Cardiac anomalies in children with congenital duodenal obstruction: a systematic review with meta-analysis.

BACKGROUND: Cardiac anomalies occur frequently in patients with congenital duodenal obstruction (DO). However, the exact occurrence and the type of associated anomalies remain unknown. Therefore, the aim of this systematic review is to aggregate the available literatures on cardiac anomalies in patients with DO. METHODS: In July 2022, a search was performed in PubMed and Embase.com. Studies describing cardiac anomalies in patients with congenital DO were considered eligible. Primary outcome was the pooled percentage of cardiac anomalies in patients with DO. Secondary outcomes were the pooled percentages of the types of cardiac anomalies, type of DO, and trisomy 21. A meta-analysis was performed to pool the reported data. RESULTS: In total, 99 publications met our eligibility data, representing 6725 patients. The pooled percentage of cardiac anomalies was 29% (95% CI 0.26-0.32). The most common cardiac anomalies were persistent foramen ovale 35% (95% CI 0.20-0.54), ventricular septal defect 33% (95% CI 0.24-0.43), and atrial septal defect 33% (95% CI 0.26-0.41). The most prevalent type of obstruction was type 3 (complete atresias), with a pooled percentage of 54% (95% CI 0.48-0.60). The pooled percentage of Trisomy 21 in patients with DO was 28% (95% CI 0.26-0.31). CONCLUSION: This review shows cardiac anomalies are found in one-third of the patients with DO regardless of the presence of trisomy 21. Therefore, we recommend that patients with DO should receive preoperative cardiac screening. LEVEL OF EVIDENCE: II.

Novel qualitative and quantitative ultrasound markers to facilitate prenatal diagnosis of congenital duodenal obstruction.

OBJECTIVE: Accurate prenatal diagnosis of congenital duodenal obstruction (CDO) is challenging. We aimed to determine new ultrasound metrics for accurate prenatal diagnosis of fetal CDO. METHODS: Data pertaining to 46 fetuses with suspected small intestinal obstruction (26 CDO; 16 high jejunal obstructions) were retrospectively analyzed. Prenatal ultrasonographic features including dilated intestinal length, stomach length, maximum intestinal dilatation, ratio of dilated intestinal length at late gestation and dilated stomach length (I/S ratio), and location of distal end of dilated bowel segment relative to spine were compared between CDO and high jejunal obstruction groups. The diagnostic performance of ultrasound indices was evaluated using receiver operating characteristics curve analysis. RESULTS: In 25 out of 26 CDO cases, the distal end of the dilated small intestine segment was located on the right side of spine, while that in the high jejunal obstruction group was located on the left side of spine. The dilated intestinal length and I/S ratio in CDO group were significantly smaller than those in high jejunal obstruction group (p < .05). Dilated intestinal length <51 mm or I/S ratio <1 showed high sensitivity (100, 100%) and specificity (96.1, 92.3%) for CDO (area under the curve: 0.995 and 0.988, respectively). There were no significant differences in the AUCs of dilated intestinal length and I/S ratio. Significant correlation of the site of obstruction in CDO with fetal dilated intestinal length and I/S ratio (r = 0.686; 0.660, p < .001, respectively) were noted. CONCLUSION: Location of the distal end of the dilated small intestine segment relative to the spine, dilated intestinal length, and I/S ratio may help differentiate fetal CDO from high jejunal obstruction. The latter two metrics were associated with the site of obstruction in CDO patients.

Delayed finding of congenital duodenal obstruction following congenital diaphragmatic hernia repair.

Congenital diaphragmatic hernia (CDH) is encountered in just under 1 in 6000 live births, while congenital duodenal obstruction is seen once every 8000 live births. These congenital anomalies have only been reported together as part of the VACTERL syndrome and therefore in isolation represent an incredibly rare occurrence. This is a case report of a girl born at 34 weeks gestation who had an antenatal diagnosis of left CDH. Five days following operative repair of this, there was extensive pneumoperitoneum and pneumothorax. Upper gastrointestinal contrast study showed a perforation of the duodenum and at laparotomy, a duodenal web was found in the fourth part of the duodenum with perforation immediately proximal. Following duodenoduodenostomy, the postoperative recovery was good. She achieved full enteral feeds and was discharged home. This case highlights the importance of considering rare associations if postoperative recovery is not as expected to prevent delay in undergoing definitive treatment.

Congenital Duodenal Obstruction: National Trends in Management and Outcomes during the Last Quarter of a Century in Norway.

