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International Journal of Surgery Case... 2020Pancreatitis is a dire clinical diagnosis with variable presentation in the paediatric population. Moreover, neonatal pancreatitis has been rarely reported in the...
INTRODUCTION
Pancreatitis is a dire clinical diagnosis with variable presentation in the paediatric population. Moreover, neonatal pancreatitis has been rarely reported in the English literature.
PRESENTATION OF CASE
A newborn, product of a poorly supervised, pre-term gestation with pre-natally diagnosed intestinal obstruction, and post-natal clinical features of jaundice, vomiting, abdominal distension, aphonation and suspected chromosomal abnormalities. There was maternal hepatitis which was untreated. Diagnosed as duodenal atresia, the baby was investigated, resuscitated and had surgery. Intra-operative findings were of an omental bubble, duodenal stenosis with annular pancreas, coagulative necrosis of the pancreas and multiple intra peritoneal cheesy deposits. Following an unfortunate demise, autopsy confirmed pancreatitis and multiple congenital abnormalities.
DISCUSSION
Paediatric caregivers should be aware of the possibility of neonatal pancreatitis in jaundiced newborns with intestinal obstruction especially with a background of maternal viraemia.
CONCLUSION
A constellation of unusual presentations as highlighted could be a pointer to an emerging syndrome. All paediatric caregivers should entertain a high index of suspicion of pancreatitis in such a case, investigate and expedite appropriate interventions to prevent mortality.
PubMed: 33395874
DOI: 10.1016/j.ijscr.2020.11.114 -
Korean Journal of Transplantation Dec 2020Biliary atresia (BA)-polysplenia syndrome (PS) is diagnosed in a small proportion of BA patients. We present a case of split liver transplantation (LT) successfully...
Biliary atresia (BA)-polysplenia syndrome (PS) is diagnosed in a small proportion of BA patients. We present a case of split liver transplantation (LT) successfully performed in a pediatric recipient with BA-PS. The recipient was 29-month-old boy who underwent Kasai procedure. The coexisting malformations included agenesis of the inferior vena cava with azygos vein continuation, polysplenia, intestinal malrotation, truncated pancreas, and preduodenal portal vein and annular pancreas. With pediatric end-stage liver disease score of 33, the patient was allocated for split LT. Under this condition, the left lateral section graft was equivalent to a graft-recipient weight ratio of 2.6%. The recipient surgery was performed according to the standard procedures of pediatric LT. The graft hepatic vein was directly anastomosed with the suprahepatic confluence of the recipient hepatic veins. An external iliac vein homograft was interposed for portal vein reconstruction. Portal collateral veins were embolized intraoperatively to secure portal vein inflow. No surgical complications were developed. Currently, the patient has been doing well for 4 years after transplantation. Our pediatric patient with BA-PS had various anatomical malformations. Thorough preoperative and intraoperative assessment of these anomalies, adoption of customized reconstruction techniques of LT, and careful posttransplant monitoring are necessary for successful LT.
PubMed: 35770105
DOI: 10.4285/kjt.20.0023 -
World Journal of Clinical Cases Nov 2020Annular pancreas (AP) is a rare congenital abnormal rotation of the pancreas. AP rarely occurs in adults. Pancreatic tumors and ampullary tumors are related to AP, so...
BACKGROUND
Annular pancreas (AP) is a rare congenital abnormal rotation of the pancreas. AP rarely occurs in adults. Pancreatic tumors and ampullary tumors are related to AP, so the discovery and treatment of AP are essential.
CASE SUMMARY
This study investigated the clinical manifestations, imaging features, complications, and treatment of six patients diagnosed with AP at the Department of Hepatobiliary and Pancreatic Surgery, First Hospital of Jilin University from January 2010 to June 2020. There were four males and two females, with an average age of 56.00 ± 9.86 years old. In this study, abdominal pain and jaundice were the main clinical manifestations. Imaging can show the "crocodile jaw sign" or "double bubble sign".
CONCLUSION
For patients with duodenal or biliary obstruction, physicians should give priority to AP when imaging examinations suggest that the duodenum is wrapped with tissue similar to the density of the pancreas. Symptomatic patients should actively undergo surgical treatment.
PubMed: 33344566
DOI: 10.12998/wjcc.v8.i22.5722 -
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 -
JGH Open : An Open Access Journal of... Oct 2020Annular pancreas is a rare entity. It clinically presents with intestinal obstruction. We present a case of a young female with incomplete annular pancreas presented...
Annular pancreas is a rare entity. It clinically presents with intestinal obstruction. We present a case of a young female with incomplete annular pancreas presented with recurrent acute pancreatitis.
PubMed: 33102782
DOI: 10.1002/jgh3.12391 -
Journal of Radiology Case Reports Mar 2020The underlying etiologies of paediatric bowel obstruction are wide ranging. It can be divided into proximal and distal bowel obstruction. Amongst the different...
