Dorsal Pancreatic Agenesis Research Articles

A Case Report of Dorsal Pancreatic Agenesis and Uterus Didelphys in a Young Woman: A Seldom- reported Association

Agenesis of the dorsal pancreas is a rare congenital anomaly that often remains asymptomatic and is frequently discovered incidentally during imaging for other conditions. Its association with Müllerian anomalies, such as uterus didelphys, suggests that both conditions may stem from similar embryological disruptions during early fetal development. This report aims to raise awareness of Agenesis of the Dorsal Pancreas (ADP) and its potential associations with Müllerian anomalies. By presenting a detailed case study, we seek to address the diagnostic challenges faced by healthcare professionals and the implications for patient management, emphasizing the importance of early detection and appropriate intervention. We present a case involving a 24-year-old Iranian female who presented with a 10-day history of abdominal pain and vomiting. Initial ultrasound imaging revealed moderate hydroureteronephrosis in the right kidney, prompting further investigation. A contrast-enhanced CT scan was performed, which not only confirmed ureteropelvic junction obstruction but also unexpectedly revealed dorsal pancreatic agenesis. In addition to these findings, the patient was diagnosed with uterus didelphys, highlighting a significant developmental anomaly. This case underscores the necessity for increased awareness of ADP and its associations with Müllerian anomalies among healthcare providers. Early and accurate diagnosis through advanced imaging modalities significantly improves patient outcomes and facilitates better management strategies for associated complications.

Isolated Partial Dorsal Pancreatic Agenesis Detected Incidentally by Contrast-enhanced Computed Tomography

Agenesis of the dorsal pancreas is a rare congenital pancreatic malformation because of failure in the development of the dorsal bud of the pancreas resulting in absent corpus and cauda of pancreas. Patients with agenesis of the dorsal pancreas often present with non-specific abdominal symptoms and very often it is an incidental finding. Here, we report the contrast enhanced imaging details of this rare entity in a 63 year old woman that was detected incidentally on computed tomography and there was no other co-existing anomaly.

Dorsal pancreatic agenesis: a case report.

Herein, we present a case of a 50-year-old woman who presented to general outpatient department with a complaint of abdominal pain. The patient was diagnosed with cholelithiasis with a contrast-enhanced computed tomography finding of dorsal pancreatic agenesis on a detailed workup. However, the patient did not have any other associated anomalies or symptoms associated with dorsal pancreatic agenesis. The patient was managed for cholelithiasis with laparoscopic cholecystectomy. Failure in development due to aberrant embryogenesis may cause partial or complete agenesis of the dorsal pancreas. The minor papilla, the accessory pancreatic duct, the body, and the tail of the pancreas are not present in the case of complete dorsal agenesis. Most cases of dorsal pancreatic agenesis are asymptomatic and diagnosed incidentally, whereas some of the cases may present with other associated abnormalities. It is almost always diagnosed via imaging modalities. Dorsal pancreatic agenesis is a very rare congenital anomaly of the pancreas. It can be diagnosed via various imaging modalities and almost always remains a radiological diagnosis with incidental findings.

Dorsal pancreatic agenesis: a case report

Dorsal pancreatic agenesis: a case report

Abstract 78

Background: Dorsal pancreatic agenesis is a rare developmental anomaly characterised by absence of pancreatic body and tail. Aims and Objectives: Case-series to describe clinical, biochemical characteristics and outcomes among dorsal pancreatic agenesis patients in our institute during the time period Feb 2021-Sept 2022. Results: During this period we found 10 subjects, of which 60% were males. The median age at diagnosis was 23.5 yr (range 16-52 yr). The median duration of diabetesat presentation was 3.5 yrs (range 1-14 yr). The most frequent clinical manifestations were found to be diabetes (100% cases) and non-specific abdominal pain (80%). Steatorrhea was seen in 30% cases. None of the patients presented with DKA. Anti GAD antibody was negative in 4 out of 4 cases tested. Serum amylase and lipase levels were normal in the cases presenting with pain abdomen. All the patients underwent CT abdomen which showed non-visibility of distal body and tail of pancreas. MRCP was done in 2 cases whichrevealed absent dorsal pancreatic duct. Next generation sequencing for MODY panel was done in 7/10 cases and no pathogenic variants were detected. Two patients are on OHA and rest 80% (8/10) cases are on treatment with insulin. Symptoms and signs of micro or macrovascular complications were not found in any of the cases. Conclusion: Dorsal pancreaticagenesis is a rare condition diagnosed during imaging for common abdominal symptoms. It is an important differential diagnosis in young patients presenting with diabetes mellitus and pain abdomen.

