Annular Pancreas Research Articles

Endoscopic Ultrasound in Pancreatic Duct Anomalies.

Embryological development of the pancreas is a complex phenomenon and, therefore, it can have multiple developmental aberrations. Fortunately, the majority of these pancreatic ductal anomalies are asymptomatic with no clinical relevance and are incidentally detected during diagnostic cross-sectional imaging or endoscopic retrograde cholangiopancreatography (ERCP) or autopsy. Occasionally, pancreatic duct anomalies can result in symptoms like abdominal pain or recurrent pancreatitis. Also, an accurate pre-operative diagnosis of ductal anomalies can prevent inadvertent duct injury during surgery. Conventionally, ERCP had been used for an accurate diagnosis of pancreatic duct anomalies. However, because it is invasive and associated with a risk of pancreatitis, it has been replaced with magnetic resonance cholangiopancreatography (MRCP). MRCP has demonstrated high sensitivity and specificity for the diagnosis of ductal anomalies, which can be further improved with the use of secretin-enhanced MRCP. Endoscopic ultrasound (EUS) is a new diagnostic and interventional tool in the armamentarium of endoscopists and has demonstrated promising results in the detection of pancreatic duct variations and anomalies. Along with the visualization of the course and configuration of the pancreatic duct, EUS can also visualize changes in the pancreatic parenchyma, thereby helping with an early diagnosis of any co-existent pancreatic disease. Absence of the stack sign and crossed duct sign are important EUS features to diagnose pancreas divisum. EUS can also help with the diagnosis of other congenital ductal anomalies like annular pancreas, ansa pancreatica, and anomalous pancreaticobiliary union, although the published experience is limited.

The Broad Spectrum of Paediatric Pancreatic Disease: A Single-center 26-years Retrospective Review

BackgroundPaediatric pancreatic pathology and its management is rarely described. We present our experience. MethodsA retrospective case-note review of all patients with pancreatic disease from 1995 to 2021 was completed. Data are quoted as median (range). ResultsTwo hundred and twelve patients were identified with 75.9% presenting with pancreatitis. Referrals for pancreatitis increased during the study period and affected a wide age range (2 months–15.6 years).Acute pancreatitis (n = 118) (age 10.6 (0.18–16.3) years). The most common causes were idiopathic (n = 60, 50.8%) and biliary (n = 28, 23.8%). About 10% required treatment for complications or underlying biliary causes.Recurrent pancreatitis (n = 14) (11.6 (0.3–14.3) years). The most common cause was hereditary pancreatitis (n = 6, 42.9%). One patient required endoscopic drainage of pseudocyst.Chronic pancreatitis (n = 29) (16 (0.38–15.5) years). The underlying diagnosis was idiopathic (n = 14, 48.4%) or hereditary pancreatitis (n = 10, 34.5%). 13 patients required active management, including pancreaticojejunostomies (n = 5).Blunt Trauma (n = 34) was managed conservatively in 24 (70.5%). 6 patients required open surgery, but 4 were managed by either endoscopy or interventional radiology.Pancreatic tumours (n = 13) presented at 11.2 (2.3–16) years. Pathology included pancreaticoblastomas (n = 3), solid pseudopapillary tumours (n = 3), neuroendocrine tumours (n = 2), acinar cell cystadenoma (n = 1), intraductal papillary mucinous neoplasm (n = 1), pancreatic insulinoma (n = 1), pancreatic ductal adenocarcinoma (n = 1), and embryonal rhabdomyosarcoma (n = 1). Others (n = 4)Pancreatic cyst (n = 3) and annular pancreas (n = 1). ConclusionPaediatric pancreatic disease spans a wide spectrum of both benign and malignant disease and benefits from access to specialist medical, surgical, endoscopic, and interventional radiology expertise. Referrals for paediatric pancreatitis are increasing, but aetiology is different to that seen in adults. Level of EvidenceIV

Diamond-shaped versus side-to-side anastomotic duodenoduodenostomy in laparoscopic management of annular pancreas in children: a single-center retrospective comparative study.

