One-year Outcomes of Congenital Duodenal Obstruction: A Population-based Study.

OBJECTIVES: Congenital duodenal obstruction (CDO) occurs in 1.2 per 10,000 live births and is frequently associated with other anomalies, most commonly cardiac. The aim of this study was to report important outcomes to 1 year following surgical repair. METHODS: This was a prospective population-based study using the British Association of Paediatric Surgeons Congenital Anomaly Surveillance System. Cases were identified at specialist pediatric surgical centres in the United Kingdom during a 12-month period starting in March 2016. Outcomes were recorded at 1 year following surgical repair. RESULTS: There were 100 infants with possible follow-up at 1 year and follow-up was achieved in 80 of these (80%) of whom 76 were alive at 1 year. The remainder had been discharged home, although one remained on parenteral nutrition. Five (6.1%) infants underwent repeat surgery for reasons related to CDO and overall 23 (23%) experienced at least 1 central venous catheter-related complication within 1 year. Overall mortality either before repair or within 1 year following surgical repair was 8.4% (95% CI 2.5%-14.4%), no deaths were related to CDO. CONCLUSIONS: One year outcomes for CDO are generally very good with poor outcomes typically related to comorbidities. These data are useful for national benchmarking and parental counselling.

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.

The DATE Association: A Separate Entity or a Further Extension of the VACTERL Association?

BACKGROUND: The combination of esophageal atresia, congenital duodenal obstruction, and anorectal malformation has seldom been reported. We describe the largest series of patients with such association, which we summed up with the mnemonic acronym DATE [D-duodenal obstruction, A-anorectal malformation (ARM), and TE-tracheoesophageal fistula with esophageal atresia]. METHODS: This was a multicenter retrospective review of 13 patients recruited from 8 institutions over a nearly 5-decade period (1968-2017). Information gathered included type of DATE malformations, other associated anomalies, type and timing of surgery, and clinical outcomes. RESULTS: The DATE association consisted of type C esophageal atresia (13), complete (9) or incomplete (4) congenital duodenal obstruction (CDO), and high or intermediate (8) or low (5) ARM. Eight patients had at least one additional component feature of VACTERL association. A total of 6 patients died. Overall, 9 patients achieved complete restoration of gastrointestinal continuity, 7 of whom are alive at a median follow-up of 4 y (range, 1 to 9). Survivors received a median of 6 major operations (range, 4 to 14) to overcome their anomalies and surgical complications. Two incomplete duodenal obstructions were initially overlooked. All survivors with high or intermediate ARM defects required some form of bowel management to keep them clean. CONCLUSIONS: The DATE association is a low-frequency entity, often occurring among the wider spectrum of VACTERL association. Functional outcomes largely depend on the severity of ARM or other major associated malformations. Awareness of the DATE association may avoid untoward diagnostic delays of subtler component features of the spectrum, such as an incomplete CDO.

Duodenal atresia and associated intestinal atresia: a cohort study and review of the literature.

PURPOSE: To determine the true incidence of associated intestinal atresia (AIA) in infants with duodenal atresia (DA) and to analyze whether the surgical approach, open versus laparoscopic, would impact on patient outcome when AIA is present. METHODS: Cohort study We review all DA infants treated at our institution (2001-2016) and analyzed the outcome of those with AIA. Systematic review/meta-analysis Using a defined search strategy and according to PRISMA guidelines, two investigators independently identified all studies on DA and searched cases of AIA to determine its incidence. Data are mean ± SD. RESULTS: Cohort study Of 140 DA infants, 10 (7%) had AIA (4 type I, 4 type III, 2 type II). All type I AIA (webs) were found in the duodenum. Systematic review/meta-analysis Of 840 studies, 18 were included (2026 infants). The incidence of AIA was 2.8 ± 1.6%. The incidence of missed AIA was 0.8 ± 2.4%. Three comparative studies (759 infants) showed higher risk of missed AIA following laparoscopic (2.9 ± 2.4%) than open repair (0.3 ± 0.1%; p < 0.01). CONCLUSIONS: The incidence of AIA in DA infants is low and the risk of missing it is higher at laparoscopy than at laparotomy. Regardless the approach, surgeons should carefully investigate bowel continuity to avoid the risk of missing AIA.