A Rare Case of Dorsal Agenesis of Pancreas, Choledochal Cyst, and Hirschsprung Disease in a Young Patient

ABSTRACTDorsal pancreatic agenesis is a rare congenital pancreatic malformation. There is just 1 reported case associating it with choledochal cyst. However, no cases have reported yet with both coexisting with Hirschsprung disease. We report a case of a 23-year-old man, presenting with on and off epigastric pain, sometimes radiating to the back. His medical records showed he had Hirschsprung disease as a neonate, for which he underwent Duhamel procedure. Ultrasound imaging revealed a choledochal cyst and a nonvisualized distal portion of the pancreas. Further cross-sectional imaging confirmed the findings—a type 1 choledochal cyst and a dorsal agenesis of the pancreas in a patient with Hirschsprung disease.

Performance of endoscopic ultrasound for diagnosis of agenesis of the dorsal pancreas: a case report

Agenesis of the dorsal pancreas is a rare congenital pancreatic malformation. We herein describe a 67-year-old woman with a 5-day history of lower back pain who was eventually diagnosed with agenesis of the dorsal pancreas. Abdominal computed tomography showed an enlarged pancreatic head, but the pancreatic body and tail were invisible. The magnetic resonance imaging findings were similar to the computed tomography findings. Magnetic resonance cholangiopancreatography showed that the major pancreatic duct was mildly dilated but otherwise normal. Endoscopic ultrasound revealed absence of the pancreatic body and tail, an enlarged head of the pancreas, and mild pancreatic duct dilation. The final diagnosis was dorsal pancreatic agenesis.

Portal Annular Pancreas with portal cavernoma formation wtih associated dorsal pancreatic agenesis-A rare case report

Portal annular pancreas is a uncommon congenital anomaly resulting from fusion of the pancreatic parenchyma around the portal vein. Its causing portal cavernoma formation and association with dorsal pancreatic agenesis is rare Case report We report a 51-year-old female who underwent contrast enhanced computed tomography for vague right hypochndrial pain.On CECT abdomen images there was presence of rind of pancreatic tissue around the portal vein causin its luminal narrowing with proximal dilation of portal vein tributaries with cavernoma formation.There was also presence of agenesis of dorsal pancreas in this patient.Conclusion This variant of portal annular pancreas with cavernoma formation associated with dorsal pancreatic agenesis has not yet been reported and we propose a new CT classification of the same.

Dorsal pancreatic agenesis in newly diagnosed type one diabetes mellitus: case report and review of literature

Dorsal pancreatic agenesis is a rare congenital anomaly that may be associated with recurrent bouts of pancreatitis and diabetes mellitus. We report the case of a 21-year-old man newly diagnosed with diabetes mellitus and agenesis of dorsal pancreas. A brief review of literature and association of both entities is presented.

Portal Annular Pancreas with Portal Cavernoma Formation with Associated Dorsal Pancreatic Agenesis - A Rare Case Report

Portal Annular Pancreas with Portal Cavernoma Formation with Associated Dorsal Pancreatic Agenesis - A Rare Case Report

PDX1 -MODY and dorsal pancreatic agenesis: New phenotype of a rare disease.