Annular pancreas is a rare congenital disorder that requires surgical management once diagnosed. Diamond-shaped and side-to-side duodenoduodenostomy are both popular worldwide nowadays in the surgical management of annular pancreas. Here we present our experience with laparoscopic management of annular pancreas in the last 5 years and compare the clinical results of the diamond-shaped versus side-to-side anastomotic techniques. Fifty-two patients diagnosed with annular pancreas who underwent duodenoduodenostomy at our medical center between January 2016 and April 2021 were included in the study. Forty-four patients underwent laparoscopic diamond-shaped duodenoduodenostomy (DS group) and eight underwent laparoscopic side-to-side duodenoduodenostomy (STS group). Clinical data, including surgical indices and early outcomes after surgery, with at least 19 months of follow-up, were collected and analyzed. Of the 52 patients, 61.5% were prenatally diagnosed, and vomiting was the most common clinical manifestation after birth. The operative time and bleeding volume were 187.5 [interquartile range (IQR), 150-228)] min and 2 (IQR, 2-5) mL in the DS group, compared to 175 (IQR, 155-270) min and 2 (IQR, 2-4.25) mL in the STS group (P=0.89 and 0.32 respectively). The mean time from surgery to initial oral feeding and full oral feeding was 6 (IQR, 4-10) and 12 (IQR, 10-15) days in the DS group, compared to 8 (IQR, 4.75-11.25) and 14.5 (IQR, 13-16.75) days in the STS group (P=0.61 and 0.46 respectively). The mean hospital stay was 16 (IQR, 14-19) and 20 (IQR, 17.75-26) days in the DS and STS groups respectively (P=0.13). No severe complications such as anastomotic leakage, anastomotic stenosis, reoperation or unsuspected rehospitalization were noted in either group. Feeding intolerance was revealed in six cases in the DS group and two cases in the STS group, and there was no significant difference between the two groups (P=0.50). Both laparoscopic diamond-shaped and side-to-side techniques showed good clinical results in treating annular pancreas. The surgical technique, trans-anastomotic tube and early feeding are not likely to increase the risk of postoperative feeding intolerance.

S2167 Annular Pancreas: An Atypical Presentation in an Adult Patient

S2167 Annular Pancreas: An Atypical Presentation in an Adult Patient

S2017 A Case of Annular Pancreas Leading to Duodenal Obstruction

S2017 A Case of Annular Pancreas Leading to Duodenal Obstruction

Ectopic bile duct: A case report and brief review

Introduction: An ectopic bile duct is the confluence of common bile duct (CBD) and main pancreatic duct away from its usual location in the posteromedial wall of the second part of duodenum. This increases risk of choledocholithiasis and pancreatitis, while proximal locations increase risk of duodenal ulceration and stenosis. Case Report: A woman in her late forties was consented to elective laparoscopic cholecystectomy for symptoms of biliary colic and ultrasonographic features of a mobile gallstone. Intraoperative cholangiogram noted dilated biliary tree and main pancreatic duct despite normal liver function values, as well as an ectopic ampulla of Vater to distal third part of duodenum or proximal fourth part of duodenum. The distal bile duct had a longer common channel. The operation was otherwise uncomplicated. Magnetic resonance cholangiopancreatography demonstrated similar findings. The patient recovered uneventfully and was discharged from surgical services. Conclusion: The gold standard for diagnosing ectopic bile ducts is via endoscopic retrograde cholangiopancreatography. Hepatobiliary anatomical variants can also occur in the location of the ampulla, thus it is important for surgeons and trainees to interpret and document this carefully during intraoperative cholangiogram, especially in patients who may require endoscopic retrograde cholangiopancreatography in the perioperative period and/or later date. The patient should be educated on the associated risks and red flags to seek medical attention.

Malignant Intraductal Papillary Mucinous Neoplasm With Underlying Annular Pancreas

Malignant Intraductal Papillary Mucinous Neoplasm With Underlying Annular Pancreas

Duodenal atresia, annular pancreas, and situs inversus totalis- rare association in a newborn: A case report

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.

Exome sequencing findings in children with annular pancreas.

Annular pancreas (AP) is a congenital defect of unknown cause in which the pancreas encircles the duodenum. Theories include abnormal migration and rotation of the ventral bud, persistence of ectopic pancreatic tissue, and inappropriate fusion of the ventral and dorsal buds before rotation. The few reported familial cases suggest a genetic contribution. We conducted exome sequencing in 115 affected infants from the California birth defects registry. Seven cases had a single heterozygous missense variant in IQGAP1, five of them with CADD scores >20; seven other infants had a single heterozygous missense variant in NRCAM, five of them with CADD scores >20. We also looked at genes previously associated with AP and found two rare heterozygous missense variants, one each in PDX1 and FOXF1. IQGAP1 and NRCAM are crucial in cell polarization and migration. Mutations result in decreased motility which could possibly cause the ventral bud to not migrate normally. To our knowledge, this is the first study reporting a possible association for IQGAP1 and NRCAM with AP. Our findings of rare genetic variants involved in cell migration in 15% of our population raise the possibility that AP may be related to abnormal cell migration.