Duodenal Crohn's Disease.

Symptomatic duodenal Crohn's disease (CD) is an uncommon disease presentation, especially in isolation. The most common duodenal disease phenotype is stricturing disease rather than inflammatory or perforating. Most patients are asymptomatic and are therefore diagnosed incidentally by cross-sectional imaging or endoscopy. Medical management includes proton pump inhibitor therapy and immunosuppressive therapy including corticosteroids, immunomodulatory therapy, and biologic therapy. Symptomatic strictures can often be treated medically or endoscopically, and do not always require surgery. Surgical options include resection with primary anastomosis, bypass with a gastrojejunostomy, and strictureplasty. Treatment recommendations are largely based on limited evidence from small series and expert opinion. Therefore, the optimal treatment algorithm remains largely subjective and undefined.

International study of endoscopic management of distal malignant biliary obstruction combined with duodenal obstruction.

OBJECTIVE: Endoscopic transpapillary or endoscopic ultrasound (EUS)-guided stent placement is used for nonresectable distal malignant biliary obstruction. We conducted a retrospective study to evaluate endoscopic biliary drainage in patients with duodenal obstruction. METHODS: We included consecutive patients who underwent endoscopic biliary drainage combined with a duodenal stent at 16 referral centers in four Asian countries. The primary outcome was time to recurrent biliary obstruction (TRBO). We assessed TRBO according to the sequence of biliary and duodenal obstruction (group 1/2/3, biliary obstruction first/concurrent/duodenal obstruction first, respectively) or the location of duodenal obstruction (type I/II/III, proximal to/affecting/distal to the ampulla, respectively). We also evaluated functional success and adverse events. RESULTS: We included 110 patients (group1/2/3, 67/29/14 patients; type I/II/III, 45/46/19 patients; endoscopic retrograde cholangiopancreatography [ERCP]/EUS-guided choledocoduodenostomy/EUS-guided hepaticogastrostomy, 90/10/10 patients, respectively). The median TRBO of all cases was 450 days (interquartile range, 212-666 days) and functional success was achieved in 105 cases (95%). The TRBO did not differ significantly by the timing or location of duodenal obstruction (p = .30 and .79, respectively). The TRBO of metal stents (n = 96) tended to be longer compared with plastic stents (n = 14, p = .083). Compared with ERCP, EUS-guided biliary drainage was associated with a higher rate of adverse events. CONCLUSION: Transpapillary or transmural endoscopic biliary drainage with a duodenal stent was effective, irrespective of the timing or location of duodenal obstruction. A prospective study is required considering the tradeoff of technical success rate, stent patency, and adverse events (ClinicalTrials.gov number, NCT02376907).

Duodenal Atresia: Open versus MIS Repair-Analysis of Our Experience over the Last 12 Years.

Objective. Duodenal atresia (DA) routinely has been corrected by laparotomy and duodenoduodenostomy with excellent long-term results. We revisited the patients with DA treated in the last 12 years (2004-2016) comparing the open and the minimally invasive surgical (MIS) approach. Methods. We divided our cohort of patients into two groups. Group 1 included 10 patients with CDO (2004-09) treated with open procedure: 5, DA; 3, duodenal web; 2, extrinsic obstruction. Three presented with Down's syndrome while 3 presented with concomitant malformations. Group 2 included 8 patients (2009-16): 1, web; 5, DA; 2, extrinsic obstruction. Seven were treated by MIS; 1 was treated by Endoscopy. Three presented with Down's syndrome; 3 presented with concomitant malformations. Results. Average operating time was 120 minutes in Group 1 and 190 minutes in Group 2. In MIS Group the visualization was excellent. We recorded no intraoperative complications, conversions, or anastomotic leakage. Feedings started on 3-7 postoperative days. Follow-up showed no evidence of stricture or obstruction. In Group 1 feedings started within 10-22 days and we have 1 postoperative obstruction. Conclusions. Laparoscopic repair of DA is one of the most challenging procedures among pediatric laparoscopic procedures. These patients had a shorter length of hospitalization and more rapid advancement to full feeding compared to patients undergoing the open approach. Laparoscopic repair of DA could be the preferred technique, safe, and efficacious, in the hands of experienced surgeons.