INTRODUCTION: During the last quarter of a century, new surgical techniques in neonates have been introduced, and neonatal intensive care has developed. Few studies have explored the implementation of new techniques and if outcomes in neonates undergoing gastrointestinal surgery have improved in the last decades. Therefore, this study aimed to investigate possible changes in postoperative outcomes and surgical techniques in all neonates operated for congenital duodenal obstruction (CDO) 1995 to 2020 in Norway. MATERIAL AND METHODS: This is a national multicenter retrospective study of all neonates undergoing surgery for CDO in Norway from 1995 to 2020. Results from three periods (1995-2003, 2004-2012, and 2013-2020) were compared. The study was approved by the local data protection officers (2020/13386) and (2020/15125). RESULTS: We included 186 patients: 41 in period 1 (1995-2003), 83 in period 2 (2004-2012), and 62 in period 3 (2013-2020). Seventy (38%) neonates had Down syndrome and 104 (62%) had additional malformations/disorders. Birth weight, gender, frequency of Down syndrome, and other malformations/disorders did not differ between the three periods. We observed an increased rate of prenatal diagnosis throughout the study period (p < 0.001). The only change in surgical technique was the increased use of transanastomotic feeding tubes (p < 0.001). Length of stay, postoperative complication rate, days with parenteral nutrition, and 30-day mortality rate were stable over time. CONCLUSION: Perioperative treatment and postoperative outcomes in neonates with CDO have been surprisingly unchanged during the last quarter of a century. Only an increased rate of prenatal diagnosis and more frequent use of transanastomotic feeding tubes were observed.

Laparoscopic versus open repair of congenital duodenal obstruction: a systematic review and meta-analysis.

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.

Dual duodenojejunostomies in the repair of megaduodenum, duodenal stenosis and prior missed diagnosis of annular pancreas.

Duodenal stenosis and atresia are some of the most common forms of congenital bowel obstruction. The gold standard approach to treatment is duodenoduodenostomy, while rare, gastrojejunostomy and duodenojejunostomies may still be used. We report a case of a 7-year-old male presenting with annular pancreas with duodenal stenosis that was diagnosed at birth as primary duodenal atresia and repaired by gastrojejunostomy with a Braun enteroenterostomy. Through successful reoperation with dual duodenojejunostomy and subsequent management, we treated the sequelae of the initial repair, including megaduodenum. Regions of duodenal obstruction must have direct anastomotic repair to prevent subsequent issues related to dysmotility. Otherwise, further surgical intervention and long-term medical management, such as the novel strategy reported, may be necessary.

Clinical significance of prenatal double bubble sign on perinatal outcome and literature review.

OBJECTIVE: To determine the diagnostic accuracy of the double bubble sign in predicting duedonal obstruction, the prevalence of anomalies associated with congenital duedonal obstruction, and to evaluate the outcome of these pregnancies and review the literature. METHODS: We analyzed that the cases with double bubble sign were detected in prenatal ultrasonography between January 2014 and December 2019. Ultrasound reports, karyotyping results, surgical confirmed diagnoses and long-term results were extracted from the hospital database. RESULTS: Duodenal obstruction was detected in all operated cases with double bubble sign detected in prenatal ultrasonography. Karyotype anomaly was found in 63.9% and additional congenital anomaly was found in 69.4%. Adverse perinatal outcome was seen in 51.1% of cases. Adverse perinatal outcome was found significantly higher in abnormal karyotype and congenital anomaly groups than isolated group. 81.8% of the isolated cases lived after the operation. Only two isolated cases died. CONCLUSION: The prenatal double bubble sign is a highly reliable predictor for duodenal obstruction and structural anomalies and/or genetic abnormalities are present in the majority of cases. Duodenal obstruction is isolated, the outcomes appear favorable.

Esophageal Atresia and Associated Duodenal Atresia: A Cohort Study and Review of the Literature.

INTRODUCTION: Esophageal atresia (EA) is associated with duodenal atresia (DA) in 3 to 6% of cases. The management of this association is controversial and literature is scarce on the topic. MATERIALS AND METHODS: We aimed to (1) review the patients with EA + DA treated at our institution and (2) systematically review the English literature, including case series of three or more patients. RESULTS: Cohort study: Five of seventy-four patients with EA had an associated DA (6.8%). Four of five cases (80%) underwent primary repair of both atresia, one of them with gastrostomy placement (25%). One of five cases (20%) had a delayed diagnosis of DA. No mortality has occurred. Systematic Review: Six of six-hundred forty-five abstract screened were included (78 patients). Twenty-four of sixty-eight (35.3%) underwent primary correction of EA + DA, and 36/68 (52.9%) underwent staged correction. Nine of thirty-six (25%) had a missed diagnosis of DA. Thirty-six of sixty-eight underwent gastrostomy placement. Complications were observed in 14/36 patients (38.9 ± 8.2%). Overall mortality reported was 41.0 ± 30.1% (32/78 patients), in particular its incidence was 41.7 ± 27.0% after a primary treatment and 37.0 ± 44.1% following a staged approach. CONCLUSION: The management of associated EA and DA remains controversial. It seems that the staged or primary correction does not affect the mortality. Surgeons should not overlook DA when correcting an EA.

