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One of the rare disorders noted during birth is Annular pancreas. In this disorder a slender band of pancreatic tissue engulfs the duodenum. This congenital anomaly in children produces symptoms like duodenal obstruction but mostly they produce symptoms in later stages of life. This is one of the rare clinical diagnosis. But once diagnosed immediate treatment can reduce the morbidity risk.

Objective To investigate the clinical utility of ultrasound parameters in diagnosing neonatal annular pancreas. Methods A total of 56 neonates with descending duodenal obstruction underwent saline-contrast ultrasound examination. Ultrasonic parameters, including dilated duodenum diameter (DDD), dilated duodenum thickness (DDT), and stenosis duodenum diameter (SDD), were measured. The cases were categorized into three groups: annular pancreas (group A, n = 28), descending duodenal atresia (group B, n = 3), and descending duodenal septum (group C, n = 25). One-way ANOVA was employed to compare statistical differences among the three ultrasonic parameters across the groups, followed by LSD-t test for pairwise comparisons between two groups showing significant differences. Receiver operating characteristics analysis was conducted to calculate the area under the curve and determine the cut-off value if there were statistically significant differences observed. Results In group A, group B, group C, the diameters of DDD were measured as(23.2 ± 2.4)mm, (25 ± 1.0)mm, (19.4 ± 2.6) mm, respectively. When comparing these three groups, there was a statistically significant difference between group A or group B and group C in terms of DDD diameter measurements (t = 5.7, P < 0.05; t = 3.793, P < 0.05). However, no statistically significant difference was observed between group A and group B for DDD diameter measurements (t = 1.232, P > 0.05). The corresponding values for DDT were measured as (3.0 ± 0.6)mm, (3.2 ± 0.4) mm, and (2.4 ± 0.3) mm, respectively. When comparing these three groups, the results showed a statistically significant difference between either Group A or Group B compared to Group C in terms of DDT measurement (t = 4.695, P < 0.05; t = 2.778, P < 0.05). However, no statistically significant difference was found between Group A and Group B regard- ing the measurement of DDT (t = 0.666, P > 0.05). The SDD measurements in the three groups were (1.9 ± 0.3) mm, (3.6 ± 0.8)mm, (5.5 ± 0.7)mm, respectively. Statistical analysis using LSD-t test revealed significant differences between the three groups (t = 5.521, P < 0.05; t = 6.142, P < 0.05; t = 25.828, P < 0.05).The sensitivity, specificity, positive predictive value, and negative predictive value for detecting annular pancreas at a cutoff of 2.3 mm were determined to be 96.4%, 100%, 100%, and 96.6%, respectively, with an area under the receiver operating characteristic curve of 0.999. Conclusion SDD could be employed for the diagnosis of neonatal annular pancreas. [ABSTRACT FROM AUTHOR]

The preduodenal portal vein, being an extremely rare anomaly that occurs with a frequency of 3:1000, is clinically manifested mainly in childhood by signs of intestinal obstruction due to duodenal compression. In adults, this anomaly is asymptomatic, but has surgical importance, increasing the risk of damage to the preduodenal vein during operations on the biliary tract and duodenum. The preduodenal portal vein is usually associated with other intestinal obstruction, such as external (annular pancreas, malrotation), internal (atresia, membrane and stenosis), as well as with cardiovascular abnormalities, reverse location. Recently, we treated a patient with a preduodenal portal vein, which stimulated a revision of the disease process including diagnosis, association with other anomalies and surgical treatment.

Annular pancreas (AP) is a rare cause of congenital gastric outlet obstruction that is usually discovered during the neonatal period. Still, clinical severities can vary over a wide range and definite diagnosis could be delayed until late childhood or adulthood. We report here a case of AP detected in a 16-year-old malnourished male patient who was admitted because of non-bilious vomiting and epigastric fullness after intake of food which was relieved after vomiting. A contrast-enhanced CT scan of the abdomen confirmed the diagnosis of AP. At operation, a complete obstruction of the second part of the duodenum was found, caused by an annular pancreas. No other congenital anomaly of the intra-abdominal organs was noted. He successfully underwent retro-colic gastro-jejunostomy with uneventful postoperative recovery. Though a rare finding, AP should be considered as a differential diagnosis in patients presenting with gastric outlet obstruction after excluding common causes. The rarity of this congenital abnormality and its successful correction by surgical means have prompted us to make the following presentation. [ABSTRACT FROM AUTHOR]

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.

