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Diagnostics (Basel, Switzerland) Oct 2023Embryological development of the pancreas is a complex phenomenon and, therefore, it can have multiple developmental aberrations. Fortunately, the majority of these... (Review)
Review
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.
PubMed: 37835872
DOI: 10.3390/diagnostics13193129 -
Insights Into Imaging Jul 2021Abdominal computed tomography (CT) is frequently performed to evaluate gastrointestinal pathologic conditions. The majority of the gastrointestinal radiology literature... (Review)
Review
Abdominal computed tomography (CT) is frequently performed to evaluate gastrointestinal pathologic conditions. The majority of the gastrointestinal radiology literature has concentrated on the colon, stomach, and distal small bowel. The duodenum is often overlooked on imaging, namely on CT, but its anatomy (intra and retroperitoneal) and location in such close proximity to other viscera results in involvement by a multitude of primary and secondary processes, some of them exclusive to this bowel segment. While some conditions, like duplications, lipomas, and diverticula, are usually asymptomatic and are incidentalomas that have no pathologic significance, others are symptomatic and very relevant and should be recognized by every general radiologist: development conditions such as annular pancreas and gut malrotation; inflammatory processes such as ulcers and secondary involvement from pancreatitis; neoplastic conditions such as adenocarcinoma, lymphoma, or local extension from adjacent malignancies. They all can be reliably diagnosed with CT. In this article, we demonstrate the typical imaging features of various diseases involving the duodenum, such as developmental, traumatic, inflammatory, infectious, neoplastic, and postsurgical pathologic conditions in alphabetical order, focusing mainly on upper gastrointestinal series (UGIS) and CT but also some radiography, ultrasound, and magnetic resonance (MR) imaging.
PubMed: 34232417
DOI: 10.1186/s13244-021-01045-y -
Journal of Applied Clinical Medical... Jan 2022To investigate annular pancreas in adults using imaging features displayed on computed tomography (CT) and magnetic resonance imaging (MRI).
BACKGROUND AND PURPOSE
To investigate annular pancreas in adults using imaging features displayed on computed tomography (CT) and magnetic resonance imaging (MRI).
METHODS
Retrospective review of annular pancreas in patients undergoing CT or MRI examinations. Two abdominal radiologists blindly reviewed the CT, MRI, and magnetic resonance cholangiopancreatography (MRCP) images from the Picture Archiving and Communication Systems (PACS). A Kruskal-Wallis test was performed to evaluate subjective scoring, with Mann-Whitney test for the comparison. A p-value less than 0.05 was considered statistically significant.
RESULTS
Eleven patients (45.8%) presented a complete ring of pancreatic tissue surrounding duodenum, displayed as circular, triangular, or sandwich sign configuration, the other 13 patients (54.2%) had incomplete annular pancreas which displayed a crocodile jaw appearance, pancreatic tissue extending in a posterolateral or anterolateral direction towards duodenum. When comparing CT images of each sequence, the arterial phase group had the highest score compared with the venous phase and the plain film group (χ = 58.21, p < 0.05). When comparing MRI enhancement volumetric interpolated breath-hold examination (VIBE) sequences, arterial phase group scores were the highest (χ = 18.98, p < 0.05).
CONCLUSIONS
Both CT and MRI could detect annular pancreas, with artery phase being the best sequence to diagnose annular pancreas.
Topics: Adult; Humans; Magnetic Resonance Imaging; Pancreas; Pancreatic Diseases; Retrospective Studies; Tomography, Spiral Computed; Tomography, X-Ray Computed
PubMed: 34845829
DOI: 10.1002/acm2.13487 -
Revista Espanola de Enfermedades... May 2023Annular pancreas is a rare congenital anatomical abnormality of the pancreas. The diagnosis is mainly based on CT and MRI. The development of endoscopic technology...
Annular pancreas is a rare congenital anatomical abnormality of the pancreas. The diagnosis is mainly based on CT and MRI. The development of endoscopic technology provides a new basis for the diagnosis of annular pancreas. We report the images of the diagnosis and classification of annular pancreas under endoscopic ultrasonography(EUS) in our center.
