A case report on agenesis of dorsal pancreas with insulin-dependent diabetes mellitus: a rare entity.

Agenesis of the dorsal pancreas (ADP) is a clinically rare entity that occasionally presents with abdominal pain. It is also association with various disorders of glucose metabolism. Case presentation: A 23-year-old male presented with continuous epigastric pain for 4 h and intermittent vomiting. He has a history of experiencing recurrent abdominal pain and diarrhoea for the past 5 years. Additionally, he has been diagnosed with type 1 diabetes mellitus for 15 years. Contrast-enhanced computed tomography of the abdomen showed the absence of body and tail of the pancreas. Discussion: ADP is caused by unknown factors, but may be linked to genetic mutations or changes in signalling pathways related to retinoic acid and hedgehog. Symptoms can be absent, but may include abdominal pain, pancreatitis, and hyperglycaemia due to beta-cell dysfunction and insulin deficiency. Imaging modalities, such as contrast tomography or magnetic resonance cholangiopancreatography, or endoscopic retrograde cholangiopancreatography, are crucial in the diagnosis of ADP. Conclusion: It is important to consider ADP as a differential diagnosis in patients with glucose metabolism disorders and associated symptoms such as abdominal pain, pancreatitis, or steatorrhea. It requires the combined use of imaging modalities such as ultrasound, contrast tomography, magnetic resonance cholangiopancreatography, or endoscopic retrograde cholangiopancreatography, as ultrasound alone may not provide a complete diagnosis.

Agenesis of Dorsal Pancreas and Solid Pseudopapillary Tumor: Ventral Pancreas Preserving Portal Vein Resection and Reconstruction Using a Peritoneal Graft.

A diabetic lady in her 40s was referred to surgical oncologists with epigastric pain associated with vomiting. Computed Tomography (CT) Abdomen with contrast demonstrated a mass arising from the head of the pancreas with the absence of dorsal pancreas, confirmed on magnetic resonance cholangio-pancreatography (MRCP). A core needle biopsy was done, and the tumor was revealed to be a solid pseudopapillary epithelial neoplasm. She underwent sub-total pancreatectomy preserving the duodenum and ventral pancreas as there was adequate free margin; however due to the tumor abutting the anterior wall of the portal vein, it was resected, and reconstruction was done using a peritoneal graft. The patient made a good recovery without any significant post-operative events.

Dorsal pancreas agenesis, an incidental finding during acute appendicitis diagnosis; A case report.

INTRODUCTION AND IMPORTANCE: Dorsal pancreas agenesis is a rare congenital anomaly characterized by the absence or severe underdevelopment of the dorsal pancreatic bud. We report a case of a man who presented with features of appendicitis only to the incidentally discovery of dorsal pancreas agenesis during the diagnosis of acute appendicitis. We describe our experience on radiological diagnostic formulation and work up. CASE PRESENTATION: We present the case of a 45-year-old male patient who presented to the emergency department with symptoms and signs suggestive of acute appendicitis. A computed tomography scan and laboratory investigations confirmed the diagnosis of appendicitis. Incidentally, the scan also revealed the absence of dorsal pancreatic tissue, leading to the incidental diagnosis of dorsal pancreas agenesis. CLINICAL DISCUSSION: Dorsal pancreas agenesis is often asymptomatic and can be incidentally discovered during imaging studies or surgical interventions for unrelated conditions. In our case, the initial presentation of acute appendicitis provided an opportunity for the fortuitous diagnosis of dorsal pancreas agenesis. This emphasizes the importance of comprehensive imaging reporting in patients who undergo imaging for other conditions. CONCLUSION: This case report highlights the fortuitous discovery of dorsal pancreas agenesis during the diagnostic workup for acute appendicitis. It emphasizes the need for thorough imaging evaluation and reporting along with the importance of considering anatomical variations in patients presenting with abdominal symptoms. Increased awareness among healthcare professionals about such congenital anomalies can lead to their early recognition and appropriate management.

