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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 -
Journal of Medical Ultrasonics (2001) Apr 2022This study aimed to evaluate the diagnostic performance of ultrasound to locate atresia/stenosis and other abdominal anomalies in pediatric patients with duodenal...
The diagnostic accuracy of ultrasound and upper gastrointestinal contrast studies for locating atresia/stenosis and intestinal malrotation and detecting annular pancreas in pediatric patients with duodenal atresia/stenosis.
PURPOSE
This study aimed to evaluate the diagnostic performance of ultrasound to locate atresia/stenosis and other abdominal anomalies in pediatric patients with duodenal atresia/stenosis, including intestinal malrotation and annular pancreas.
METHODS
We classified 36 retrospective cases of duodenal atresia/stenosis based on intestinal malrotation status, and evaluated the diagnostic performance of ultrasound/upper gastrointestinal contrast studies to locate atresia/stenosis and intestinal malrotation, as well as ultrasound for detecting additional anomalies such as annular pancreas. The incidence of annular pancreas was compared between groups using Fisher's exact test.
RESULTS
Atresia/stenosis was correctly located by ultrasound in 33 (91.7%) cases and by upper gastrointestinal contrast study in 36 (100%) cases. Of the eight cases with intestinal malrotation, five and two were correctly diagnosed by ultrasound and upper gastrointestinal contrast study, respectively. Ultrasound correctly diagnosed annular pancreas in 6/14 cases. The incidence of annular pancreas was significantly different between the groups (present/absent in groups with vs. without intestinal malrotation: 6/2 vs. 8/20, P = 0.036).
CONCLUSIONS
Ultrasound has a relatively high capability in locating atresia/stenosis. However, some cases are misdiagnosed. In clinical practice, upper gastrointestinal contrast studies should be used complementarily during diagnosis. Additional anomalies may not be detected by preoperative examinations; therefore, surgeons should carefully evaluate for additional anomalies during surgery, especially coexisting intestinal malrotation and annular pancreas.
Topics: Child; Constriction, Pathologic; Contrast Media; Digestive System Abnormalities; Duodenal Obstruction; Humans; Intestinal Atresia; Intestinal Volvulus; Pancreas; Pancreatic Diseases; Retrospective Studies
PubMed: 34988762
DOI: 10.1007/s10396-021-01176-1 -
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 -
Journal of Minimal Access Surgery 2020Annular pancreas is a rare, congenital, rotational anomaly of pancreas, seen usually in newborns who present with features of duodenal obstruction. However, in adults,...
BACKGROUND
Annular pancreas is a rare, congenital, rotational anomaly of pancreas, seen usually in newborns who present with features of duodenal obstruction. However, in adults, only 24% of cases are present with duodenal obstruction. Surgery remains the procedure of choice in patients in whom symptoms can be attributed to duodenal obstruction and the goal of surgery is to relieve obstruction by bypassing the annulus. Laparoscopic Roux-en Y duodenostomy (DJ) is our preferred bypass approach for this condition. Literature search revealed that very few case reports have been published about laparoscopic management of annular pancreas, especially about duodenojejunal anastomosis. We present our experience in the laparoscopic management of symptomatic annular pancreas in adults and technique of the laparoscopic Roux-en Y DJ for annular pancreas.
MATERIALS AND METHODS
Between 1996 and 2016, a total of six adult patients underwent laparoscopic management for symptomatic annular pancreas. The demographic, perioperative and follow-up details were documented.
RESULTS
All surgeries were successfully performed by laparoscopic approach with no conversion to open. Five cases underwent Roux-en Y DJ and one underwent gastrojejunostomy. No major perioperative events occurred. The mean length of hospital stay was 5.6 days. Five out of six patients were followed up for 24 months, and no symptom recurrence was seen.
CONCLUSION
Laparoscopic Roux-en Y duodenojejunostomy could be used as a safe and physiological treatment for annular pancreas in adult patients and should be preferred for the treatment of duodenal obstruction due to annular pancreas.
PubMed: 30618433
DOI: 10.4103/jmas.JMAS_245_18 -
Gene Expression Patterns : GEP Sep 2021The pancreas development depends on complex regulation of several signaling pathways, including the Hedgehog (Hh) signaling via a receptor complex component, Smoothened,...