Maturity-Onset Diabetes of the Young (MODY) type 4 or PDX1 -MODY is a rare form of monogenic diabetes caused by heterozygous variants in PDX1 . Pancreatic developmental anomalies related to PDX1 are reported only in neonatal diabetes cases. Here, we describe dorsal pancreatic agenesis in 2 patients with PDX1 -MODY. The proband presented with diabetes since 14 years of age and maintained regular glycemic control with low doses of basal insulin and detectable C-peptide levels after 38 years with diabetes. A diagnosis of MODY was suspected. Targeted next-generation sequencing identified a heterozygous variant in PDX1 : c.188delC/p.Pro63Argfs*60. Computed tomography revealed caudal pancreatic agenesis. Low fecal elastase indicated exocrine insufficiency. His son had impaired glucose tolerance, presented similar pancreatic agenesis, and harbored the same allelic variant. The unusual presentation in this Brazilian family enabled expansion upon a rare disease phenotype, demonstrating the possibility of detecting pancreatic malformation even in cases of PDX1 -related diabetes diagnosed after the first year of life. This finding can improve the management of MODY4 patients, leading to precocious investigation of pancreatic dysgenesis and exocrine dysfunction.

An Unusual Presentation of Pancreatic Dorsal Agenesis

Dorsal pancreatic agenesis is a rare congenital anomaly. Besides, polysplenia syndrome concomitant with situs inversus totalis and distal pancreatic hypoplasia are rare congenital anomalies. We aimed to present such a rare congenital variation with computed tomography, magnetic resonance, and magnetic resonance cholangiopancreatography findings

Factors Predicting Readmission Within 30 Days of Acute Pancreatitis Attack: A Prospective Study

Introduction: Acute pancreatitis is the most common pancreatic disease and the leading gastrointestinal reason for hospitalization in the United States. Although one-third of discharged acute pancreatitis patients are readmitted risk factors for 30-day readmission after acute pancreatitis is not well documented. This study seeks to determine whether individualized discharge planning can help in reducing the risk for readmission within 30 days. Methods: In this prospective study, data was collected and analyzed on 113 patients who were hospitalized at three different community hospitals for an attack of acute pancreatitis. Verbally consented patients, enrolled from July 2015 through June 2016, were contacted thirty days after discharge and questioned regarding readmissions. Information collected from electronic medical records included demographics, clinical profile, and details of readmissions. Results: The total study included 113 patients, with a mean age of 55.3 years, 61% male, 64% white, and mean BMI of 29.9. Etiologies for the initial acute pancreatitis admission included biliary (37%), alcohol-associated (31%), idiopathic (22%) and other (10%). Local complications were common, found in 39% of patients. Persistent organ failure (7%), ICU admission (10%), BISAP≥3 (6%) and transfers from another hospital (2%) were also noted. The average length of stay was 6.9 days. A total of 19 patients (17%) were readmitted within 30 days of discharge. This group's average age was 53.6 years, male 68% and white 79%. At initial discharge 32% had local complications, 42% were given short-term pain medications, 37% had abnormal vital signs, 21% had SIRS, 11% were given antibiotics. One patient had GI symptoms, was on non-solid diet and was transferred to another hospital. Of the 19 readmitted patients 74% had the same etiology for both admissions: 32% biliary, 21% alcohol-associated, 16% idiopathic and 5% dorsal pancreatic agenesis. Of the five patients readmitted for a different etiology, four were for non-pancreatic reasons. Conclusion: Nearly one-fifth of patients hospitalized with acute pancreatitis are readmitted within 30 days of discharge. Although they tended to be male, white, with local complications and pain medications at discharge there was no statistically significant association between 30-day readmission and any of the traits studied. The complexity of factors that impact the health of patients presenting with acute pancreatitis continues to make this diagnosis difficult to treat.

Complete Dorsal Pancreatic Agenesis and Unilateral Renal Agenesis: First Case Report and Review of Literature

Complete Dorsal Pancreatic Agenesis and Unilateral Renal Agenesis: First Case Report and Review of Literature

Agenesis of the dorsal pancreas: systematic review of a clinical challenge.

