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Radiology Case Reports Mar 2023Annular pancreas is an uncommon congenital anomaly which is a rare cause of congenital duodenal obstruction. It is normally identified during the neonatal period, but...
Annular pancreas is an uncommon congenital anomaly which is a rare cause of congenital duodenal obstruction. It is normally identified during the neonatal period, but may also be identified in adolescence or adulthood. This diagnosis is often overlooked in adult patients who present with symptoms suggestive of duodenal obstruction. We present a case of AP detected in a 23-year-old man, with complaints of continuous vomiting and abdominal discomfort over the last 6 months. An upper gastrointestinal study revealed a constricted second part of the duodenum. A computed tomography scan revealed a complete ring of pancreatic tissue around the second part of the duodenum. Diagnostic and therapeutic surgery decompresses the external obstruction. The patient had an early post-operative activation. No specific guidelines and protocols exist about the management of such cases. Given the rarity of this congenital anomaly, presenting with chronic partial duodenal obstruction, and its successful surgical treatment, have prompted us to report the case along with a brief review of literature about the subject.
PubMed: 36747589
DOI: 10.1016/j.radcr.2022.11.083 -
African Journal of Paediatric Surgery :... 2023Annular pancreas (AP) is a congenital anomaly and a recognised cause of duodenal obstruction which can affect all age groups. It may manifest early in the neonatal...
Annular pancreas (AP) is a congenital anomaly and a recognised cause of duodenal obstruction which can affect all age groups. It may manifest early in the neonatal period, but it may also have varied and often delayed symptoms. We report the case of AP in an 18-month-old girl with a long history of recurrent post-prandial non-bilious vomiting treated for a long time with the clinical diagnosis of gastroesophageal reflux. Upper gastrointestinal study was suggestive of partial duodenal obstruction and computed tomography revealed a complete ring of pancreatic parenchyma surrounding the second part of the duodenum. Diamond-shaped duodenoduodenostomy was achieved successfully and the post-operative period was uneventful. Although rare, AP must be kept in mind of any paediatric surgeon while confronted to symptoms of partial duodenal obstruction.
Topics: Female; Infant, Newborn; Humans; Child; Infant; Duodenal Obstruction; Duodenum; Pancreas; Vomiting
PubMed: 36722572
DOI: 10.4103/ajps.ajps_86_21 -
Congenital Short Bowel Syndrome With Annular Pancreas Presenting as Neonatal Intestinal Obstruction.Cureus Nov 2022Congenital short bowel syndrome (CSBS) is a very rare gastrointestinal anomaly of unknown etiology. We report a case of a six-week-old male with CSBS who presented with...
Congenital short bowel syndrome (CSBS) is a very rare gastrointestinal anomaly of unknown etiology. We report a case of a six-week-old male with CSBS who presented with features of intestinal obstruction and failure to thrive. The abdominal radiograph was suggestive of a central gasless abdomen, and a provisional diagnosis of malrotation of the gut with volvulus was considered. On exploration, the duodenum was hugely dilated with annular pancreas, which was not obstructing the duodenum. The length of the intestine was 20 cm from the duodenojejunal junction to the ileocecal junction. Duodenal web was ruled out. Total parenteral nutrition (TNP) was started postoperatively. Early and long-term parenteral nutrition and referral to specialist centers with intestinal rehabilitation programs have improved the overall outcome; however, the challenges are entirely different in developing countries.
PubMed: 36579202
DOI: 10.7759/cureus.31802 -
Revista Medica Del Instituto Mexicano... Jan 2023The presence of duodenal atresia related to type IIIb intestinal atresia is a rare association, with few cases reported in the literature, representing a surgical...
BACKGROUND
The presence of duodenal atresia related to type IIIb intestinal atresia is a rare association, with few cases reported in the literature, representing a surgical challenge considering that even isolated cases of type IIIb intestinal atresia are a challenge. The objective was to report the successful surgical management of a case of a complex intestinal malformation, characterized by duodenal occlusion secondary to annular pancreas and type IIIb intestinal atresia, with intestinal malrotation by definition and the presence of Meckel's diverticulum.
CLINICAL CASE
We present the case report of a newborn sent to the second level of care with a diagnosis of duodenal obstruction not diagnosed prenatally, which resulted in duodenal atresia due to annular pancreas and type IIIb intestinal atresia according to the Grosfeld classification. The presence of duodenal atresia with type IIIb intestinal atresia is an extremely rare condition, even more so associated with annular pancreas. These cases are a challenge considering the short length of the small intestine and its consequent need for total parenteral nutrition for a prolonged period.
CONCLUSIONS
The surgical management of this complex intestinal malformation resulted in a case with an adequate post-surgical evolution, based on the immediate start of enteral feeding with a short period of need for total parenteral nutrition that finally resulted in a short hospital stay.
Topics: Infant, Newborn; Humans; Duodenal Obstruction; Intestinal Atresia; Pancreas
PubMed: 36542807
DOI: No ID Found -
International Journal of Surgery Case... Dec 2022Annular pancreas is an uncommon congenital abnormality characterized by a ring of pancreatic tissue around the duodenum descending part. It is exceptionally rare in...
INTRODUCTION
Annular pancreas is an uncommon congenital abnormality characterized by a ring of pancreatic tissue around the duodenum descending part. It is exceptionally rare in adults and is increasingly detected after the investigation of symptoms caused by its consequences, which include recurrent pancreatitis, duodenal stenosis at the site of the annulus, or duodenal or gastric ulcers. In this study, we present a case report of symptomatic annular pancreas in an adult patient.