Clinical significance of the prenatal double bubble sign - single institution experience.

OBJECTIVE: The objective of this study is to determine the risk of aneuploidy, associated structural anomalies, and pregnancy outcome in the presence of prenatal double bubble sign detection. METHOD: We have performed a retrospective study on ultrasound reports and pregnancy outcomes in 42 fetuses with double bubble sign examined in our ultrasound department between 1998 and 2013. RESULTS: Additional major defects or aneuploidy markers were present in 24 fetuses. Chromosomal abnormalities occurred in 21 cases. There were 35 live births; two pregnancies ended in intrauterine fetal demise; five patients opted for termination of pregnancy because of additional anomalies or abnormal karyotype. Postnatal or postmortem verification revealed duodenal obstruction secondary to intrinsic or extrinsic cause in 40 cases; in one case, proximal jejunal atresia was diagnosed, and one neonate had normal bowel. CONCLUSIONS: Sonographic double bubble sign is associated with a significant rate of major anatomic defects, abnormal karyotype, and unfavorable pregnancy outcome. It is strongly indicative of duodenal obstruction.

Risk of adhesive obstruction after colorectal surgery: the benefits of the minimally invasive approach may extend well beyond the perioperative period.

BACKGROUND: Risk of adhesive small-bowel obstruction (SBO) is high following open colorectal surgery. Laparoscopic surgery may induce fewer adhesions; however, the translation of this advantage to a reduced rate of bowel obstruction has not been well demonstrated. This study evaluates whether SBO is lower after laparoscopic compared with open colorectal surgery. METHODS: Patients who underwent laparoscopic abdominal colorectal surgery, without any previous history of open surgery, from 1998 to 2010 were identified from a prospective laparoscopic database. Details regarding occurrence of symptoms of SBO (colicky abdominal pain; nausea and/or vomiting; constipation; abdominal distension not due to infection or gastroenteritis), admissions to hospital with radiological findings confirming SBO, and surgery for obstruction after the laparoscopic colectomy were obtained by contacting patients and mailed questionnaires. Patients undergoing open colorectal surgery for similar operations during the same period and without a history of previous open surgery also were contacted and compared with the laparoscopic group for risk of obstruction. RESULTS: Information pertaining to SBO was available for 205 patients who underwent an elective laparoscopic procedure and 205 similar open operations. The two groups had similar age, gender, and sufficiently long duration of follow-up. Despite a significantly longer duration of follow-up for the laparoscopic group, admission to hospital for SBO was similar between groups. Patients who underwent laparoscopic surgery also had significantly lower operative intervention for SBO (8% vs. 2%, p = 0.006). CONCLUSIONS: Although the rate of SBO was similar after laparoscopic and open colorectal surgery, the need for operative intervention for SBO was significantly lower after laparoscopic operations. These findings especially in the context of the longer follow-up for laparoscopic patients suggests that the lower incidence of adhesions expected after laparoscopic surgery likely translates into long-term benefits in terms of reduced SBO.

Laparoscopic repair of duodenal atresia: revisited.

BACKGROUND: Since the initial reports of laparoscopic repair of duodenal atresia in neonates, further reports have been scant. Could this be because of unacceptable rates of complications, like anastomotic leakage, as mentioned in later reports? In the present study the laparoscopic repair of duodenal atresia in neonates is revisited. PATIENTS: Group 1 consisted of 22 patients with duodenal obstruction between 2000-2005 until the laparoscopic approach was abandoned. Of these 22 patients, 10 had Down syndrome and 8 had concomitant malformations. In this group 18 patients were operated laparoscopically. Four patients underwent an open procedure. Group 2 consisted of six patients that underwent operation between 2008 and February 2010. RESULTS: In group 1 there were four conversions. In 14 patients the procedure could be completed laparoscopically. In five patients postoperative leakage occurred. The complication rate was found to be unacceptably high, and the laparoscopic approach was abandoned. After gaining additional experience in intracorporeal suturing and adjusting the technique, the procedure was started up again in 2008. Since then six consecutive neonates have undergone laparoscopic repair of duodenal atresia without complications. CONCLUSIONS: Laparoscopic repair of duodenal atresia is one of the most demanding pediatric laparoscopic surgical procedures. After initial promising results at the beginning of the twenty-first century a relative "radio silence" followed, apparently caused by unsatisfactory results. Only considerable adjustments in technique and extensive improvement in experience has led to acceptable outcomes more recently. Laparoscopic repair of duodenal atresia should therefore be restricted to pediatric centers with extensive experience in laparoscopic surgery and intracorporeal suturing.

