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Annular pancreas is a fairly uncommon condition in which the head of the gland surrounds the second portion of the duodenum with risk of obstruction.

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Encirclement may be complete or incomplete.

Annular pancreas may be associated with duodenal atresia, an anomaly that requires surgery soon after birth.

Such infants infrequently have other congenital anomalies, including trisomy 21 (Down syndrome).

Many patients with annular pancreas do not require surgery in early life but develop symptoms at 60 or 70 years of age.

More commonly, the diagnosis is made incidental to radiologic studies for a duodenal ulcer.

This anomaly is probably due to a failure of the ventral anlage  to migrate behind the duodenum.

Since this migration is due to increased focal growth of the duodenal wall, the annular pancreas reflects impaired development of the duodenum.

Failure of fusion of the rudiments of the pancreas leads to two separate glands, each with its separate duct draining into the duodenum. Persistence of two separate ducts from dorsal and ventral pancreas predisposes to recurrent pancreatitis.

                  

EUS features of annular pancreas (with video).Gastrointest Endosc. 2007 Feb;65(2):340-4.

BACKGROUND: An annular pancreas is a rare congenital anomaly that results in a band of pancreatic tissue, either partially or completely encircling the duodenum. OBJECTIVES: In patients referred for an upper-GI (UGI) EUS (1) to determine the prevalence of an annular pancreas identified by EUS versus CT and (2) to describe the EUS features of an annular pancreas. DESIGN: After review of UGI EUS procedures from January 1, 2000, through June 1, 2006, we conducted a retrospective review of EUS annular pancreas images to identify characteristic EUS features. PATIENTS: This is the largest report describing the EUS findings in 5 patients. RESULTS: Of 9776 patients undergoing UGI EUS, 5 patients (0.05%) (2 men; median age, 64 years; range, 44-69 years) were found to have an annular pancreas. EUS detected a band of pancreatic tissue that encircled the duodenum by 360 degrees (n = 3), 270 degrees (n = 1), or 180 degrees (n = 1). Within this band of tissue, the pancreatic duct was identified in 4 of 5 patients. The ductal and parenchymal features within the annular band were identical to those within the remaining pancreas in all patients. None of the patients had hypoechoic ventral anlage. LIMITATIONS: Whereas, firm EUS criteria of an annular pancreas cannot be established based on 5 patients, certain features may prove useful for diagnosis. CONCLUSIONS: Although a diagnosis of an annular pancreas is rare, it should be considered in situations when a patient with possible associated symptoms has a normal CT.

Ampullary carcinoma associated with an annular pancreas.JOP. 2007 Jan 9;8(1):50-4.

CONTEXT: Annular pancreas is an uncommon congenital abnormality. Co-existence of this condition with a pancreaticobiliary malignancy is an exceptionally rare occurrence. CASE REPORT: We present a case report of a 78-year-old woman with jaundice due to an ampullary carcinoma associated with an annular pancreas treated by pancreaticoduodenectomy. CONCLUSIONS: A collection of previously reported cases is reviewed together with the relevant literature. Obstructive jaundice is an uncommon feature of annular pancreas; hence the possibility of co-existent pancreaticobiliary malignancy should be excluded.

Annular pancreas in identical twin newborns.J Pediatr Surg. 2006 Aug;41(8):e19-21.

Annular pancreas is a rare congenital anomaly occurring in 1 of every 12,000 to 15,000 live births [Nerwich N, Shi E. Neonatal duodenal obstruction: a review of 30 consecutive cases. Pediatr Surg Int 1994;9:47-50]. It may remain asymptomatic throughout life, present in adulthood, or present in infancy as a high intestinal obstruction. Review of the literature demonstrates only 8 cases of familial annular pancreas and no case of twins exhibiting the disease. We will describe a case of identical (monochorionic diamniotic) female twins with neonatal presentation of duodenal obstruction and annular pancreas, and discuss data supportive of a genetic etiology.

Annular pancreas in the adult: management with laparoscopic gastrojejunostomy.Am Surg. 2006 Jan;72(1):71-3.

