stillbirths, and 11 (4.4%) terminations. There was no significant change in the prevalence rates between 2004 and 2017. Live birth prevalence was 2.75/10,000 and total prevalence was 3.08/10,000 births. A total of 100 (40.2%) cases were isolated, 67 (26.9%) had other multiple congenital anomalies, and 83 (33.3%) were syndromic. There were no terminations in isolated DA. Most associated anomalies were cardiac (36.1%), followed by other gastrointestinal tract anomalies (23.7%) and limb deformities/defects (7.2%). Trisomy 21 was observed in 63 cases (25.3%). Neonatal mortality was 3.6% (n = 8) and at 1 year 95.0% were alive. Both neonatal and infant mortalities were associated with cardiac anomalies (p < 0.001 and p = 0.001, respectively). All neonatal deaths had associated cardiac defect(s). Conclusions The prevalence of DA in Finland remains stable and among the highest reported. DA is often associated with cardiac anomalies, which portend a high risk for mortality. Despite the burden of associated anomalies, overall survival is high. [ABSTRACT FROM AUTHOR]

Objectives Our objective was to investigate the prevalence of small intestinal atresia and Hirschsprung's disease (HD) in infants with Down syndrome (DS) and its impact on outcomes. Study Design We analyzed the National Inpatient Sample dataset. We included infants with DS, small intestinal atresia, HD, and the concomitant occurrence of both conditions. Regression analysis was used to control clinical and demographic variables. Results A total of 66,213,034 infants were included, of whom, 99,861 (0.15%) had DS. The concomitant occurrence of small intestinal atresia and HD was more frequent in infants with DS compared with the general population, adjusted odds ratio (aOR): 122, 95% confidence interval (CI): 96–154, (p < 0.001). Infants with DS and concomitant small intestinal atresia and HD had higher mortality compared with those without these conditions, aOR: 8.59, 95% CI: 1.95–37.8. Conclusion Infants with DS are at increased risk of concomitant small intestinal atresia and HD, and this condition is associated with increased mortality. Key Points Infants with Down syndrome are at increased risk of congenital GI anomalies. Infants with Down syndrome are at increased risk of necrotizing enterocolitis. Increased mortality in Down syndrome infants with concomitant small intestinal atresia and Hirschsprung's disease. [ABSTRACT FROM AUTHOR]

A rare case of bilateral fetal meromelia with duodenal atresia (DA) was diagnosed on three-dimensional (3D) sonography. A 32-year-old G3P2002 with a large for date uterus, was reviewed at 26 weeks' gestation. The 3D sonogram confirmed the presence of the humerus, olecranon process, and only the proximal third of the ulna bone, bilaterally. The distal ulna, radius, metacarpals, and phalanges were absent on both upper limbs. The bones of the lower limbs were present and appropriate for gestational age. Further evaluation revealed the "double bubble" sign, which is characteristic of DA within the fetal abdomen. The patient was induced 2 weeks later, due to intrauterine fetal demise. The suspected fetal anomalies were confirmed at birth. A high index of suspicion and a detailed sonographic evaluation are essential for detecting rare fetal anomalies, such as meromelia. [ABSTRACT FROM AUTHOR]

Background and Objectives: Duodenal atresia and stenosis are common causes of intestinal obstruction. Associated anomalies significantly influence early postoperative mortality, while postoperative complications impact long-term survival. Materials and Methods: Over a 13-year period from January 2010 to August 2023, a total of 74 infants and children with congenital duodenal obstruction were treated at "Grigore Alexandrescu" Children's Emergency Hospital and met the inclusion criteria. All patients diagnosed with duodenal obstruction (both instrinsic and extrinsic causes) were included. Analysed data included congenital anomalies, Apgar scores, birth weights, surgical techniques, and complications. Results: The associated anomalies included cardiac (n = 33), Down syndrome (n = 13), neurological (n = 11), pulmonary (n = 7), renal (n = 4), skeletal (n = 1), and gastrointestinal and hepatobiliopancreatic anomalies (n = 25). In total, 12 patients experienced perioperative ventilation problems. Early postoperative complications (within 30 days) occurred in 21 patients, while 6 had late postoperative complications (after 30 days). Among non-surgical complications, we noted ventilation problems, sepsis (n = 7), and pneumothorax (n = 1). Surgical complications included adhesive bowel obstruction (n = 7), incisional hernia (n = 3), peritonitis (n = 3), dysfunctional duodenoduodenostomy or duodenojejunostomy (n = 3), pneumoperitoneum (n = 5), enteric fistula (n = 3), and volvulus (n = 4). Conclusions: Surprisingly, this retrospective study revealed that an Apgar score below 8, along with neurological and pulmonary abnormalities, is associated with postoperative complications. Conversely, other congenital anomalies, low birth weight, and age at admission do not serve as prognostic factors. [ABSTRACT FROM AUTHOR]

Background: Laparoscopic repair of duodenal atresia (LRDA) remains a technically challenging procedure and its benefits ambiguous. To assess the safety and efficacy of LRDA, we performed a systematic review of techniques and material for LRDA and a meta-analysis comparing outcomes with open repair (OR). Methods: Comprehensive search of EMBASSE, PubMed and Cochrane was performed from 2000 to 2023. Studies comparing LRDA with OR were identified and outcomes extracted included operative time, time to enteral feeds, length of hospitalisation, anastomotic leaks and stricture and total complications. χ2 was used to assess associations between complications and conversions rates of different LRDA approaches (laparoscopic technique, suturing technique). Comprehensive meta-analysis was used for Meta-analysis. Results: Twelve studies were identified and 1731 patients were enrolled in the study (398 [LRDA] and 1325 [OR]). Total rate of complications and conversion for LRDA was 15.58% and 18.84%, respectively. Complication rates were not significantly affected by operative technique and suturing technique. Conversion rates were not significantly affected operative technique; using a combination of interrupted and running suturing was significantly higher than using running or interrupted (χ2 = 7.45, p < 0.05). Anastomotic leaks, strictures and total complications were equivocal between LRDA and OR (OR 1.672, 95% CI 0.796–3.514; OR 2.010, 95% CI 0.758–5.333; OR 1.172, 95% CI 0.195–7.03). Operative time was significantly greater for LRDA (SDM 1.035, 95% CI 0.574–1.495, p < 0.001). Time to initial and full enteral feeds and length of hospitalisation were shorter in the LRDA group (SDM − 0.493, 95% CI − 2.166 to 1.752, p = 0.466; SDM − 0.207, 95% CI − 1.807 to 0.822, p = 0.019; SDM − 0.111, 95% CI − 1.101 to 0.880, p = 0.466, respectively). Conclusions: LRDA showed equivalent complication rates compared to OR with an additional benefit of quicker establishment of feeds. There was no significant difference in complication and conversion rates between laparoscopic techniques. Despite a longer operative time, LRDA provides a safe minimal access approach for neonates after this consistent implementation of the technique in the past decade. [ABSTRACT FROM AUTHOR]

