-
Journal of Clinical Gastroenterology Feb 2022Annular pancreas is a rare congenital condition where the second part of the duodenum is encircled by pancreatic tissue. There is a scarcity of data on its natural...
BACKGROUND AND AIMS
Annular pancreas is a rare congenital condition where the second part of the duodenum is encircled by pancreatic tissue. There is a scarcity of data on its natural history therefore, we aimed to evaluate the epidemiology of annular pancreas and identify underlying associations using a large database.
METHODS
A multi-institutional database (Explorys) was surveyed. A cohort of patients with a Systematized Nomenclature of Medicine-Clinical Terms diagnosis of "MRI, CT, EUS and/or ERCP" between April 2015 and April 2020 was identified. Subsequently a cohort of patients with history of "annular pancreas" was identified.
RESULTS
There were a total of 40,075,980 individuals in the database with 6,162,600 (15.38%) who had an magnetic resonance imaging, computed tomography, endoscopic retrograde cholangiopancreatography, and/or endoscopic ultrasound. There were 210 (3.4/100,000) had a diagnosis of annular pancreas. When compared with the control group, patients with annular pancreas were more likely to have a history of alcohol use, smoking, acute pancreatitis, chronic pancreatitis, gastritis, duodenitis, jaundice, and gastrointestinal outlet obstruction.
CONCLUSIONS
This is the largest study evaluating the epidemiology of annular pancreas. We found the estimated prevalence rate of annular pancreas to be 3.4/100,000 in those who had abdominal imaging. Annular pancreas was more likely to be diagnosed in patients with symptoms pertaining to acute or chronic pancreatitis, biliary, and gastric outlet obstruction.
Topics: Acute Disease; Cholangiopancreatography, Endoscopic Retrograde; Humans; Pancreas; Pancreatic Diseases; Pancreatitis; United States
PubMed: 33769394
DOI: 10.1097/MCG.0000000000001531 -
Radiology Jun 2023
Topics: Humans; Tomography, X-Ray Computed; Pancreatic Diseases; Pancreas; Imaging, Three-Dimensional
PubMed: 37158717
DOI: 10.1148/radiol.230010 -
HPB : the Official Journal of the... Apr 2024The annular pancreas (AP) is a rare gastrointestinal congenital malformation, in which malrotation of the pancreatic ventral bud in the seventh week of embryonic... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
The annular pancreas (AP) is a rare gastrointestinal congenital malformation, in which malrotation of the pancreatic ventral bud in the seventh week of embryonic development manifests in a partial or complete ring of tissue around the second part of the duodenum.
METHODS
The main online medical databases such as PubMed, ScienceDirect, Wiley online library, Web of Science, and EBSCO discovery service were used to gather all relevant studies on the AP.
RESULTS
A total of 12,729,118 patients were analyzed in relation to the prevalence of AP. The pooled prevalence of AP was 0.0045% (95% CI: 0.0021%-0.0077%). The most frequent comorbidity in adults and children was duodenal obstruction, with a pooled prevalence of 24.04% and 52.58%, respectively (95% CI: 6.86%-46.48% and 35.56%-69.31%, respectively). The most frequent operation in adult patients with AP was duodenojejunostomy, with pooled prevalence established at 3.62% (95% CI: 0.00%-10.74%).
CONCLUSION
The diagnostic complexity of AP is accentuated by its nonspecific clinical symptoms, making accurate identification reliant on imaging studies. Therefore, having a thorough knowledge of the clinical characteristics of the AP and its associated anomalies becomes paramount when faced with this rare congenital condition.
Topics: Adult; Child; Humans; Prevalence; Pancreas; Pancreatic Diseases; Duodenal Obstruction; Digestive System Abnormalities
PubMed: 38310080
DOI: 10.1016/j.hpb.2024.01.006 -
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 -
JPMA. the Journal of the Pakistan... Dec 2019Circumportal annular pancreas (CAP) also known as portal annular pancreas (PAP) is an uncommon pancreatic anatomic variant in which normal pancreatic tissue completely...
