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JGH Open : An Open Access Journal of... Dec 2020Pancreaticobiliary anomalies are rare and often present with cryptic signs and symptoms, thus delaying appropriate treatment.
BACKGROUND AND AIM
Pancreaticobiliary anomalies are rare and often present with cryptic signs and symptoms, thus delaying appropriate treatment.
METHODS
Endoscopic retrograde cholangiopancreatography (ERCP) was used to define pancreaticobiliary anomalies. A retrospective review was performed of 5522 ERCPs conducted at a tertiary care center from 1972 to 2015.
RESULTS
There were 249 (4.5%) patients with pancreaticobiliary anomalies, including 179 patients with pancreas divisum (PD), 44 patients with choledochal cyst (CC) (Todani's classification Type I: extrahepatic cyst 31, Type III; choledochocele 9, Type V: Caroli's disease 4), 20 patients with anomalous pancreaticobiliary ductal union (APDU), and 6 patients with other abnormalities. Of 179 patients with pancreas divisum, 8 (4.5%) required minor sphincterotomies for multiple unexplained acute pancreatitis. Of the 31, 15 (48%) Type I CC patients underwent an operation. In patients with Type III CC (choledochocele), seven of the nine were treated by endoscopic sphincterotomy, and two patients were treated by surgery. Four patients with Type V CC (Caroli's disease) were managed nonoperatively. Of the 20 patients with APDU, 8 (40%) required operative intervention. Six patients were found to have other anomalies: two with pancreas bifidum, one with a duplication of the gallbladder, one with a cystic duct diverticulum, one with an annular pancreas, and one with an abnormal cystic duct origin. These patients were treated based on their etiology.
CONCLUSION
Pancreaticobiliary anomalies are rare and can be defined using ERCP. The appreciation of these abnormalities is important for the proper diagnosis and treatment of these rare biliary and pancreatic disorders.
PubMed: 33319053
DOI: 10.1002/jgh3.12418 -
Frontiers in Genetics 2021The annular pancreas (AP) is a congenital anomaly of the pancreas that can cause acute abdominal pain and vomiting after birth. However, the genetic cause of AP is still...
The annular pancreas (AP) is a congenital anomaly of the pancreas that can cause acute abdominal pain and vomiting after birth. However, the genetic cause of AP is still unknown, and no study has reported AP in patients with 17q12 duplication. This study retrospectively analyzed the next-generation sequencing (NGS) data of individuals from January 2016 to June 2020 for 17q12 duplication. To identify the function of the key gene of in the 17q12 duplication region, human mRNA was microinjected into LiPan zebrafish transgenic embryos. A total of 19 cases of 17q12 duplication were confirmed. AP was diagnosed during exploratory laparotomy in four patients (21.1%). The other common features of 17q12 duplication included intellectual disability (50%), gross motor delay (50%), and seizures/epilepsy (31.58%). The ratio of the abnormal pancreas in zebrafish was significantly higher in the overexpression models. In conclusion, we first reported AP in patients with duplication of the 17q12 region, resulting in the phenotype of 17q12 duplication syndrome. Furthermore, our zebrafish studies verified the role of the gene in pancreatic development.
PubMed: 34025713
DOI: 10.3389/fgene.2021.615072 -
Congenital Short Bowel Syndrome With Annular Pancreas Presenting as Neonatal Intestinal Obstruction.Cureus Nov 2022Congenital short bowel syndrome (CSBS) is a very rare gastrointestinal anomaly of unknown etiology. We report a case of a six-week-old male with CSBS who presented with...
Congenital short bowel syndrome (CSBS) is a very rare gastrointestinal anomaly of unknown etiology. We report a case of a six-week-old male with CSBS who presented with features of intestinal obstruction and failure to thrive. The abdominal radiograph was suggestive of a central gasless abdomen, and a provisional diagnosis of malrotation of the gut with volvulus was considered. On exploration, the duodenum was hugely dilated with annular pancreas, which was not obstructing the duodenum. The length of the intestine was 20 cm from the duodenojejunal junction to the ileocecal junction. Duodenal web was ruled out. Total parenteral nutrition (TNP) was started postoperatively. Early and long-term parenteral nutrition and referral to specialist centers with intestinal rehabilitation programs have improved the overall outcome; however, the challenges are entirely different in developing countries.