Background and Objectives: Duodenal atresia and stenosis are common causes of intestinal obstruction. Associated anomalies significantly influence early postoperative mortality, while postoperative complications impact long-term survival. Materials and Methods: Over a 13-year period from January 2010 to August 2023, a total of 74 infants and children with congenital duodenal obstruction were treated at “Grigore Alexandrescu” Children’s Emergency Hospital and met the inclusion criteria. All patients diagnosed with duodenal obstruction (both instrinsic and extrinsic causes) were included. Analysed data included congenital anomalies, Apgar scores, birth weights, surgical techniques, and complications. Results: The associated anomalies included cardiac (n = 33), Down syndrome (n = 13), neurological (n = 11), pulmonary (n = 7), renal (n = 4), skeletal (n = 1), and gastrointestinal and hepatobiliopancreatic anomalies (n = 25). In total, 12 patients experienced perioperative ventilation problems. Early postoperative complications (within 30 days) occurred in 21 patients, while 6 had late postoperative complications (after 30 days). Among non-surgical complications, we noted ventilation problems, sepsis (n = 7), and pneumothorax (n = 1). Surgical complications included adhesive bowel obstruction (n = 7), incisional hernia (n = 3), peritonitis (n = 3), dysfunctional duodenoduodenostomy or duodenojejunostomy (n = 3), pneumoperitoneum (n = 5), enteric fistula (n = 3), and volvulus (n = 4). Conclusions: Surprisingly, this retrospective study revealed that an Apgar score below 8, along with neurological and pulmonary abnormalities, is associated with postoperative complications. Conversely, other congenital anomalies, low birth weight, and age at admission do not serve as prognostic factors.

Introduction Coexistent congenital duodenal obstruction and esophageal atresia (EA) is known to have significant morbidity and mortality. Management strategies are not well-defined for this association. The data from the Turkish EA registry is evaluated. Materials and Methods A database search was done for the years 2015 to 2022. Results Among 857 EA patients, 31 (3.6%) had congenital duodenal obstruction. The mean birth weight was 2,104 (± 457) g with 6 babies weighing less than 1,500 g. Twenty-six (84%) had type C EA. The duodenal obstruction was complete in 15 patients and partial in 16. Other anomalies were detected in 27 (87%) patients. VACTERL-H was present in 15 (48%), anorectal malformation in 10 (32%), a major cardiac malformation in 6 (19%), and trisomy-21 in 3 (10%). Duodenal obstruction diagnosis was delayed in 10 (32%) babies for a median of 7.5 (1–109) days. Diagnosis for esophageal pathologies was delayed in 2. Among 19 babies with a simultaneous diagnosis, 1 died without surgery, 6 underwent triple repair for tracheoesophageal fistula (TEF), EA, and duodenal obstruction, and 3 for TEF and duodenal obstruction in the same session. A staged repair was planned in the remaining 9 patients. In total, 15 (48%) patients received a gastrostomy, the indication was long-gap EA in 8. Twenty-five (77%) patients survived. The cause of mortality was sepsis (n = 3) and major cardiac malformations (n = 3). Conclusion Congenital duodenal obstruction associated with EA is a complex problem. Delayed diagnosis is common. Management strategies regarding single-stage repairs or gastrostomy insertions vary notably depending on the patient characteristics and institutional preferences. [ABSTRACT FROM AUTHOR]

Neonates with duodenal atresia (DA) are often born prematurely and undergo repair soon after birth, while others are delayed to allow for growth until closer to term corrected gestational age (cGA). Premature infants have been demonstrated to experience worse outcomes, but it is unclear whether delaying surgery mitigates the increased morbidity. This study evaluates the association of timing of DA repair with postoperative morbidity. We retrospectively evaluated neonates undergoing DA repair from the National Surgical Quality Improvement Program-Pediatric database (2015–2020). A multivariable regression analyzed factors associated with composite morbidity, including cGA and age in days of life (DOL) at surgery. A propensity score matched analysis was completed in premature neonates born at ≤35 weeks gestation to compare outcomes at similar birth gestational ages (bGA) and birth weight who underwent early (<7 DOL) versus delayed (≥7 DOL) repair. 809 neonates were included with a median bGA of 36 weeks (IQR 34–38), birth weight of 2.46 kg (IQR 1.96–2.95), and DOL at surgery of 2 (IQR 1–5). Infants born ≤35 weeks represented 35.23% of the cohort. On multivariable analysis, increasing cGA at surgery was associated with decreased morbidity (OR: 0.91, CI [0.84, 0.99]), and increasing DOL at surgery was associated with increased morbidity (OR: 1.02, CI [1.00, 1.04]). On propensity score matched analysis, delayed repairs were associated with increased postoperative ventilation (6 days vs. 2 days, p < 0.05); however, there were no differences in composite or surgical morbidity between early and delayed repairs. Morbidity after DA repair in neonates ≤35 weeks cGA is primarily driven by non-surgical causes, but delaying surgery does not appear to mitigate the risks associated with prematurity. It seems reasonable to consider repair in neonates around 33–34 weeks gestation without prohibitive risk factors. Optimal timing of DA repair requires a delicate balance between these factors. Level III. Retrospective Cohort Study. [ABSTRACT FROM AUTHOR]

Tracheal atresia/agenesis (TA) is associated with other congenital anomalies (TACRD and/or VACTERL). In this study, a female newborn with TA who had all the elements associated with TACRD and VACTERL was reported. The patient with TA, tracheo-esophageal fistula (TEF) connecting to the esophagus at the level of the carina, persistent left superior vena cava, duodenal atresia, vertebral and limb defects, bilateral hydronephrosis, and persistent cloaca was operated on postnatal 5th day. Band ligation to the abdominal esophagus, gastrostomy, duodenal atresia repair and diverting colostomy due to persistent cloaca were performed. The lungs were bilaterally expanded in the first postnatal, preoperative and postoperative chest radiographs. Oxygen saturation remained above 90% until the patient died suddenly on the postnatal 8th day. TA is a very rare congenital anomaly that causes postnatal respiratory distress. This case is unique among the literature on TACRD and VACTERL associations for many reasons. Esophageal band ligation in TA cases with TEF is a method that keeps the pressure in the esophagus at an appropriate level and provides air passage to the trachea via fistula.