Distinct clinical characteristics of patients with congenital duodenal obstruction in a medical center in Taiwan.

BACKGROUND: Patients with congenital duodenal obstruction (CDO) predominantly have a postampullary lesion site and a high association with Down syndrome, according to previous reports from Western countries. This study aimed to determine whether there are racial differences in the clinical characteristics of congenital duodenal obstruction, based on the experience from a Taiwanese medical center. METHODS: The charts were retrospectively reviewed among neonates with CDO who received surgery at National Taiwan University Hospital during the period 1985- 2008. Patients were grouped into a preampullary or postampullary group according to the obstruction in relation to the ampulla of Vater. Other characteristics, including sex, time of diagnosis, operative findings, as well as associated anomalies, were recorded for further analysis. RESULTS: A total of 30 patients with CDO including 16 with atresia, 10 with mucosal webs, and 4 with stenosis, were recruited; among them, 16 were boys and 14 were girls. In 15 patients (50%), the diagnosis of duodenal obstruction was made prenatally. A total of 11 of the 30 patients (37%) were in the preampullary group and 19 (63%) were in the postampullary group. Seventeen patients (56.7%) had at least one additional anomaly, including four (13%) who had trisomy 21. The preampullary group had significantly fewer associated congenital anomalies than in the postampullary group (27%vs. 74%, p = 0.012). CONCLUSION: Our cohort showed a relatively lower incidence of postampullary lesions and associated Down syndrome in patients with CDO compared with Western countries. Additionally, patients with preampullary lesions had significantly less association with other anomalies.

Gallstone ileus: management options and results on a series of 40 patients.

INTRODUCTION: Controversy remains about the management of gallstone ileus. While some authors propose enterotomy, others defend the one-stage procedure (simultaneously fistula repair). The objective of the present study was to analyze management options and comparative study their results. MATERIAL AND METHODS: Retrospective and descriptive study with revision of clinical stories of patients with the diagnosis of gallstone ileus between 1987 and 2008. All the following variables were recorded: dates of hospital admission, surgery and discharge, age, sex, pathological antecedents, preoperative or intraoperative diagnosis, treatment, location of the fistula and location of the obstruction. End-result variables were: postoperative complications, mortality, complications during the follow-up and biliary complications. RESULTS: A total of 40 patients were included of 46,648 admissions. Age, comorbidity, and intraoperative diagnosis were related with poorer short- and long-outcomes. The percentage of postoperative complications was similar for groups with and without fistula repair. Mortality was higher in the group with fistula repair (15 vs. 25%). Biliary complications were more frequent in the group without fistula repair (11 vs. 0%). Sex, location of the fistula and location of the obstruction did not be related with the prognosis. CONCLUSION: One-stage procedure is related with higher mortality rate than enterotomy alone. Nevertheless, fistula repair reduces the number of biliary complications during the follow-up.

Prenatal diagnosis of duodenal obstruction selects cases with a higher risk of maternal-foetal complications and demands in utero transfer to a tertiary centre.