Topics: Adult; Humans; Endosonography; Pancreas; Pancreatic Diseases; Endoscopy; Ultrasonography
PubMed: 36148679
DOI: 10.17235/reed.2022.9103/2022 -
African Journal of Paediatric Surgery :... 2022The annular pancreas is a rare congenital disorder of the pancreas first recognized in 1818. It is believed to result from faulty rotation of the ventral pancreatic bud... (Review)
Review
BACKGROUND
The annular pancreas is a rare congenital disorder of the pancreas first recognized in 1818. It is believed to result from faulty rotation of the ventral pancreatic bud in its course around the posterior aspect of the duodenal anlage. The duodenum is encircled and might be obstructed by normal pancreatic tissue. The management of the annular pancreas is still developing and under revision.
CASE PRESENTATION
Six cases of neonatal intestinal obstruction secondary to the annular pancreas diagnosed, operated on, and involved in our study. Age, gender, maturity, age at referral, birth weight, clinical presentation, imaging findings, associated congenital anomalies, treatment, complications, and hospital stay were all studied. Polyhydramnios is found in 3 cases (50%). Down syndrome was diagnosed in one case. One patient has associated malrotation. Symptoms started earlier within the first 24 hours. Vomiting was bile stained in 4 cases (66.7%). Passing meconium, sometimes frequent, does not exclude the annular pancreas. Most patients show double bubbles sign on plain abdominal X-ray. All six neonates were treated with duodenoduodenostomy with excellent results. Survival was 100% and complications were minimum.
CONCLUSION
The annular pancreas, although rare, is an important cause of neonatal duodenal obstruction. The accurate diagnosis is usually performed during laparotomy. Vomiting may contain bile or not, furthermore, passing meconium does not exclude this condition. The best and the excellent surgical option is diamond duodenoduodenostomy. This case series might be added to the registered cases of the annular pancreas to standardize the method of diagnosis and to define the best management.
Topics: Anastomosis, Surgical; Duodenal Obstruction; Humans; Infant, Newborn; Pancreas; Pancreatic Diseases
PubMed: 35017379
DOI: 10.4103/ajps.AJPS_180_20 -
Polish Journal of Radiology 2020Herein we reviewed the computed tomography (CT) findings of a spectrum of pathological entities affecting the duodenum. We discuss the CT findings of some congenital,... (Review)
Review
Herein we reviewed the computed tomography (CT) findings of a spectrum of pathological entities affecting the duodenum. We discuss the CT findings of some congenital, inflammatory, traumatic, and neoplastic pathologies of the duodenum along with the conventional barium studies of selected conditions. Pathologies of this C-shaped intestinal segment, derived from both foregut and midgut, are often overlooked in clinical practice and radiological literature. While congenital anomalies like duplication cysts and diverticula are usually asymptomatic, annular pancreas and malrotation may manifest in the first decade of life. Primary as well as secondary involvement of the duodenum by various disease processes can be evaluated by careful CT technique and proper attention to the duodenum. Among congenital conditions, annular pancreas, duplication cyst, superior mesenteric artery syndrome, midgut volvulus, and diverticula are presented. Duodenal involvement in adenocarcinoma, lymphoma, gastrointestinal stromal tumours, Crohn's disease, and groove pancreatitis are discussed. Duodenal wall haematoma and traumatic duodenal perforation causing pneumoretroperitoneum in two patients after blunt trauma of the abdomen are also illustrated. CT provides superb anatomic detail and offers high diagnostic specificity for the detection of duodenal pathologies because it allows direct imaging of the intestinal wall, secondary signs of bowel disease within the surrounding mesentery, and abnormal findings in adjacent structures. Primary duodenal malignancies and local extension from adjacent malignancies can be diagnosed by CT reliably. CT also plays a vital role in the diagnosis of traumatic duodenal injury by differentiating between mural haematoma and a duodenal perforation because the latter requires immediate surgical intervention.