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. [ABSTRACT FROM AUTHOR]

Embryological development of the pancreas is a complex phenomenon and, therefore, it can have multiple developmental aberrations. Fortunately, the majority of these pancreatic ductal anomalies are asymptomatic with no clinical relevance and are incidentally detected during diagnostic cross-sectional imaging or endoscopic retrograde cholangiopancreatography (ERCP) or autopsy. Occasionally, pancreatic duct anomalies can result in symptoms like abdominal pain or recurrent pancreatitis. Also, an accurate pre-operative diagnosis of ductal anomalies can prevent inadvertent duct injury during surgery. Conventionally, ERCP had been used for an accurate diagnosis of pancreatic duct anomalies. However, because it is invasive and associated with a risk of pancreatitis, it has been replaced with magnetic resonance cholangiopancreatography (MRCP). MRCP has demonstrated high sensitivity and specificity for the diagnosis of ductal anomalies, which can be further improved with the use of secretin-enhanced MRCP. Endoscopic ultrasound (EUS) is a new diagnostic and interventional tool in the armamentarium of endoscopists and has demonstrated promising results in the detection of pancreatic duct variations and anomalies. Along with the visualization of the course and configuration of the pancreatic duct, EUS can also visualize changes in the pancreatic parenchyma, thereby helping with an early diagnosis of any co-existent pancreatic disease. Absence of the stack sign and crossed duct sign are important EUS features to diagnose pancreas divisum. EUS can also help with the diagnosis of other congenital ductal anomalies like annular pancreas, ansa pancreatica, and anomalous pancreaticobiliary union, although the published experience is limited. [ABSTRACT FROM AUTHOR]

Annular pancreas is a rare congenital abnormality that is often considered a neonatal condition, though in few cases it can present in adulthood. Patients diagnosed with annular pancreas in adulthood are often asymptomatic and diagnosed incidentally from radiologic studies. While there is no existing treatment protocol for symptomatic annular pancreas, multiple surgical approaches have been documented in the literature. In this case study we present a 49-year-old female patient who presented with gastric outlet obstruction secondary to annular pancreas. The patient was treated with nasogastric decompression and open side-to-side gastrojejunostomy with an antecolic approach to bypass the obstructed duodenal segment. The surgery was successful, followed by an uneventful postoperative course and discharge on postoperative Day 4. By 5-week outpatient follow-up the patient had resolution of her symptoms and achieved weight gain. [ABSTRACT FROM AUTHOR]

Background: Congenital duodenal obstruction occur with other anomalies in 50% of cases. Although predictable, its association with biliary/pancreatic anomalies is rarely reported. All cases of duodenal atresia (DA) and stenosis (DS) associated with biliary and/or pancreatic abnormalities were analyzed. Methods: A systematic review searched all studies reporting association between DA/DS and biliary/pancreatic abnormalities. The study was performed according to PRISMA guidelines. Results: Of 522 studies reviewed, 285 were analyzed after removing the duplicates, and 104 (249 patients) meeting the inclusion criteria, were included. Anomalous bifurcated bile duct was described in 24 cases (20 DA, 4 DS): 6 at necropsy, and 20 at perinatal surgery. Choledochal cyst was found in 7 patients (5 DA, 2 DS): 1 perinatally, 2 in the first year, 4 at median age of 6.5 years (range 4–27 years). Biliary atresia was diagnosed perinatally in 13 patients with DA and in 1 at 9 months. Annular pancreas was discovered in 146 patients (15 DA, 113 DS, 18 not specified): 113 perinatally, 4 children, and 28 adults (median age 33 years, range 4–72 years). In the remaining 58 cases, a combination of biliary and pancreatic abnormalities was diagnosed in 41 DA, 16 DS, 1 not specified duodenal obstruction, in 35 patients perinatally, in 4 infants, and in 19 at median age of 3 years (range 1–40 years). Conclusions: Presence of DA/DS should rise the alert for a possible association with biliary/pancreatic abnormalities. Therefore, a careful intraoperative approach should be adopted when repairing DA/DS to avoid damaging biliary and pancreatic ducts. In addition, appropriate imaging looking for biliary/pancreatic anomalies should be considered in symptomatic patients.