Delayed presentation of a congenital duodenal web managed successfully with incision of web.

One of the causes of congenital incomplete duodenal obstruction is the presence of congenital duodenal web with fenestration. This condition requires a high index of suspicion for an early and accurate diagnosis. We present an unusual presentation of duodenal web in a 6-year-old girl who presented with a 3-year history of cyclical and seasonal vomiting and abdominal pain. The diagnosis of congenital duodenal web with fenestration was made on contrast study and endoscopy and was treated surgically by incision of the web.

Management of duodenal atresia associated with situs inversus abdominus: A case report.

RATIONALE: Duodenal atresia in association with situs inversus abdominus is extremely rare. Care should be taken when selecting appropriate surgical methods, and caution should be exercised during the surgery to avoid misdiagnosis and mistreatment. With prompt recognition of the condition, the surgical procedure should be performed in a timely manner to achieve positive results. PATIENT CONCERNS: A newborn affected by situs inversus abdominus associated with duodenal atresia, midgut malrotation, and volvulus. DIAGNOSIS: Congenital duodenal atresia with situs inversus abdominis. INTERVENTIONS: Diamond-shaped duodenoduodenostomy with appendectomy was performed, with the release of Ladd band and correction of the malrotation. OUTCOMES: The baby boy is thriving well with no abdominal complaints at 4 years of surgical follow-up. LESSONS: Although several theories are put forward to clarify this matter, the proper cause of duodenal atresia is not well defined. Clinical symptoms and examinations can assist diagnosis, the definitive cause should be ascertained by surgical approach. And the operating surgeon must be aware of the "mirror anatomy" to prevent unnecessary injuries. Additionally, long-term prognosis for duodenal atresia are very good, therefore, careful attention in postoperative management are important in such a case.

The value of saline-aided ultrasound in diagnosing congenital duodenal obstruction.

PURPOSE: The aim of the study is to assess the value of saline-aided ultrasound (US) in diagnosing congenital duodenal obstruction (CDO). METHODS: The neonates with CDO were enrolled in this study, including the neonates confirmed with annular pancreas (AP) by operation, the neonates confirmed with duodenal atresia, the neonates confirmed with duodenum web, and the neonates confirmed with malrotation. Pertinent data were recorded, including the US features, intraoperative findings, and surgical procedures. The methodology of this study is a diagnostic test study which means the US feature is the test and the intraoperative finding is the gold standard. RESULTS: A total of 95 neonates were enrolled, including 33 neonates with AP, 6 neonates with duodenal atresia, 29 neonates with duodenum web, and 27 neonates with malrotation. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of the hyperechogenic band for the detection of AP were 78.8% (26/33), 90.3% (56/62), 81.2% (26/32), 88.8% (56/63), and 86.3% (82/95), respectively. The sensitivity, specificity, NPV, and accuracy for the detection of duodenal atresia were 0% (0/6), 100% (89/89), 93.6% (89/95), and 93.6% (89/95), respectively. The sensitivity, specificity, PPV, NPV, and accuracy for the detection of duodenum web were 100% (29/29), 100% (66/66), 100% (29/29), 100% (66/66), and 100% (95/95), respectively. The sensitivity, specificity, PPV, NPV, and accuracy for the detection of malrotation were 100% (27/27), 100% (68/68), 100% (27/27), 100% (68/68), and 100% (95/95), respectively. CONCLUSION: Saline-aided US is a feasible tool to diagnose CDO.

The application of prenatal ultrasound in the diagnosis of congenital duodenal obstruction.

