-
American Journal of Medical Genetics.... Feb 2024
Topics: Humans; Pancreas; Pancreatic Diseases
PubMed: 37789234
DOI: 10.1002/ajmg.a.63432 -
AJR. American Journal of Roentgenology Mar 2022. Use of upper gastrointestinal (UGI) series to diagnose UGI obstruction in neonates and infants has raised concern about increased radiation sensitivity of developing... (Comparative Study)
Comparative Study
Saline-Aided Ultrasound Versus Upper Gastrointestinal Series in Neonates and Infants With Suspected Upper Gastrointestinal Obstruction: A Prospective Multicenter Comparative Study.
. Use of upper gastrointestinal (UGI) series to diagnose UGI obstruction in neonates and infants has raised concern about increased radiation sensitivity of developing organs. . The purpose of this study was to assess the diagnostic performance of saline-aided ultrasound (US) in comparison with UGI series in evaluation for UGI obstruction in neonates and infants. . In this prospective multicenter study at three hospitals, inpatients were enrolled who were younger than 1 year and had suspected UGI obstruction between June 2015 and May 2018; patients with US evidence of malrotation or pyloric stenosis were ineligible. Enrolled patients underwent both saline-aided US (saline solution administered through a nasogastric tube) and UGI series. Surgical findings or at least 1-year of clinical follow-up findings served as the reference for presence of UGI obstruction. UGI obstruction was classified in terms of level (proximal vs distal) and cause. Two radiologists independently interpreted saline-aided US examinations to assess interobserver agreement and then reached consensus. Two other radiologists assessed upper GI series in consensus. Diagnostic performance for the presence and level of UGI obstruction was compared between modalities. Causes of obstruction were assessed with saline-aided US. . A total of 209 neonates were included (116 boys, 93 girls; median age, 5 days; 124 (59.3%) patients had UGI obstruction (proximal in 108 patients). Saline-aided US had strong interobserver agreement for presence (κ = 0.87) and level (κ = 0.85) of obstruction. For presence of UGI obstruction, accuracy, sensitivity, and specificity were 94.7%, 98.4%, and 89.4% for saline-aided US and 89.5%, 95.2%, and 81.2% for UGI series. For obstruction level, accuracy, sensitivity, and specificity were 90.3%, 97.2%, and 56.3% for saline-aided US versus 87.1%, 92.6%, and 50.0% for UGI series. Accuracy for presence was significantly higher for saline-aided US ( = .02); otherwise, these metrics were not different between tests ( > .05). For causes of UGI obstruction (annular pancreas, duodenal web, duodenal atresia, and duodenal stenosis), the accuracy of saline-aided US ranged from 75.0% to 95.2%. . Saline-aided US has high diagnostic performance for presence and level of UGI obstruction in neonates and infants, comparing favorably with UGI series. Saline-aided US may have additional utility in evaluating causes of obstruction. . Saline-aided US may serve as an initial screening modality for UGI obstruction in neonates and infants. . Chinese Clinical Trial Registry ChiCTR-DCC-15006232.
Topics: Female; Gastrointestinal Tract; Humans; Infant, Newborn; Intestinal Obstruction; Male; Prospective Studies; Saline Solution; Ultrasonography
PubMed: 34643106
DOI: 10.2214/AJR.21.26621 -
Journal of the Belgian Society of... 2024A complete annular pancreas, a rare congenital anomaly, can lead to duodenal obstruction, causing recurrent symptoms like vomiting and often requiring surgical...
A complete annular pancreas, a rare congenital anomaly, can lead to duodenal obstruction, causing recurrent symptoms like vomiting and often requiring surgical intervention for relief.
PubMed: 38312145
DOI: 10.5334/jbsr.3432 -
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 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 -
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 -
Fetal and Pediatric Pathology Aug 2022The presence of hepatic parenchyma at ectopic locations is infrequently reported in neonatal age. A male neonate presented with clinical signs and symptoms of duodenal...
The presence of hepatic parenchyma at ectopic locations is infrequently reported in neonatal age. A male neonate presented with clinical signs and symptoms of duodenal obstruction. At exploration, an annular pancreas was found as the causative factor and he underwent a Kimura's duodeno-duodenostomy. A pedicled cyst was attached to the stomach's greater curvature, was excised, and histologically was a mesothelial-lined cyst with ectopic liver, complete with bile ducts, in the cyst wall. Ectopic liver tissue may be clinically silent and found within the wall of a mesothelial cyst. Long-term complications of this ectopic tissue are not known.
Topics: Cysts; Duodenal Obstruction; Duodenostomy; Humans; Infant, Newborn; Liver; Male; Pancreas
PubMed: 33945385
DOI: 10.1080/15513815.2021.1919809