Abstract 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.

Annular pancreas is an uncommon congenital anomaly which is a rare cause of congenital duodenal obstruction. It is normally identified during the neonatal period, but may also be identified in adolescence or adulthood. This diagnosis is often overlooked in adult patients who present with symptoms suggestive of duodenal obstruction. We present a case of AP detected in a 23-year-old man, with complaints of continuous vomiting and abdominal discomfort over the last 6 months. An upper gastrointestinal study revealed a constricted second part of the duodenum. A computed tomography scan revealed a complete ring of pancreatic tissue around the second part of the duodenum. Diagnostic and therapeutic surgery decompresses the external obstruction. The patient had an early post-operative activation. No specific guidelines and protocols exist about the management of such cases. Given the rarity of this congenital anomaly, presenting with chronic partial duodenal obstruction, and its successful surgical treatment, have prompted us to report the case along with a brief review of literature about the subject.

Annular pancreas (AP) is a congenital anomaly and a recognised cause of duodenal obstruction which can affect all age groups. It may manifest early in the neonatal period, but it may also have varied and often delayed symptoms. We report the case of AP in an 18-month-old girl with a long history of recurrent post-prandial non-bilious vomiting treated for a long time with the clinical diagnosis of gastroesophageal reflux. Upper gastrointestinal study was suggestive of partial duodenal obstruction and computed tomography revealed a complete ring of pancreatic parenchyma surrounding the second part of the duodenum. Diamond-shaped duodenoduodenostomy was achieved successfully and the post-operative period was uneventful. Although rare, AP must be kept in mind of any paediatric surgeon while confronted to symptoms of partial duodenal obstruction.

Our purpose is to illustrate the radiological aspects of the annular pancreas as an etiology of duodenal obstruction in infants. We report the case of a 4-month-old girl, who was admitted to our department with postprandial vomiting evolving since birth. The initial examination found a severely dehydrated patient. Abdominal ultrasound showed gross dilatation of the stomach and duodenum, it also showed pancreatic tissue surrounding the duodenum, suggesting a diagnosis of annular pancreas as the cause of the duodenal obstruction. Post-contrast abdominal CT showed the gastric and duodenal dilatation, and a ring of pancreatic tissue surrounding uncompletly the second portion of the duodenum. The patient underwent a bypass surgery which consisted in a duodeno-duodenostomy with simple post-operative follow-up and no recurrence of digestive symptoms. Annular pancreas is a rare pathology to be sought in neonatal obstruction. A good knowledge of radiological semiology is essential for a good diagnostic approach. However, surgery is the only effective way to diagnose and treat this pathology.

Background: Simultaneous occurrence of duodenal atresia, annular pancreas, and situs inversus totalis is an exceedingly rare clinical condition. This may pose management-related challenges to the treating surgeon. Case Presentation: We report a case with a preoperative diagnosis of duodenal atresia and situs inversus. Intraoperatively, an annular pancreas encircling the duodenum was found. Instead of duodenoduodenostomy, a duodenojejunal anastomosis was performed. The postoperative course remained uneventful. The patient is doing fine on a 6-month follow-up. Conclusion: The simultaneous presence of multiple anomalies in this patient prompted us to utilize an alternative approach to relieve the duodenal obstruction. This report highlights the importance of tailoring surgical decisions based on the individualized needs of the patient.

Bronchoesophageal fistulas (BEFs) are rare malformations characterized by abnormal communication between the bronchus and the esophagus. Herein, we report a successful management of a child with BEF discovered as an intraoperative surprise and associated with the short gut, annular pancreas, and disorder of intestinal fixation.

