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BMJ Case Reports Feb 2022Duodenal stenosis and atresia are some of the most common forms of congenital bowel obstruction. The gold standard approach to treatment is duodenoduodenostomy, while...
Duodenal stenosis and atresia are some of the most common forms of congenital bowel obstruction. The gold standard approach to treatment is duodenoduodenostomy, while rare, gastrojejunostomy and duodenojejunostomies may still be used. We report a case of a 7-year-old male presenting with annular pancreas with duodenal stenosis that was diagnosed at birth as primary duodenal atresia and repaired by gastrojejunostomy with a Braun enteroenterostomy. Through successful reoperation with dual duodenojejunostomy and subsequent management, we treated the sequelae of the initial repair, including megaduodenum. Regions of duodenal obstruction must have direct anastomotic repair to prevent subsequent issues related to dysmotility. Otherwise, further surgical intervention and long-term medical management, such as the novel strategy reported, may be necessary.
Topics: Child; Duodenal Obstruction; Duodenum; Fetal Diseases; Humans; Infant, Newborn; Intestinal Atresia; Male; Missed Diagnosis; Pancreas; Pancreatic Diseases; Urinary Bladder
PubMed: 35228246
DOI: 10.1136/bcr-2021-248219 -
Journal of Clinical and Diagnostic... Nov 2015Intestinal atresia accounts for approximately one third of all cases of neonatal intestinal obstruction. There is controversy regarding pathogenesis of congenital...
INTRODUCTION
Intestinal atresia accounts for approximately one third of all cases of neonatal intestinal obstruction. There is controversy regarding pathogenesis of congenital atresia and stenosis of small bowel. Studies regarding clinical manifestations and specific histopathological features of neonatal intestinal atresia are scarce in Indian literature.
AIM
To understand the histomorphological features and thus suggest pathophysiology of cases with Intestinal Atresia.
MATERIALS AND METHODS
Out of 147 cases, of intestinal obstruction in newborn studied over a period of 5 years, 39 cases of intestinal atresia were found. Their histomorphological details with clinical manifestations were studied.
RESULTS
Type II was the commonest type of atresia. Associated anomalies noted were gastroschisis, volvulus, anal stenosis, microcolon, annular pancreas, meconium cyst and duplication cyst. Histological changes observed were ulceration, flattening, abnormal villous configuration, luminal obliteration, narrowing, haemangiomatous proliferation of blood vessels, fibrosis, haemorrhage, calcification, and mesenchymal condensation around the blood vessels. Gangrene and perforation has also noted in some cases.
CONCLUSION
An intrauterine intestinal ischemia due to vascular pathology followed by resorption of the bowel is the possible explanation for the development of intestinal atresia.
PubMed: 26674207
DOI: 10.7860/JCDR/2015/13320.6838 -
Surgical and Radiologic Anatomy : SRA May 2021Annular pancreas encountered in adults and jejunal arterial variations are rare. Anatomical variations can cause conflicts between oncology and surgical safety.
PURPOSE
Annular pancreas encountered in adults and jejunal arterial variations are rare. Anatomical variations can cause conflicts between oncology and surgical safety.
METHODS
Case report of a 68-year-old man suffering from vomiting because of an annular pancreas and a ductal adenocarcinoma of the pancreas head invading the second portion of the duodenum.
RESULTS
Contrast-enhanced computed tomography showed multiple arterial variations describing the absence of the coeliac trunk such that the left gastric artery (LGA), splenic artery and superior mesenteric artery (SMA) were arising separately from the aorta. The accessory left hepatic artery arose from the LGA; and both the common hepatic artery and combined trunk of the replaced right hepatic artery with the higher replaced first jejunal artery separately arose close to the root of the SMA. The patient underwent curative pancreaticoduodenectomy which achieved 3 years of recurrence-free survival.
CONCLUSION
This was an extraordinary case of annular pancreas with first jejunal arterial variation detailing an embryological interpretation as well as considerations for balancing short- and long-term outcomes.
Topics: Aged; Carcinoma, Pancreatic Ductal; Computed Tomography Angiography; Humans; Imaging, Three-Dimensional; Male; Mesenteric Artery, Superior; Pancreas; Pancreatic Diseases; Pancreatic Neoplasms; Pancreaticoduodenectomy; Treatment Outcome
PubMed: 33481131
DOI: 10.1007/s00276-020-02671-9 -
Journal of Indian Association of... 2023Bronchoesophageal fistulas (BEFs) are rare malformations characterized by abnormal communication between the bronchus and the esophagus. Herein, we report a successful...
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.
PubMed: 38173639
DOI: 10.4103/jiaps.jiaps_79_23 -
Surgical Case Reports Feb 2019Although annular pancreas concurrent with pancreaticobiliary maljunction has rarely been reported, some reports have pointed out a possibility that both anomalies have a...
