-
Surgical Oncology Jun 2022Portal annular pancreas (PAP), also called circumportal pancreas, is a congenital pancreatic anomaly in which the portal and/or mesenteric veins are surrounded by...
BACKGROUND
Portal annular pancreas (PAP), also called circumportal pancreas, is a congenital pancreatic anomaly in which the portal and/or mesenteric veins are surrounded by pancreatic parenchyma [1,2]. Joseph et al. classified PAP into three types (according to the fusion pattern of the pancreatic parenchyma and ductal system [1]), each of which they subdivided (based on the relation to the portal confluence) into the suprasplenic, infrasplenic, and mixed type [1,3]. The most common type is IIIa [1,4], where the portal vein (suprasplenic) is encased by the uncinate process with an anteportal main pancreatic duct.
METHODS
The patient was a 78-year-old woman who had undergone left nephrectomy for renal cell carcinoma five years prior. We performed laparoscopic pancreatoduodenectomy for a metastatic tumor of the head of a type IIIa PAP (Fig. 1). The anteportal pancreas was transected, and dissection was performed around the superior mesenteric artery using a right approach. The retroportal pancreas was transected using a linear stapler with bioabsorbable polyglycolic acid felt. We performed pancreatojejunostomy for the anteportal stump of the pancreas containing a main pancreatic duct; the retroportal stump was not reconstructed, because it had no major pancreatic ducts on preoperative imaging.
RESULTS
The operative time was 505 minutes, and the blood loss was 70 ml. The postoperative course was uneventful, and the patients was discharged on postoperative day 12.
CONCLUSION
Laparoscopic pancreatoduodenectomy was performed successfully in a patient with a type IIIa PAP. The retroportal pancreas can be transected using a linear stapler, without reconstruction.
Topics: Aged; Carcinoma, Renal Cell; Female; Humans; Kidney Neoplasms; Laparoscopy; Neoplasms, Second Primary; Pancreas; Pancreatic Diseases; Pancreatic Neoplasms; Pancreaticoduodenectomy; Portal Vein
PubMed: 35462237
DOI: 10.1016/j.suronc.2022.101772 -
Radiology Case Reports Jun 2022VACTERL Syndrome affects multiple body systems and can include various anomalies of the Vertebral column, Anus and/or rectum, heart (Cardiac), Tracheo-Esophagus, kidneys...
VACTERL Syndrome affects multiple body systems and can include various anomalies of the Vertebral column, Anus and/or rectum, heart (Cardiac), Tracheo-Esophagus, kidneys (Renal), and Limbs. Patients with VACTERL syndrome are at increased risk of having a congenital duodenal obstruction that may be extrinsic in the form of an annular pancreas or intrinsic in the form of duodenal atresia, stenosis or web. Simultaneous presentation of both the annular pancreas and duodenal web is a rare clinical entity and typically presents in neonates. However, late presentation of annular pancreas combined with a duodenal web is exceedingly uncommon. We present a case of late diagnosis of annular pancreas with duodenal web resulting in an entrapped ingested foreign body.
PubMed: 35401909
DOI: 10.1016/j.radcr.2022.02.062 -
BMJ Case Reports Feb 2022Duodenal stenosis and atresia are some of the most common forms of congenital bowel obstruction. The gold standard approach to treatment is duodenoduodenostomy, while...
Duodenal stenosis and atresia are some of the most common forms of congenital bowel obstruction. The gold standard approach to treatment is duodenoduodenostomy, while rare, gastrojejunostomy and duodenojejunostomies may still be used. We report a case of a 7-year-old male presenting with annular pancreas with duodenal stenosis that was diagnosed at birth as primary duodenal atresia and repaired by gastrojejunostomy with a Braun enteroenterostomy. Through successful reoperation with dual duodenojejunostomy and subsequent management, we treated the sequelae of the initial repair, including megaduodenum. Regions of duodenal obstruction must have direct anastomotic repair to prevent subsequent issues related to dysmotility. Otherwise, further surgical intervention and long-term medical management, such as the novel strategy reported, may be necessary.