Comparison of endoscopic balloon dilation and surgery for duodenal stricture in patients with Crohn’s disease

Background Few studies have compared endoscopic balloon dilation (EBD) and surgery in the treatment of duodenal stricture in patients with Crohn’s disease (CD). Methods We performed a retrospective study to compare the efficacy and safety among patients with CD-associated duodenal stricture treated with EBD or surgery from October 2013 to December 2021. Univariate and multivariate analyses were performed to evaluate factors associated with recurrence-free or surgery-free survival. Results A total of 48 eligible patients were included, including 30 patients treated with EBD only and 18 patients treated with surgery. Patients treated with surgery experienced more symptomatic improvement (100% vs. 63.33%, p = 0.003) and significantly longer recurrence-free survival (6.31 [IQR: 3.00–8.39] years vs. 2.96 [IQR: 1.06–5.42] years, p = 0.01) but suffered more postprocedural adverse events (16.67% vs. 0.74% per procedure, p = 0.001). In patients initially treated with EBD (n = 41), a total of 11 (26.83%) required subsequent surgical intervention. Younger age at CD diagnosis (HR = 0.90, 95% CI: 0.81–1.00, p = 0.04) was associated with a higher risk for subsequent surgery. Conclusions Surgery for CD-associated duodenal strictures was associated with a longer recurrence-free survival. EBD was safe and effective with minimal postprocedural adverse events but led to a high frequency of recurrence.

Endoscopic Ultrasonography-Guided Biliary Drainage: Experience of a Moroccan Center

Background: Endoscopic ultrasound-guided biliary drainage (EUS-BD) is a relatively new therapeutic modality in the arsenal of endoscopists for attaining satisfactory biliary drainage when traditional ERC fails. The aim of our study is to describe the first Moroccan experience with EUS-BD in patients with malignant biliary obstruction after a failed or inaccessible ERCP. Methods: This is a retrospective study conducted at Ibn Sina Hospital, from Mars 2018 to July 2022, which include 27 patients with an inoperable or metastatic malignant biliary obstruction who underwent endoscopic ultrasonography-guided biliary drainage (EUS-BD) in case of endoscopic retrograde cholangiopancreatography (ERCP) failure. Results: From a total of 288 patients with malignant biliary obstruction,27 underwent EUS-BD (9,3%), 15 EUS-guided hepaticogastrostomy (55,5%) and 12 EUS-guided choledocoduodenostomy (44,4%) were performed. The mean age was 62.5 years (age range: 23-74 years), a male predominance of 59% was noted, cholestatic jaundice was present in 100% of patients and associated with pruritus in 92.59% of cases. The mean level of Total Bilirubin was 190 mg/l. The causes of biliary obstruction were: an inoperable pancreatic head mass in 13 patients(48,1%), a cholangiocarcinoma in 7 patients (25.9%), an ampullary mass in 4 patients(14,8%), a gallbladder cancer with hepatic and duodenal invasion in one patient (3,7%), a gastric antropyloric adenocarcinoma with pancreatic head invasion in one patient (3,7%) and Duodenal adenocarcinoma in one patient (3,7%).The leading causes of failed ERCP were secondary to tumor infiltration of the duodenal wall and critical duodenal stenosis in 11 cases (40,7%), in 9 cases due to a difficult cannulation (33.3%) and in 7 cases due to a failure to pass the stricture by ERCP (25.9%). In EUS-HG The technical and the clinical success rates were 86.6% (13/15) and 84.6% (11/13), respectively, the complication rate was 13.3% (2/15) including one case of stent migration complicated with bile leak and one case of cholangitis. In EUS-CD The technical and the clinical success rates were 91.6% (11/12) and 81.8% (9/11)), respectively, the complication rate was 16.6% (2/12) including two cases of cholangitis. The technical and the clinical success rates in all EUS-BD were 85.1% (24/27) and 83.3% (20/24), respectively. The complication rate was 14.8% (4/27). Conclusion: EUS-BD appears to be an effective and safe therapeutic option for biliary decompression in cases where ERCP fails.