BACKGROUND
Although annular pancreas concurrent with pancreaticobiliary maljunction has rarely been reported, some reports have pointed out a possibility that both anomalies have a common pathogenesis in pancreatic development. We herein report a case with pancreaticobiliary maljunction diagnosed long after surgical treatment for annular pancreas.
CASE PRESENTATION
A 34-year-old female, with a surgical history of duodenal obstruction due to annular pancreas in the neonatal period, was referred to our hospital for further examination of chronic pancreatitis. Endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography revealed choledocholithiasis, pancreatic lithiasis, and pancreaticobiliary maljunction without biliary dilatation. Choledocholithotomy and cholecystectomy were performed, and highly elevated levels of amylase in bile from the common bile duct were found intraoperatively.
CONCLUSION
The present case highlights a possible association of pancreaticobiliary maljunction in a patient with annular pancreas.
PubMed: 30707318
DOI: 10.1186/s40792-019-0572-2 -
European Journal of Pediatric Surgery... Jan 2021Communicating bronchopulmonary foregut malformations (CBPFM) are extremely rare. We present a complex case of type IB CBPFM with esophageal atresia and distal...
Communicating bronchopulmonary foregut malformations (CBPFM) are extremely rare. We present a complex case of type IB CBPFM with esophageal atresia and distal tracheoesophageal fistula (EA/TOF), duodenal atresia/annular pancreas (DA/AP), and intestinal malrotation who underwent primary repair for EA/TOF on day 3. Bilious aspirates on day 8 prompted an upper gastrointestinal (GI) contrast revealing a duodenal obstruction and communication between the right lung lower lobe and the esophagus (T8-T9 level). DA/AP and malrotation were repaired by a gastrojejunostomy and Ladd's procedure. A repeat contrast swallow identified a 2nd communication from the esophagus into the right lower lobe (T5-T6 level) raising the suspicion of a recurrent TOF. Computed tomography (CT) thorax confirmed above findings with an anomalous blood supply to right lung. An exploratory thoracotomy identified a three-lobed lung. However, the lower lobe was enlarged and connected in two separate locations to the esophagus. The child recovered after the disconnection of the esophageal connections and partial right lower lobectomy. CBPFM are extremely rare anomalies requiring a high index of suspicion, use of an upper GI contrast series, and CT scans for diagnosis. The treatment of choice is resection of the affected lung and disconnection of the esophageal communications.
PubMed: 34917448
DOI: 10.1055/s-0041-1740321 -
Journal of Pediatric Surgery Jun 2023The purpose of this study was to assess diagnostic accuracy and neonatal outcomes in fetuses with a suspected proximal gastrointestinal obstruction (GIO).
BACKGROUND
The purpose of this study was to assess diagnostic accuracy and neonatal outcomes in fetuses with a suspected proximal gastrointestinal obstruction (GIO).
METHODS
After IRB approval, a retrospective chart review was conducted on prenatally suspected and/or postnatally confirmed cases of proximal GIO at a tertiary care facility (2012-2022). Maternal-fetal records were queried for presence of a double bubble ± polyhydramnios, and neonatal outcomes were assessed to calculate the diagnostic accuracy of fetal sonography.
RESULTS
Among 56 confirmed cases, the median birthweight and gestational age at birth were 2550 g [interquartile range (IQR) 2028-3012] and 37 weeks (IQR 34-38), respectively. There was one (2%) false-positive and three (6%) false-negatives by ultrasound. Double bubble had a sensitivity, specificity, positive predictive value, and negative predictive value for proximal GIO of 85%, 98%, 98%, and 83%, respectively. Pathologies included 49 (88%) with duodenal obstruction/annular pancreas, three (5%) with malrotation, and three (5%) with jejunal atresia. The median postoperative length of stay was 27 days (IQR 19-42). Cardiac anomalies were associated with significantly higher complications (45% vs 17%, p = 0.030).
CONCLUSIONS
In this contemporary series, fetal sonography has high diagnostic accuracy for detecting proximal gastrointestinal obstruction. These data are informative for pediatric surgeons in prenatal counseling and preoperative discussions with families.
LEVEL OF EVIDENCE
Diagnostic Study, Level III.
Topics: Pregnancy; Infant, Newborn; Female; Child; Humans; Retrospective Studies; Digestive System Abnormalities; Ultrasonography, Prenatal; Parturition; Duodenal Obstruction
PubMed: 36907770
DOI: 10.1016/j.jpedsurg.2023.02.029 -
Journal of Surgical Case Reports Apr 2021Annular pancreas is defined by a ring of pancreatic tissue encircling the descending portion of the duodenum. It is exceptionally rare in adults and commonly diagnosed...