Bronchoesophageal fistulas (BEFs) are rare malformations characterized by abnormal communication between the bronchus and the esophagus. Herein, we report a successful management of a child with BEF discovered as an intraoperative surprise and associated with the short gut, annular pancreas, and disorder of intestinal fixation. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Annular pancreas (AP) is a congenital anomaly and a recognised cause of duodenal obstruction which can affect all age groups. It may manifest early in the neonatal period, but it may also have varied and often delayed symptoms. We report the case of AP in an 18-month-old girl with a long history of recurrent post-prandial non-bilious vomiting treated for a long time with the clinical diagnosis of gastroesophageal reflux. Upper gastrointestinal study was suggestive of partial duodenal obstruction and computed tomography revealed a complete ring of pancreatic parenchyma surrounding the second part of the duodenum. Diamond-shaped duodenoduodenostomy was achieved successfully and the post-operative period was uneventful. Although rare, AP must be kept in mind of any paediatric surgeon while confronted to symptoms of partial duodenal obstruction. [ABSTRACT FROM AUTHOR]

VACTERL Syndrome affects multiple body systems and can include various anomalies of the Vertebral column, Anus and/or rectum, heart (Cardiac), Tracheo-Esophagus, kidneys (Renal), and Limbs. Patients with VACTERL syndrome are at increased risk of having a congenital duodenal obstruction that may be extrinsic in the form of an annular pancreas or intrinsic in the form of duodenal atresia, stenosis or web. Simultaneous presentation of both the annular pancreas and duodenal web is a rare clinical entity and typically presents in neonates. However, late presentation of annular pancreas combined with a duodenal web is exceedingly uncommon. We present a case of late diagnosis of annular pancreas with duodenal web resulting in an entrapped ingested foreign body.

Annular pancreas is a rare congenital anomaly in which a thin band of pancreatic tissue partially or completely surrounds the duodenum. It is challenging to diagnose due to its variable clinical presentation. Approximately two-thirds of patients have no symptoms in their lifetime, and most symptomatic cases are seen in neonates and infants. Symptomatic adult patients present with upper gastrointestinal symptoms, such as epigastric pain, vomiting, and postprandial fullness associated with gastric outlet obstruction. Complications associated with annular pancreas include peptic ulcer disease, pancreatitis, pancreatic head carcinoma, and biliary obstruction. Annular pancreas is also a rare cause of upper gastrointestinal bleeding in adults, but it should be considered as one of the differential diagnoses in patients presenting with a peptic ulcer and duodenal stricture. Here, we report the case of a 60-year-old man who presented with melena and was subsequently diagnosed with an annular pancreas.

Embryological development of the pancreas is a complex phenomenon and, therefore, it can have multiple developmental aberrations. Fortunately, the majority of these pancreatic ductal anomalies are asymptomatic with no clinical relevance and are incidentally detected during diagnostic cross-sectional imaging or endoscopic retrograde cholangiopancreatography (ERCP) or autopsy. Occasionally, pancreatic duct anomalies can result in symptoms like abdominal pain or recurrent pancreatitis. Also, an accurate pre-operative diagnosis of ductal anomalies can prevent inadvertent duct injury during surgery. Conventionally, ERCP had been used for an accurate diagnosis of pancreatic duct anomalies. However, because it is invasive and associated with a risk of pancreatitis, it has been replaced with magnetic resonance cholangiopancreatography (MRCP). MRCP has demonstrated high sensitivity and specificity for the diagnosis of ductal anomalies, which can be further improved with the use of secretin-enhanced MRCP. Endoscopic ultrasound (EUS) is a new diagnostic and interventional tool in the armamentarium of endoscopists and has demonstrated promising results in the detection of pancreatic duct variations and anomalies. Along with the visualization of the course and configuration of the pancreatic duct, EUS can also visualize changes in the pancreatic parenchyma, thereby helping with an early diagnosis of any co-existent pancreatic disease. Absence of the stack sign and crossed duct sign are important EUS features to diagnose pancreas divisum. EUS can also help with the diagnosis of other congenital ductal anomalies like annular pancreas, ansa pancreatica, and anomalous pancreaticobiliary union, although the published experience is limited.