Annular pancreas is an uncommon congenital anomaly associated with duodenal atresia in neonates. Rarely, the condition may manifest later in life. These symptoms include abdominal pain, nausea, and vomiting and usually arise due to obstruction to gastric emptying. Abdominal CT scan with high resolution and angiography protocol and magnetic resonance imaging are useful in confirming the presence of annular pancreas. Operative management involves bypassing the obstructed duodenum. Duodenoduodenostomy is routinely performed in neonates with annular pancreas. In adults, the duodenum is less mobile, and duodenojejunostomy or gastrojejunostomy are recommended. We report two cases of annular pancreas in adults treated with laparoscopic gastrojejunostomy.

Annular pancreas - a rare cause of gastric obstruction in an 82-year-old patient. Ann R Coll Surg Engl. 2005 Jan;87(1):W13-5.

Annular pancreas is an uncommon and rarely reported congenital anomaly and thus is rarely suspected. A case is reported of an 82-year-old patient who presented with a 3-month history of nausea and vomiting associated with weight loss who at laparotomy was found to have an annular pancreas.

Annular pancreas combined with distal stenosis. A report of four cases and review of the literature.Pediatr Med Chir. 2004 Jul-Aug;26(4):256-9.

BACKGROUND: Congenital duodenal obstruction (CDO) in combination with more distal duodenal obstructions is a rare anomaly occurring in 4% of neonates with duodenal atresia. The experience of two European Pediatric Centers in treatment of congenital double duodenal obstruction (CDDO) is reported and the pertinent literature is reviewed. MATERIALS AND METHODS: During the last 15 years a total of 86 neonates were operated upon for CDO at the department of pediatric surgery of "St. Sophia" Children's Hospital of Athens in Greece and the department of pediatric surgery of "Federico II" Children's University Hospital of Naples in Italy; four of them had a CDDO. These ones presented with nonbilious vomiting and the plain film of the abdomen showed the typical "double bubble". Our cases with CDDO presented annular pancreas causing complete obstruction of the second part of the duodenum and dilatation of the duodenum distal to this obstruction due to an additional congenital stenosis (two cases) or a membranous web (two cases). A diamond shaped duodeno-duodenal (DDD) anastomosis was carried out to relieve the proximal obstruction and a Heinecke-Mikulicz plasty was used to relieve the distal stenosis. RESULTS: All patients with double obstruction underwent successful surgery with no complications. The postoperative course was uneventfuL An upper gastrointestinal barium study at one month postoperatively showed no blind loop, megaduodenum, anastomotic stenosis or malfunction. CONCLUSIONS: a) The combination of duodenal atresia with annular pancreas and distal duodenal stenosis or web is extremely rare. b) Patency of the duodenum distal to the usual obstruction should always be checked in order to avoid misdiagnosis of this combination.

Annular pancreas in two consecutive siblings: an extremely rare case.Eur J Pediatr Surg. 2005 Oct;15(5):364-8.

Annular pancreas is the rare congenital anomaly where the pancreas forms a full or incomplete ring around the second segment of the duodenum, causing various degrees of stenosis or atresia. It is estimated that it appears in 1 out of 12 000-15 000 births of living neonates and until now, in the literature, only 6 cases have been reported among individuals of the same family. We present the case of two siblings, a boy and a girl, with annular pancreas from consecutive pregnancies of the same couple. Both neonates had a prenatal diagnosis of duodenal obstruction and they underwent duodenoduodenal, proximal transverse to distal longitudinal anastomosis. Furthermore, the girl had a mobile ascending colon. Their postoperative condition was perfect. The case we are reporting is an addition to the other 6 cases of familial presentation of annular pancreas and is similar to one of them. In these families, a total of 16 persons present this congenital anomaly while 14 are seemingly healthy. Twelve of the affected persons are female and 4 male. In conclusion, it can be stated that female individuals seem to have a greater propensity to transmit the disease to their descendants, compared to males, suggesting the possible action of an autosomal recessive sex-influenced gene. The recording of such rare family cases should be encouraged, in order to fully recognize a possible type of inherited transmission.

Symptomatic adult annular pancreas: report of two cases and a review of the literature.Hepatobiliary Pancreat Dis Int. 2005 Aug;4(3):468-71.