Failure to Thrive in a Middle-Aged Female: A Case of Congenital Incomplete Pancreas From a Rare Genetic Defect.

Hepatocyte nuclear factor-1 beta (HNF1B) gene is predominantly expressed in the liver, kidney, lung, genitourinary tract, and pancreas. It is an important transcription factor that regulates pancreas development. Mutation or absence of this gene is rare and can cause incomplete pancreatic development known as the agenesis of the dorsal pancreas. This rare genetic abnormality is associated with other disorders like maturity-onset diabetes of the young, abnormal liver function tests, genitourinary tract malformation, pancreatitis, and renal cysts. Diagnosing this genetic abnormality is difficult, especially in patients presenting with symptoms specific to only one system. Management is based on disease manifestation and involves a multidisciplinary approach. Our case describes a 51-year-old female with poorly controlled diabetes mellitus and Mullerian duct anomalies who presented with abdominal pain, fatigue, dizziness, and electrolyte derangement. Contrast-enhanced computed tomography (CECT) of the abdomen showed a multicystic kidney and a pancreatic head with a missing body and tail. Further workup revealed an HNF1B mutation.

Agenesis of the Dorsal Pancreas: A Rare Cause of Diabetes and Recurrent Upper Abdominal Pain.

Agenesis of the dorsal pancreas is a rare congenital disorder with only a handful of cases described in the literature. It presents a diagnostic dilemma. Cross-sectional imaging is the cornerstone for diagnosis. It could have a syndromic association with polysplenia and cardiac anomalies. Pancreas divisum and chronic pancreatitis may present with similar symptoms and must be ruled out. We present a case of a 55-year-old male with recurrent non-specific abdominal pain and diabetes mellitus. He was managed with insulin and painkillers for symptomatic relief. We also reviewed approximately 68 cases described in the literature to date.

Laparoscopic total gastrectomy for concurrent gastric cancer and complete agenesis of the dorsal pancreas.

We present an unusual case of laparoscopic total gastrectomy with lymph node dissection in a 56-year-old woman with gastric cancer and agenesis of the dorsal pancreas (ADP). Esophagogastroduodenoscopy revealed erosive lesions with thickened gastric folds; biopsy specimens revealed a poorly differentiated adenocarcinoma. Abdominal contrast-enhanced computed tomography showed a thickened wall of the gastric body; the distal pancreas was not visualized. Under the clinical diagnosis of gastric cancer with ADP, the patient underwent laparoscopic total gastrectomy with standard lymphadenectomy. The absence of a pancreatic neck, body, and tail was confirmed; lymph nodes along the splenic artery were dissected. Pathological analysis demonstrated a poorly differentiated adenocarcinoma invading the serosa, with five lymph node metastases. The postoperative course was unremarkable; postoperative adjuvant chemotherapy was performed using S-1 plus oxaliplatin. No symptom recurrence was observed at the 6-month follow-up. Laparoscopic surgery, with careful preoperative anatomic evaluation, can be considered for concurrent gastric cancer and ADP.

Agenesia del páncreas dorsal: reporte de caso y revisión del tema / Agenesis of the dorsal pancreas: case report and topic review

Complete agenesis of the dorsal pancreas (ADP) is an exceedingly rare congenital anomaly, compatible with life. It may be asymptomatic and usually incidentally diagnosed. In symptomatic cases, the clinical manifestations vary from abdominal pain, pancreatitis and diabetes mellitus to exocrine insufficiency with steatorrhea. We present a case report of a 28 year old female with ADP, diagnosed incidentally during radiological evaluation for hyperglycemias in SARS COV2 concomitant affection. Magnetic resonance cholangiopancreatography confirmed the absence of, neck, body and tail of the pancreas. Knowing the pancreatic embryogenesis, the clinical presentation of their malformations and the main radiological characteristics is important for the proper diagnosis of these anomalies.

Association of Agenesis of the Dorsal Pancreas With HNF1B Heterozygote Mutation: A Case Report.

