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.

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.

Research progress of pancreatic head cancer arising from dorsal and ventral pancreas / 中华消化外科杂志

Pancreas arises from dorsal and ventral anlagens on an embryological basis,with a difference in histocytology.Immunohistochemical staining for an anti-pancreatic polypeptide can be performed to discriminate between the dorsal and ventral pancreas because of the difference in the amount of pancreatic polypeptide contained by the dorsal and ventral pancreas.Differences of survival in patients were approved due to the difference in histocytology between the dorsal and ventral pancreas,including the ability of local invasion,lymph node metastases,and nerve plexus invasion.

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.

Research progress of pancreatic head cancer arising from dorsal and ventral pancreas / 中华消化外科杂志

Pancreas arises from dorsal and ventral anlagens on an embryological basis,with a difference in histocytology.Immunohistochemical staining for an anti-pancreatic polypeptide can be performed to discriminate between the dorsal and ventral pancreas because of the difference in the amount of pancreatic polypeptide contained by the dorsal and ventral pancreas.Differences of survival in patients were approved due to the difference in histocytology between the dorsal and ventral pancreas,including the ability of local invasion,lymph node metastases,and nerve plexus invasion.

A Case of Agenesis of the Dorsal Pancreas with Concomitant Pancreatic Ductal Adenocarcinoma in an Adult Patient / 대한췌담도학회지

Agenesis of dorsal pancreas (ADP) is an extremely rare congenital anomaly defined as an absence of the dorsal ductal system resulting from failure in the embryologic development of the pancreatic dorsal bud. Most of ADP patients are asymptomatic but some of them suffer recurrent pancreatitis and diabetes. Few number of pancreatic adenocarcinoma in association with ADP has been published previously in other countries. There was no such case reported in Korea. We report a case diagnosed as pancreatic adenocarcinoma with ADP.

Heterotaxy syndrome: a case report / Síndrome heterotáxica: relato de caso

Heterotaxy syndrome is defined as an abnormal arrangement of some organs and vessels in association with dysmorphism. The authors describe the case of a patient with heterotaxy syndrome with poliesplenia incidentally diagnosed during imaging evaluation (computed tomography and small bowel barium study) of unrelated pathological condition.

Síndrome heterotáxica é definida como um arranjo anormal de alguns órgãos e vasos em associação a dismorfismo. Descrevemos o caso de uma paciente com síndrome heterotáxica diagnosticada incidentalmente durante avaliação por imagem (tomografia computadorizada e radiografia contrastada do intestino delgado) de condição patológica não relacionada.

Polysplenia Syndrome with Congenital Agenesis of Dorsal Pancreas Presenting as Acute Pancreatitis and the Role of Endoscopic Ultrasonography in Its Diagnosis / 대한소화기학회지

A 49-year-old female was admitted to our hospital for acute pancreatitis. The abdomen CT scan incidentally showed midline liver with hepatomegaly, centrally located gallbladder, pancreas truncation, right sided small bowel, left sided large bowel, interruption of the inferior vena cava with azygos continuation, preduodenal portal vein, and multiple spleens in the left upper quadrant. In MRCP, the head of pancreas was enlarged and short main pancreatic duct without accessory duct was showed. EUS revealed enlarged ventral pancreas with a main pancreatic duct of normal caliber, absence of the accessory pancreatic duct and the dorsal pancreas. She was diagnosed as polysplenia syndrome with agenesis of dorsal pancreas. It is a rare congenital anomaly frequently associated with various visceral anomalies including multiple spleens, impaired visceral lateralization, congenital heart diseases, gastrointestinal abnormalities and azygos continuation of the inferior vena cava. We report a case of polysplenia syndrome with agenesis of dorsal pancreas presenting acute pancreatitis.

A Case of Complete Agenesis of the Dorsal Pancreas / 대한소화기내시경학회지

The pancreas with the complete absence of its body and tail is the result of underdevelopment or agenesis of the dorsal pancreatic bud during embryogenesis, and this is a rare anomaly. We report here on a case of a 38-year-old man who had a pancreas with the total absence of the body and tail. On the abdominal computed tomography (CT), only a pancreatic head portion with speckled calcifications was seen, and the pancreatic body and tail were not visualized at all. Endoscopic retrograde cholangiopancreatography (ERCP) showed only a short major pancreatic duct with smooth tapering and terminal arborization. The Ampulla of Vater had a normal appearance and it was located at the medial side of the second portion of the duodenum. There was no difficulty to perform cannulation. Any minor papilla was not found. On magnetic resonance cholangiopancreatography (MRCP), the duct of Santorini and the duct in the body and tail were not visualized.