Topics: Child; Duodenal Obstruction; Duodenum; Fetal Diseases; Humans; Infant, Newborn; Intestinal Atresia; Male; Missed Diagnosis; Pancreas; Pancreatic Diseases; Urinary Bladder
PubMed: 35228246
DOI: 10.1136/bcr-2021-248219 -
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 -
Clinical Journal of Gastroenterology Apr 2022The present study aims to report a rare case of obstructive choledocholithiases found in an elderly female with heterotaxy syndrome (HS) with polysplenia, and to review... (Review)
Review
Cholangitis secondary to obstructive choledocholithiases in an elderly woman with heterotaxy syndrome with polysplenia: report of a case and brief review of the literature.
The present study aims to report a rare case of obstructive choledocholithiases found in an elderly female with heterotaxy syndrome (HS) with polysplenia, and to review the available literature on hepatobiliary pathologies associated with this syndrome. We present the case of an 82-year-old female patient with multiple comorbidities who presented to our hospital with abdominal pain and jaundice. An abdominal ultrasound and subsequent computed tomography showed several obstructive choledocholithiases. Incidentally, an abnormal visceral arrangement consistent with HS with polysplenia was observed, including midline liver, semi-annular pancreas, intestinal malrotation, preduodenal portal vein and other vascular anomalies. Accordingly, the patient was diagnosed with cholangitis secondary to obstructive choledocholithiases and HS with polysplenia. She was admitted to the hospital for endoscopic retrograde cholangiopancreatography after medical stabilization. Unfortunately, her condition rapidly worsened and died a few hours after hospitalization due to septic shock. Only one case of choledocholithiases in HS has been previously reported in the literature. It is unclear whether the abnormal arrangement of abdominal organs may have a significant role in the development of this complication. Although biliary drainage might be hindered in the presence of anatomical variants such as (semi-)annular pancreas, intestinal malrotation and preduodenal portal vein, further studies are needed to determine if there is an association between these anomalies and the development of choledocholithasis.
Topics: Aged; Aged, 80 and over; Cholangitis; Digestive System Abnormalities; Female; Heterotaxy Syndrome; Humans; Pancreas; Pancreatic Diseases
PubMed: 35066797
DOI: 10.1007/s12328-021-01574-5 -
African Journal of Paediatric Surgery :... 2022The annular pancreas is a rare congenital disorder of the pancreas first recognized in 1818. It is believed to result from faulty rotation of the ventral pancreatic bud... (Review)
Review
BACKGROUND
The annular pancreas is a rare congenital disorder of the pancreas first recognized in 1818. It is believed to result from faulty rotation of the ventral pancreatic bud in its course around the posterior aspect of the duodenal anlage. The duodenum is encircled and might be obstructed by normal pancreatic tissue. The management of the annular pancreas is still developing and under revision.
CASE PRESENTATION
Six cases of neonatal intestinal obstruction secondary to the annular pancreas diagnosed, operated on, and involved in our study. Age, gender, maturity, age at referral, birth weight, clinical presentation, imaging findings, associated congenital anomalies, treatment, complications, and hospital stay were all studied. Polyhydramnios is found in 3 cases (50%). Down syndrome was diagnosed in one case. One patient has associated malrotation. Symptoms started earlier within the first 24 hours. Vomiting was bile stained in 4 cases (66.7%). Passing meconium, sometimes frequent, does not exclude the annular pancreas. Most patients show double bubbles sign on plain abdominal X-ray. All six neonates were treated with duodenoduodenostomy with excellent results. Survival was 100% and complications were minimum.
CONCLUSION
The annular pancreas, although rare, is an important cause of neonatal duodenal obstruction. The accurate diagnosis is usually performed during laparotomy. Vomiting may contain bile or not, furthermore, passing meconium does not exclude this condition. The best and the excellent surgical option is diamond duodenoduodenostomy. This case series might be added to the registered cases of the annular pancreas to standardize the method of diagnosis and to define the best management.
Topics: Anastomosis, Surgical; Duodenal Obstruction; Humans; Infant, Newborn; Pancreas; Pancreatic Diseases
PubMed: 35017379
DOI: 10.4103/ajps.AJPS_180_20 -
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 -
Acta Medica (Hradec Kralove) 2022The aim of our manuscript is to report of a successful perinatal outcome after treatment of acute polyhydramnios caused by duodenal atresia. A 34-year-old G3P1 was...