The underlying etiologies of paediatric bowel obstruction are wide ranging. It can be divided into proximal and distal bowel obstruction. Amongst the different etiologies of the proximal bowel obstructions at the level of the duodenum, there are a few etiologies including duodenal atresia, internal hernias, intestinal malrotation, annular pancreas etc. Superior mesenteric artery syndrome is amongst one of these differential diagnoses which is more prevalent in the adolescent age group. We describe the imaging features of this entity and its demographics, imaging characteristics, treatment and prognosis.
Topics: Adolescent; Diagnosis, Differential; Duodenal Obstruction; Duodenum; Humans; Intestinal Atresia; Superior Mesenteric Artery Syndrome
PubMed: 33082917
DOI: 10.3941/jrcr.v14i3.3830 -
Children (Basel, Switzerland) Aug 2020The concomitant occurrence of duodenal atresia (DA) and a choledochal cyst (CC) has rarely been reported. Knowledge of both the presentation and management of this rare...
The concomitant occurrence of duodenal atresia (DA) and a choledochal cyst (CC) has rarely been reported. Knowledge of both the presentation and management of this rare co-occurrence is imperative in avoiding potential complications and sequelae, such as biliary metaplasia. Herein we describe a female infant born at 32 weeks gestational age who was diagnosed with duodenal atresia and annular pancreas postnatally, who had subsequent findings of malrotation and a choledochal cyst, as seen from contrast imaging. Uncomplicated repair of the DA and obstruction was performed at 4 days of life. She re-presented 2 years later with non-bloody, nonbilious emesis and was found to have elevated amylase, lipase and liver enzymes. Imaging revealed dilated intra-hepatic ducts, a distended gallbladder and a large choledochal cyst. She underwent a cholecystostomy tube placement followed by a definitive choledochal cyst excision with immediate improvement following surgery and full resolution of symptoms before discharge.
PubMed: 32824642
DOI: 10.3390/children7080099 -
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 -
Polish Journal of Radiology 2020Herein we reviewed the computed tomography (CT) findings of a spectrum of pathological entities affecting the duodenum. We discuss the CT findings of some congenital,... (Review)
Review
Herein we reviewed the computed tomography (CT) findings of a spectrum of pathological entities affecting the duodenum. We discuss the CT findings of some congenital, inflammatory, traumatic, and neoplastic pathologies of the duodenum along with the conventional barium studies of selected conditions. Pathologies of this C-shaped intestinal segment, derived from both foregut and midgut, are often overlooked in clinical practice and radiological literature. While congenital anomalies like duplication cysts and diverticula are usually asymptomatic, annular pancreas and malrotation may manifest in the first decade of life. Primary as well as secondary involvement of the duodenum by various disease processes can be evaluated by careful CT technique and proper attention to the duodenum. Among congenital conditions, annular pancreas, duplication cyst, superior mesenteric artery syndrome, midgut volvulus, and diverticula are presented. Duodenal involvement in adenocarcinoma, lymphoma, gastrointestinal stromal tumours, Crohn's disease, and groove pancreatitis are discussed. Duodenal wall haematoma and traumatic duodenal perforation causing pneumoretroperitoneum in two patients after blunt trauma of the abdomen are also illustrated. CT provides superb anatomic detail and offers high diagnostic specificity for the detection of duodenal pathologies because it allows direct imaging of the intestinal wall, secondary signs of bowel disease within the surrounding mesentery, and abnormal findings in adjacent structures. Primary duodenal malignancies and local extension from adjacent malignancies can be diagnosed by CT reliably. CT also plays a vital role in the diagnosis of traumatic duodenal injury by differentiating between mural haematoma and a duodenal perforation because the latter requires immediate surgical intervention.
PubMed: 32612721
DOI: 10.5114/pjr.2020.95477 -
The American Journal of Case Reports Jun 2020BACKGROUND Heterotaxy is a syndrome of abnormal arrangement of the internal thoracic-abdominal structures across the left-right axis of the body. It is a primary...
BACKGROUND Heterotaxy is a syndrome of abnormal arrangement of the internal thoracic-abdominal structures across the left-right axis of the body. It is a primary disorder with 2 main settings - bilateral left sidedness (polysplenia syndrome) or right sidedness (asplenia syndrome) - although some overlapping or uncertainties may occur. Patients with right heterotaxy typically present with asplenia, complex heart disease, and other thoracoabdominal organ situs abnormalities. CASE REPORT We present a unique case of congenital asplenia syndrome with complex heart disease, annular pancreas, and other extra-heterotaxic anomalies (e.g., musculoskeletal) in the form of a radius aplasia and partial syndactyly of the thumb and index finger of the left hand. These associated anomalies have not been reported before. CONCLUSIONS This case shows the need for paying increased attention to the implications of other extracardiac anomalies that can be associated with heterotaxy syndrome.
Topics: Female; Heart Defects, Congenital; Heterotaxy Syndrome; Humans; Infant, Newborn; Intensive Care Units; Limb Deformities, Congenital; Pancreas; Pancreatic Diseases; Spleen
PubMed: 32491997
DOI: 10.12659/AJCR.923341