Agenesis of the Dorsal Pancreas: A Case Report and Review of the Literature

Partial or complete agenesis of the dorsal pancreas is a rare congenital anomaly that results from the embryological failure of the dorsal pancreatic bud to form the body and tail of the pancreas. To date, four cases have been reported in Korea. We report an additional case; a 25-year-old woman presented with diabetes mellitus and abdominal pain. Abdominal computed tomography (CT) revealed a normal-appearing pancreatic head, but the body and tail were not visualized. Endoscopic cholangiopancreatogram (ERCP) revealed a short pancreatic duct in the uncinate process and the head and the duct of Santorini draining into the minor papilla. Abdominal magnetic resonance imaging (MRI) findings were similar to the CT and ERCP results. The patient was diagnosed with partial agenesis of the dorsal pancreas by CT, ERCP and MRI.

Establishment of the Guideline for the Anatomical Resection of the Ventral and Dorsal Portion of the Pancreatic Head

PURPOSE: This study was designed to delineate the anatomical details of the pancreatic head for a ventral or dorsal segmental pancreatic resection along the embryological fusion plane, and to determine the feasibility of both procedures. METHODS: The resected pancreaticoduodenectomy specimens were analyzed (n=8), with the pancreatic and distal common bile ducts visualized by pancreatography (n=8). Immunohistochemical staining, with pancreatic polypeptide (PP), was performed in serially sliced specimens (n=3). The immunohistochemical and H&E staining were performed to evaluate the composition of the anatomical structures of the two differentially stained pancreas. RESULTS: What was presumed to be the embryological fusion plane was discovered between two differentially stained segments. This started just above the anterior inferior pancreaticoduodenal artery, directed to the posterior superior part of the pancreatic head and ended at the anterior surface of the distal common bile duct. The duct of Wirsung and the distal common bile duct were included in the posterior segment of the pancreas (ventral pancreas). There were two types of pancreatic duct arrangement, with the differences between the two types being; (1) the distance between the fusion point of the ventral and dorsal pancreatic ducts and the papilla of Vater, and (2) the stream of the Santorini duct. The branches of the pancreatic ducts were scattered over the entire pancreatic head region in multiple-directions. CONCLUSION: The fusion plane of the ventral and dorsal pancreas seems to initiate just above the anterior inferior pancreaticoduodenal artery, in a posterior-superior direction along the anterior surface of the distal common bile duct. A ventral pancreatectomy seems an impractical procedure with regard to the postoperative morbidity and operative difficulty, while a dorsal pancreatectomy seems to be more practical and feasible in its clinical aspects.

A Case of Complete Agenesis of Dorsal Pancreas / 대한소화기내시경학회지

Agenesis of dorsal pancreas is a rare congenital anomaly that arises from the failure of the dorsal pancreatic bud of endodermal cells to form the body and tail of the pancreas. It may be associated with diabetes mellitus, pancreatic exocrine dysfunction, or abdominal pain. Complete or partial agenesis of dorsal pancreas has been reported in a small number of pediatric and adult patients. A case is herein described involving a complete agenesis of dorsal pancreas and diabetes mellitus. A 38-year-old man with a 7-months history of non-insulin dependent diabetes mellitus was admitted due to weight loss and abdominal pain. Abdominal ultrasonography and computed tomography showed a normal biliary tree and enlarged head of the pancreas without visualization of the pancreatic body and tail. Endoscopic retrograde cholangiopancreatography (ERCP) revealved the short duct of Wirsung in the uncinate process and a head without opacification of any ducts in the pancreatic body or tail. The patient underwent explo-laparotomy for evaluation of the suspected pancreatic cancer. The patient was diagnosed as having complete agenesis of the dorsal pancreas by ERCP, CT, and surgery.

Agenesis of the Dorsal Pancreas: An autopsy case

Agenesis of the dorsal pancreas is one of the rare congenital anomalies of the pancreas. Six cases of them have been reported. We have experienced an autopsy case of agenesis of the dorsal pancreas associated with fetal death in the uterus. Grossly, the body and tail of the pancreas and uncinate process were not found and those were partially replaced by adipose tissue. No abnormality was noted in the other organs. Microscopically, pancreatic tissue with autolytic change was identified only in the head portion of the pancreas.