OBJECTIVES: It was the aim of this study to determine if prenatal diagnosis of congenital duodenal obstruction (CDO) selects high-risk pregnancies and demands special perinatal attention. METHODS: Medical records of 62 neonates with intrinsic CDO, admitted since 1981 in 2 institutions, were reviewed and divided into 2 groups: 39 cases, detected in utero by a prenatal ultrasonogram (group A), and 23 diagnosed at birth (group B). Prevalence of complete CDO, mean gestational age, mean birth weight, hydramnios, as well as maternal-foetal complications requiring emergency Caesarean section and associated with premature delivery were compared in the 2 groups. RESULTS: A complete CDO was found in 77% of patients in group A versus 48% of patients in group B (p < 0.02). Differences in mean gestational age and mean birth weight in the 2 groups were non-significant. Prenatally diagnosed patients presented a higher prevalence of hydramnios, maternal-foetal complications and premature delivery. CONCLUSIONS: Prenatal diagnosis selects patients with complete CDO and hydramnios. These pregnancies present a high incidence of maternal-foetal complications, which may require an emergency Caesarean section, and are frequently associated with premature delivery. These aspects must be considered in prenatal counselling. In utero transfer to a tertiary centre for delivery and appropriate perinatal care should be recommended.

Laparoscopic treatment of duodenal obstruction: report on first experiences in Latin America.

BACKGROUND: There are few reports in the literature on the use of a laparoscopic approach for duodenal obstruction, particularly for duodenal atresia. We report here the results of 4 cases treated laparoscopically, and discuss the safety, feasibility and long-term results of this approach. PATIENTS AND METHODS: Four pediatric patients presented with duodenal obstruction, one with duodenal atresia, one with annular pancreas, and two with duodenal obstruction due to Ladd's bands but without malrotation. Diagnosis was made by clinical evaluation, simple X-ray film in the Ladd's patients, and contrast gastroduodenal X-ray series for the annular pancreas. All procedures were performed using 3-mm instruments and 3 trocars. Two duodeno-duodenal anastomoses were performed and two lyses of Ladd's bands; all procedures were carried out laparoscopically. RESULTS: An upper GI contrast excluded obstruction or leakage in all patients 5 - 7 days after surgery and feedings were started. The patient with annular pancreas died of cardiovascular complications after one months. The other 3 patients are asymptomatic and tolerating feedings after a follow-up of 36 months. CONCLUSIONS: We conclude that a laparoscopic approach for duodenal obstruction can be performed safely and effectively and achieves a short hospital stay. Laparoscopic lysis of Ladd's bands is easy to perform, but duodenoduodenostomy requires advanced laparoscopic skills.

The influence of trisomy 21 on the incidence and severity of congenital heart defects in patients with duodenal atresia.

Duodenal atresia is associated with a wide variety of congenital malformations. Trisomy 21 occurs in approximately one-thirds of infants with duodenal atresia. Congenital heart disease in patients with trisomy 21 and duodenal atresia is well known. However, the frequency and spectrum of congenital heart defects in infants with duodenal atresia and a normal karyotype has not been outlined in the literature. Therefore, we conducted a retrospective chart review to clarify our knowledge about this population. Retrospective review of the medical record was performed on patients with duodenal atresia/stenosis from January 1995 to September 2007. Demographic data included birth weight and gestational age. Variables of interest included cardiac defects and karyotype. Surgical repair for duodenal and cardiac malformations were reviewed. Ninety-four patients with duodenal atresia/stenosis were identified. Average gestational age was 36 weeks and birth weight was 2,536 g. Trisomy 21 was identified in 39 (41%) patients. Overall, 37 patients (39.3%) had a congenital heart defect. Defects were identified in 24 (61.5%) patients with trisomy 21, when compared to 13 (23.6%) patients with a normal karyotype. Of the patients with congenital heart defects and trisomy 21, 11 (28.2%) required operative repair compared to the 6 (10.9%) patients with a defect and normal karyotype. Therefore, in patients with duodenal atresia, the presence of trisomy 21 carries a relative risk of 2.61 for congenital heart defects, and relative risk of 2.59 for open heart surgery. In patients with duodenal atresia, the presence of trisomy 21 carries a 2.5-fold increased risk of cardiac defect and the same increased risk for repairing a cardiac defect.

Congenital duodenal obstruction in children: a decade's experience.