Annular pancreas is defined by a ring of pancreatic tissue encircling the descending portion of the duodenum. It is exceptionally rare in adults and commonly diagnosed during the investigation of symptoms arising due to its complications. Treatment usually involves the surgical correction with a duodenoduodenostomy, gastrojejunostomy or duodenojejunostomy. We discuss the case of a 66-year-old male patient who presented with symptoms of gastric outlet obstruction and was found to have an annular pancreas encircling the pylorus and the first and second portions of the duodenum and was treated by performing a gastrojejunostomy. Upper gastrointestinal series, computerized tomography (CT) scans, and magnetic resonance cholangeopancreatographys can all be used for preoperative diagnosis; however, endoscopic retrograde cholangiopancreatography (ERCP) is the diagnostic modality of choice. Nonetheless, many patients may only be diagnosed intraoperatively, especially those who cannot undergo an ERCP due to stenosis proximal to the duodenum or patients in whom the annulus may not be visible on CT scan.
PubMed: 33927863
DOI: 10.1093/jscr/rjab125 -
Nature Communications Oct 2019Human embryonic stem cell-derived beta cells offer a promising cell-based therapy for diabetes. However, efficient stem cell to beta cell differentiation has proven...
Human embryonic stem cell-derived beta cells offer a promising cell-based therapy for diabetes. However, efficient stem cell to beta cell differentiation has proven difficult, possibly due to the lack of cross-talk with the appropriate mesenchymal niche. To define organ-specific niche signals, we isolated pancreatic and gastrointestinal stromal cells, and analyzed their gene expression during development. Our genetic studies reveal the importance of tightly regulated Hedgehog signaling in the pancreatic mesenchyme: inactivation of mesenchymal signaling leads to annular pancreas, whereas stroma-specific activation of signaling via loss of Hedgehog regulators, Sufu and Spop, impairs pancreatic growth and beta cell genesis. Genetic rescue and transcriptome analyses show that these Sufu and Spop knockout defects occur through Gli2-mediated activation of gastrointestinal stromal signals such as Wnt ligands. Importantly, inhibition of Wnt signaling in organoid and human stem cell cultures significantly promotes insulin-producing cell generation, altogether revealing the requirement for organ-specific regulation of stromal niche signals.
Topics: Cell Culture Techniques; Cell Differentiation; Cell- and Tissue-Based Therapy; Diabetes Mellitus; Down-Regulation; Embryonic Stem Cells; Hedgehog Proteins; Humans; Insulin-Secreting Cells; Nuclear Proteins; Organoids; Repressor Proteins; Wnt Proteins
PubMed: 31604927
DOI: 10.1038/s41467-019-12624-5 -
Nigerian Journal of Surgery : Official... 2019Intestinal obstruction in a newborn remains a significant emergency in pediatric surgery. Clinical presentation is often subtle with sudden deterioration of their...
BACKGROUND
Intestinal obstruction in a newborn remains a significant emergency in pediatric surgery. Clinical presentation is often subtle with sudden deterioration of their clinical states. Clinical outcome in the developing countries is poor owing to a variety of factors.
OBJECTIVE
The objective of this study is to identify the factors affecting the management outcome in our environment.
PATIENTS AND METHODS
Data on clinical presentation, management, and outcome of all neonates managed for intestinal obstruction over a 13-year period at a tertiary center in Nigeria were retrospectively reviewed. Analysis of factors affecting the management outcome was also performed.
RESULTS
One hundred and seventeen neonates comprising 85 (72.7%) boys and 32 (27.3%) girls were managed for intestinal obstruction. The age at presentation ranged from 0 to 29 days, with a mean of 6.86 ± 8.4 days. Seventy-five (64.1%) patients presented within a week of onset of symptoms and 42 (35.9%) patients later. Eighty-five patients (72.6%) presented with symptoms from birth. The most common causes of intestinal obstruction included anorectal malformation in 62 (53%) neonates and Hirschsprung's disease in 16 (13.7%) neonates. Other causes included obstructed inguinoscrotal hernias, duodenal atresia, jejunoileal atresia, malrotation, and annular pancreas. Eleven patients died with a mortality rate of 9.4%. The age at presentation ( = 0.001) and the presence of postoperative complications ( = 0.009) were significantly related to the duration of hospital stay. Furthermore, the presence of postoperative complications ( = 0.012) was significantly associated with postoperative mortality.
CONCLUSION
Early presentation and postoperative complications significantly affected the morbidity and mortality associated with the management of neonates with intestinal obstruction.
PubMed: 31579370
DOI: 10.4103/njs.NJS_11_19