PRESENTATION OF CASE
A 23-year-old woman hospitalized for epigastric pain and recurrent vomiting. Radiological examination was consistent with an annular pancreas. At operation, a partial obstruction of the second part of the duodenum was found, caused by an annular pancreas. No other congenital anomaly of the intra-abdominal organs was noted. A gastrojejunostomy was performed and she was discharged after 8 days with good recovery.
CONCLUSION
Because annular pancreas in adults is a rare clinical scenario, it should be included in the differential diagnosis of adult patients with gastrointestinal obstructive symptoms for a prolonged period of time, given that surgical intervention has a reliable outcome.
PubMed: 36455345
DOI: 10.1016/j.ijscr.2022.107804 -
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 -
Radiology Case Reports Nov 2022Our purpose is to illustrate the radiological aspects of the annular pancreas as an etiology of duodenal obstruction in infants. We report the case of a 4-month-old...
Our purpose is to illustrate the radiological aspects of the annular pancreas as an etiology of duodenal obstruction in infants. We report the case of a 4-month-old girl, who was admitted to our department with postprandial vomiting evolving since birth. The initial examination found a severely dehydrated patient. Abdominal ultrasound showed gross dilatation of the stomach and duodenum, it also showed pancreatic tissue surrounding the duodenum, suggesting a diagnosis of annular pancreas as the cause of the duodenal obstruction. Post-contrast abdominal CT showed the gastric and duodenal dilatation, and a ring of pancreatic tissue surrounding uncompletly the second portion of the duodenum. The patient underwent a bypass surgery which consisted in a duodeno-duodenostomy with simple post-operative follow-up and no recurrence of digestive symptoms. Annular pancreas is a rare pathology to be sought in neonatal obstruction. A good knowledge of radiological semiology is essential for a good diagnostic approach. However, surgery is the only effective way to diagnose and treat this pathology.
PubMed: 36188077
DOI: 10.1016/j.radcr.2022.08.063 -
Revista Espanola de Enfermedades... May 2023Annular pancreas is a rare congenital anatomical abnormality of the pancreas. The diagnosis is mainly based on CT and MRI. The development of endoscopic technology...
Annular pancreas is a rare congenital anatomical abnormality of the pancreas. The diagnosis is mainly based on CT and MRI. The development of endoscopic technology provides a new basis for the diagnosis of annular pancreas. We report the images of the diagnosis and classification of annular pancreas under endoscopic ultrasonography(EUS) in our center.
Topics: Adult; Humans; Endosonography; Pancreas; Pancreatic Diseases; Endoscopy; Ultrasonography
PubMed: 36148679
DOI: 10.17235/reed.2022.9103/2022 -
Cureus Aug 2022Congenital anomalies of the pancreas are relatively uncommon. Most of these are asymptomatic and are detected incidentally, but can present with a variety of clinical...
BACKGROUND AND OBJECTIVE
Congenital anomalies of the pancreas are relatively uncommon. Most of these are asymptomatic and are detected incidentally, but can present with a variety of clinical manifestations like pancreatitis, duodenal obstruction, biliary obstruction, and rarely malignancy. Here in our study, we describe various congenital anomalies of the pancreas associated with various clinical manifestations, its management strategies, and outcomes. The aim was to study the various clinical manifestations of and management strategies for pancreatic diseases associated with congenital anomalies of the pancreas and their outcomes.
METHODS
A retrospective analysis of a prospectively maintained institutional database of 14 patients, admitted over a period of three years from June 2019 to May 2022, who were treated for different clinical manifestations of various congenital anomalies of the pancreas and their outcomes was done at our institution.
RESULTS
The total number of congenital anomalies of the pancreas in our study was 14 out of whom 7 (50%) were males and 7 (50%) females. The mean age of the patients was 37 years. The most common congenital anomaly was pancreatic divisum in six (42.9%) cases. The most common clinical manifestation was acute pancreatitis in four (28.6%) cases. One (7.1%) case was incidentally detected intraoperatively for another condition. Eight (57.1%) patients underwent surgical intervention and six (42.9%) patients were medically managed. Mortality occurred in two (14.3%) cases. Associated alcohol consumption was seen in 2 (14.3%) cases; 10 (71.4%) patients had no comorbidities while 4 (28.6%) patients had diabetes mellitus. Out of eight (57.1%) surgical patients, two (25%) had Clavien-Dindo grade I and one patient (12.5%) grade V complications.
CONCLUSION
Congenital anomalies of the pancreas can be associated with a variety of clinical manifestations; their management strategies and outcomes are no different from patients with the same clinical manifestations with normal pancreatic development.
PubMed: 36110453
DOI: 10.7759/cureus.27915 -
DEN Open Apr 2022Annular pancreas is a congenital abnormality in which part of the pancreatic head completely or partially surrounds the duodenum in a ring-like manner. The condition is...
Annular pancreas is a congenital abnormality in which part of the pancreatic head completely or partially surrounds the duodenum in a ring-like manner. The condition is thought to be an abnormality of the ventral pancreatic bud. While pancreatitis is a common complication of the annular pancreas, its recurrence may be prevented by improving the outflow of pancreatic juice. The present case report describes a 23-year-old woman who had been referred to our hospital for recurrent pancreatitis since childhood. An endoscopic incision was made on the orifice of the annular pancreas, after which pancreatitis of the annular pancreas did not recur for 6 years. The patient subsequently exhibited pancreatolithiasis in the dorsal pancreatic duct, which was successfully treated with endoscopic treatment. Endoscopic pancreatic sphincterotomy may prevent the recurrence of pancreatitis and avoid further surgical interventions by improving the flow of pancreatic juice.
PubMed: 35873513
DOI: 10.1002/deo2.122