-
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 -
Acta Medica Okayama Feb 2023An annular pancreas is a rare anomaly of the pancreas, defined as pancreatic tissue that totally or partly encircles the duodenum, usually the descending portion. A...
An annular pancreas is a rare anomaly of the pancreas, defined as pancreatic tissue that totally or partly encircles the duodenum, usually the descending portion. A 76-year-old man who was diagnosed with gastric cancer cT3N0M0 Stage IIB underwent laparoscopic distal gastrectomy with D2 lymph node dissection. Intraoperatively, the dorsal half of the duodenal bulb was seen to be half surrounded by the pancreas, and a non-typical annular pancreas was diagnosed. Because of the risk to the pancreas, it was considered impossible to perform anastomosis by a linear stapler as in the usual laparoscopic procedure. Therefore, we performed laparoscopically assisted distal gastrectomy and Billroth-I reconstruction using a circular stapler, and the surgery was completed without difficulties. His postoperative course was good despite the development of a pancreatic fistula, which was an International Study Group for Pancreas Fistula biochemical leak. Some APs can be diagnosed preoperatively, but the rarer subtypes such as ours are more difficult to visualize on imaging. In gastrectomy, it is both oncologically important and technically challenging to perform lymph node dissection around the pancreas. In this case with an especially proximal pancreas, a circular stapler was considered better suited for gastroduodenal anastomosis and required a broader field than that afforded by laparoscopy. A case of non-typical annular pancreas diagnosed during laparoscopic gastric surgery is described.
Topics: Male; Humans; Aged; Laparoscopy; Pancreas; Gastrectomy; Stomach Neoplasms
PubMed: 36849152
DOI: 10.18926/AMO/64368 -
The Indian Journal of Surgery Oct 2016Carcinoma of the ampulla of Vater associated with annular pancreas is a rare entity. Only seven cases have been reported so far in the English literature. We herein...
Carcinoma of the ampulla of Vater associated with annular pancreas is a rare entity. Only seven cases have been reported so far in the English literature. We herein report one such case in a 42-year-lady who presented with progressively increasing jaundice and pain in the upper abdomen for past 2 months. A magnetic resonance cholangiopancreaticography (MRCP) and MRI revealed an annular pancreas with an ampullary mass and a dilated CBD. An upper gastrointestinal endoscopic biopsy from the ampullary mass revealed a moderately differentiated adenocarcinoma. She was diagnosed as having annular pancreas with periampullary cancer that was subsequently confirmed at laparotomy. Histological examination confirmed a complete annular pancreas with a 2 cm × 2 cm moderately differentiated adenocarcinoma of the ampulla of Vater. The management, however, remains as in any case of periampullary malignancy. This highlights the importance that obstructive jaundice in an adult patient presenting with annular pancreas may be associated with a coexisting periampullary malignancy.
PubMed: 27994339
DOI: 10.1007/s12262-016-1529-3 -
Radiology Case Reports Jan 2024Congenital duodenal obstruction is a common cause of bowel obstruction. It is relatively easy to diagnose in the neonatal period. However, if the obstruction is due to a...
Congenital duodenal obstruction is a common cause of bowel obstruction. It is relatively easy to diagnose in the neonatal period. However, if the obstruction is due to a duodenal diaphragm, diagnosis may be delayed until later in infancy or even adulthood, depending on the size of the aperture in the diaphragm. Congenital duodenal obstruction may be associated with other gastrointestinal and biliary anomalies. The association of a duodenal diaphragm and a partial annular pancreas is a rare clinical entity. We present an unusual case of late presentation of duodenal diaphragm with partial annular pancreas in a 10-year-old girl with a 3-month history of abdominal distension and vomiting. The plain abdominal X-ray showed the classic picture of a double bubble. The CT images showed narrowing of the third duodenal portion and dilatation of the stomach and duodenum. The pancreatic tissue incompletely surrounded the second part of the duodenum. Intraoperatively, both the duodenal diaphragm and the partial annular pancreas were confirmed. Excision of the diaphragm and duodenoplasty were performed. A duodenal diaphragm should be suspected in patients with a history of abdominal distension and vomiting, even in late childhood. Associated gastrointestinal and biliary anomalies should always be excluded.
PubMed: 38028306
DOI: 10.1016/j.radcr.2023.10.003 -
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 -
Endoscopy Dec 2023
Topics: Humans; Pancreas Divisum; Pancreas; Pancreatic Diseases; Cholangiopancreatography, Endoscopic Retrograde; Pancreatic Ducts
PubMed: 36958350
DOI: 10.1055/a-2032-3462 -
Frontiers in Pediatrics 2022Primary ciliary dyskinesia (PCD) is a rare genetic disorder characterized by motile ciliary dysfunction and impaired ultrastructure. Despite numerous studies, the...
