Pancreaticoduodenectomy with Anatomical Vascular Variant in Groove Pancreatitis-A Case Report.

Groove pancreatitis represents a chronic focal form of pancreatitis affecting the zone between the pancreatic head and the duodenal "C" loop, known as the groove area. This is a rare condition that affects the pancreatic periampullary part, including the duodenum and the common bile duct, which is usually associated with long-term alcohol and tobacco misuse, and is more frequent in men than in women. The most common clinical symptoms of groove pancreatitis include weight loss, acute abdominal pain, nausea, and jaundice. This report is about a 66-year-old woman with a history of heavy smoking, presenting with weight loss, nausea, and upper abdominal pain. Contrast-enhanced computed tomography revealed the existence of chronic pancreatitis as well as the dilatation of the main pancreatic duct, a cyst of the pancreatic head, and enlargement of the biliary tract. Conservatory treatment was initiated but with no improvement of symptoms. Since endoscopic retrograde cholangiopancreatography was not possible due to the local changes, we decided to perform pancreatoduodenectomy, as surgery appears to be the single effective treatment.

Development of a CT-based radiomics-clinical model to diagnose acute pancreatitis on nonobvious findings on CT in children with pancreaticobiliary maljunction.

OBJECTIVES: Since neither abdominal pain nor pancreatic enzyme elevation is specific for acute pancreatitis (AP), the diagnosis of AP in patients with pancreaticobiliary maljunction (PBM) may be challenging when the pancreas appears normal or nonobvious on CT. This study aimed to develop a quantitative radiomics-based nomogram of pancreatic CT for identifying AP in children with PBM who have nonobvious findings on CT. METHODS: PBM patients with a diagnosis of AP evaluated at the Children's Hospital of Soochow University from June 2015 to October 2022 were retrospectively reviewed. The radiological features and clinical factors associated with AP were evaluated. Based on the selected variables, multivariate logistic regression was used to construct clinical, radiomics, and combined models. RESULTS: Two clinical parameters and 6 radiomics characteristics were chosen based on their significant association with AP, as demonstrated in the training (area under curve [AUC]: 0.767, 0.892) and validation (AUC: 0.757, 0.836) datasets. The radiomics-clinical nomogram demonstrated superior performance in both the training (AUC, 0.938) and validation (AUC, 0.864) datasets, exhibiting satisfactory calibration (P > .05). CONCLUSIONS: Our radiomics-based nomogram is an accurate, noninvasive diagnostic technique that can identify AP in children with PBM even when CT presentation is not obvious. ADVANCES IN KNOWLEDGE: This study extracted imaging features of nonobvious pancreatitis. Then it developed and evaluated a combined model with these features.

Refractory postoperative pancreatic fistula following laparoscopic distal pancreatectomy for pancreatic cancer caused by incomplete pancreas divisum: a case report.

Pancreas divisum (PD) represents a prevalent congenital pancreatic variant, typically arising from the failure of fusion between the ventral and dorsal pancreatic ducts. This condition is frequently associated with recurrent pancreatitis. We herein present a case involving an incomplete PD diagnosis following the identification of a refractory postoperative pancreatic fistula (POPF) after laparoscopic distal pancreatectomy (DP) for pancreatic cancer. A 74-year-old female patient, who had undergone laparoscopic DP for pancreatic cancer, developed a POPF accompanied by intraabdominal bleeding, necessitating urgent intervention radiology to avert life-threatening complications. Following this, intraabdominal drainage was performed through an intraoperative drainage root. Subsequent fistulography and endoscopic retrograde pancreatography unveiled the presence of an incomplete PD for the first time. Consequently, a stent was placed in the Santorini duct. However, the volume of pancreatic juice from the intraabdominal drainage tube exhibited no reduction. Despite repeated attempts to access the pancreatic duct via a guidewire through the drainage tube, these endeavors proved futile. Paradoxically, the removal of the external drainage tube led to a recurrence of intraabdominal abscess formation. Consequently, reinsertion of the drainage tube became imperative. Consideration was given to draining the abscess under endoscopic ultrasonography and performing pancreatic duct drainage. However, due to the diminution of the abscess cavity through the external fistula drainage procedure, coupled with the absence of pancreatic duct dilation and its tortuous course, it was deemed a formidable challenge. the patient necessitated a lifestyle adaptation with a permanently placed percutaneous drainage tube.