Introduction. Annular pancreas is a rare congenital anomaly in which a band of the pancreatic tissue, in continuity with the pancreatic head, completely or incompletely surrounds the descending part of the duodenum. An abnormal pancreatic development can cause complete annular pancreas, partial annular pancreas, and pancreas divisum. Complete annular pancreas is diagnosed in newborns, while the diagnosis of the partial annular pancreas is more frequently established in adults. The most reliable diagnostic methods are computed tomography and magnetic resonance cholangiopancreatography. The anomaly is treated surgically, using bypass procedures. Case report. A 12-year-old girl presented malnourished, with occasional feeding problems, vomiting, heartburn, and pain from infancy. The upper gastrointestinal series showed an extremely dilated stomach, the first and the second part of the duodenum. An endoscopic exam revealed the dilated stomach, pylorus, and the first and the second part of the duodenum with retained contrast, while the entrance of the endoscope into the third part of the duodenum was not possible. Computed tomography showed pancreatic tissue encircling the second part of the duodenum and the characteristic "crocodile jaw" sign. Roux-en-Y duodenojejunostomy was performed as a bypass procedure. Conclusion. The complete annular pancreas is a well-known and easily diagnosed anomaly in newborns. The partial annular pancreas is often poorly recognized, especially in patients who do not present with marked duodenal obstruction. Unrevealed, it causes chronic problems in food intake, with possible serious complications. Although a very rare condition in the pediatric population, partial annular pancreas should be taken into consideration in unclear cases of chronic poor oral food intake and vomiting.

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

The authors presented a case of duodenal obstruction in a 61-year-old man, resulting from an annular pancreas diagnosed on imaging (computed tomography, magnetic resonance cholangiopancreatography, and endoscopic ultrasound). The patient underwent a diagnostic laparoscopy. Intraoperatively, given a straightforward appearance and anatomy of the annular pancreas overlying the second part of the duodenum, and due to extensive adhesions in the abdomen, a primary division of the annular pancreas was performed, instead of a bypass procedure such as gastrojejunostomy. He had some residual symptoms 1 week postoperatively which was treated with duodenal dilatation endoscopically. On review and follow-up at 1 year, he has remained well with resolution of symptoms, supported by radiological improvement on a computed tomography performed at 4 months post-operatively. We believe this approach has resulted in less morbidity and a shorter period of recovery as compared to a bypass procedure and represents a reasonable therapeutic option for annular pancreas., Competing Interests: The authors disclose no conflicts. The authors confirm that the material has not been published previously and is not submitted for publication elsewhere., (Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2024.)

Objective: Reinforce that, despite being a rare condition, annular pancreas must be considered as a differential diagnosis for intestinal obstructions in the newborn period. Case description: This case report refers to a 13-days newborn admitted to the emergency room presenting with postprandial vomiting and progressive weight loss since maternity discharge. The patient did not respond to initial interventions that included IV antibiotics and insertion of a nasogastric tube. A contrasted study of the upper digestive tract (esophagus, stomach and duodenum) found a luminal narrowing on the second duodenal portion. The patient was submitted to an exploratory laparotomy which found a pancreatic-tissue ring involving the second part of the duodenum. Despite rare, we reinforce that annular pancreas must be considered as a differential diagnosis for intestinal obstructions in the newborn period. Comments: Annular pancreas is a rare congenital defect in which a ring of pancreatic tissue encircles the duodenum, causing different degrees of intestinal obstruction.

Primary ciliary dyskinesia (PCD) is a rare genetic disorder characterized by motile ciliary dysfunction and impaired ultrastructure. Despite numerous studies, the genetic basis for about 30% of PCD cases remains to be elucidated. Here, we present the identification and functional analysis of two novel mutations in the gene encoding coiled-coil domain-containing protein 40 (CCDC40), which are found in a familial case of PCD. These novel CCDC40 mutations, NM_017950.4: c.2236-2delA and c.2042_2046delTCACA, NP_060420.2: p.(Ile681fs), were identified by whole-exome sequencing (WES). Sanger sequencing was then performed to confirm the WES results and determine the CCDC40 gene sequences of the proband’s parents. The c.2042_2046delTCACA mutation disrupts the reading frame of the protein and is therefore predicted to produce a non-functional protein. Using a minigene assay with the pcDNA3.1(+) plasmid, we further investigated the potential pathogenic effects of the c.2236-2delA mutation and found that this mutation leads to formation of a truncated protein via splicing disruption. Thus, in summary, we identified two mutations of the CCDC40 gene that can be considered pathogenic compound heterozygous mutations in a case of familial PCD, thereby expanding the known mutational spectrum of the CCDC40 gene in this disease.