INTRODUCTION: Congenital duodenal obstruction (CDO) is one of the most common anomalies in newborns and infants. In 38-55% of patients, intrinsic duodenal obstruction is associated with another significant congenital anomaly. We report on a retrospective study of all children born with CDO presented to our institution over a 10-year period. PATIENTS AND METHODS: A retrospective analysis was carried out of the records of all children admitted to our center from January 1996 to December 2005 with the diagnosis of congenital duodenal obstruction. Seventy-seven patients were enrolled in the study. Age ranged from 1 day to 3 months; there were 44 males and 33 females. Weight ranged from 800 g to 4.5 kg. Five babies (6.49%) were born prematurely between 31 and 34 weeks' gestation. For the purposes of comparison, duodenal obstruction was divided into 3 groups according to the intraoperative findings. The follow-up period ranged from 6 months to 5 years. The collected data were tabulated, compared, and statistically analyzed. RESULTS: Patients were divided into 3 groups: group 1 (duodenal atresia) consisted of 32 patients, group 2 (annular pancreas) consisted of 30 patients and group 3 (duodenal web) consisted of 15 patients. Twenty-five patients (32.4%) were diagnosed with pure duodenal obstruction, while 52 cases (67.5%) had other associated anomalies. Cardiac anomalies, Down syndrome, GIT anomalies and renal anomalies were the most commonly occurring associated anomalies. CONCLUSION: Congenital duodenal obstruction repair can have a very good outcome, as our study shows. Associated congenital cardiac, GIT, and respiratory anomalies were the main cause of postoperative morbidity. Down syndrome did not influence morbidity. Mortality was unrelated to duodenal obstruction.

Stent collapse as a delayed complication of placement of a covered gastroduodenal stent.

OBJECTIVE: The purpose of this study was retrospective evaluation of the incidence, predictive factors, and interventional management of stent collapse after placement of a covered metallic stent in patients with obstruction of the gastric outlet or duodenum due to malignant disease. MATERIALS AND METHODS: Among 259 patients with symptomatic malignant gastroduodenal obstruction successfully treated with stent placement, stent collapse occurred in 12 (4.6%) of the patients 34-270 days (mean, 101.8 days) after stent placement. Multivariate analysis was performed to evaluate factors predictive of stent collapse. Interventional management of stent collapse also was evaluated. RESULTS: Multivariate analysis showed that presence of the stent in the peripyloric region (odds ratio, 27.745; p = 0.036), longer survival time (odds ratio, 1.016; p < 0.001), and absence of chemotherapy after stent placement (odds ratio, 31.661; p = 0.048) were independent predictors of stent collapse. Eleven patients with stent collapse were successfully treated with placement of a second bare stent. The twelfth patient refused further treatment. CONCLUSION: Stent collapse is an uncommon delayed complication of placement of covered metallic stents in patients with malignant gastroduodenal obstruction. Collapse occurs most commonly in the peripyloric region, in patients with longer survival times, and in patients who do not undergo chemotherapy after stent placement. Stent collapse can be managed by coaxial placement of a second bare stent into the collapsed stent.

Analysis of postoperative reoperation for congenital duodenal obstruction.

OBJECTIVE: To analyse the risk factors for reoperation after initial surgical repair of congenital duodenal obstruction and demonstrate that they can be decreased with more careful attention and more advanced techniques during surgery. METHODS: The records of newborns and infants (aged 0-2 months) who had surgical therapy for congenital duodenal obstruction in the past 30 years were reviewed and analysed. Of the 298 patients, 132 (44%) were boys and 166 (56%) were girls. All patients who underwent repeat surgery postoperatively were evaluated by the reasons for surgery. The number of patients with various combination lesions of congenital duodenal obstruction was also calculated and the relationship to postoperative reoperation was analysed. RESULTS: Twenty patients (6.7%) had congenital duodenal obstruction with combination lesions including duodenal web, malrotation, annular pancreas and multiple duodenal web. Twelve patients required further operation 5 days to 2 years postoperatively for complications (n = 5) and other duodenal atresias that were not discovered initially (n = 7). CONCLUSION: More than half of reoperated patients (7/12) had multiple lesions of duodenal obstruction that were missed during the primary operation. The postoperative reoperation rate for congenital duodenal obstruction could be decreased with more careful attention to operative details and more preoperative and intraoperative evaluation of the gastrointestinal tract.