Circumportal annular pancreas (CAP) also known as portal annular pancreas (PAP) is an uncommon pancreatic anatomic variant in which normal pancreatic tissue completely surrounds the portal vein and can be mistaken for mass of pancreatic head. We present here a case of a 65 years old woman who was a diagnosed case of endometrial carcinoma, underwent CT scan for further metastatic workup which revealed this rare pancreatic variant.
Topics: Aged; Female; Humans; Pancreas; Pancreatic Diseases; Tomography, X-Ray Computed
PubMed: 31853133
DOI: No ID Found -
Annals of Surgical Oncology Sep 2023Portal Annular Pancreas (PAP) is a relatively uncommon entity with 4% reported incidence. Pancreaticoduodenectomy is challenging in cases with PAP and is associated with...
BACKGROUND
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.
METHODS
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.
RESULTS
Patient had a smooth intraoperative course & the postoperative recovery was also uneventful. Pathology reported pT3 duodenal cancer with negative margins and uninvolved lymph nodes.
CONCLUSION
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.
Topics: Humans; Pancreaticoduodenectomy; Pancreatic Fistula; Pancreas; Anastomosis, Surgical; Postoperative Complications; Pancreatic Hormones; Pancreatic Neoplasms; Portal Vein
PubMed: 37402974
DOI: 10.1245/s10434-023-13782-z -
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 -
Khirurgiia 2023Portal annular pancreas is a little-known anatomical variant of annular pancreas. In these patients, pancreatic parenchyma encircles the portal vein annularly. This...
Portal annular pancreas is a little-known anatomical variant of annular pancreas. In these patients, pancreatic parenchyma encircles the portal vein annularly. This anomaly is associated with high risk of postoperative pancreatic fistula in pancreatic surgery. Considering small incidence of anomaly and characteristics of surgery, we describe laparoscopic distal pancreatectomy with preservation of spleen and splenic vessels in a patient with solid pseudopapillary tumor combined with portal annular pancreas. A 33-year-old woman underwent laparoscopic surgery for cystic-solid pancreatic tumor. Spleen-sparing distal pancreatectomy was performed. Portal annular pancreas was visualized intraoperatively and confirmed retrospectively after analysis of MR data. Ventral and dorsal parts of portal annular pancreas were transected using stapler device. Pancreatic fistula developed in postoperative period. The patient was discharged after 6 days with a drainage tube. Surgeons need to be aware of portal annular pancreas. This anomaly increases the risk of postoperative fistula. Transection of ventral and dorsal parts of annular pancreas using stapler device is the most acceptable option to reduce the risk of postoperative fistula.
Topics: Female; Humans; Adult; Pancreatectomy; Pancreatic Fistula; Retrospective Studies; Laparoscopy; Postoperative Complications
PubMed: 37313708
DOI: 10.17116/hirurgia2023061108 -
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 -
Abdominal Radiology (New York) Jul 2023Given its crucial location at the crossroads of the gastrointestinal tract, the hepatobiliary system and the splanchnic vessels, the duodenum can be affected by a wide... (Review)
Review
Given its crucial location at the crossroads of the gastrointestinal tract, the hepatobiliary system and the splanchnic vessels, the duodenum can be affected by a wide spectrum of abnormalities. Computed tomography and magnetic resonance imaging, in conjunction with endoscopy, are often performed to evaluate these conditions, and several duodenal pathologies can be identified on fluoroscopic studies. Since many conditions affecting this organ are asymptomatic, the role of imaging cannot be overemphasized. In this article we will review the imaging features of many conditions affecting the duodenum, focusing on cross-sectional imaging studies, including congenital malformations, such as annular pancreas and intestinal malrotation; vascular pathologies, such as superior mesenteric artery syndrome; inflammatory and infectious conditions; trauma; neoplasms and iatrogenic complications. Because of the complexity of the duodenum, familiarity with the duodenal anatomy and physiology as well as the imaging features of the plethora of conditions affecting this organ is crucial to differentiate those conditions that could be managed medically from the ones that require intervention.
Topics: Humans; Duodenum; Tomography, X-Ray Computed; Magnetic Resonance Imaging; Neoplasms; Pancreatic Diseases
PubMed: 37099183
DOI: 10.1007/s00261-023-03909-x