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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 -
Clinical Journal of Gastroenterology Feb 2023A 65-year-old woman presented with epigastric pain persisting for more than 3 months. She was diagnosed with autoimmune pancreatitis (AIP), based on high serum IgG4...
A 65-year-old woman presented with epigastric pain persisting for more than 3 months. She was diagnosed with autoimmune pancreatitis (AIP), based on high serum IgG4 levels (981 mg/dL) and diffuse pancreatic enlargement with a capsule-like rim on computed tomography (CT). Additionally, the main pancreatic duct was indistinct on magnetic resonance cholangiopancreatography. CT, esophagogastroduodenoscopy, and upper gastrointestinal radiography revealed stenosis with gastric outlet obstruction (GOO) in the second part of the duodenum. Prednisolone administration was initiated as treatment; on day 3 of treatment, the patient's symptoms improved. After 2 weeks, CT and endoscopic ultrasonography of the duodenal bulbs revealed improvement of the enlarged pancreas. The second part of the duodenum ran into the pancreatic head, and no malignant lesions were observed. Based on the above findings, we suspect that she developed AIP in the annular pancreas (AnnP), where duodenal stenosis worsened with diffuse pancreatic enlargement, resulting in GOO. She is currently under careful observation with tapering of prednisolone-without surgical treatment for AnnP. The pathogenesis of GOO caused by AIP without malignancy is rare. One case of GOO caused by AIP, wherein AIP developed in the AnnP (similar to the present case), has been reported, highlighting the novelty of our report.
Topics: Adult; Humans; Female; Aged; Pancreatitis; Autoimmune Pancreatitis; Autoimmune Diseases; Pancreas; Gastric Outlet Obstruction; Prednisolone
PubMed: 36333486
DOI: 10.1007/s12328-022-01727-0 -
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 -
The British Journal of Surgery Sep 2022
Topics: Humans; Pancreas; Pancreatic Diseases
PubMed: 36084336
DOI: 10.1093/bjs/znac167 -
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 -
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