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Radiology Case Reports Nov 2023Complete annular pancreas (AP) is a congenital anomaly, in which the duodenum is either completely or partially encircled by the ring of pancreatic tissue, which...
Complete annular pancreas (AP) is a congenital anomaly, in which the duodenum is either completely or partially encircled by the ring of pancreatic tissue, which eventually becomes continuous with the head of the pancreas. The incidence of AP is estimated to be around 0.02%. Reports from endoscopic retrograde cholangiopancreatography show an incidence of 0.4%. Partial AP (PAP) is a rarer entity, in which a band of pancreatic tissue in continuity with the head of the pancreas, incompletely encircles the duodenum. It may be asymptomatic or may present later in life with complications. We report a case of a 72-years old male with features of intermittent bowel obstruct which was later found to be having PAP on contrast-enhanced computed tomography imaging at the level of renal hilum characterized by "crocodile-jaw appearance." The possibility of PAP should also be considered at the back of mind of the treating physician, if a patient presents with a long-term history of postprandial abdominal discomfort.
PubMed: 37680665
DOI: 10.1016/j.radcr.2023.08.044 -
Molecular Genetics & Genomic Medicine Oct 2023Annular pancreas (AP) is a congenital defect of unknown cause in which the pancreas encircles the duodenum. Theories include abnormal migration and rotation of the...
BACKGROUND
Annular pancreas (AP) is a congenital defect of unknown cause in which the pancreas encircles the duodenum. Theories include abnormal migration and rotation of the ventral bud, persistence of ectopic pancreatic tissue, and inappropriate fusion of the ventral and dorsal buds before rotation. The few reported familial cases suggest a genetic contribution.
METHODS
We conducted exome sequencing in 115 affected infants from the California birth defects registry.
RESULTS
Seven cases had a single heterozygous missense variant in IQGAP1, five of them with CADD scores >20; seven other infants had a single heterozygous missense variant in NRCAM, five of them with CADD scores >20. We also looked at genes previously associated with AP and found two rare heterozygous missense variants, one each in PDX1 and FOXF1.
CONCLUSION
IQGAP1 and NRCAM are crucial in cell polarization and migration. Mutations result in decreased motility which could possibly cause the ventral bud to not migrate normally. To our knowledge, this is the first study reporting a possible association for IQGAP1 and NRCAM with AP. Our findings of rare genetic variants involved in cell migration in 15% of our population raise the possibility that AP may be related to abnormal cell migration.
PubMed: 37635636
DOI: 10.1002/mgg3.2233 -
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 -
Annals of Surgical Oncology Sep 2023Portal Annular Pancreas (PAP) is a relatively uncommon entity with 4% reported incidence. Pancreaticoduodenectomy is challenging in cases with PAP and is associated with...
BACKGROUND
Portal Annular Pancreas (PAP) is a relatively uncommon entity with 4% reported incidence. Pancreaticoduodenectomy is challenging in cases with PAP and is associated with higher postoperative pancreatic fistula rate and overall morbidity. PAP is classified according to the pattern and location of fusion around the portal vein as-supra-splenic, infra-splenic & mixed fusion type. Also, the ductal anatomy can vary as pancreatic duct present only in the ante-portal portion or only in the retro-portal portion or ducts in both ante and retro-portal portion. At present, ideal surgical strategy is not defined as per the PAP types.
METHODS
The case demonstrated in the video presented with a localized, large duodenal mass with type IIA PAP (supra-splenic fusion with both ante and retro-portal ducts) detected on the preoperative triphasic CT scan. To achieve a single pancreatic cut surface with a single pancreatic duct for anastomosis, an extended pancreatic resection was performed using meso-pancreas triangle approach.
RESULTS
Patient had a smooth intraoperative course & the postoperative recovery was also uneventful. Pathology reported pT3 duodenal cancer with negative margins and uninvolved lymph nodes.
CONCLUSION
A preoperative knowledge of PAP and its various types is extremely important in order to tailor intraoperative management, specially of the retro-portal portion. In patients with retro-portal duct or both ante and retro-portal ducts (as the case presented in the video), an extended resection is recommended to mitigate postoperative pancreatic fistula.
Topics: Humans; Pancreaticoduodenectomy; Pancreatic Fistula; Pancreas; Anastomosis, Surgical; Postoperative Complications; Pancreatic Hormones; Pancreatic Neoplasms; Portal Vein
PubMed: 37402974
DOI: 10.1245/s10434-023-13782-z -
Endoscopy Dec 2023
Topics: Humans; Pancreas Divisum; Pancreas; Pancreatic Diseases; Cholangiopancreatography, Endoscopic Retrograde; Pancreatic Ducts
PubMed: 36958350
DOI: 10.1055/a-2032-3462