The pancreas development depends on complex regulation of several signaling pathways, including the Hedgehog (Hh) signaling via a receptor complex component, Smoothened, which deficiency blocks the Hh signaling. Such a defect in birds and mammals results in an annular pancreas. We showed that in developing zebrafish, the mutation of Smoothened or inhibition of Hh signaling by its antagonist cyclopamine caused developmental defects of internal organs, liver, pancreas, and gut. In particular, the pancreatic primordium was duplicated. The two exocrine pancreatic primordia surround the gut. This phenomenon correlates with a significant reduction of the gut's diameter, causing the annular pancreas phenotype.
Topics: Animals; Hedgehog Proteins; Pancreas; Signal Transduction; Smoothened Receptor; Zebrafish; Zebrafish Proteins
PubMed: 34087472
DOI: 10.1016/j.gep.2021.119185 -
BMC Pregnancy and Childbirth Jul 2020The purpose of this research is to summarize the prenatal ultrasound characteristics of congenital duodenal obstruction (CDO), especially in the diagnosis of duodenal...
BACKGROUND
The purpose of this research is to summarize the prenatal ultrasound characteristics of congenital duodenal obstruction (CDO), especially in the diagnosis of duodenal diaphragm and annular pancreas. At present, few researchers have summarized the specific ultrasound features of duodenal diaphragm and annular pancreas.
METHODS
In this study, a retrospective analysis of 40 patients diagnosed with CDO between January 2016 and December 2019 was carried out. Data on the diagnosis, ultrasound images and outcomes of the patients were gathered, and the features of the patients were analyzed.
RESULTS
The results showed that there were 17 patients (42.5%) of congenital duodenal diaphragm, all with a 'rat tail' sign on the ultrasound images. Moreover, there were 4 patients (10.0%) of CDO caused by annular pancreas, all with a 'pliers' sign on the ultrasound images. We summarized the imaging features of the 'rat tail' sign and the 'pliers' sign.
CONCLUSION
The main conclusion of this study was that the 'rat tail' sign could be used as an indirect ultrasound feature to diagnose duodenal diaphragm. The 'pliers' sign could be used as a direct ultrasound feature in the diagnosis of annular pancreas in CDO.
Topics: Adult; Duodenal Obstruction; Female; Gestational Age; Humans; Male; Pancreas; Pancreatic Diseases; Pregnancy; Retrospective Studies; Ultrasonography, Prenatal
PubMed: 32620086
DOI: 10.1186/s12884-020-03078-5 -
The Korean Journal of Gastroenterology... Apr 2022Annular pancreas is a rare congenital anomaly in which a thin band of pancreatic tissue partially or completely surrounds the duodenum. It is challenging to diagnose due...
Annular pancreas is a rare congenital anomaly in which a thin band of pancreatic tissue partially or completely surrounds the duodenum. It is challenging to diagnose due to its variable clinical presentation. Approximately two-thirds of patients have no symptoms in their lifetime, and most symptomatic cases are seen in neonates and infants. Symptomatic adult patients present with upper gastrointestinal symptoms, such as epigastric pain, vomiting, and postprandial fullness associated with gastric outlet obstruction. Complications associated with annular pancreas include peptic ulcer disease, pancreatitis, pancreatic head carcinoma, and biliary obstruction. Annular pancreas is also a rare cause of upper gastrointestinal bleeding in adults, but it should be considered as one of the differential diagnoses in patients presenting with a peptic ulcer and duodenal stricture. Here, we report the case of a 60-year-old man who presented with melena and was subsequently diagnosed with an annular pancreas.
Topics: Adult; Gastrointestinal Hemorrhage; Humans; Infant, Newborn; Male; Middle Aged; Pancreas; Pancreatic Diseases; Pancreatic Neoplasms; Peptic Ulcer
PubMed: 35473777
DOI: 10.4166/kjg.2022.012 -
Pancreas Apr 2021Portal annular pancreas (PAP) is a rare congenital pancreatic anomaly, in which the uncinate process of the pancreas fuses to the body of pancreas behind the portal... (Review)
Review
Portal annular pancreas (PAP) is a rare congenital pancreatic anomaly, in which the uncinate process of the pancreas fuses to the body of pancreas behind the portal vein. Here, we report a case of PAP with common hepatic arterial anomaly, which was identified during surgery. A 57-year-old man who had branch type intraductal papillary mucinous neoplasm in the head of the pancreas developed a nodule in the cystic lesion. We planned pylorus preserving pancreaticoduodenectomy. The common hepatic artery from the celiac artery passing behind the portal vein was revealed in preoperative examinations. During surgery, we discovered that the uncinate process of the pancreas was fused with the body of the pancreas behind the portal vein. We divided the pancreas at the anterior and posterior of the portal vein. The main pancreatic duct was present in the anterior pancreatic stump. We performed pancreaticojejunostomy in the anterior stump and closed the posterior stump by interrupted suture. Forty-four surgical cases of PAP have been reported in the English medical literature. There are few previous reports of PAP which involved an arterial anomaly. Clinicians should consider PAP preoperatively to ensure that the surgeon can appropriately plan pancreatic resection to avoid postoperative complications.