Background: The use of laparoscopy in the repair of duodenal atresia has been increasing. However, there is no consensus regarding which surgical approach has better outcomes. We aimed to compare the different surgical approaches and types of anastomoses for duodenal atresia repair. Methods: Patients who underwent duodenal atresia repair at a single pediatric center were identified between January 2006 and June 2022. Those with concomitant gastrointestinal anomalies or who required other simultaneous operations were excluded. The primary outcome was rate of complications, defined as rate of leak, stricture, and re-operation by surgical approach and technique of anastomosis. Results: A total of 78 patients were included. The majority were female (51.3%, n = 40), with a median age of 4 days (IQR 3.0,8.0) and a median weight of 2.7 kg (IQR 2.2,3.3) at repair. The re-operation rate was 7.7% (n = 6), of which two were anastomotic leaks, and four were anastomotic strictures. The leak rate was 5.6% (n = 1/18) for the open handsewn and 4.8% (n = 1/21) for the laparoscopic handsewn technique. The stricture rate was 12.5% (n = 1/8) for the laparoscopic-assisted handsewn, 9.1% (n = 2/22) for the laparoscopic U-clip, 4.8% (n = 1/21) for the laparoscopic handsewn, and none with laparoscopic stapled and laparoscopic converted to open handsewn techniques. No differences were found in complication rate when controlling for surgical approach. Conclusion: The method of surgical approach did not affect the outcomes or complications in the repair of duodenal atresia. [ABSTRACT FROM AUTHOR]

The combination of duodenal atresia (DA) and esophageal atresia (EA) is very rare. With improvements in prenatal sonography and the use of fetal magnetic resonance imaging (MRI), these malformations can be diagnosed in a more accurate and timely manner; polyhydramnios remains the most common sign despite having a low specificity. The high rate of associated anomalies (in 85% of cases) can also impact neonatal management and increase the morbidity rate; thus, it is of paramount importance to look for every possible associated malformation, such as VACTERL and chromosomic anomalies. The surgical management of this combination of atresias is not well defined and changes according to the patient's clinical status, the type of EA, and the other associated malformations. Management ranges from a primary approach for one of the atresias with delayed correction of the other (56.8%) to a simultaneous repair of both atresias (33.8%) with or without gastrostomy, or total abstention (9.4%). We suggest that a simultaneous approach can be safely performed on patients in good physical condition, with a birth weight over 1500 g, and with no major respiratory distress; this method begins by closing the tracheoesophageal fistula to protect the lung and then repairing the DA. The mortality rate has decreased over the years, dropping from 71% before 1980 to 24% after 2001. In this review, we present the available evidence on these conditions, focusing mostly on the epidemiology, prenatal diagnosis, neonatal management strategies, and outcome, with the aim of determining how the different clinical features and surgical approaches may impact on morbidity and mortality. [ABSTRACT FROM AUTHOR]

Tracheal atresia/agenesis (TA) is associated with other congenital anomalies (TACRD and/or VACTERL). In this study, a female newborn with TA who had all the elements associated with TACRD and VACTERL was reported. The patient with TA, tracheo-esophageal fistula (TEF) connecting to the esophagus at the level of the carina, persistent left superior vena cava, duodenal atresia, vertebral and limb defects, bilateral hydronephrosis, and persistent cloaca was operated on postnatal 5th day. Band ligation to the abdominal esophagus, gastrostomy, duodenal atresia repair and diverting colostomy due to persistent cloaca were performed. The lungs were bilaterally expanded in the first postnatal, preoperative and postoperative chest radiographs. Oxygen saturation remained above 90% until the patient died suddenly on the postnatal 8th day. TA is a very rare congenital anomaly that causes postnatal respiratory distress. This case is unique among the literature on TACRD and VACTERL associations for many reasons. Esophageal band ligation in TA cases with TEF is a method that keeps the pressure in the esophagus at an appropriate level and provides air passage to the trachea via fistula. [ABSTRACT FROM AUTHOR]

A newborn presented with a rare combination of esophageal atresia with tracheoesophageal fistula (EA/TEF) and duodenal atresia (DA), which was successfully managed using minimally invasive surgical techniques. The patient was a 1-day-old male for whom passing a feeding tube was infeasible and who had a double bubble sign on radiography. The neonate underwent a thoracoscopic ligation of the tracheoesophageal fistula (TEF) and a laparoscopic duodeno-duodenostomy on the same day, resulting in stabilized vital signs. Ten days after the initial operation, a thoracoscopic esophago-esophagostomy was successfully performed. The patient demonstrated full feeding capability and normal weight gain after the surgeries. The co-occurrence of EA/TEF and DA is a rare and complex anomaly. This case indicates that minimally invasive techniques can effectively manage this condition.

Background: Simultaneous occurrence of duodenal atresia, annular pancreas, and situs inversus totalis is an exceedingly rare clinical condition. This may pose management-related challenges to the treating surgeon. Case Presentation: We report a case with a preoperative diagnosis of duodenal atresia and situs inversus. Intraoperatively, an annular pancreas encircling the duodenum was found. Instead of duodenoduodenostomy, a duodenojejunal anastomosis was performed. The postoperative course remained uneventful. The patient is doing fine on a 6-month follow-up. Conclusion: The simultaneous presence of multiple anomalies in this patient prompted us to utilize an alternative approach to relieve the duodenal obstruction. This report highlights the importance of tailoring surgical decisions based on the individualized needs of the patient.