Development of human pancreas

Pancreas is an exocrine and endocrine organ. Exocrine portion secretes digestive fluid. Endocrine portion secreting insulin and glucagon etc. Pancreas developed from dorsal and ventral pancreatic buds, which arise from either side of distal foregut. When duodenum rotates to right, ventral pancreatic bud(VPB) rotates posterior along with common bile duct(CBD) and finally lies below and behind the dorsal pancreatic bud(DPB). In humans, DPB forms major part and VPB forms inferior part of head and uncinate process of pancreas. Main duct (Duct of Wirsung) is derived from whole of ventral pancreatic duct(VPD) and distal part of dorsal pancreatic duct(DPD). Main duct joins with CBD and it perforated posteromedial side of second part of duodenum at major duodenal papilla. Occasionally accessory duct might originate from proximal part of DPD and open into minor papilla. Sometimes two buds do not fuse and lead to pancreatic divisum. Abnormal rotation and fusion of buds might lead to annular pancreas. A few endodermal pancreatic evaginations may remain and migrate in bowel wall and form accessory(heterotopic) pancreas. Islets originating from DPB have more insulin synthesis. Neck, body and tail of pancreas is supplied by coeliac trunk. Derivatives of right VPB are supplied by branches of superior mesenteric artery. During rotation of VPB, superior mesenteric vessels(SMV) were engaged in between DPB and VPB. Lastly, SMV is located posterior to neck but it is anterior to uncinate process of pancreas. Detailed study of development of exocrine and endocrine portions is required for successful management of pathology of the pancreas.

Annular Pancreas in Adults: Clinical Presentations, Diagnosis, and Management

Annular Pancreas in Adults: Clinical Presentations, Diagnosis, and Management

Wolfram Syndrome-2, a Cause of Severe Gastrointestinal Bleeding: A Case Series and a Literature Review.

There are very few reports of Wolfram syndrome-2 (WFS2) in the literature, and understanding of involvement of the gastrointestinal (GI) tract in the syndrome is limited. Objectives: This study aims to describe the clinical presentations of a large number of WFS2 patients with specific focus on their GI manifestations. This is a retrospective case series study. Patients who were homozygous for the CISD2 gene mutation were identified through the genetic department of Al-Makassed hospital. Their medical records were reviewed, and biometric data have been obtained. The data were collected and arranged on a data sheet, and descriptive analysis was done using SPSS. Thirteen patients from 9 families were identified; diabetes mellitus was present in 6 of them, optic atrophy in 5, diabetes insipidus (DI) in 5, and deafness in 2. All of the patients had GI manifestations with abnormal findings on upper endoscopy. Dysmorphic facial features and abnormal findings on brain MRI were present in 3 of our patients. The GI manifestations including GI bleeding and severe ulcerations were the first to appear in 9 of them, while anemia in the remaining 4. This is the largest study to date describing patients with WFS2. This study's evidence shows the prominent presence of GI involvement, and the severe findings on endoscopy, including duodenal, gastric, and esophageal ulcerations and strictures. Unlike in the Jordanian report, some of the patients in our report also have DI.

Annulling an Annulled Annular Pancreas: A Case Report

Annular Pancreas (AP) is a rare congenital developmental anomaly which results from failure of the ventral bud to rotate to fuse with the dorsal bud, resulting in a ring of pancreatic parenchyma that surrounds the second portion of the duodenum. This rare entity can be partial or complete and the partial is even rarer. Most cases manifest in the infancy but may remain asymptomatic and various nonspecific clinical manifestations are seen in adults. We report a rare case of partial annular pancreas with atrophy of body and tail of pancreas which presented with upper gastrointestinal bleeding and obstruction. The challenge in this case was the annular pancreas was the only functioning pancreatic tissue.

Chronic duodenal obstruction complicated by the course of chronic pancreatitis

The annular pancreas (CPJ) is mainly considered in pediatric surgical practice as a pathology of the newborn period, manifested by a picture of duodenal obstruction and having the potential for complete cure. Nevertheless, during the course of life, patients with this defect may develop complications such as chronic pancreatitis, peptic ulcer, pancreatic tumors, changes in the biliary tract, etc., which develop in adulthood and often cause complications and deaths. There are very few descriptions of such complications in children in the available literature, and therefore, we want to provide a clinical case of an 8-year-old boy with a complicated course of CPJ.