PubMed: 32612721
DOI: 10.5114/pjr.2020.95477 -
Cureus Aug 2022Congenital anomalies of the pancreas are relatively uncommon. Most of these are asymptomatic and are detected incidentally, but can present with a variety of clinical...
BACKGROUND AND OBJECTIVE
Congenital anomalies of the pancreas are relatively uncommon. Most of these are asymptomatic and are detected incidentally, but can present with a variety of clinical manifestations like pancreatitis, duodenal obstruction, biliary obstruction, and rarely malignancy. Here in our study, we describe various congenital anomalies of the pancreas associated with various clinical manifestations, its management strategies, and outcomes. The aim was to study the various clinical manifestations of and management strategies for pancreatic diseases associated with congenital anomalies of the pancreas and their outcomes.
METHODS
A retrospective analysis of a prospectively maintained institutional database of 14 patients, admitted over a period of three years from June 2019 to May 2022, who were treated for different clinical manifestations of various congenital anomalies of the pancreas and their outcomes was done at our institution.
RESULTS
The total number of congenital anomalies of the pancreas in our study was 14 out of whom 7 (50%) were males and 7 (50%) females. The mean age of the patients was 37 years. The most common congenital anomaly was pancreatic divisum in six (42.9%) cases. The most common clinical manifestation was acute pancreatitis in four (28.6%) cases. One (7.1%) case was incidentally detected intraoperatively for another condition. Eight (57.1%) patients underwent surgical intervention and six (42.9%) patients were medically managed. Mortality occurred in two (14.3%) cases. Associated alcohol consumption was seen in 2 (14.3%) cases; 10 (71.4%) patients had no comorbidities while 4 (28.6%) patients had diabetes mellitus. Out of eight (57.1%) surgical patients, two (25%) had Clavien-Dindo grade I and one patient (12.5%) grade V complications.
CONCLUSION
Congenital anomalies of the pancreas can be associated with a variety of clinical manifestations; their management strategies and outcomes are no different from patients with the same clinical manifestations with normal pancreatic development.
PubMed: 36110453
DOI: 10.7759/cureus.27915 -
Molecular Genetics & Genomic Medicine Oct 2023Annular pancreas (AP) is a congenital defect of unknown cause in which the pancreas encircles the duodenum. Theories include abnormal migration and rotation of the...
BACKGROUND
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.
METHODS
We conducted exome sequencing in 115 affected infants from the California birth defects registry.
RESULTS
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.
CONCLUSION
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.
PubMed: 37635636
DOI: 10.1002/mgg3.2233 -
Pathologica Feb 2022Congenital anomalies of the liver, biliary tree and pancreas are rare birth defects, some of which are characterized by a marked variation in geographical incidence.... (Review)
Review
Congenital anomalies of the liver, biliary tree and pancreas are rare birth defects, some of which are characterized by a marked variation in geographical incidence. Morphogenesis of the hepatobiliary and pancreatic structures initiates from two tubular endodermal evaginations of the most distal portion of the foregut. The pancreas develops from a larger dorsal and a smaller ventral outpouching; emergence of the two buds will eventually lead to the fusion of the duct system. A small part of the remaining ventral diverticulum divides into a "pars cystica" and "pars hepatica", giving rise to the cystic duct and gallbladder and the liver lobes, respectively. Disruption or malfunctioning of the complex mechanisms leading to the development of liver, gallbladder, biliary tree and pancreas can result in numerous, albeit fortunately relatively rare, congenital anomalies in these organs. The type and severity of anomalies often depend on the exact moment in which disruption or alteration of the embryological mechanisms takes place. Many theories have been brought forward to explain their embryological basis; however, no agreement has yet been reached for most of them. While in some cases pathological evaluation might be more centered on macroscopic evaluation, in other instances small biopsies will be the keystone to understanding organ function and treatment results in the context of congenital anomalies. Thus, knowledge of the existence and histopathological characteristics of some of the more common conditions is mandatory for every pathologist working in the field of gastrointestinal pathology.
Topics: Biliary Tract; Gallbladder; Gastrointestinal Tract; Humans; Liver; Pancreas
PubMed: 35212316
DOI: 10.32074/1591-951X-709