Background: Agenesis of the dorsal pancreas (ADP) is a rare disease, the pathogenic mechanism of which is partially related to variants of hepatocyte nuclear factor 1B (HNF1B) gene. Case Presentation: We report a case of ADP, which presented with acute ketoacidosis, hyperuricemia, and liver dysfunction. In this case, the HNF1B score was estimated as 16 and a heterozygous variant of HNF1B in exon 2 (c.513G>A-p.W171X) was identified through gene sequencing. Conclusions: A good understanding of the clinical comorbidities of ADP is essential for avoiding missed diagnosis to a great extent. Moreover, estimation of HNF1B score is recommended before genetic testing.

Complete agenesis of dorsal pancreas with pancreatic cyst: A case report.

Agenesis of the dorsal pancreas (ADP) is extremely rare disease with no specific symptoms and there is no clear pathogenesis. Approximately half of the affected individuals develop diabetes resulting from reduced islet cell mass secondary to lack of endocrine structures. In this case, we aimed to present a 17-year-old female patient with ADP accompanied by a pancreatic cyst.

Frequency of Obliteration of the Dorsal and Ventral Ducts of the Pancreas in Islet Transplantation.

BACKGROUND: Islet isolation is an essential process in every human islet transplantation protocol. Intraductal enzyme delivery followed by adequate distention of the pancreas is the most critical step in islet isolation. Anomalies of the pancreatic duct system can significantly affect this process. Thus, identification and characterization of ductal patency is of paramount importance to achieve optimal islet isolation. AIMS: To investigate the frequency of duct obliteration in the human pancreas and explore donor/patient characteristics associated with specific ductal variations. METHODS: We examined ductal patency of pancreata allocated for islet allotransplantation (n = 597) and autotransplantation (n = 21) after removal of the duodenum during islet isolation procedure. Donor/patient factors were reviewed from the batch files. RESULTS: Among 559 deceased donor pancreata without pancreas divisum (n = 38, 6.4%), both ducts were patent in 50.1%, only ventral duct was patent in 46.7%, and only dorsal duct was patent in 3.2%. Donor age was not associated with the frequency of obliterated dorsal duct. Black race tended to have the higher frequency of patent dorsal duct. As expected, pancreas divisum was more frequent in chronic pancreatitis cases (n = 6, 28.6%). Within 7 cases of chronic pancreatitis with unknown etiology, we encountered one case of ventral duct obliteration. CONCLUSIONS: The minor duodenal papilla and aging do not likely play an important role in the occurrence of dorsal duct obliteration. Although frequency of obliterated ventral duct was low in our population, physicians, including gastroenterologists and endoscopists, as well as islet transplantation researchers should be aware of this possibility.

Partial agenesis of dorsal pancreas. Report of two cases.

INTRODUCTION: Agenesis of the dorsal pancreas (ADP) is a rare congenital anomaly resulting in missing corpus and cauda of the pancreas. The possibility of finding this disorder is increasing with the use of advanced radiological techniques like CT scan or MRI. PRESENTATION OF CASE: We reported 2 cases of a partial ADP as radiological finding: the first one was a 79-year-old asymptomatic patient who presented to perform a CT staging scan for bladder tumor, while the second case was a 73-year-old patient with obstructive jaundice and with suspected common bile duct calculi. In the second patient US, CT scan and MRI were performed, and after that also an ERCP was scheduled. DISCUSSION: The prevalence of agenesis of the dorsal pancreas is not exactly known; in the literature, only 50 cases have been reported. Its cause and pathogenesis are not fully understood. Some patients experience no symptoms, while others may develop hyperglycemia, diabetes mellitus, bile duct obstruction, abdominal pain, pancreatitis, or other conditions. CONCLUSION: Considering that dorsal agenesis is sporadically found, often do not have related symptoms and it does not require a specific treatment, whether further examinations are needed to determine the type of agenesis remains questioned.