Abstract Background Findings from manometry studies and contrast imaging reveal functioning gastric physiology in newborns with duodenal atresia and stenosis. Stomach reservoir function should therefore be valuable in aiding the postoperative phase of gastric feeding. The aim of this study was therefore to compare the feasibility of initiating oral or large volume(s) gavage feeds vs small volume bolus feeds following operation for congenital duodenal anomalies. Methods Single-center electronic medical records of all babies with duodenal atresia and stenosis admitted to a university surgical center during January 1997–September 2021 were analyzed. A fast-fed group (FF) included newborns fed with oral or gavage feeds advanced at a rate of at least 2.5 ml/kg and then progressed more than once a day vs slow-fed group (SF) fed with gavage feeds at incremental rate less than 2.5 ml/kg/day for each time period of oral tolerance or by drip feeds. Total feed volume was limited to 120–150 ml/kg/day in the respective study cohort populations. Results Fifty-one eligible patients were recruited in the study - twenty-six in FF group and twenty-five in SF group. Statistically significant differences were observed in the (i) date of first oral feeds (POD 7.7 ± 3.2 vs 16.1 ± 7.7: p

Abstract Background Amniotic fluid (AF) was thought of just as a mechanical cushioning to the foetus. Nowadays, its role during pregnancy is getting more attention, suggesting hitherto unknown aspects. The aim of the study is to speculate on AF nutritional functions and its clinical repercussions based on what digestive tract (DT) atresias seem to suggest. Methods A retrospective analysis of the patients admitted to our department for DT atresias between 2000 and 2020 was conducted. Patients’ birth weight (BW), gestational age (GA) at birth and diagnosis were recorded. The following were excluded from the study: oesophageal atresias (OA) with tracheoesophageal fistula (TOF), colonic and anal atresias and patients with associated major comorbidities. A control group was made of patients admitted to our ward in the same period for congenital pulmonary airway malformations (CPAM). To standardize the BW, it was coupled with birth GA calculating the newborn percentiles. The mean newborn percentiles of OAs, duodenal atresias (DAs), jejunal atresias (JAs), and ileal atresias (IAs) were independently compared with the control group using Student’s t-test. Lastly, the significance of the frequencies’ distribution of newborns born small for gestational age (SGA) between the DT atresias and the control group was evaluated with the χ 2 test, and the OR were calculated. A p-value < 0.05 was considered statistically significant. Results A total of 231 patients were eligible for the study: 36 OAs without TOF, mean BW 2488.8 ± 491 g (range 1630–3750 g), mean GA 36.8 ± 2.1 weeks (31–40 weeks), mean newborn percentile 18 ± 22 (1–75); 20 DAs, mean BW 2586.8 ± 577.9 g (1250–3462 g), mean GA 36.2 ± 2.5 weeks (31–40 weeks), mean newborn percentile 31 ± 23 (3–79); 17 JAs, mean BW 2483.5 ± 621.7 g (900–3205 g), mean GA 34.8 ± 2.1 weeks (30–38 weeks), mean newborn percentile 44 ± 28 (4–96); 17 IAs, mean BW 2646.1 ± 769.8 g (1162.0–3888 g), mean GA 35.9 ± 3.2 weeks (30–41 weeks), mean newborn percentile 44 ± 26 (1–82); and 141 CPAMs with mean BW 3287.4 ± 492.0 g (980–4580 g), mean GA 38.7 ± 1.8 weeks (26–41 weeks), mean newborn percentile 43 ± 26 (1–99). The number of SGA neonates was 18 between OA patients (50%), 4 between DAs (20%), 1 between JAs (6%), 2 between IAs (12%) and 11 between CPAMs (8%). The mean percentile of the OAs and DAs was lower than the control group with a p of < .01 and .03 while no statistical significance was found in the comparison between DAs, JAs, IAs and CPAMs with a p of .06, .86 and .59. The incidence of SGA in the control group resulted lower than the one in the DT atresias where it becomes higher the more proximal the atresia is (p < .05). The OR of SGA newborn in the OA group was 11.8, in DA 3.0, in JA 0.7 and in IA 1.6. Conclusion AF showed to have a great impact on foetal growth, and its preferred site of absorption seemed to be the stomach and duodenum. Its nutritional role could have an important predictive value in diagnosing DT atresia both prenatally and postnatally.

Congenital duodenal atresia with situs inversus is occasionally accompanied by a preduodenal portal vein (PDPV), which is incidentally diagnosed during surgery. Duodenoduodenostomy is the most common and effective treatment. However, some patients require other anastomoses. Here, we present two cases of laparoscopic gastrojejunostomy for congenital duodenal atresia with situs inversus and PDPV and describe the reason for selecting gastrojejunostomy. The optimal surgical strategy is patient specific and should be determined based on the patient's general and physical condition.

Background: This study assessed the characteristics of neonates with duodenal atresia (DA) who developed transient postoperative cholestasis which resolved spontaneously, without identifiable congenital anomalies or surgical complications, and identified potential risk factors for cholestasis. Methods: Neonates with DA who underwent surgery at our institution between January 2009 and July 2022 were retrospectively reviewed. Demographic factors, intraoperative findings, placement of a trans-anastomotic tube (TAT), and postoperative outcomes were compared between patients who developed cholestasis (conjugated hyperbilirubinemia >2.0 mg/dL) after surgery (Group A) and those who did not (Group B). This report is a retrospective cohort study and complies with the STROBE statement. Results: Among 19 neonates with DA, 6 (31.6%) developed cholestasis after surgery, with the highest direct bilirubin value being 4.3 (2.4-6.5) mg/dL (median, [range]) on postoperative day 14.5 (2-23) that persisted for 67 (47-116) days until spontaneous resolution. Neonates in Group A had a significantly younger gestational age (36.6 vs. 38.0 weeks) (median) (p=0.038), a higher rate of Down syndrome (66.7 vs. 15.4%) (p=0.046), a higher rate of TAT placement (66.7 vs. 15.4%) (p=0.046), and longer administration of total parenteral nutrition (15.5 vs. 7.0 days) (p=0.027) than those in Group B. Conclusion: Transient cholestasis after surgery for DA seemed to be associated with prematurity, Down syndrome, parenteral nutrition, and TAT placement. [ABSTRACT FROM AUTHOR]