Topics: Hepatic Artery; Humans; Male; Middle Aged; Pancreas; Pancreatic Diseases; Pancreatic Neoplasms; Pancreaticoduodenectomy; Portal Vein
PubMed: 33939680
DOI: 10.1097/MPA.0000000000001766 -
Cureus Mar 2023One of the main causes of proximal bowel obstruction in neonates is congenital duodenal obstruction. It can be grouped by intrinsic and extrinsic factors and the...
One of the main causes of proximal bowel obstruction in neonates is congenital duodenal obstruction. It can be grouped by intrinsic and extrinsic factors and the presentation may differ depending on whether the obstruction is complete or partial. The intrinsic factors include duodenal atresia, duodenal stenosis, or duodenal web. The extrinsic factors include malrotation with Ladd's band, annular pancreas, anterior portal vein, and duodenal duplication. Malrotation may present with or without midgut volvulus. We are sharing a rare presentation of congenital duodenal obstruction with combined intrinsic and extrinsic causes, namely, duodenal stenosis with gastrointestinal malrotation in a neonate. The patient underwent successful exploratory laparotomy, corrective Kimura's procedure (duodenostomy), Ladd's procedure, and appendicectomy. Early recognition of signs and symptoms, prompt corrective surgery, and adequate optimization of metabolic components post-operatively are important to determine the decreased morbidity and mortality of neonates.
PubMed: 37065346
DOI: 10.7759/cureus.36137 -
World Journal of Gastroenterology Jul 2019Congenital duodenal obstruction (CDO) can be complete (CCDO) or incomplete (ICDO). To date there is no outcome analysis available that compares both subtypes. (Comparative Study)
Comparative Study
BACKGROUND
Congenital duodenal obstruction (CDO) can be complete (CCDO) or incomplete (ICDO). To date there is no outcome analysis available that compares both subtypes.
AIM
To quantify and compare the association between CCDO and ICDO with outcome parameters.
METHODS
We retrospectively reviewed all patients who underwent operative repair of CCDO or ICDO in our tertiary care institution between January 2004 and January 2017. The demographics, clinical presentation, preoperative diagnostics and postoperative outcomes of 50 patients were compared between CCDO ( = 27; atresia type 1-3, annular pancreas) and ICDO ( = 23; annular pancreas, web, Ladd´s bands).
RESULTS
In total, 50 patients who underwent CDO repair were enrolled and followed for a median of 5.2 and 3.9 years (CCDO and ICDO, resp.). CCDO was associated with a significantly higher prenatal ultrasonographic detection rate (88% versus 4%; CCDO ICDO, < 0.01), lower gestational age at birth, lower age and weight at operation, higher rate of associated congenital heart disease (CHD), more extensive preoperative radiologic diagnostics, higher morbidity according to Clavien-Dindo classification and comprehensive complication index (all ≤ 0.01). The subgroup analysis of patients without CHD and prematurity showed a longer time from operation to the initiation of enteral feeds in the CCDO group ( < 0.01).
CONCLUSION
CCDO and ICDO differ with regard to prenatal detection rate, gestational age, age and weight at operation, rate of associated CHD, preoperative diagnostics and morbidity. The degree of CDO in mature patients without CHD influences the postoperative initiation of enteral feeding.
Topics: Age Factors; Child; Child, Preschool; Digestive System Surgical Procedures; Duodenal Obstruction; Duodenum; Enteral Nutrition; Female; Gestational Age; Humans; Infant; Infant, Low Birth Weight; Infant, Newborn; Infant, Premature; Laparoscopy; Male; Postoperative Period; Retrospective Studies; Treatment Outcome
PubMed: 31391773
DOI: 10.3748/wjg.v25.i28.3787