The aim of our manuscript is to report of a successful perinatal outcome after treatment of acute polyhydramnios caused by duodenal atresia. A 34-year-old G3P1 was referred due to polyhydramnios in the 30th week of pregnancy. Ultrasound revealed polyhydramnios, amniotic fluid index (AFI) 28, and a double bubble sign that indicated duodenal atresia and dilatated oesophagus. In the 32nd week of gestation, the volume of amniotic fluid increases, AFI 35, along with symptoms of dyspnea and abdominal pain. Due to the clinical picture and the early gestational age, it was decided to perform an amnioreduction. In the 36th week of gestation cesarean section was performed. The baby was taken for exploratory laparotomy and found to have a simultaneous complete duodenal atresia and annular pancreas with associated dilated the first portion of the duodenum and the stomach. A side-to-side duodenoduodenostomy via single-layer hand-sewn anastomosis was performed over a transanastamotic feeding tube (TAFT). The postoperative course was uneventful. Amnioreduction is useful and safe in the treatment of acute polyhydramnios caused by duodenal atresia and thus has a significant role in prolonging gestation until fetal maturity.
Topics: Pregnancy; Humans; Female; Adult; Polyhydramnios; Pregnancy Outcome; Cesarean Section; Amniotic Fluid
PubMed: 36942706
DOI: 10.14712/18059694.2023.6 -
Revista de Gastroenterologia de Mexico... 2022
Topics: Adenocarcinoma; Ampulla of Vater; Humans; Pancreas; Pancreatic Diseases
PubMed: 34969645
DOI: 10.1016/j.rgmxen.2021.12.007 -
Medicine Dec 2021Portal annular pancreas (PAP) is a rare pancreatic anomaly characterized by portal vein encasement in the pancreatic parenchyma. Due to its rarity, PAP may often be...
RATIONALE
Portal annular pancreas (PAP) is a rare pancreatic anomaly characterized by portal vein encasement in the pancreatic parenchyma. Due to its rarity, PAP may often be missed on preoperative computed tomography (CT) review, and surgeons may face challenges in dealing with an unexpected intraoperative encounter with PAP. We documented 2 such intraoperatively diagnosed cases and illustrated their surgical management.
PATIENTS CONCERNS
In case 1, a 70-year-old man was found to have a 15-mm mass in the pancreatic body and dilatation of the peripheral main pancreatic duct on enhanced CT. Case 2 involved a 46-year-old woman with a history of familial adenomatous polyposis, and rectal cancer with a mass in the duodenal papilla.
DIAGNOSES
The patient in case 1 was diagnosed with resectable pancreatic cancer. In case 2, the patient was diagnosed with duodenal papillary carcinoma.
INTERVENTIONS
In case 1, the patient underwent distal pancreatectomy with lymph node dissection. In case 2, the patient underwent pancreaticoduodenectomy. Intraoperatively, PAP was observed in both cases. In case 1, after the usual transection at the right border of the portal vein, an additional dissection was performed on the dorsal pancreas using a powered linear stapler. In case 2, an additional section was made in the pancreatic body caudal to the cricoid pancreatic junction so that the pancreatic cross-section was oriented in 1 plane.
OUTCOMES
The patient in case 1 was discharged without complications. In case 2, although the patient had a grade-B pancreatic fistula (International Study Group of Pancreatic Fistula Classification), the patient recovered conservatively and was discharged without significant complications. In both cases, a retrospective review identified PAP in patients' preoperative CT images.
LESSONS
Both cases required ingenuity during pancreatectomy. Awareness about PAP and its management will enable surgeons to prepare for unexpected encounters with the condition. Moreover, surgeons (especially pancreatic surgeons) should consider the possibility of PAP while managing pancreatic anomalies to make appropriate treatment decisions.
Topics: Aged; Female; Humans; Lymph Node Excision; Male; Middle Aged; Pancreas; Pancreatectomy; Pancreatic Diseases; Pancreatic Fistula; Pancreatic Neoplasms; Pancreaticoduodenectomy; Portal Vein; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 34918681
DOI: 10.1097/MD.0000000000028204