Duodenal atresia and stenosis: long-term follow-up over 30 years.

BACKGROUND: Duodenal atresia and stenosis is a frequent cause of congenital, intestinal obstruction. Current operative techniques and contemporary neonatal critical care result in a 5% morbidity and mortality rate, with late complications not uncommon, but unknown to short-term follow-up. METHODS: A retrospective review of patients with duodenal anomalies was performed from 1972 to 2001 at a tertiary, children's hospital to identify late morbidity and mortality. RESULTS: Duodenal atresia or stenosis was identified in 169 patients. Twenty children required additional abdominal operations after their initial repair with average follow-up of 6 years (range, 1 month to 18 years) including fundoplication (13), operation for complicated peptic ulcer disease (4), and adhesiolysis (4). Sixteen children underwent revision of their initial repair: tapering duodenoplasty or duodenal plication (7), conversion of duodenojejunostomy to duodenoduodenostomy (3), redo duodenojejunostomy (3), redo duodenoduodenostomy (2), and conversion of gastrojejunostomy to duodenoduodenostomy (1). There were 10 late deaths (range, 3 months to 14 years) attributable to complex cardiac malformations (5), central nervous system bleeding (1), pneumonia (1), anastomotic leak (1), and multisystem organ failure (2). CONCLUSIONS: Late complications occur in 12% of patients with congenital duodenal anomalies, and the associated late mortality rate is 6%, which is low but not negligible. Follow-up of these patients into adulthood is recommended to identify and address these late occurrences.

Management of biliary and duodenal complications of chronic pancreatitis.

Biliary stricture and duodenal obstruction have been increasingly recognized as complications of chronic pancreatitis. The anatomical relationship of the distal common bile duct and the duodenum with the head of the pancreas is the main factor for their involvement in chronic pancreatitis. In hospitalized patients with pancreatitis, the incidence of biliary stricture and duodenal obstruction is reported to be about 6% and 1.2%, respectively. For patients requiring an operation for chronic pancreatitis the incidence increases to 35% for biliary stricture and 12% for duodenal obstruction. Fibrosis around the distal common bile duct can cause stenosis with obstruction of bile flow. Clinically, the presentation of these patients ranges from being asymptomatic with elevated alkaline phosphatase or bilirubin, or both, to being septic with cholangitis. Jaundice, cholangitis, hyperbilirubinemia, and persistent elevation of serum alkaline phosphatase occur more frequently in patients with pancreatitis with a biliary stricture. A twofold elevation of alkaline phosphatase is a marker of possible common duct stenosis in patients with chronic pancreatitis. The incidence of both biliary cirrhosis and cholangitis in these patients is about 10%. ERCP reveals a characteristic long, smoothly tapered stricture of the intrapancreatic common bile duct. In duodenal obstruction, the factors that convert self-limiting edema to chronic fibrosis and stricture formation are unknown, but ischemia superimposed on inflammation may be the major cause. These patients present with a prolonged history of nausea and vomiting. Barium studies typically show a long constricting lesion of the duodenum, and endoscopy reveals reactive inflammatory changes in a narrowed duodenum. Operation is indicated in patients with common bile duct strictures secondary to chronic pancreatitis when there is evidence of cholangitis, biliary cirrhosis, common duct stones, progression of stricture, elevation of alkaline phophatase and/or bilirubin for over a month, and an inability to rule out cancer. The operation of choice is either choledochoduodenostomy or choledochojejunostomy. A cholecystoenterostomy is less favored because of its higher failure rate (23%). Endoscopic stenting plays a role in patients who are unfit for surgery, but it is not recommended as definitive therapy. For duodenal obstruction, failure to resolve the obstruction with 1-2 weeks of conservative therapy is an indication for bypass. The operation of choice is a gastrojejunostomy. Not uncommonly, combined obstruction of the pancreatic duct, common bile duct, and duodenum will develop. Combined drainage procedures or resection are used to manage these problems.