Background: Duodenal and pyloric web (DW/PW) can present at any age, symptoms depend upon the location of the web along with the presence and size of the opening in the web. The surgical management is not straightforward always. Here, in this study, we aim to assess clinical characteristics, management, and outcome of children with DW/PW. Materials and Methodology: This was a retrospective study from 2005 to 2019, and data were collected from record registers. All children of DW/PW presented between this duration were included in this study. Results: A total of 45 patients (age range = 1 day to 11 years) included in the study, 40 had DW while 5 had PW. Seven patients were diagnosed antenatally and 20 patients had associated congenital anomalies. Most patients presented with vomiting either bilious or nonbilious. Plain X-ray was sufficient for the diagnosis in 60% of patients, the rest diagnosed on contrast study. The web excision and pyloroplasty were done for PW. The web excision and Heineke-Mikulicz type enteroplasty was the preferred surgery for DW but some patients were required Kimura's duodeno-duodenostomy. For postoperative nutrition, enteral feeding was established through the placement of a feeding tube beyond anastomosis. Ten patients died due to septicemia and associated anomalies. Four patients had a minor leak which was managed by conservative means. Four patients required redo surgery, adhesive obstruction was the most common indication. During follow-up, all 35 patients were doing well with no major complaints. Conclusion: DW/PW has different presentations as compared to other intestinal atresia and can present at any age. A contrast study confirms the diagnosis when plain X-ray is inconclusive. Associated anomalies and septicemia are the poor prognostic indicators. Postoperative enteral feeding helps in maintaining adequate nutrition and improves the outcome even in children with a minor anastomotic leak.

The prognosis for duodenal atresia (DA) is generally good if radical surgery is performed. However, some cases of DA may be complicated by morphological abnormalities in the pancreaticobiliary system, which may cause problems in the distant period. Here, we report a case of gallbladder cancer (GC) that developed more than 20 years after the initial DA operation. A female infant was admitted to our institution with an anorectal malformation after birth. She had Down syndrome, a ventricular septal defect, and DA. She underwent duodenoduodenostomy for DA, during which a Y-shaped biliary duct was found. The patient grew up well postoperatively, and she was followed up at our institution until the age of 12 years. At the age of 24, she was reintroduced to our institution for multiple hepatic tumors and was diagnosed with GC and multiple liver metastases. Simultaneously, a pancreaticobiliary maljunction (PBM) without a choledochal cyst was also identified. She received palliative care and died two months later. Although reports of combined DA and PBM are rare, the combination should not be overlooked, given the carcinogenic risk of PBM. DA with pancreaticobiliary anomalies requires attention to the possibility of PBM during the postoperative follow-up.

Intestinal obstruction is one of the most common surgical emergencies in the neonatal period. Early diagnosis is vital for proper management and good outcome. Intestinal obstruction can be divided into high, for example, duodenal atresia and jejunal atresia, or low, for example, ileal atresia, colonic atresia, and Meckel's diverticulum. The most common cause of intestinal obstruction in neonates is midgut atresia. Surgical correction is needed and is a challenge in the developing countries where there is lack of pediatric surgeons, anesthesiologists, and intensive care. More research and data is also needed across countries to show the uneven distribution of the available resources. [ABSTRACT FROM AUTHOR]

Background: Duodenal obstruction (DO) is a congenital anomaly that is highly associated with other anomalies, such as cardiac anomalies and trisomy 21. However, an overview of additional anomalies and patient-specific risk factors for cardiac anomalies is lacking. Potential association with the vertebral, anorectal, cardiac, trachea-esophageal, renal and limb anomalies (VACTERL) spectrum remains unknown. Therefore, we aim to examine the incidence of associated anomalies, a VACTERL-spectrum association and patient-specific risk factors for cardiac anomalies in patients with DO. Methods: A retrospective cohort study was performed between 1996 and 2021. Outcomes were the presence of any additional anomalies. Risk factors for cardiac anomalies were analyzed using multivariate logistic regression. Results: Of 112 neonates with DO, 47% (N = 53/112) had one associated anomaly and 38% (N = 20/53) had multiple anomalies. Cardiac anomalies (N = 35/112) and trisomy 21 (N = 35/112) were present in 31%. In four patients, VACTERL-spectrum was discovered, all with cardiac anomalies. Trisomy 21 was found to be a risk factor for cardiac anomalies (OR:6.5; CI-95%2.6–16.1). Conclusion: Associated anomalies were present in half of patients with DO, of which cardiac anomalies and trisomy 21 occurred most often, and the VACTERL-spectrum was present in four patients. Trisomy 21 was a significant risk factor for cardiac anomalies. Therefore, we recommend a preoperative echocardiogram in patients with DO. In case a cardiac anomaly is found without trisomy 21, VACTERL-screening should be performed. [ABSTRACT FROM AUTHOR]

Laparoscopic repair of duodenal atresia in neonates has gained popularity among some pediatric surgeons. Single-center studies suggest comparable short-term outcomes to open surgery. The purpose of this study was to utilize a large, multi-institutional pediatric dataset to examine 30-day post-operative outcomes by operative approach for newborns who underwent duodenal atresia repairs. We identified neonates aged ≤1 wk in the 2016-2018 National Surgical Quality Improvement Program-Pediatric -database that underwent a laparoscopic or open repair for duodenal atresia. Preoperative characteristics were compared between operative approaches. Postoperative complications, operative time, postoperative length of stay (LOS), and supplemental nutrition at discharge were assessed using multivariate regressions. There were 267 neonates who met inclusion criteria. There were 233 (87%) infants who underwent open repairs and 34 (13%) who underwent laparoscopic repairs. Ten (29%) children who had laparoscopy were converted to open. After adjusting for confounding, laparoscopy was associated with an increase in operative time by 65 min (95% confidence interval 45-87 min, P < 0.001) but a five-day shorter LOS (95% confidence interval −9 to −2, P = 0.006) when compared to laparotomy. There were no significant differences in postoperative complications or supplemental nutrition at discharge. Our findings suggest that laparoscopic repairs of duodenal atresia are associated with shorter postoperative LOS but longer operative times when compared to open repairs. Although the conversion rate to laparotomy remained relatively high, the laparoscopic approach was associated with comparable 30-day postoperative outcomes. • Laparoscopic duodenal atresia repair was uncommon with high conversion rates. • Laparoscopy had comparable postoperative complications to open procedures. • Laparoscopy was associated with longer operative times but reduced lengths of stay. • Laparoscopy had comparable rates of nutrition supplementation at discharge. • Overall results support continued use of laparoscopy for duodenal atresia repairs. [ABSTRACT FROM AUTHOR]