PubMed: 36579202
DOI: 10.7759/cureus.31802 -
The Indian Journal of Surgery Jun 2017The postbulbar segment also known as the distal duodenum is a separate clinical entity in terms of the intrinsic pathologies of this region and its varied anatomical...
The postbulbar segment also known as the distal duodenum is a separate clinical entity in terms of the intrinsic pathologies of this region and its varied anatomical relations. The common bile duct and the pancreatic duct open through the major papilla, which marks the beginning of this segment. Distal duodenal obstruction can be defined as a clinicoradiological entity that is characterized by features of gastric outlet obstruction with recurrent bilious vomiting and a radiological evidence of postbulbar obstruction. A Medline search for distal duodenal obstruction revealed 1409 entries, mostly in the form of case reports. In the last 10 years, 310 cases are reported. Clinical features like abdominal pain, nausea, and vomiting are non-specific and quite similar to gastric outlet obstruction. Clinical findings are also non-specific and do not aid in diagnosis. Laboratory findings also do not add much. Contrast-enhanced CT scan and MR enterography are diagnostic modalities of choice. Distal duodenal obstruction is a clinical entity that has a wide list of differential diagnosis which requires due consideration in terms of its management and follow-up. Most of these lesions require adequate workup with the help of a multidisciplinary team consisting of radiologists, gastroenterologists, and pathologists to adequately diagnose and stage the disease before a treatment plan is formulated. A thorough knowledge is a must regarding the treatment options available for each of the conditions so that the management can be personalized leading to better results.
PubMed: 28659679
DOI: 10.1007/s12262-017-1604-4 -
Asian Journal of Surgery Jan 2022
Topics: Foot; Hand; Humans; Pancreas; Pancreatic Diseases
PubMed: 34823993
DOI: 10.1016/j.asjsur.2021.11.035 -
Cureus Mar 2018Portal annular pancreas is a rare congenital anomaly in which the portal vein and/or the splenoportal confluence are completely encircled by aberrant pancreatic...
Portal annular pancreas is a rare congenital anomaly in which the portal vein and/or the splenoportal confluence are completely encircled by aberrant pancreatic parenchyma. It is an asymptomatic condition and is usually an incidental finding. It is, however, important to a surgeon because the postoperative pancreatic fistula (POPF) rates following pancreatic resection are higher in patients with this anomaly. A 47-year-old male presented with features of obstructive jaundice. He was diagnosed to have periampullary carcinoma, and pancreatoduodenectomy was planned. During surgery, uncinate process was seen extending posterior to the portal vein and was communicating with the body of pancreas to the left of the portal vein. After transection, there were two pancreatic stumps. The pancreatic duct was identified in the stump anterior to the portal vein. No duct was present in the posterior pancreatic stump. We closed the posterior pancreatic stump with interrupted polypropylene sutures and performed a duct to mucosa pancreaticojejunostomy in the anterior stump. On reviewing the preoperative computed tomography (CT) scan, we were able to identify the pancreatic tissue encasing the portal vein superior to the splenic vein. Circumportal pancreas is classified based on the orientation of pancreatic duct to the portal vein and the relationship of the aberrant pancreatic tissue with the splenoportal confluence. Following pancreatoduodenectomy, the surgeon has to manage two pancreatic stumps, one anterior and the other posterior to the portal vein. No standardised technique has been described for management of the pancreatic stumps. Every surgeon planning pancreatic surgery should be aware of this rare anomaly, and look for the same in the preoperative CT scan so that appropriate plan can be made regarding the type of pancreatic anastomosis.
PubMed: 29805935
DOI: 10.7759/cureus.2366 -
Pediatric Gastroenterology, Hepatology... May 2020An 18-year-old woman with annular pancreas and duodenal duplication presented with recurrent acute pancreatitis and underwent a resection of duodenal duplication....
An 18-year-old woman with annular pancreas and duodenal duplication presented with recurrent acute pancreatitis and underwent a resection of duodenal duplication. However, the patient experienced recurrent abdominal pain after resection. Abdominal computed tomography and magnetic resonance imaging showed a dilatation of the peripheral pancreatic duct and stenosis and malformation of both the Wirsung's and Santorini's duct due to multiple stones. The modified puestow procedure was performed. The main pancreatic ducts in the body and tail were opened, and the intrapancreatic common bile duct was preserved. A Roux-en-Y pancreatico-jejunostomy was performed for reconstructing the pancreaticobiliary system after removing the ductal protein plug. The patient experienced no abdominal pain, no significant elevation of the serum amylase and lipase levels, and no stone formation during the 2 years of follow-up. This procedure is considered to be beneficial for pediatric patients with chronic pancreatitis due to annular pancreas and duodenal duplication.