BACKGROUND: Annular pancreas in adults is a rare embryologic abnormality detected after development of complications. Embryology, diagnosis and treatment strategies for symptomatic adult annular pancreas remain controversial. In this paper we reevaluated these problems in view of the technological and theoretical advances. METHODS: In 2 patients with annular pancreas, one(36-year-old male patient) presenting with duodenal obstruction and duodenal ulcer associated with duodenocolic fistula underwent Billroth II gastrectomy and fistula ectomy and the other(17-year-old male patient) presenting with duodenal obstruction and duodenal ulcer underwent Billroth II gastrectomy. English language literature about annular pancreas etiology, diagnosis and treatment was reviewed. RESULTS: Both of the patients had uneventfully recovered. Abdominal computed tomography, endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography showed typical images of annular pancreas. Duodenal bypass procedure, choledochojejunostomy, endoscopic sphincterotomy or biliary stenting, and pancreatic resection were alternative to treat this sort of anomaly. CONCLUSIONS: Annular pancreas in adults is a rare congenital abnormality, while newer imaging modalities and an index of suspicion may assist in finding more cases. The management of this congenital anomaly should be individualized according to the associated complications.

Islet isolation and transplantation from an annular pancreas: a case report.JOP. 2005 May 10;6(3):274-6.

CONTEXT: Annular pancreas is an uncommon congenital anomaly formed by a thin band of normal pancreatic tissue encircling the duodenum. CASE REPORT: We report the first case of an islet isolation and transplantation from an annular pancreas. The pancreas together with duodenum was procured from a 32-year-old previously healthy man after diagnosis of brain death. The pancreas including the annular portion was distended well after intraductal collagenase perfusion. A total of 276,064 islet equivalent was recovered and transplanted into a type 1 diabetic patient. CONCLUSIONS: Bearing in mind the shortage of donors, patients with this anomalous condition should not be excluded as potential organ donors.

A case of annular pancreas in a male adult.Fukushima J Med Sci. 2004 Dec;50(2):75-81.

Annular pancreas is a rare congenital anomaly, which consists of a ring of pancreatic tissue partially or completely encircling the descending portion of the duodenum. We reported a case of symptomatic annular pancreas in a 40 year old man admitted to our hospital complaining of abdominal pain, nausea and vomiting without body weight loss in January 2000. The patient underwent laparoscopic cholecystectomy for acalculous cholecystitis in September 1996. Initially, he was diagnosed with duodenal stenosis due to a duodenal ulcer scar, but laboratory data showed no abnormalities. His symptoms did not improve with medication or endoscopic balloon dilatation. Duodenograpy revealed a narrow segment with a smooth mucosal surface in the 2nd portion of the duodenal loop in the duodenum, and a computed tomography (CT) scan demonstrated a thickened pancreas head around this narrow segment. We were therefore able to diagnose annular pancreas. A duodeno-duodenostomy was performed in March 2000. The patient's postoperative course was uneventful, and he was discharged from our hospital on the 19th postoperative day. Although define diagnosis of annular pancreas is frequently made at laparotomy, the development of a recurrent imaging modality might assist in the preoperative diagnosis.

Unusual clinical presentation of annular pancreas in the adult. Pancreatology. 2005;5(1):81-5. Epub 2005 Mar 16.

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.

Annular pancreas in children: a recent decade's experience.J Pediatr Surg. 2004 Nov;39(11):1654-7.

PURPOSE: Annular pancreas is a recognized cause of duodenal obstruction in children. The authors sought to characterize the clinical, radiologic, and prognostic findings in this disorder through a 10-year review of all patients with annular pancreas. METHODS: A retrospective review of all annular pancreas cases identified between 1993 and 2002 at 2 tertiary pediatric surgical centers was completed. Factors analyzed included patient demographics, presenting symptoms, associated anomalies, radiologic findings, operation performed, postoperative outcomes, and complications. RESULTS: Sixteen patients were identified. Four patients (25%) were premature. Twelve patients (75%) presented during the first week of life and the remainder within the first year. All patients presented with vomiting, which was nonbilious in 15 (94%). Five patients (31 %) had chromosomal anomalies, and 6 (38%) had other major congenital malformations. Eleven patients (69%) presented with partial duodenal obstruction. Operations performed were duodenoduodenostomy in 14 (88%), duodenojejunostomy in 1 (6%), and Ladd's procedure in 1 (6%). Enteral feedings were started, and full enteral feeding was achieved at an average of 8.4 days and 15.9 days after operation, respectively. All patients survived and were discharged after an average hospitalization of 24.0 days. CONCLUSIONS: Patients with annular pancreas have preampullary duodenal obstruction, which is more commonly partial. Duodenoduodenostomy is the appropriate treatment. Prognosis is excellent, despite the frequent association with chromosomal anomalies and major congenital malformations.