Purpose: To assess the safety and efficacy of laparoscopic versus open repair of congenital duodenal obstruction (CDO), we conducted a systematic review and meta-analysis (CDO).Methods: A literature search was conducted to identify studies that compared laparoscopic surgery (LS) and open surgery (OS) for neonates with CDO. Meta-analysis was used to pool and compare variables such as operative time, time to feeding, length of hospital stay, anastomotic leak or stricture, postoperative ileus, wound infection, and overall postoperative complications.Results: Among the 1348 neonatal participants with CDO in the ten studies, 304 received LS and 1044 received OS. When compared to the OS approach, the LS approach resulted in shorter hospital stays, faster time to initial and full feeding, longer operative time, and less wound infection. However, no significant difference in secondary outcomes such as anastomotic leak or stricture, postoperative ileus, and overall postoperative complications was found between LS and OS.Conclusions: LS is a safe, feasible and effective surgical procedure for neonatal CDO when compared to OS. Compared with OS, LS has a faster time to feeding, a shorter hospital stay, and less wound infection. Furthermore, in terms of anastomotic leak or stricture, postoperative ileus, and overall postoperative complications, LS is equivalent to OS. We conclude that LS should be considered an acceptable option for CDO. [ABSTRACT FROM AUTHOR]

Bilious (dark green) vomiting in the newborn is a surgical emergency as the underlying diagnosis may be volvulus of the entire midgut secondary to malrotation. This diagnosis is time-critical because if untreated, volvulus will lead to midgut necrosis resulting in short gut syndrome or death. While a range of other diagnoses are possible, transfer to a paediatric surgical centre should be undertaken promptly so that malrotation volvulus can be excluded or treated. This review focuses on the causes, investigation, and management of proximal bowel obstruction in the neonate that present primarily with bilious vomiting. [ABSTRACT FROM AUTHOR]

Background: There are few evidence-based guidelines for perioperative antibiotic management in neonates who undergo enteric operations. We sought to assess antibiotic administration practices in a large population of patients who underwent operations involving enteric anastomoses and evaluate the incidence of postoperative infection and other outcomes based on antibiotic approach., Methods: The Pediatric Health Information Systems database was queried for patients who underwent repair of esophageal, duodenal or jejuno-ileal atresia in 2021. The type and number of consecutive days of perioperative antibiotics was determined and ICD-10 codes corresponding to infection were noted. The incidences of post operative infections (bacterial and fungal), antibiotic-resistant infections and anti-fungal medication administration were determined., Results: 516 infants were identified. A wide variety of antibiotics were administered and 39 % of patients received more than one day of treatment. There were no differences in the incidence of postoperative infection between those who received more or less than one day of perioperative antibiotics for any of the operations assessed. The incidence of bacterial infection in patients treated with cefazolin or cefoxitin monotherapy was no different than that for all other regimens. There were no significant differences in the incidence of post-operative fungal infection based on antibiotic type or duration., Conclusion: There was substantial variation in the duration and type of antibiotics administered after neonatal enteric operations. We identified a low incidence of infection with only one day of perioperative antibiotics and there was no evidence that longer treatment reduced infection risk. Cefazolin monotherapy was likewise associated with a low risk for perioperative infections., Study Type: Non-interventional observational database study., Competing Interests: Conflicts of interest The authors have no conflicts of interest to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Abstract Background Homozygous mutations in the transcription factor RFX6 are the cause of the Mitchell–Riley syndrome (MRS) associating neonatal diabetes, congenital digestive system, such as biliary atresia, pancreatic hypoplasia, duodenal and/or jejunal atresia, intestinal malrotation, gallbladder aplasia, cholestasis. A constitutive inactivation of RFX6 leads also to gastric heterotopia. Application of RNA-seq in human diseases may help to better understand pathogenic mechanism of diseases and to predict the risk of developing chronic disorders and personalizing their prevention and treatment. We evaluated oncogenic patterns and cancer predisposition using the transcriptomic profile in a case of MRS with neonatal diabetes, duodenal atresia, and extensive intestinal tract gastric heterotopia. Results We signalled the interactors of RFX6 with other up and downregulated genes, that may be interested in severity of diabetic condition, in multi-organs impairment and cancer predisposition. Furthermore, several dysregulated genes are involved in biological processes that can lead to promote cancer including “Evading apoptosis” (BAD, BBC3, EGF, FGFR2, FLT3LG, HMOX1, HRAS, IFNAR2, IGF1R, IL12RB1, IL13RA1, IL15, IL2RB, IL2RG, IL6R, KEAP1, MGST1, PDGFA, PDGFRB, PIK3R3, RALB, RALGDS, RASSF1, SOS1, TGFA, TXNRD3), “Proliferation” (APC, BRAF, CCND2, CCND3, CCNE2, FGFR2, FLT3LG, FZD1, FZD6, HMOX1, HRAS, IGF1R, KEAP1, LRP6, MAPK3, MGST1, PDGFA, PDGFB, PDGFRB, RB1, SOS1, TGFA, TXNRD3, WNT10B), “Sustained angiogenesis” (BRAF, FGFR2, FLT3LG, HRAS, IGF1R, JAG1, MAPK3, NOTCH2, PDGFA, PDGFB, PDGFRB, SOS1, TGFA, TGFB1), “Genomic instability” (BAD, BBC3) and “Insensitivity to anti-growth signals” (SMAD2, TGFB1). We also inspected the signalings and their related genes in cancer, such as “PI3K signaling”, “ERK signaling”, “JAK-STAT signaling”, “Calcium signaling”, “Other RAS signaling”, “WNT signaling”. Conclusions In our MRS patient, we signaled the interactors of RFX6 with other up- and downregulated genes that may be related to severe diabetic condition, multi-organ impairment, and cancer predisposition. Notably, many dysregulated genes may lead to triggering carcinogenesis. The possibility of the patient developing cancer degeneration in heterotopic gastric mucosa and/or additional long-term tumoral sequelae is not excluded. Personalized prevention and treatment strategies should be proposed.

