Symptomatic adult annular pancreas.
Chen Yi-Cheng,Yeh Chun-Nan,Tseng Jeng-Hwei
Journal of clinical gastroenterology
Annular pancreas is most often found in neonates and infants younger than 1 year of age. Annular pancreas in adults is rare and clinically identifiable when symptomatic. Upper gastrointestinal radiography and surgical confirmation were the most common methods for diagnosing annular pancreas in the 1950s to 1980s. However, the revolution in image modalities has enabled preoperative diagnosis in the last 20 years. Many surgical methods have also been developed, and the one used depends on the presentation and extent of the pancreas annulus. We report two cases of symptomatic adult annular pancreas at Chang Gung Memorial Hospital in Taiwan.
Unusual clinical presentation of annular pancreas in the adult.
Cunha José Eduardo M,de Lima Marcelo Simas,Jukemura José,Penteado Sonia,Jureidini Ricardo,Patzina Rosely A,Siqueira Sheila Aparecida C
Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.]
Annular pancreas (AP) is a rare congenital anomaly, usually present in childhood, with symptoms due to duodenal obstruction; however, this condition can manifest in adulthood with abdominal pain, pancreatitis and pancreatic head mass. The authors present a case of AP observed in a 22-year-old patient that presented an unusual dual-phase clinical manifestation of duodenal obstruction in infancy that was treated by a duodenojejunostomy, and abdominal pain due to chronic pancreatitis in the adult age. MRI with cholangiopancreatography played a decisive role in achieving the correct diagnosis. The patient was treated by a pylorus-preserving Whipple procedure, with resection of the previous duodenojejunostomy. Pancreatic changes characteristic of chronic pancreatitis were demonstrated both in the AP and in the resected pancreatic segment. A marked biliopancreatic ductal anomaly not previously described in the literature was demonstrated by radiologic examination of the surgical specimen. The pathogenesis of AP, the importance of its association with benign and malignant pancreatic disease and the treatment alternatives are discussed by the authors.
10.1159/000084493
Annular pancreas: a review of its molecular embryology, genetic basis and clinical considerations.
Etienne Denzil,John Alana,Menias Christine O,Ward Robert,Tubbs R Shane,Loukas Marios
Annals of anatomy = Anatomischer Anzeiger : official organ of the Anatomische Gesellschaft
The annular pancreas remains an intriguing congenital anomaly. Many theories have sought to explain its embryological basis; however, no consensus has yet been reached regarding the exact mechanism of aberration. More recently, molecular investigations have shed light on some of these theories, confirming the origination of annular tissue from the ventral pancreatic bud. These studies highlight the role of the hedgehog signaling pathway in the development of this anomaly. Overexpression of the ventral-specific gene transmembrane 4 superfamily member 3 (tm4sf3) has also been associated with annular formation. Moreover, isolated case reports of familial annular pancreas have also been documented, suggesting a genetic basis for the development of this anomaly. Annular pancreas can initially present in childhood or adulthood with symptoms of duodenal obstruction and is diagnosed using a variety of imaging modalities. Treatment usually involves surgical correction, with bypass procedures (e.g. duodenoduodenostomy, gastrojejunostomy or duodenojejunostomy) currently favored over annular resection due to complications associated with the latter approach.
10.1016/j.aanat.2012.04.006
Annular pancreas: dramatic differences between children and adults.
Zyromski Nicholas J,Sandoval John A,Pitt Henry A,Ladd Alan P,Fogel Evan L,Mattar Wissam E,Sandrasegaran Kumar,Amrhein David W,Rescorla Fredrick J,Howard Thomas J,Lillemoe Keith D,Grosfeld Jay L
Journal of the American College of Surgeons
BACKGROUND:Annular pancreas is rare; only 737 cases have been reported in the English literature. In addition, no large analysis has compared children and adults. Recently, prenatal diagnosis and advances in imaging have led to increased experience with this condition. STUDY DESIGN:Data from 103 patients (48 children, 55 adults) with annular pancreas, managed from 1992 to 2006, were reviewed. Patients with isolated duodenal atresia, stenosis, or webs were excluded. RESULTS:Median ages at diagnosis were 1 day in children and 47 years in adults. Annular pancreas was more common in girls and women (children, 58%; adults, 69%). Congenital anomalies were more frequent (p < 0.01) in children (71%) than in adults (16%); Down syndrome, cardiac, and intestinal anomalies were most common. Prenatal diagnosis was suspected in 56% of infants, and adults presented with pain (75%), vomiting (24%), pancreatitis (22%), or abnormal liver tests (11%). All children were managed with duodenal bypass. Children were more likely (p < 0.01) to require surgery for associated anomalies. In contrast, adults had fewer duodenal bypass procedures (24%) but more often required endoscopic pancreatobiliary procedures (67%), cholecystectomy (56%), and other pancreatobiliary surgery (20%; p < 0.01). Adults more commonly (p < 0.01) had pancreas divisum (29%) and pancreatobiliary neoplasia (11%). Five children (6%) with multiple anomalies died; all adults survived their operations. Late deaths occurred in 2 children (4%) with multiple anomalies and 3 adults (5%) with pancreatobiliary cancer. CONCLUSIONS:Annular pancreas is associated with a spectrum of disease that differs in children and adults. Congenital anomalies are more common in children with annular pancreas; complex pancreatobiliary disorders and malignancy are more frequent in adults.