Pancreatic neuroendocrine tumour resection in circumportal pancreas: a rare anatomical anomaly with important surgical implications.

Various congenital anomalies of the pancreas have been reported due to its complex embryological development involving the fusion of two separate buds. Circumportal pancreas is a rare anatomical anomaly where the pancreatic head and uncinate process fuse abnormally with the pancreatic body, encasing the portal vein and/or superior mesenteric vein completely. This anomaly poses several challenges to hepatobiliary surgeons, as the encasement of the portal vein by the abnormal pancreatic tissue makes an additional parenchymal transection necessary. Vascular variants have also been reported with circumportal pancreas, which, if not recognised preoperatively, can be catastrophic. Therefore, careful preoperative evaluation and planning are essential, to ensure safe pancreatic resection and recovery in a patient with circumportal pancreas. We present a case of a successful subtotal pancreatectomy and splenectomy in a patient with circumportal pancreas, for a suspected pancreatic duct adenocarcinoma. The aim of this case report is to contribute valuable insights that can aid hepatobiliary surgeons in enhancing their preoperative planning when encountered with patients with similar anatomical variances.

Pancreatic congenital anomalies and their features on CT and MR imaging: a pictorial review.

OBJECTIVE: To review the congenital anomalies of the pancreas with their main clinical manifestations and key imaging findings on CT and MRI. BACKGROUND AND CLINICAL SIGNIFICANCE: Anomalies of pancreatic development are frequent and generally asymptomatic, but can mimic and predispose individuals to pancreatic or peripancreatic pathologies, such as pancreatitis or malignancy. Their correct diagnosis may help avoid unnecessary further investigations and procedures, or establish adequate treatment when they manifest clinically. Differentiating pancreatic congenital anomalies from their main radiological mimics constitutes a challenge for the radiologist and requires familiarity with key imaging findings. CONCLUSION: The imaging findings of CT and MRI are essential for the correct diagnosis of congenital pancreatic anomalies.

Exploring the clinical characteristics and prevalence of the annular pancreas: a meta-analysis.

BACKGROUND: The annular pancreas (AP) is a rare gastrointestinal congenital malformation, in which malrotation of the pancreatic ventral bud in the seventh week of embryonic development manifests in a partial or complete ring of tissue around the second part of the duodenum. METHODS: The main online medical databases such as PubMed, ScienceDirect, Wiley online library, Web of Science, and EBSCO discovery service were used to gather all relevant studies on the AP. RESULTS: A total of 12,729,118 patients were analyzed in relation to the prevalence of AP. The pooled prevalence of AP was 0.0045% (95% CI: 0.0021%-0.0077%). The most frequent comorbidity in adults and children was duodenal obstruction, with a pooled prevalence of 24.04% and 52.58%, respectively (95% CI: 6.86%-46.48% and 35.56%-69.31%, respectively). The most frequent operation in adult patients with AP was duodenojejunostomy, with pooled prevalence established at 3.62% (95% CI: 0.00%-10.74%). CONCLUSION: The diagnostic complexity of AP is accentuated by its nonspecific clinical symptoms, making accurate identification reliant on imaging studies. Therefore, having a thorough knowledge of the clinical characteristics of the AP and its associated anomalies becomes paramount when faced with this rare congenital condition.

Monogenic Diabetes with GATA6 Mutations: Characterization of a Novel Family and a Comprehensive Analysis of the GATA6 Clinical and Genetics Traits.

Monogenic diabetes caused by GATA6 mutations were almost described as neonatal diabetes, and the phenotypic spectrum has expanded since then. Our study underscores the broad phenotypic spectrum by reporting a de novo GATA6 mutation in a family. Furthermore, we reviewed related literature to summarize the clinical and genetic characteristics of monogenic diabetes with GATA6 mutations (n = 39) in order to improve clinicians' understanding of the disease. We conclude that the GATA6 missense mutation (c. 749G > T, p. Gly250Val) is not reported presently, characterized by adult-onset diabetes with pancreatic dysplasia and located in transcriptional activation region. Carries with GATA6 mutations (n = 55) have a variable spectrum of diabetes, ranging from neonatal (72.7%), childhood-onset (20%) to adults-onset (7.5%). 83.5% of patients with abnormal pancreatic development. Heart and hepatobillary defects are the most common abnormalities of extrapancreatic features. Most mutations with GATA6 are loss of function (LOF, 71.8%) and located in functional region. Functional studies mostly support loss-of-function as the pathophysiological mechanism. In conclusion, there are various types of diabetes with GATA6 mutations, which can also occur in adult diabetes. Phenotypic defects with GATA6 mutations are most frequently malformations of pancreas and heart. This highlights the importance of comprehensive clinical evaluation of identified carriers to evaluate their full phenotypic spectrum.