Agenesis of the dorsal pancreas is a rare malformation. Since 1911 and until 2008, 53 cases have been reported. Several authors have recently described the association of this anomaly with neoplasia of the ventral pancreas, thus we performed a systematic review of the literature from 2008 to 2015. A systematic review of the MedLine and ISI Web of Science Databases from 2008 until 2015 was carried out, and 30 articles which met the inclusion criteria were identified that included a total of 53 patients: 7 children and 46 adults. Although dorsal pancreatic agenesis is a rare malformation, given its association with non-alcoholic pancreatitis and neoplasia of the residual pancreas, physicians should maintain an expectant attitude.

A triad of complete dorsal pancreatic agenesis, pancake kidney and bicornuate uterus. An association or an incidental finding: first case in literature.

Agenesis of the dorsal pancreas is a rare congenital anomaly which can be detected either incidentally during imaging or encountered during investigation for pancreas related pathologies such as pancreatitis or diabetes mellitus. Few associations of this condition with heterotaxy syndrome, pancreatic tumors and congenital heart disease have been described. We report a novel association of genitourinary anomalies in a case of complete dorsal pancreatic agenesis not previously described in the literature. Knowledge about dorsal pancreatic agenesis is essential for radiologist in patients presenting with unexplained pain abdomen and its association with various conditions may help radiologist in assisting clinician in management of these patients.

Study of Dorsal Pancreatic Agenesis on Cross Sectional Imaging

Objective: Agenesis of the dorsal pancreas is an extremely rare anomaly which results from defective development of pancreas. It may be asymptomatic and incidentally detected on cross sectional imaging. Methods: Four cases of dorsal pancreatic agenesis were found during one year study in the Department of Radiology, Gauhati Medical College and Hospital, Guwahati. Results: All the cases were detected incidentally during imaging which was done for some non-specific symptoms. Conclusions: Due to the incidental finding of dorsal pancreatic agenesis, more attention should be paid for pancreas during imaging.

Screening of diabetes of youth for hepatocyte nuclear factor 1 mutations: clinical phenotype of HNF1β-related maturity-onset diabetes of the young and HNF1α-related maturity-onset diabetes of the young in Japanese.

To compare the prevalence and clinical features of HNF1β-related MODY and HNF1α-related MODY in Japanese. We enrolled 230 Japanese patients with suspected MODY and examined them for HNF1α and HNF1β mutations. We characterized the clinical features of HNF1β-related MODY (HNF1β-MODY) and HNF1α-related MODY (HNF1α-MODY). Six patients had HNF1β mutations, four of which were large gene deletions and 24 patients had HNF1α mutations, which included one gene deletion. The mean fasting plasma glucose level at onset of HNF1β-MODY was considerably higher and the age of onset of HNF1β-MODY was considerably older than they were for HNF1α-MODY, while the mean BMI and C-peptide index at onset were similar. Three patients with HNF1β-MODY were found to have dorsal pancreatic agenesis and four of them had whole-gene deletion. Five of the patients with HNF1β-MODY had insulin secretion defects and were treated with insulin, and four of these did not have a parent with overt diabetes. HNF1β-MODY may present as β-cell dysfunction in Japanese rather than as hyperinsulinaemia, which it does among European/American. This dysfunction might result from an intrinsically lower capacity for insulin secretion in Japanese. HNF1β-MODY has an older age of onset than HNF1α-MODY, which may suggest lower penetrance of the disease. In addition, HNF1β-MODY has a broad spectrum of clinical manifestations, some of which are detectable by imaging. This may be helpful in some cases for selecting HNF1β-MODY candidates for genetic testing.

Agenesis of the dorsal pancreas

Developmental anomalies of the pancreas have been reported but dorsal pancreatic agenesis is an extremely rare entity. We report an asymptomatic 62-year-old woman with complete agenesis of the dorsal pancreas. Abdominal computed tomography (CT) revealed a normal pancreatic head, but pancreatic body and tail were not visualized. Magnetic resonance imaging (MRI) findings were similar to CT. At magnetic resonance cholangiopancreatography (MRCP), the major pancreatic duct was short and the dorsal pancreatic duct was not visualized. The final diagnosis was dorsal pancreatic agenesis.