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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 -
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 -
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 -
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 -
South Dakota Medicine : the Journal of... Feb 2019Annular pancreas is an uncommon congenital cause of gastric outlet obstruction. The incidence is usually referenced at between five and 15 per 100,000 based on autopsy... (Review)
Review
Annular pancreas is an uncommon congenital cause of gastric outlet obstruction. The incidence is usually referenced at between five and 15 per 100,000 based on autopsy series. When present, this rare condition surfaces with symptoms in the pediatric population during the first few months of life. An adult presenting with symptoms of gastric outlet obstruction due to annular pancreas is an unusual incident. This case describes gastric outlet obstruction due to a partial annular pancreatic band in an otherwise healthy 32-year-old male. Given the scarcity of this pathological process in adults; no specific guidelines exist about the management of this condition. Continued reporting of this pathology is essential for development of such guidelines. Literature review, embryology and treatment options will be discussed.
Topics: Adult; Gastric Outlet Obstruction; Humans; Male; Pancreas; Pancreatic Diseases
PubMed: 30855730
DOI: No ID Found -
Korean Journal of Radiology 2015This pictorial review aims to illustrate the magnetic resonance imaging (MRI) findings and presentation patterns of anatomical variations and various benign and... (Review)
Review
This pictorial review aims to illustrate the magnetic resonance imaging (MRI) findings and presentation patterns of anatomical variations and various benign and malignant pathologies of the duodenum, including sphincter contraction, major papilla variation, prominent papilla, diverticulum, annular pancreas, duplication cysts, choledochocele, duodenal wall thickening secondary to acute pancreatitis, postbulbar stenosis, celiac disease, fistula, choledochoduodenostomy, external compression, polyps, Peutz-Jeghers syndrome, ampullary carcinoma and adenocarcinoma. MRI is a useful imaging tool for demonstrating duodenal pathology and its anatomic relationships with adjacent organs, which is critical for establishing correct diagnosis and planning appropriate treatment, especially for surgery.
Topics: Ampulla of Vater; Choledochal Cyst; Diverticulum; Duodenal Diseases; Duodenum; Humans; Magnetic Resonance Imaging; Pancreas; Pancreatic Diseases; Radiography
PubMed: 26576112
DOI: 10.3348/kjr.2015.16.6.1240 -
Journal of Gastrointestinal Surgery :... May 2021Portal annular pancreas (PAP) is an anatomic variation due to aberrant fusion of the ventral and dorsal pancreatic buds around the portal vein. In this article, we... (Review)
Review
INTRODUCTION
Portal annular pancreas (PAP) is an anatomic variation due to aberrant fusion of the ventral and dorsal pancreatic buds around the portal vein. In this article, we present a case report with a systematic review of literature of patients undergoing major pancreatic surgery with associated PAP. We also intend to discuss and suggest possible surgical strategies to minimise major postoperative complications.
METHODS
A systematic literature search was conducted using the terms "circumportal," "periportal," "pancreas," "annular pancreas," "portal annular pancreas" and "pancreas anomaly." All articles describing portal annular pancreas with surgical resection were included.
RESULTS
We identified a total of 53 patients of PAP from 29 articles, who underwent pancreatic resection with a median age of 65 years. POPF (postoperative pancreatic fistula) was demonstrated in 42.55% of patients and 34% had CR (clinically relevant)-POPF. Following pancreaticoduodenectomy, pancreatic stump was reconstructed in all patients with either pancreaticojejunostomy or pancreaticogastrostomy. Standard line of pancreatic transection, i.e., division of anteportal portion at the pancreatic neck and stapling of the retroportal process, resulted in 71% incidence of CR-POPF, whereas it was only 16% when extended resection was performed to achieve single pancreatic stump and 12.5% when retroportal portion was sutured or ligated. Amongst distal pancreatic resections, 66% had POPF and 33% developed CR-POPF.
CONCLUSION
It is of utmost importance for pancreatic surgeons to diligently look for and identify PAP in the preoperative imaging. Additional imaging in the form of MRCP helps to define abnormal pancreatic ductal anatomy. Surgeons need to be cognisant of pancreatic stump management in patients with PAP to reduce associated higher rates of POPF.
Topics: Aged; Humans; Pancreas; Pancreatic Diseases; Pancreatic Fistula; Pancreaticoduodenectomy; Pancreaticojejunostomy; Postoperative Complications
PubMed: 33555524
DOI: 10.1007/s11605-021-04927-0