Symptomatic annular pancreas in newborns.Med Sci Monit. 2002 Jun;8(6):CR434-7.

BACKGROUND: The pancreas exhibits various types of anomalies, including aplasia, dysplasia, pancreatic cysts, duplication, and ectopia, of which the most common is annular pancreas. This study describes the characteristic features of 7 cases of annular pancreas diagnosed during exploration.MATERIAL/METHODS: Seven newborns undergoing surgery for annular pancreas from 1990 to 1998 were analyzed. The cases were evaluated according to birth weight, sex, symptoms, methods of diagnosis, associated anomalies, and surgical treatment modalities.RESULTS: The mean birth weight of the patients was 2385 +/- 1002 g, the mean gestational age was 37.5 +/- 3.1 weeks. Four patients (42.8%) had associated anomalies, including intestinal malrotation (42.8%), intrinsic duodenal obstruction (28.5%), trisomy 21 karyotype (14.2%), cardiac malformation (14.2%), and Meckel's diverticulum (14.2%). Surgical treatment included duodenoduodenostomy (DD) in four patients and DD plus tapering enteroplasty (TE) plus placement of transanastomotic jejunal tube (TJT) in three patients.CONCLUSIONS: Infants with annular pancreas associated with duodenal obstruction are generally premature or small for their gestational age. The symptoms observed in annular pancreas are related not only to extrinsic compression of the ectopic tissue, but also the duodenal stenosis associated with this malformation. Annular pancreas is most commonly associated with intestinal malrotation. It does not correlate as strongly with trisomy 21 karyotype as do the duodenal atresias, and oral feeding tolerance time is nearly the same between the DD and DD+TE+TJT groups.

An annular pancreas derived from paired ventral pancreata, supporting Baldwin's hypothesis.Pancreas. 2000 May;20(4):408-10.

An annular pancreas is a rare malformation. It is generally accepted that the ring formation originates from a single ventral pancreas, as suggested by Lecco. No reports of resected and/or autopsied annular pancreata derived from paired ventral pancreata, thus supporting Baldwin's hypothesis, have been published. We describe an annular pancreas originating from paired ventral pancreata, thus supporting Baldwin's hypothesis, and attempt to clarify the pathogenesis of an annular pancreas. The patient was a 1-day-old Japanese male newborn, born at 32 weeks of pregnancy. He died from respiratory failure owing to esophageal atresia the next day. Autopsy incidentally revealed an annular pancreas that was examined histologically. Multiple 4 microm thick serial sections were obtained from paraffin-embedded pancreatic tissue. Sections for histologic analysis were subjected to hematoxylin-eosin staining and pancreatic polypeptide immunostaining. An unusually large pancreatic duct encircled by pancreatic tissue ran around the duodenum, and the duct was confirmed to flow into the major papilla. The islets of the encircling pancreas were positive for pancreatic polypeptide. A normal main and accessory pancreatic duct were also identified. Histologic and immunohistochemical evaluation revealed that the ring formation originated from the left lobe of paired ventral pancreata. An annular pancreas that was investigated histopathologically and immunohistochemically and found to support Baldwin's hypothesis is described.

Annular pancreas associated with pancreatolithiasis: a case report. Hepatogastroenterology. 1999 Jan-Feb;46(25):527-31.