Annular pancreas is a rare congenital intestinal obstruction to be found in infants. It is characterized by nonbilious vomiting, abdominal distention, and feeding intolerance that share similar symptoms with other intestinal obstructions. We reported a case of 11-month-old female infant with a history of nonbilious vomiting after coughing as much as 6 times per day 1 day before admission. The patient had an incorrect provisional diagnosis of hypertrophic pyloric stenosis based on ultrasound. Later on, an upper gastrointestinal series showed dilatation of the second part of the duodenum which was successfully treated by a duodenostomy during laparotomy procedure. It is suggested that treating an intestinal obstruction needs a further investigation when the symptoms were continuously repeated.

Annular pancreas (AP) is a rare condition that usually is not associated with symptoms in adults. However, in some patients, AP may cause non-specific symptoms such as abdominal pain and vomiting, making its diagnosis challenging. The current case report presents a challenging diagnosis of an AP case and surgical management of it by performing duodenoduodenostomy. A 47-year-old female presented with chronic abdominal pain and vomiting after meals. The examination using CT showed a complete ring of pancreatic tissue encircling the descending part of the duodenum, confirming the diagnosis of AP. Therefore, the patient underwent duodenoduodenostomy, in which the obstruction was bypassed. AP is a rare condition characterized by a band of pancreatic tissue that encircles the second part of the duodenum. Most cases of AP in adults remain asymptomatic. However, when AP is symptomatic, it is associated with vague abdominal symptoms. The primary management of symptomatic AP in adults involves surgical bypass of the annulus through performing gastrojejunostomy or duodenojejunostomy. While duodenoduodenostomy is less favorable, we opted for it due to the limitation of the obstruction to a specific segment of the duodenum. This case underscores the importance of considering AP as a potential cause in the differential diagnosis of vague and persistent gastrointestinal symptoms. Moreover, most studies concerning the management of AP have consisted of case reports or small case series. This emphasizes the need for further studies to enhance our understanding of the most appropriate approach for managing each case of AP. • Annular pancreas (AP) is a rare congenital anomaly that is not usually associated with symptoms in adults. • In symptomatic cases, AP causes non-specific symptoms such as abdominal pain. • AP is diagnosed by laparotomy or radiologic studies such as CT in adults. • Duodenoduodenostomy may be an effective approach in managing of AP in adults. [ABSTRACT FROM AUTHOR]

The present study aims to report a rare case of obstructive choledocholithiases found in an elderly female with heterotaxy syndrome (HS) with polysplenia, and to review the available literature on hepatobiliary pathologies associated with this syndrome. We present the case of an 82-year-old female patient with multiple comorbidities who presented to our hospital with abdominal pain and jaundice. An abdominal ultrasound and subsequent computed tomography showed several obstructive choledocholithiases. Incidentally, an abnormal visceral arrangement consistent with HS with polysplenia was observed, including midline liver, semi-annular pancreas, intestinal malrotation, preduodenal portal vein and other vascular anomalies. Accordingly, the patient was diagnosed with cholangitis secondary to obstructive choledocholithiases and HS with polysplenia. She was admitted to the hospital for endoscopic retrograde cholangiopancreatography after medical stabilization. Unfortunately, her condition rapidly worsened and died a few hours after hospitalization due to septic shock. Only one case of choledocholithiases in HS has been previously reported in the literature. It is unclear whether the abnormal arrangement of abdominal organs may have a significant role in the development of this complication. Although biliary drainage might be hindered in the presence of anatomical variants such as (semi-)annular pancreas, intestinal malrotation and preduodenal portal vein, further studies are needed to determine if there is an association between these anomalies and the development of choledocholithasis. [ABSTRACT FROM AUTHOR]

Abstract Annular pancreas is a congenital abnormality in which part of the pancreatic head completely or partially surrounds the duodenum in a ring‐like manner. The condition is thought to be an abnormality of the ventral pancreatic bud. While pancreatitis is a common complication of the annular pancreas, its recurrence may be prevented by improving the outflow of pancreatic juice. The present case report describes a 23‐year‐old woman who had been referred to our hospital for recurrent pancreatitis since childhood. An endoscopic incision was made on the orifice of the annular pancreas, after which pancreatitis of the annular pancreas did not recur for 6 years. The patient subsequently exhibited pancreatolithiasis in the dorsal pancreatic duct, which was successfully treated with endoscopic treatment. Endoscopic pancreatic sphincterotomy may prevent the recurrence of pancreatitis and avoid further surgical interventions by improving the flow of pancreatic juice.