Intraluminal endoscopic complex approach in the treatment of a patient with adenoma of the major duodenal papilla, choledocholithiasis and stricture of the terminal common bile duct

Introduction. Adenomas of the major duodenal papilla are benign neoplasms, but due to their high tendency to malignancy, they must be removed. At present, minimally invasive methods of operations are increasingly being considered as an alternative to surgical interventions, while cases of a combination of a neoplasm of the major duodenal papilla with choledocholithiasis and bile duct strictures are of particular difficulty in endoscopic treatment. Clinical case. A 62-year-old patient underwent complex endoscopic treatment at the Vishnevsky National Research Medical Center for Surgery about adenoma of the major duodenal papilla type IV, extending to the walls of the duodenum and the terminal section of the common bile duct, in combination with choledocholithiasis and cicatricial stricture of the common bile duct. Results. The patient successfully applied a complex approach of minimally invasive treatment in the amount of endoscopic lithoextraction, fragmentary removal of adenoma of the major duodenal papilla with intraductal high-frequency electrosurgical ablation of residual adenomatous tissue and subsequent staged biliary stenting for cicatricial stricture of the common bile duct, as well as stenting of the main pancreatic duct to prevent post-manipulation pancreatitis. Discussion. An integrated approach in the treatment of a patient with adenoma of the major duodenal papilla IV, choledocholithiasis and stricture of the common bile duct, made it possible to achieve clinical success in the absence of complications and the need for long-term rehabilitation.

IgG4-related disease of duodenal obstruction due to multiple ulcers in a 12-year-old girl

BackgroundImmunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory disease and affected individuals typically present with an increased infiltration of IgG4-positive plasma cells in the pancreas, hepatobiliary tract, and liver but rarely in the gastrointestinal tract.Case presentationA 12-year-old girl presented with vomiting and poor weight gain. Gastroscopy revealed duodenal stenosis and ulceration. Computed tomography revealed edematous duodenal wall thickening and air-fluid levels on the right side of the duodenum, which suggested duodenal perforation or penetration. She underwent pancreaticoduodenectomy, and IgG4-RD was diagnosed via histopathology.ConclusionsThis is the first pediatric case of isolated duodenal IgG4-RD resulting in duodenal obstruction after multiple ulcers. Gastrointestinal IgG4-RD should be among the differential diagnoses of unexplained gastrointestinal obstruction or ulceration even in children.

Pancreaticoduodenectomy in the Portal Annular Pancreas-Mesopancreas Triangle Approach (with Video).

Portal Annular Pancreas (PAP) is a relatively uncommon entity with 4% reported incidence. Pancreaticoduodenectomy is challenging in cases with PAP and is associated with higher postoperative pancreatic fistula rate and overall morbidity. PAP is classified according to the pattern and location of fusion around the portal vein as-supra-splenic, infra-splenic & mixed fusion type. Also, the ductal anatomy can vary as pancreatic duct present only in the ante-portal portion or only in the retro-portal portion or ducts in both ante and retro-portal portion. At present, ideal surgical strategy is not defined as per the PAP types. The case demonstrated in the video presented with a localized, large duodenal mass with type IIA PAP (supra-splenic fusion with both ante and retro-portal ducts) detected on the preoperative triphasic CT scan. To achieve a single pancreatic cut surface with a single pancreatic duct for anastomosis, an extended pancreatic resection was performed using meso-pancreas triangle approach. Patient had a smooth intraoperative course & the postoperative recovery was also uneventful. Pathology reported pT3 duodenal cancer with negative margins and uninvolved lymph nodes. A preoperative knowledge of PAP and its various types is extremely important in order to tailor intraoperative management, specially of the retro-portal portion. In patients with retro-portal duct or both ante and retro-portal ducts (as the case presented in the video), an extended resection is recommended to mitigate postoperative pancreatic fistula.

Resection of Late Recurrence of Gastric Cancer with Duodenal and Bile Duct Strictures 22 Years after Surgery for Gastric Cancer

Resection of Late Recurrence of Gastric Cancer with Duodenal and Bile Duct Strictures 22 Years after Surgery for Gastric Cancer