Despite data to suggest benefit of trans- anastomotic tube (TAT) feeding in infants following repair of congenital duodenal obstruction (CDO), TAT usage is limited. We aimed to report a large series of infants with CDO treated with or without TAT in order to improve the evidence underlying this simple intervention. Single centre retrospective review of all infants CDO over a 20-year period (January 1999 – November 2020, inclusive). Important outcomes were compared between infants treated with or without TAT. Data are median [IQR]. Ninety-six infants were included. A TAT was placed in 54 infants (56%). Median time to full enteral feed was significantly shorter in the TAT group (6 [5–8] days vs 10 [7.5–12], p <0.001). Time to first feed was shorter in the TAT group (2 [2–2.8] days vs 3 [2–5], p <0.001). Significantly fewer infants with a TAT placed received a central venous catheter (CVC, 15% vs 76%, p <0.001). Infants without a TAT received parenteral nutrition (PN) for longer (0 [0–0] vs 7 [0–11] days, p <0.001). There was no change in length of stay between TAT and no TAT group (16 [13–21.8] vs 15 [12–21.8] days, p = 0.722). Eight infants (15%) in the TAT group required a CVC and PN. One infant in the TAT group developed a perforation that required surgical management and nine infants in the non-TAT group had complications related to the CVC (21%), including one infant that required general anaesthetic for tunnelled central line placement (2.3%). In infants with CDO, TAT use was associated with earlier establishment of full enteral feeds, reduced need for CVC and PN and reduced complications. Further research should focus on the barriers to wider use of TAT by surgeons and neonatologists in infants with CDO. Level III evidence [ABSTRACT FROM AUTHOR]

Background and Objective: Intestinal atresia is a life-threatening problem requiring early active intervention. The aim of the study was to compare management outcomes between Duodenal-Atresia (DA) and Jejunoileal-Atresias (JIA). Methods: Retrospective descriptive analysis of patients operated between March 2015 to February 2020 in a tertiary-care Pediatric Surgery unit of Armed Forces Medical College Pune, India. The data was obtained from the hospital records, operation theatre notes, discharge summary and follow-up notes. Demographic and clinical information and data were analyzed with SPSS version 26 with appropriate statistical tools. Findings: Forty-eight neonates were included (DA=23; JIA=25). There were 18 (37.5%) males and 30 (62.5%) females. The mean age, mean birth weight, and time to feed were statistically significant and better in JIA compared to DA. A total of 45% of newborns had associated anomalies. The primary surgery performed in patients with DA was Kimura's duodenoduodenostomy, while Resection-anastomosis with or without tapering enteroplasty was performed in JIA patients. In the subgroup analysis of JIA, the length of hospital stay in the tapering enteroplasty was statistically significant compared to resection anastomosis.(p=0.048). The average weight gain and survival in Jejunoileal-atresia at six months is statistically significant compared to Duodenal-atresia. Conclusion: The management of intestinal atresias is challenging and requires a dedicated team in a specialized neonatal intensive-care unit. Intensive investigation of other congenital anomalies associated with the condition is equally critical and in the subgroup analysis of JejunoIlial -atresia, the patients undergoing tapering enterostomy showed better survival compared to resection and end to end anastomosis only. [ABSTRACT FROM AUTHOR]

Background: Amniotic fluid (AF) was thought of just as a mechanical cushioning to the foetus. Nowadays, its role during pregnancy is getting more attention, suggesting hitherto unknown aspects. The aim of the study is to speculate on AF nutritional functions and its clinical repercussions based on what digestive tract (DT) atresias seem to suggest. Methods: A retrospective analysis of the patients admitted to our department for DT atresias between 2000 and 2020 was conducted. Patients' birth weight (BW), gestational age (GA) at birth and diagnosis were recorded. The following were excluded from the study: oesophageal atresias (OA) with tracheoesophageal fistula (TOF), colonic and anal atresias and patients with associated major comorbidities. A control group was made of patients admitted to our ward in the same period for congenital pulmonary airway malformations (CPAM). To standardize the BW, it was coupled with birth GA calculating the newborn percentiles. The mean newborn percentiles of OAs, duodenal atresias (DAs), jejunal atresias (JAs), and ileal atresias (IAs) were independently compared with the control group using Student's t-test. Lastly, the significance of the frequencies' distribution of newborns born small for gestational age (SGA) between the DT atresias and the control group was evaluated with the χ2 test, and the OR were calculated. A p-value < 0.05 was considered statistically significant. Results: A total of 231 patients were eligible for the study: 36 OAs without TOF, mean BW 2488.8 ± 491 g (range 1630–3750 g), mean GA 36.8 ± 2.1 weeks (31–40 weeks), mean newborn percentile 18 ± 22 (1–75); 20 DAs, mean BW 2586.8 ± 577.9 g (1250–3462 g), mean GA 36.2 ± 2.5 weeks (31–40 weeks), mean newborn percentile 31 ± 23 (3–79); 17 JAs, mean BW 2483.5 ± 621.7 g (900–3205 g), mean GA 34.8 ± 2.1 weeks (30–38 weeks), mean newborn percentile 44 ± 28 (4–96); 17 IAs, mean BW 2646.1 ± 769.8 g (1162.0–3888 g), mean GA 35.9 ± 3.2 weeks (30–41 weeks), mean newborn percentile 44 ± 26 (1–82); and 141 CPAMs with mean BW 3287.4 ± 492.0 g (980–4580 g), mean GA 38.7 ± 1.8 weeks (26–41 weeks), mean newborn percentile 43 ± 26 (1–99). The number of SGA neonates was 18 between OA patients (50%), 4 between DAs (20%), 1 between JAs (6%), 2 between IAs (12%) and 11 between CPAMs (8%). The mean percentile of the OAs and DAs was lower than the control group with a p of <.01 and.03 while no statistical significance was found in the comparison between DAs, JAs, IAs and CPAMs with a p of.06,.86 and.59. The incidence of SGA in the control group resulted lower than the one in the DT atresias where it becomes higher the more proximal the atresia is (p <.05). The OR of SGA newborn in the OA group was 11.8, in DA 3.0, in JA 0.7 and in IA 1.6. Conclusion: AF showed to have a great impact on foetal growth, and its preferred site of absorption seemed to be the stomach and duodenum. Its nutritional role could have an important predictive value in diagnosing DT atresia both prenatally and postnatally. [ABSTRACT FROM AUTHOR]