BACKGROUND: The purpose of this research is to summarize the prenatal ultrasound characteristics of congenital duodenal obstruction (CDO), especially in the diagnosis of duodenal diaphragm and annular pancreas. At present, few researchers have summarized the specific ultrasound features of duodenal diaphragm and annular pancreas. METHODS: In this study, a retrospective analysis of 40 patients diagnosed with CDO between January 2016 and December 2019 was carried out. Data on the diagnosis, ultrasound images and outcomes of the patients were gathered, and the features of the patients were analyzed. RESULTS: The results showed that there were 17 patients (42.5%) of congenital duodenal diaphragm, all with a 'rat tail' sign on the ultrasound images. Moreover, there were 4 patients (10.0%) of CDO caused by annular pancreas, all with a 'pliers' sign on the ultrasound images. We summarized the imaging features of the 'rat tail' sign and the 'pliers' sign. CONCLUSION: The main conclusion of this study was that the 'rat tail' sign could be used as an indirect ultrasound feature to diagnose duodenal diaphragm. The 'pliers' sign could be used as a direct ultrasound feature in the diagnosis of annular pancreas in CDO.

Enhanced recovery after surgery for the treatment of congenital duodenal obstruction.

BACKGROUND: Enhanced recovery after surgery (ERAS) has been widely used in adult surgery. However, ERAS has not been reported in neonatal surgery. The present prospective study explored the application value of ERAS in treating congenital duodenal obstruction (CDO). METHODS: A total of 68 cases of CDO were collected from October 1, 2017 to July 31, 2019. We divided patients with a prenatal diagnosis of congenital duodenal obstruction into the ERAS group and those who were diagnosed the disease after birth into the control group. The ERAS group adopted ERAS-related measures, and the control group followed the usual measures. The study compared the differences in the gestational age, birth weight, length of hospital stay (LOS), complications, feeding intolerance, and weight one month after surgery between the two groups. RESULTS: A total of 49 patients were included in the analysis, including 23 who were allocated to the ERAS group and 26 to the control group. The LOS was 9.696±1.222 days in the ERAS group and 12.654±1.686 days in the control group, resulting in a significantly shorter LOS in the ERAS group than in the control group (p<0.001). One month after surgery, the neonates in the ERAS group weighted significantly more than those in the control group. No differences were observed in birth weight, gestational age, and the incidence of complications or feeding intolerance between the two groups. CONCLUSION: In this single-center study, the implementation of neonate-specific ERAS for CDO surgery was feasible and safe and led to a shorter LOS without increasing the incidence of complications or feeding intolerance. TYPE OF STUDY: Treatment Study LEVEL OF EVIDENCE: Level III.

The impact of trisomy 21 on epidemiology, management, and outcomes of congenital duodenal obstruction: a population-based study.

PURPOSE: Congenital duodenal obstruction (CDO) is associated with trisomy 21 (T21), or Down's syndrome, in around a third of infants. The aim of this study was to explore the impact of T21 on the epidemiology, management, and outcomes of infants with CDO. METHODS: Data were prospectively collected from specialist neonatal surgical centres in the United Kingdom over a 12 month period from March 2016 using established population-based methodology for all babies with CDO. Infants with T21 were compared to those without any chromosomal anomaly. RESULTS: Of 102 infants with CDO that underwent operative repair, T21 was present in 33 [32% (95% CI 23-41%)] babies. Cardiac anomalies were more common in those with T21 compared to those without a chromosomal anomaly (91 vs 17%, p < 0.001), whereas associated gastrointestinal anomalies were less common in infants with T21 (3 vs 12%, p = 0.03). Surgical management was not influenced by T21. Time to achieve full enteral feed, need for repeat related surgery, and mortality were similar between groups. Infants with T21 had a longer median initial inpatient stay (23 vs 16.5 days, p = 0.02). CONCLUSIONS: Infants with T21 have a higher incidence of cardiac anomalies and a longer initial inpatient stay; however, it does not change CDO management or outcomes. This information is important for prenatal and postnatal counselling of parents of infants with CDO and T21.

Potential Benefits of Laparoscopic Repair of Duodenal Atresia: Insights from a Retrospective Comparative Study.