We present a case of annular pancreas associated with pancreatolithiasis. A 41 year-old Japanese man with epigastric pain was admitted to the surgical service at Miyazaki Medical College Hospital. Contrast duodenography revealed severe stenosis of the descending duodenum. Cholangiography showed a stenotic segment of the intrapancreatic common bile duct surrounded by calcifications. Computed tomography of the abdomen revealed calcifications in the posterior region of the pancreatic head. Percutaneous cannulation of the pancreatic ductal system, using ultrasonic guidance, demonstrated a slightly dilated pancreatic duct in the body, stenosis of the duct of Wirsung in the pancreatic head, a normal duct of Santorini, and calcifications in the duct of an annular pancreas which communicated with the duct of Wirsung. At surgery, the second portion of the duodenum was completely encircled by the annular pancreas, and a Whipple procedure was performed. Including this patient, 170 adult cases of annular pancreas have been reported in Japan since 1922. Surgery was performed on 122 patients; 106 of these procedures were well documented. A Whipple procedure was performed on 16 patients, including the present case. Nine of these 16 patients had associated malignant disease, while the others had benign pancreatic disease. This is the fifth reported case of pancreatolithiasis associated with an annular pancreas in Japan. This case emphasizes that an annular pancreas may predispose to localized chronic pancreatitis and pancreatolithiasis.


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Annular pancreas with obstructive jaundice: beware of underlying neoplasm.Pancreas. 2002 Oct;25(3):314-6.

INTRODUCTION: Annular pancreas is a rare congenital abnormality, and in adult patients it presents with clinical features that differ from those seen in newborns. Features in the adult patient include peptic ulceration, duodenal obstruction, acute pancreatitis, and obstructive jaundice. Treatment strategies for annular pancreas with obstructive jaundice remain controversial. AIM: To present three cases involving adult patients with annular pancreas and obstructive jaundice, due to carcinoma of the ampulla of Vater in two patients and chronic pancreatitis in the third. METHODOLOGY AND RESULTS: Pancreaticoduodenectomy was performed on all patients, and the postoperative courses were uneventful. CONCLUSION: Our experience suggests that for adult patients with annular pancreas presenting with obstructive jaundice, it is necessary to consider the possibility of associated or coexisting periampullary malignancy.

A new embryologic hypothesis of annular pancreas. Hepato gastroenterology. 2001 Jan-Feb;48(37):277-8.

Annular pancreas is a developmental anomaly of the pancreas. There are two major hypotheses concerning development of the annular pancreas from the ventral pancreatic anlage; adhesion of the right ventral anlage to the duodenal wall (Lecco's theory), and persistence of the left ventral anlage (Baldwin's theory) reported in 1910, but each theory has some problems and can account for only a few types of annular pancreas. We report a new embryologic hypothesis of annular pancreas which can account for the developmental mechanism of three types of arrangement of annular ducts. The tip of the left ventral anlage adheres to the duodenum and stretches to form a ring. Whether the tip is proximal or distal to the bile duct creates several arrangements of the annular duct.

Annular pancreas in adults: embryological development, morphology and clinical significance.Surg Radiol Anat. 2001;23(6):437-42.

Three cases (two male, one female) of annular pancreas are presented, which were found and operated on within the last three decades. Diagnosis was made using imaging techniques, especially ultrasonography and computed tomography. In two cases there was incomplete obstruction, while in the other obstruction was complete. It is characteristic that in the case of complete obstruction the annular portion of the pancreas attached to a circular band of connective tissue, while in the two cases of incomplete obstruction there was a complete ring of pancreatic tissue. In all cases surgical intervention was needed in order to create a bypass. In the first case gastro enterostomy and truncal vagotomy was performed, in the second latero-lateral duodeno-jejunostomy and in the third latero-lateral antropyloroduodeno-jejunostomy. The embryology and morphology of the annular pancreas, the morphology of its duct system, diagnosis, differential diagnosis and the surgical treatment of this rare condition are reviewed.

Annular pancreas in adults: diagnostic considerations on a case.G Chir. 2000 May;21(5):243-7.

Annular Pancreas (AP) is a rare congenital anomaly that usually presents in childhood with symptoms referable to duodenal obstruction; nonetheless, this condition can manifest in adulthood with abdominal pain, pancreatitis, duodenal ulcer, pancreatic head mass. The Authors hereby discuss a case of AP observed in a 63 year-old patient in which EUS played a decisive role in achieving a certain diagnosis.