PubMed: 32483552
DOI: 10.5223/pghn.2020.23.3.304 -
Case Reports in Gastroenterology 2016The patient was a 56-year-old woman who had experienced epigastralgia and dorsal pain several times over the last 20 years. She was admitted for a diagnosis of acute...
The patient was a 56-year-old woman who had experienced epigastralgia and dorsal pain several times over the last 20 years. She was admitted for a diagnosis of acute cholecystitis, and severe intra- and extrahepatic bile duct dilatation with inner air density was noted. No papilla of Vater was present in the descending duodenum, and 2 small holes were present in the pyloric ring. Bile excretion from one of the small holes was observed under forward-viewing endoscope. It was considered that the pancreatic and bile ducts separately opened into the pyloric ring. Based on these findings, malformation of the pancreaticobiliary duct was diagnosed. She did not wish treatment, but the obstruction associated with duodenal stenosis was noted after 2 years. Pancreatoduodenectomy was performed as curative treatment for duodenal stenosis and retrograde biliary infection through the bile duct opening in the pyloric ring. The ventral pancreas encompassed almost the entire circumference of the pyloric ring, suggesting a subtype of annular pancreas. Generally, lesions are present in the descending part of the duodenum in an annular pancreas, and the pancreatic and bile ducts join in the papillary region. However, in this patient, (1) the pancreas encompassed the pyloric ring, (2) the pancreatic and bile ducts opened separately, and (3) the openings of the pancreatic and bile ducts were present in the pyloric ring. The pancreas and biliary tract develop through a complex process, which may cause various types of malformation of the pancreaticobiliary system, but no similar case report was found on a literature search. This case was very rare and could not be classified in any type of congenital anomaly of the pancreas. We would classify it as a subtype of annular pancreas with separate ectopic opening of the pancreatic and bile ducts into the pyloric ring.
PubMed: 27721721
DOI: 10.1159/000447292 -
International Journal of Clinical and... 2015Annular pancreas is a rare congenital anomaly characterized by pancreatic tissues wrapping completely or incompletely around the descending duodenum. In most patients...
OBJECTIVES
Annular pancreas is a rare congenital anomaly characterized by pancreatic tissues wrapping completely or incompletely around the descending duodenum. In most patients with annular pancreas, onset occurs in early childhood. Adults with annular pancreas are prone to duodenal ulcers and pancreatitis. Intraductal papillary mucinous neoplasm (IPMN) is a type of papillary mucinous secretory epithelial tumor, which originates in the main pancreatic duct and/or branch duct. We report a case of annular pancreas accompanied with intraductal papillary mucinous neoplasm.
METHODS
A 52-year-old male patient hospitalized due to recurrent upper abdominal pain for one and a half years was enrolled in this study.
RESULTS
One case of annular pancreas accompanied with intraductal papillary mucinous neoplasm which manifested as recurrent chronic pancreatitis was found. After pancreaticoduodenectomy, the patient died from uncontrollable gastrointestinal bleeding.
CONCLUSIONS
To the best of our knowledge, this is the first case in China and the second case worldwide of annular pancreas accompanied with IPMN in English literature.
PubMed: 26064223
DOI: No ID Found -
DEN Open Apr 2022Annular pancreas is a congenital abnormality in which part of the pancreatic head completely or partially surrounds the duodenum in a ring-like manner. The condition is...
Annular pancreas is a congenital abnormality in which part of the pancreatic head completely or partially surrounds the duodenum in a ring-like manner. The condition is thought to be an abnormality of the ventral pancreatic bud. While pancreatitis is a common complication of the annular pancreas, its recurrence may be prevented by improving the outflow of pancreatic juice. The present case report describes a 23-year-old woman who had been referred to our hospital for recurrent pancreatitis since childhood. An endoscopic incision was made on the orifice of the annular pancreas, after which pancreatitis of the annular pancreas did not recur for 6 years. The patient subsequently exhibited pancreatolithiasis in the dorsal pancreatic duct, which was successfully treated with endoscopic treatment. Endoscopic pancreatic sphincterotomy may prevent the recurrence of pancreatitis and avoid further surgical interventions by improving the flow of pancreatic juice.
PubMed: 35873513
DOI: 10.1002/deo2.122