Primary ciliary dyskinesia (PCD) is a rare genetic disorder characterized by motile ciliary dysfunction and impaired ultrastructure. Despite numerous studies, the genetic basis for about 30% of PCD cases remains to be elucidated. Here, we present the identification and functional analysis of two novel mutations in the gene encoding coiled-coil domain-containing protein 40 (CCDC40), which are found in a familial case of PCD. These novel mutations, NM_017950.4: c.2236-2delA and c.2042_2046delTCACA, NP_060420.2: p.(Ile681fs), were identified by whole-exome sequencing (WES). Sanger sequencing was then performed to confirm the WES results and determine the gene sequences of the proband's parents. The c.2042_2046delTCACA mutation disrupts the reading frame of the protein and is therefore predicted to produce a non-functional protein. Using a minigene assay with the pcDNA3.1(+) plasmid, we further investigated the potential pathogenic effects of the c.2236-2delA mutation and found that this mutation leads to formation of a truncated protein splicing disruption. Thus, in summary, we identified two mutations of the gene that can be considered pathogenic compound heterozygous mutations in a case of familial PCD, thereby expanding the known mutational spectrum of the gene in this disease.
PubMed: 36245716
DOI: 10.3389/fped.2022.996332 -
Medicine Dec 2017Portal annular pancreas is a rare anatomic variation, where the uncinated process of the pancreas connects with the dorsal pancreas and the pancreas tissue encases the...
Radical antegrade modular pancreatosplenectomy for adenocarcinomaof the body of the pancreas in a patient with portal annular pancreas, aberrant hepatic artery, and absence of the celiac trunk: A case report.
RATIONALE
Portal annular pancreas is a rare anatomic variation, where the uncinated process of the pancreas connects with the dorsal pancreas and the pancreas tissue encases the portal vein (PV), superior mesenteric vein (SMV) or splenic vein (SV). Malignancies are quite uncommon in the patients, who have an annular pancreas especially portal annular pancreas. Ectopic common hepatic artery and absence of the celiac trunk (CT) are the other infrequent abnormalities.
PATIENT CONCERNS
A 74-year-old man suffered from upper abdominal and back pain.
DIAGNOSES AND INTERVENTIONS
Contrast enhanced computed tomography indicated a low-density mass in the body of the pancreas. Pathological report showed adenocarcinoma of the body of pancreas after radical antegrade modular pancreatosplenectomy (RAMPS).
OUTCOMES
In the operation, we found the superior vein and portal vein was surrounded by the pancreatic tissue. The left gastric artery and splenic artery originated respectively from abdominal aorta, and celiac trunk was not viewed. In addition, the common hepatic artery was a branch from the superior mesenteric artery.
LESSONS
In general, this is a novel clinical case of pancreatic carcinoma happening in the portal annular pancreas which was accompanied with aberrant hepatic artery and absence of the celiac trunk at the same time. Confronted with the pancreatic neoplasms, the possibility of coexistent annular pancreas and arterial variations should be considered.
Topics: Adenocarcinoma; Aged; Celiac Artery; Contrast Media; Hepatic Artery; Humans; Male; Pancreas; Pancreatectomy; Pancreatic Neoplasms; Portal Vein; Splenectomy; Tomography, X-Ray Computed
PubMed: 29310347
DOI: 10.1097/MD.0000000000008738 -
Radiologia Brasileira 2019
PubMed: 31435094
DOI: 10.1590/0100-3984.2017.0196 -
Journal of Surgical Case Reports Jul 2023The annular pancreas (AP) is an uncommon congenital anomaly, characterised by a circumferential envelope in the second portion of the duodenum. In recent years, some...
The annular pancreas (AP) is an uncommon congenital anomaly, characterised by a circumferential envelope in the second portion of the duodenum. In recent years, some genetic component has been found in the etiology. A newborn full-term male, weighing at 1910 g at birth, had a history of intrauterine growth restriction and diagnosis of tetralogy of Fallot, Down syndrome and congenital hypothyroidism. Duodenal membrane is suspected after persistent postprandial vomiting and abdominal distension; his abdomen was distended, hyperresonant and soft. The gastroduodenal series showed data compatible with a duodenal membrane so exploratory laparotomy was performed, finding the pancreas completely wrapping the second portion of the duodenum, so a diamond-shaped-duodenoduodenostomy anastomosis was performed. The AP should be considered, especially in male neonates with postprandial vomiting, abdominal distension, who show some other congenital anomaly, and in the abdominal X-ray, the sign of the double bubble is observed.
PubMed: 37489161
DOI: 10.1093/jscr/rjad407