Polysplenia with agenesis of the dorsal pancreas and preduodenal portal vein, about a case.

PURPOSE OF THE STUDY: Polysplenia is a complex polymalformative syndrome that is frequently accompanied by an inconsistent spectrum of visceral and vascular anatomical abnormalities and is extremely undiscovered in adulthood. The objective of this article is to limit the intraoperative risks generated by the lack of knowledge of these anatomical variations by insisting on the inconstancy of all these variations and the perfect knowledge of the reference anatomy and the exploration of the anatomical variations in imaging before the surgery. PATIENT AND METHODS: The patient was 50-year-old who was hospitalized in our department for gastric adenocarcinoma. During her extension assessment, a polysplenia syndrome was accidentally discovered on the scan images, which showed an exceptional association between preduodenal portal vein, agenesis of the dorsal pancreas, and polysplenia. CONCLUSION: There are neither specific clinical symptoms of the polysplenia syndrome, nor any biological sign; hence, the interest of recognizing this pathology is to avoid diagnostic errors, but also to guide the surgeon during the surgical act performed.

Two cases of agenesis of the dorsal pancreas and a review of the literature.

BACKGROUND: Agenesis of the dorsal pancreas (ADP) is a very rare disease with no specific symptoms, and the pathogenesis is not clear. Some patients will be accompanied by other diseases, such as pancreatic tumor or pancreatitis. But most cases are very atypical and difficult to distinguish. Some syndromes of pancreatic exocrine insufficiency are common in patients with ADP. Here, we report two cases of ADP and summarize the clinical features, diagnosis, and treatment of ADP. CASE PRESENTATION: Case A is a 65-year-old Chinese woman who presented with abdominal pain accompanied by nausea, bloating and acid reflux. The enhanced abdominal CT scan found nothing meaningful except the absence of the body and tail of the pancreas. The diagnosis was considered as gastrointestinal dysfunction cause by exocrine pancreatic insufficiency and recovered after symptomatic treatment. Case B is a 61-year-old Chinese woman who presented with abdominal pain accompanied by fever, vomiting and bloating. The abdominal CT showed multiple stones in the gallbladder, and the body and tail of the patient's pancreas were absent. She was diagnosed with cholelithiasis and recovered after laparoscopic cholecystectomy. CONCLUSION: Agenesis of the dorsal pancreas (ADP) is a rare congenital disease with an unclear pathogenesis that presents multiple symptoms. It should be considered when the patients have non-specific, persistent and unexplained symptoms such as bloating or uncontrolled blood sugar. Imaging examination is helpful for diagnosis. And it does not require surgical intervention unless it accompanies other diseases, EPI need to be considered when the non-specific gastrointestinal symptoms appear.

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.

Agenesis of the dorsal pancreas presenting with diabetic ketoacidosis - a case report and literature review.

BACKGROUND: Agenesis of the dorsal pancreas (ADP) is clinically rare, and it is usually accompanied by abdominal pain. Various disorders of glucose metabolism associating with ADP have been reported, but there are only two studies reporting a correlation between ADP and DKA in English literature. CASE PRESENTATION: We present a case of a patient with ADP accompanied by abdominal pain and diabetic ketoacidosis as the initial clinical presentation. A 30-year-old man presented with a 3-month history of recurrent onset of persistent mild epigastric pain, which worsen when eating. Laboratory tests revealed metabolic acidosis, hyperglycemia, and ketonuria. Phase contrast CT and MRCP showed the absence of the body and tail of the pancreas, as well as the dorsal pancreatic duct. The C-peptide release test indicated ß-cell dysfunction. A combination therapy of insulin, pancreatic enzyme supplements, and mosapride citrate were administrated and the pain gradually resolved. CONCLUSIONS: As glucose metabolism disorders can vary across different individuals, we advise clinicians to consider the diagnosis of ADP for a patient who presents with a glucose metabolism disorder accompanied by abdominal pain, pancreatitis or steatorrhea.