INTRODUCTION: Congenital duodenal obstruction (CDO) repair can be performed open or laparoscopically. We aimed to determine the potential benefit of laparoscopic repair regarding tolerance of enteral feeding, postoperative pain, hospital stay, and complication rate. MATERIALS AND METHODS: In a single-center retrospective cohort study, we compared neonates with isolated CDO operated open versus laparoscopically from 2010 to 2019. No transanastomotic tubes were used, and anastomoses were created in a side-to-side fashion in all cases. An early feeding policy is applied for all cases operated at our institution. Statistical comparison was performed using the Mann-Whitney's test or Fisher's exact test where appropriate. RESULTS: Forty-one patients analyzed were similar regarding body weight, gestational age, and proportion of patients with trisomy 21. Median follow-up was 21 months. Four (20%) out of 20 laparoscopic procedures started laparoscopically were converted to open. Comparing the 21 open with the 16 laparoscopically completed patients, median anesthetic duration was shorter by 18% in the open versus laparoscopic completed group (218 vs. 179 minutes, respectively; p = 0.025). Median postoperative time to full enteral feeds was shorter by 4 days in the first group (7 vs. 11 days, respectively; p = 0.028). In accordance, the median duration of parenteral nutrition (PN) was less than half in the laparoscopic completed compared with the open group (5 vs. 11.5 days, respectively; p = 0.031). Postoperative opioids were required for only half the duration in the laparoscopically completed group compared with open (2 vs. 4 days, respectively; p = 0.026). Outcomes such as length of stay, the occurrence of strictures or adhesions requiring reintervention, or line sepsis were similar in both groups. CONCLUSION: Patients undergoing laparoscopic CDO repair at our institution benefited from shorter time to full enteral feeds, and reduced the need for PN as well as postoperative pain medication.

Obstrucción duodenal congénita de etiología múltiple: reporte de un caso con malrotación intestinal, bandas de Ladd y membrana duodenal intrínseca como causantes de obstrucción intestinal en el neonato / Congenital duodenal obstruction of multiple etiology: case report with intestinal malrotation, Ladd bands and intrinsic duodenal membrane as causes of intestinal obstruction in the neonate

Resumen: La obstrucción duodenal congénita agrupa un amplio espectro de anomalías en el desarrollo del intestino delgado; se clasifica según su etiología tanto en extrínseca como intrínseca. Su presentación es relativamente común, teniendo una incidencia de 1: 2.500-10.000 nacidos vivos y normalmente presenta asociación con aneuploidías (como la trisomía 21) y malformaciones cardíacas. Las imágenes tienen un papel importante en la aproximación diagnóstica y la radiografía de abdomen simple, es el primer escalón en la aproximación de la obstrucción intestinal. El tratamiento involucra una exploración quirúrgica del abdomen con corrección de los defectos encontrados. Este es un reporte de caso de un neonato que presentó obstrucción duodenal congénita de etiología tanto extrínseca (malrotacion intestinal y bandas de Ladd) como obstrucción intrínseca (membranas duodenales).

Abstract: Congenital duodenal obstruction groups together a broad spectrum of abnormalities in the development of the small intestine; it is classified according to its etiology as both extrinsic and intrinsic. Its presentation is relatively common, having an incidence of 1: 2.500-10.000 live births and it is normally associated with aneuploidy (such as trisomy 21) and cardiac malformations. Images play an important role in the diagnostic approach, with simple abdominal radiography being the first step in the approximation of intestinal obstruction. The treatment corresponds to a surgical exploration of the abdomen with resolution of the defects found. This is a case report of a newborn who presented congenital duodenal obstruction of both extrinsic etiology (intestinal malrotation and Ladd bands) and intrinsic obstruction (duodenal membranes).

Comparison of outcomes between complete and incomplete congenital duodenal obstruction.

BACKGROUND: Congenital duodenal obstruction (CDO) can be complete (CCDO) or incomplete (ICDO). To date there is no outcome analysis available that compares both subtypes. AIM: To quantify and compare the association between CCDO and ICDO with outcome parameters. METHODS: We retrospectively reviewed all patients who underwent operative repair of CCDO or ICDO in our tertiary care institution between January 2004 and January 2017. The demographics, clinical presentation, preoperative diagnostics and postoperative outcomes of 50 patients were compared between CCDO (n = 27; atresia type 1-3, annular pancreas) and ICDO (n = 23; annular pancreas, web, Ladd´s bands). RESULTS: In total, 50 patients who underwent CDO repair were enrolled and followed for a median of 5.2 and 3.9 years (CCDO and ICDO, resp.). CCDO was associated with a significantly higher prenatal ultrasonographic detection rate (88% versus 4%; CCDO vs ICDO, P < 0.01), lower gestational age at birth, lower age and weight at operation, higher rate of associated congenital heart disease (CHD), more extensive preoperative radiologic diagnostics, higher morbidity according to Clavien-Dindo classification and comprehensive complication index (all P ≤ 0.01). The subgroup analysis of patients without CHD and prematurity showed a longer time from operation to the initiation of enteral feeds in the CCDO group (P < 0.01). CONCLUSION: CCDO and ICDO differ with regard to prenatal detection rate, gestational age, age and weight at operation, rate of associated CHD, preoperative diagnostics and morbidity. The degree of CDO in mature patients without CHD influences the postoperative initiation of enteral feeding.