Purpose: We aimed to evaluate possible positive and negative effects of postoperative use of transanastomotic feeding tube (TAFT) in neonates operated for congenital duodenal obstruction (CDO). Methods: This is a retrospective study reviewing medical records of neonates operated for CDO during 2003–2020 and comparing postoperative feeding outcomes and complications in patients with and without TAFT. Approval from the hospital's data protection officer was obtained. Results: One hundred patients, 59% girls, were included, and 37% received TAFT. Mean birth weight and gestational age were 2628 (675.1) grams and 36.6 (2.4) weeks, respectively. Furthermore, 45% had no other malformations, and 36% had Down syndrome. Patient demographics were similar for TAFT and not-TAFT patients, except that not-TAFT neonates weighed median 335 g less (p = 0.013). The TAFT group got parenteral nutrition 2 days shorter (p < 0.001) and started enteral feeds 1.5 days earlier (p < 0.001) than the not-TAFT group. Fewer neonates with TAFT got a central venous catheter [65 vs 89%, (p = 0.008)]. In the TAFT group, 67% were breast fed at discharge compared to 49% in the not-TAFT group (p = 0.096). Conclusion: Neonates with TAFT had earlier first enteral feed, fewer days with parenteral nutrition and fewer placements of central venous catheters. [ABSTRACT FROM AUTHOR]

Background and purpose: To investigate annular pancreas in adults using imaging features displayed on computed tomography (CT) and magnetic resonance imaging (MRI). Methods: Retrospective review of annular pancreas in patients undergoing CT or MRI examinations. Two abdominal radiologists blindly reviewed the CT, MRI, and magnetic resonance cholangiopancreatography (MRCP) images from the Picture Archiving and Communication Systems (PACS). A Kruskal–Wallis test was performed to evaluate subjective scoring, with Mann–Whitney test for the comparison. A p‐value less than 0.05 was considered statistically significant. Results: Eleven patients (45.8%) presented a complete ring of pancreatic tissue surrounding duodenum, displayed as circular, triangular, or sandwich sign configuration, the other 13 patients (54.2%) had incomplete annular pancreas which displayed a crocodile jaw appearance, pancreatic tissue extending in a posterolateral or anterolateral direction towards duodenum. When comparing CT images of each sequence, the arterial phase group had the highest score compared with the venous phase and the plain film group (χ2 = 58.21, p < 0.05). When comparing MRI enhancement volumetric interpolated breath‐hold examination (VIBE) sequences, arterial phase group scores were the highest (χ2 = 18.98, p < 0.05). Conclusions: Both CT and MRI could detect annular pancreas, with artery phase being the best sequence to diagnose annular pancreas. [ABSTRACT FROM AUTHOR]

Background: Annular pancreas is a rare, congenital, rotational anomaly of pancreas, seen usually in newborns who present with features of duodenal obstruction. However, in adults, only 24% of cases are present with duodenal obstruction. Surgery remains the procedure of choice in patients in whom symptoms can be attributed to duodenal obstruction and the goal of surgery is to relieve obstruction by bypassing the annulus. Laparoscopic Roux-en Y duodenostomy (DJ) is our preferred bypass approach for this condition. Literature search revealed that very few case reports have been published about laparoscopic management of annular pancreas, especially about duodenojejunal anastomosis. We present our experience in the laparoscopic management of symptomatic annular pancreas in adults and technique of the laparoscopic Roux-en Y DJ for annular pancreas. Materials and Methods: Between 1996 and 2016, a total of six adult patients underwent laparoscopic management for symptomatic annular pancreas. The demographic, perioperative and follow-up details were documented. Results: All surgeries were successfully performed by laparoscopic approach with no conversion to open. Five cases underwent Roux-en Y DJ and one underwent gastrojejunostomy. No major perioperative events occurred. The mean length of hospital stay was 5.6 days. Five out of six patients were followed up for 24 months, and no symptom recurrence was seen. Conclusion: Laparoscopic Roux-en Y duodenojejunostomy could be used as a safe and physiological treatment for annular pancreas in adult patients and should be preferred for the treatment of duodenal obstruction due to annular pancreas.