Single-cell transcriptomic analyses reveal distinct dorsal/ventral pancreatic programs.

The pancreas of vertebrates is separately derived from both the dorsal and ventral endodermal domains. However, the difference between these two programs has been unclear. Here, using a pancreatic determination gene, Pdx1, driven GFP transgenic mouse strain, we identified Pdx1-GFP highly expressing cells (Pdx1high) and Pdx1-GFP lowly expressing cells (Pdx1low) in both embryonic dorsal Pdx1-expressing region (DPR) and ventral Pdx1-expressing region (VPR). We analyzed the transcriptomes of single Pdx1low and Pdx1high cells from the DPR and VPR. In the VPR, Pdx1low cells have an intermediate progenitor identity and can generate hepatoblasts, extrahepatobiliary cells, and Pdx1high pancreatic progenitor cells. In the DPR, Pdx1high cells are directly specified as pancreatic progenitors, whereas Pdx1low cells are precocious endocrine cells. Therefore, our study defines distinct road maps for dorsal and ventral pancreatic progenitor specification. The findings provide guidance for optimization of current ß-cell induction protocols by following the in vivo dorsal pancreatic specification program.

Fusion plane between dorsal and ventral pancreas and it's clinical significance / 中华肝胆外科杂志

Pancreas arises from dorsal and ventral anlagen,and there is an embryological fusion plane between them.The embryological fusion plane can be discriminated by immunohistochemical staining for an anti-pancreatic polypeptide or computed tomography.The embryological fusion plane can not only guide the management of benign or low malignant potential tumors,but also determine the clinicopathological characteristics of pancreatic head cancer and patients'survival,and the embryological fusion plane plays an important role in the management of pancreatic disease.In this review,the research progressions and clinical significance in the embryological fusion plane of dorsal and ventral pancreas are described.

A Rare Case of Complete Agenesis of Dorsal Pancreas.

Agenesis of dorsal pancreas (ADP) is an extremely rare congenital anomaly that results from defective development of pancreas. Most ADP patients are asymptomatic; if symptomatic, they present with epigastric pain. About half of affected individuals develop diabetes mellitus (DM), resulting from reduced islet cell mass secondary to the absence of endocrine structures. Being very rare, it is generally not kept in mind while dealing these cases and are not suspected until imaging investigations are not done. In our case study, ADP was diagnosed during evaluation of the patient for recurrent pain abdomen and generalized weakness. How to cite this article: Jain A, Singh M, Dey S, Kaura A, Diwakar G. A Rare Case of Complete Agenesis of Dorsal Pancreas. Euroasian J Hepato-Gastroenterol 2017;7(2):183-184.

Diabetes mellitus due to agenesis of the dorsal pancreas in a patient with heterotaxy syndrome.

Heterotaxy syndrome (HS) is a congenital disorder resulting from an abnormal arrangement of visceral organs across the normal left-right axis in the embryonic period. HS is usually associated with multiple anomalies, including defects of the major cardiovascular system and the extracardiovascular system such as intestinal malrotation, abnormal lung lobulation, bronchus anomalies, and pancreatic dysplasia. Although pancreatic dysplasia is occasionally accompanied with HS, the occurrence of diabetes mellitus (DM) due to pancreatic dysplasia in HS is rarely reported. We here report a case involving 13-year-old girl with DM caused by agenesis of the dorsal pancreas and HS diagnosed on the basis of the presence of a double-outlet right ventricle with bilateral pulmonary stenosis and intestinal malrotation with duodenal cyst. Timely diagnosis and treatment with insulin improved glycemic control.

Coincidence of Polysplenia, Kartagener Syndrome, Dorsal Pancreas Agenesis, and Polycystic Kidney Disease in an Adult.

Here we present the case of an adult male patient with the anomalies of polysplenia, Kartagener syndrome, dorsal pancreas agenesis, and adult polycystic kidney disease. Familiarity with this extremely rare coincidence may improve diagnostic accuracy and patient management.