10.1016/j.jamcollsurg.2007.12.009
Investigation of annular pancreas through multiple detector spiral CT (MDCT) and MRI.
Zhou Yongxia,Li Xiaoyan
Journal of applied clinical medical physics
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.
10.1002/acm2.13487
Annular pancreas: endoscopic and pancreatographic findings from a tertiary referral ERCP center.
Gromski Mark A,Lehman Glen A,Zyromski Nicholas J,Watkins James L,El Hajj Ihab I,Tan Damien,McHenry Lee,Easler Jeffrey J,Tirkes Temel,Sherman Stuart,Fogel Evan L
Gastrointestinal endoscopy
BACKGROUND AND AIMS:Annular pancreas is a congenital anomaly whereby pancreatic tissue encircles the duodenum. Current knowledge of endoscopic findings of annular pancreas is limited to small case series. The aim of this study was to describe the endoscopic and pancreatographic findings of patients with annular pancreas at a large tertiary care ERCP center. METHODS:This is a retrospective observational study. Our Institutional Review Board-approved, prospectively collected ERCP database was queried for cases of annular pancreas. The electronic medical records were searched for patient and procedure-related data. RESULTS:From January 1, 1994, to December 31, 2016, 46 patients with annular pancreas underwent ERCP at our institution. Index ERCP was technically successful in 42 patients (91.3%), and technical success was achieved in all 46 patients (100%) after 2 attempts, when required. A duodenal narrowing or ring was found in most patients (n = 39, 84.8%), yet only 2 (4.3%) had retained gastric contents. Pancreas divisum was found in 21 patients (45.7%), 18 of which were complete divisum. Pancreatobiliary neoplasia was the indication for ERCP in 7 patients (15.2%). Pancreatographic findings consistent with chronic pancreatitis were noted in 15 patients (32.6%) at the index ERCP. CONCLUSION:This is the largest series describing the endoscopic and pancreatographic findings of patients with annular pancreas. We found that 45.7% of patients had concurrent pancreas divisum. Endoscopic therapy was successful in most patients at our institution after 1 ERCP, and in all patients after a second ERCP. Nearly one-third of patients had findings consistent with chronic pancreatitis at the time of index ERCP. It is unclear whether this may be a feature of the natural history of annular pancreas.
10.1016/j.gie.2018.09.008
The Epidemiology of Annular Pancreas in the United States: A Population-based Study.
Journal of clinical gastroenterology
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.
10.1097/MCG.0000000000001531
Exome sequencing findings in children with annular pancreas.
Molecular genetics & genomic medicine
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.
10.1002/mgg3.2233
Exploring the clinical characteristics and prevalence of the annular pancreas: a meta-analysis.
HPB : the official journal of the International Hepato Pancreato Biliary Association
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.
10.1016/j.hpb.2024.01.006
Annular pancreas and obstructive jaundice.
Benger J R,Thompson M H
The American journal of gastroenterology
Annular pancreas is a rare abnormality which, in some cases, will be an incidental finding. Recently, two case reports have highlighted the possibility that annular pancreas is responsible for extrahepatic biliary obstruction in the absence of pancreatitis. We present a case of obstructive jaundice in which an annular pancreas was noted at both ERCP and CT scanning, but in which the cause of the obstruction was a carcinoma of the ampulla of Vater. Obstructive jaundice should not be attributed to an annular pancreas until the alternative differential diagnoses have been positively excluded.