Endoscopic minor papilla sphincterotomy in patients with complete pancreas divisum and acute recurrent pancreatitis: a metanalysis.

BACKGROUND AND AIMS: Pancreas divisum (PD) is a congenital variant of the pancreatic ductal system and a potential cause of acute recurrent pancreatitis (ARP). Endoscopic minor papilla sphincterotomy (MiES) is the most common procedure performed in the management of PD-related ARP. The aim of this study is to perform a meta-analysis estimating the efficacy and the safety of MiES in the management of patients with PD-related ARP. METHODS: A research was performed in Pubmed, EMBASE and Web of science, the studies were reviewed and selected according to inclusion and exclusion criteria. Evaluation of Heterogeneity and publication bias was performed, and a random effect model was used to estimate the effect size of each study. RESULTS: One hundred and thirteen articles were selected and reviewed, 13 met the inclusion criteria. All the studies were retrospective with a mean follow-up duration of 45.9 months. A total of 323 patients with PD-related ARP treated with MiES were included in the meta-analysis. The overall clinical success rate of MiES (defined as no further episodes of ARP, reduction of episodes of ARP, or improvement in quality of life) was of 77% (95%CI: 72%-81%; p = 0.30). Evaluating only the studies with clinical success rate defined as "no further AP in the follow-up" the clinical success rate was of 69.8% (95%CI: 61.3%-77.2%; p = 0.57), while evaluating the studies with other definitions (reduction of episodes of ARP or improvement in quality of life) the clinical success rate was of 81.2% (95%CI: 75.2%-86.1%; p = 0.45). The common fixed effects model disclosed a 25.5% overall adverse events rate (95%CI: 19.3%-32.8%; p = 0.42): acute pancreatitis in 14.3% (95%CI: 9.7%-20.6%; p = 0.36), bleeding in 5.6% (95%CI: 2.9%-10.4%; p = 0.98), and other adverse events in 5.6% (95%CI: 2.9%-10.4%; p = 0.67). CONCLUSION: MiES is an effective and relatively safe treatment in the management of PD-related ARP. The retrospective nature of the studies selected is the main limitations of this metanalysis. Prospective trials are needed to confirm these data.

Complete agenesis of dorsal pancreas: A rare cause of insulin-dependent diabetes mellitus.

ABSTRACT: We report a 16-year-old boy who presented with abdominal pain and severe hyperglycemia. His random blood glucose on admission was above 300 mg/dL, without ketosis. Urine examination revealed mild ketonuria. Renal function tests were normal. Antibodies to glutamate acid decarboxylase (anti-GAD) and islet tyrosine phosphatase 2 (anti-IA2) were negative. Findings on enhanced abdominal computed tomography scan were compatible with complete dorsal pancreatic agenesis. The body and tail of the pancreas were not visualized. Additionally, ectopic and malrotated left kidney was detected. Magnetic resonance cholangiopancreatography confirmed the absence of the dorsal pancreatic duct. The patient was treated with multiple subcutaneous insulin injections and attained good glycemic control. He is currently doing well on follow-up. The present case is a rare combination of complete agenesis of the dorsal pancreas with an ectopic, malrotated kidney. Clinical awareness of this rare association will help improve patient management.

Primate-specific ZNF808 is essential for pancreatic development in humans.