Background: Congenital duodenal obstruction (CDO) is commonly detected antenatally through the presence of the "double bubble" sign on prenatal ultrasound, denoting dilatation of the stomach and duodenum. Subsequent postnatal ultrasonography plays a pivotal role in determining the causes of obstruction, thereby informing surgical strategies and neonatal management. The aim of this study was to investigate the diagnostic accuracy of postnatal ultrasonography in comparison to that of prenatal ultrasound and surgical findings in a cohort of 43 patients with fetal double bubble sign., Methods: A total of 43 patients, comprising 24 males and 19 females, who exhibited double bubble sign on prenatal ultrasound were subjected to postnatal ultrasound assessment at a tertiary care facility during the 2018-2023 period. The accuracy of both pre-and postnatal ultrasonography in the identification and diagnosis of CDO, as well as its underlying causes, was compared to that of the established gold standard of surgical findings., Results: The accuracy rates for prenatal and postnatal ultrasonic diagnosis of CDO were 97.7% (42/43) and 100% (42/42), respectively. In terms of etiological diagnosis, prenatal and postnatal ultrasound correctly identified the causes of obstruction in 45.2% (19/42) and 81.0% (34/42) of cases, respectively, as confirmed by surgical intervention., Conclusions: The presence of the prenatal double bubble sign serves as a highly reliable indicator for CDO. Additionally, postnatal ultrasonography proved to be a valuable tool in refining the diagnosis and determining the underlying causes of obstruction in neonates., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://qims.amegroups.com/article/view/10.21037/qims-24-445/coif). The authors have no conflicts of interest to declare., (2024 Quantitative Imaging in Medicine and Surgery. All rights reserved.)

Annular pancreas is a rare congenital disorder, which is characterized by partial or complete pancreatic tissue surrounding the second part of the duodenum. It can be diagnosed antenatally on ultrasound. We are reporting a case where a double bubble sign on an antenatal scan was seen, which was followed up after the delivery of the baby, and radiography also confirmed the findings. Both investigations suggested duodenal obstruction (duodenal atresia and annular pancreas were kept as possible causes). The child was taken for surgery and pancreatic tissue surrounding the second part of the duodenum was seen, confirming the diagnosis of annular pancreas., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Panpalia et al.)

Annular pancreas in adults is a rare congenital abnormality, often detected after the onset of complications or incidentally during autopsy. Diagnosis in adults is challenging due to the similarity of symptoms with other conditions. We report a case of a 55-year-old female who presented with a six-month history of intermittent colicky pain in the epigastric region, radiating to the back, accompanied by periodic nonbilious vomiting. Abdominal examination revealed mild tympany and succussion splash in the epigastric region, with no organomegaly, lumps, or visible peristalsis. The patient was treated successfully with gastrojejunostomy (GJ) and jejunojejunostomy (JJ), leading to a good recovery. This case highlights the importance of considering the annular pancreas in the differential diagnosis of adults presenting with similar symptoms, underscoring the diagnostic challenges faced by radiologists and surgeons., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Ali et al.)

Introduction: Duodenal obstruction due to annular pancreas is a rare entity. Among the causes of neonatal obstruction described by Robert Gross, annular pancreas comprises less than 1% of total group (Gross and Chisholm, 1944) [1]. Herewith, we present a case of annular pancreas causing duodenal obstruction in a neonate for which we performed laparoscopic duodeno-jejunostomy and placed a trans anastomotic naso-jejunal feeding tube. Technique description: A 2-day old neonate presented with complaints of bilious vomiting since birth. Clinical findings and radiological imaging were suggestive of duodenal obstruction. The patient was planned for diagnostic laparoscopy. Intraoperatively, a large annular pancreas was noted to obstruct the second part of duodenum. Duodeno-jejunal anastomosis was performed using polydioxanone 5-0 in a single layer. After completion of the posterior layer, a 6Fr infant feeding tube was placed trans anastomotic reaching mid jejunum. The patient tolerated the procedure well and feeds were started on POD 2 via the naso-jejunal trans-anastomotic tube. Conclusion: Laparoscopic duodeno-jejunostomy with the placement of a trans-anastomotic tube is beneficial for the patient as feeds can be started within 48 hours or even earlier. Moreover, laparoscopic approach increases precision in dissection due to greater magnification, decreases overall morbidity and improves cosmesis. Laparoscopic duodeno-jejunostomy, though technically challenging, is feasible in the neonatal age group.