Background: Findings from manometry studies and contrast imaging reveal functioning gastric physiology in newborns with duodenal atresia and stenosis. Stomach reservoir function should therefore be valuable in aiding the postoperative phase of gastric feeding. The aim of this study was therefore to compare the feasibility of initiating oral or large volume(s) gavage feeds vs small volume bolus feeds following operation for congenital duodenal anomalies.Methods: Single-center electronic medical records of all babies with duodenal atresia and stenosis admitted to a university surgical center during January 1997-September 2021 were analyzed. A fast-fed group (FF) included newborns fed with oral or gavage feeds advanced at a rate of at least 2.5 ml/kg and then progressed more than once a day vs slow-fed group (SF) fed with gavage feeds at incremental rate less than 2.5 ml/kg/day for each time period of oral tolerance or by drip feeds. Total feed volume was limited to 120-150 ml/kg/day in the respective study cohort populations.Results: Fifty-one eligible patients were recruited in the study - twenty-six in FF group and twenty-five in SF group. Statistically significant differences were observed in the (i) date of first oral feeds (POD 7.7 ± 3.2 vs 16.1 ± 7.7: p < 0.001), and (ii) first full feeds (POD 12.5 ± 5.3 vs 18.8 ± 9.7: p < 0.01) in FF vs SF study groups.Conclusion: Initial feeding schedules with oral or incremental gavage-fed rates of at least 2.5 ml/kg in stepwise increments and multi-steps per day is wholly feasible in the postoperative feeding regimens of neonates with congenital duodenal disorders. Significant health benefits are thus achievable in these infants allowing an earlier time to acquiring full enteral feeding and their hospital discharge. [ABSTRACT FROM AUTHOR]

Background: Congenital duodenal atresia (CDA) is a common and surgically treated digestive tract anomaly that develops in the early stage of pregnancy. It often coexists with trisomy 21 and other inborn defects. Surgery is the only way of treatment. This study aimed to investigate the relationship of CDA with early diagnosis, course of pregnancy, coexisting congenital defects, and further development of children. Methods: The data were collected using the medical history and a self-designed survey which consisted of 22 questions about the perinatal interview, coexisting inborn defects, after birth symptoms, time and methods of diagnosis, as well as the treatment outcome. Results: The surveys were sent to 31 patients who were diagnosed and treated because of CDA in our clinic between 2004 and 2019. According to the collected data, 73.7% of the patients were diagnosed prenatally with the mean time of diagnosis at 28th weeks of gestation. Moreover, 37% of the patients were preborn, and almost half of the patients had low and very low body weight. More than 2/3 of children presented coexisting inborn abnormalities. Among after birth symptoms, the most common ones were abdominal distention and lack of meconium. The majority of children were operated within 24 hours after birth. Conclusions: Prenatal diagnosis of CDA results in an earlier time of primary operation. Newborns diagnosed antenatally present fewer symptoms after the operation, fewer reoperations, and a shorter time of oral feeding toleration. The coexistence of other congenital defects increases patients’ mortality. The important factors that deteriorate the postoperative course are prematurity and low body weight. In order to avoid long-term complications, it is of utmost importance to provide follow-up.

A full-term female infant is born via vaginal delivery to a 26-year-old primigravida with unexplained polyhydramnios and antenatal diagnosis of duodenal atresia. After birth, bilious fluid was aspirated through the orogastric tube and the initial abdominal radiograph was suggestive of double bubble sign with the absence of air in the distal bowel. Baby passed meconium at 4 h of life and repeat abdominal radiograph showed double bubble sign with the presence of air in distal bowel. Upper gastrointestinal contrast study done revealed distension of the stomach and duodenal bulb with an abrupt narrowing. It also revealed flow of contrast into the jejunal loops on the right side of the spine which was suggestive of intestinal malrotation as the leading diagnosis. However, intra-operative findings confirmed a Type III Duodenal atresia with bile proximal and distal to atretic. Occasionally, distal bowel gases can be seen in radiographs of infants with duodenal atresia due to abnormal development of the biliary system that can allow passage of air from the proximal duodenal segment to the distal. Here, we report a case and discuss the embryology of this rare entity.

Orofacial clefts are common birth defects and Van der Woude syndrome (VWS) is the most common form of orofacial cleft syndrome, accounting for approximately 2% of patients with a cleft.[1],[2] The cardinal features of VWS are lower lip pits associated with cleft lip and/or palate.[2],[3],[4] Lip pits are reported to occur in over 80% of individuals with VWS.[2] Most reported cases of VWS have been linked to chromosome 1q32-q41.[5],[6],[7] The interferon regulatory factor 6 (IRF6) gene, which is located at 1q32-p41 region, has been implicated in several studies.[2],[8] There are over 300 IRF6 variants that have been identified in patients with VWS, with approximately 50% of these being missense variants.[9] This paper describes a 7.5-year-old male patient that is heterozygous for a missense variant in C.101A >C p.(Lys34Thr) which is likely to be pathogenic.[9] This patient has the cardinal features for VWS but also has duodenal atresia. Neither the truncating variant identified in this patient nor other variants associated with VWS have been previously linked to duodenal atresia.

Purpose: This study aimed to evaluate the diagnostic performance of ultrasound to locate atresia/stenosis and other abdominal anomalies in pediatric patients with duodenal atresia/stenosis, including intestinal malrotation and annular pancreas. Methods: We classified 36 retrospective cases of duodenal atresia/stenosis based on intestinal malrotation status, and evaluated the diagnostic performance of ultrasound/upper gastrointestinal contrast studies to locate atresia/stenosis and intestinal malrotation, as well as ultrasound for detecting additional anomalies such as annular pancreas. The incidence of annular pancreas was compared between groups using Fisher's exact test. Results: Atresia/stenosis was correctly located by ultrasound in 33 (91.7%) cases and by upper gastrointestinal contrast study in 36 (100%) cases. Of the eight cases with intestinal malrotation, five and two were correctly diagnosed by ultrasound and upper gastrointestinal contrast study, respectively. Ultrasound correctly diagnosed annular pancreas in 6/14 cases. The incidence of annular pancreas was significantly different between the groups (present/absent in groups with vs. without intestinal malrotation: 6/2 vs. 8/20, P = 0.036). Conclusions: Ultrasound has a relatively high capability in locating atresia/stenosis. However, some cases are misdiagnosed. In clinical practice, upper gastrointestinal contrast studies should be used complementarily during diagnosis. Additional anomalies may not be detected by preoperative examinations; therefore, surgeons should carefully evaluate for additional anomalies during surgery, especially coexisting intestinal malrotation and annular pancreas. [ABSTRACT FROM AUTHOR]