Identifying genes linked to extreme phenotypes in humans has the potential to highlight biological processes not shared with all other mammals. Here, we report the identification of homozygous loss-of-function variants in the primate-specific gene ZNF808 as a cause of pancreatic agenesis. ZNF808 is a member of the KRAB zinc finger protein family, a large and rapidly evolving group of epigenetic silencers which target transposable elements. We show that loss of ZNF808 in vitro results in aberrant activation of regulatory potential contained in the primate-specific transposable elements it represses during early pancreas development. This leads to inappropriate specification of cell fate with induction of genes associated with liver identity. Our results highlight the essential role of ZNF808 in pancreatic development in humans and the contribution of primate-specific regions of the human genome to congenital developmental disease.

An Unusual Variant of Anomalous Pancreaticobiliary Junction.

A 27-year-old previously healthy African American female presented to the Emergency Department with an acute onset of epigastric abdominal pain and nausea. Laboratory studies proved unremarkable. CT scan demonstrated intrahepatic and extrahepatic biliary ductal dilation with possible stones within the common bile duct. The patient was discharged with a surgery follow-up appointment. Laparoscopic cholecystectomy with intraoperative cholangiography was performed 3 weeks later due to concern for choledocholithiasis. The intraoperative cholangiogram showed multiple abnormalities, concerning for an infectious or inflammatory process. Magnetic resonance cholangiopancreatography (MRCP) demonstrated a suspected anomalous pancreaticobiliary junction and cystic lesion near the pancreatic head. Endoscopic retrograde cholangiopancreatography (ERCP) for cholangioscopy showed normal-appearing pancreaticobiliary mucosa with 3 tributaries directly from the pancreas entering the bile duct and an ansa orientation to the pancreatic duct. Biopsies of the mucosa were benign. Annual MRCP and MRI to assess for findings concerning for neoplasm given the anomalous pancreaticobiliary junction were recommended.

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.

Adult annular pancreas and its classification under endoscopic ultrasonography.

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.

[Surgical management of duodenal atresia due to annular pancreas and intestinal atresia IIIb]. / Manejo quirúrgico de atresia duodenal por páncreas anular y atresia intestinal IIIb.

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.

Introducción: la presencia de atresia duodenal relacionada con atresia intestinal tipo IIIb es una asociación rara, con pocos casos reportados en la literatura, y representa un reto quirúrgico si se toma en cuenta que incluso los casos aislados de atresia intestinal tipo IIIb lo representan. El objetivo fue reportar el manejo quirúrgico exitoso del caso de una malformación intestinal compleja, caracterizada por una oclusión duodenal secundaria a páncreas anular y atresia intestinal tipo IIIb, con una malrotación intestinal por definición y la presencia de divertículo de Meckel. Caso clínico: reportamos el caso de un recién nacido enviado de segundo nivel de atención con un diagnóstico de obstrucción duodenal no diagnosticado prenatalmente, que resultó en atresia duodenal por páncreas anular y atresia intestinal tipo IIIb, según la clasificación de Grosfeld. La presencia de atresia duodenal con atresia intestinal tipo IIIb es una condición extremadamente rara y todavía lo es más asociada con páncreas anular. Estos casos son un desafío si se toma en cuenta la corta longitud de intestino delgado y su consiguiente necesidad de nutrición parenteral total por un periodo prolongado. Conclusiones: el manejo quirúrgico de esta malformación intestinal compleja resultó en un caso con una adecuada evolución postquirúrgica, basada en el inicio mediato de alimentación enteral con un periodo corto de necesidad de nutrición parenteral total que finalmente resultó en una corta estancia hospitalaria.

Agenesis of Dorsal Pancreas in a Young Adult: A Case Report.

Dorsal agenesis of the pancreas is an exceedingly rare congenital anomaly where the dorsal part of the pancreas fails to develop properly during embryonic development. We report a case of partial agenesis of the dorsal pancreas in a 27-year-old female who presented with progressively worsening abdominal pain, nausea, vomiting, generalized weakness, easy fatigability, and dizziness. Physical examination revealed fair general condition with stable vital signs and normal abdominal and other system findings. Laboratory evaluations and abdominal computed tomography scan revealed an absence of the pancreatic body, tail, and duct of Santorini, confirming the diagnosis of partial agenesis of the dorsal pancreas. Only around 100 cases have been reported in the literature, making it a diagnostic challenge. Our case illustrates the rarity and challenges in diagnosing dorsal agenesis of the pancreas. Further research is needed to fully understand its causes and associations. Keywords: agenesis; case reports; congenital; pancreas.