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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 -
Case Reports in Gastroenterology 2024Portal annular pancreas (PAP) is a congenital anomaly resulting from aberrant fusion of the ventral and dorsal pancreatic buds around the portal vein (PV). PAP was...
INTRODUCTION
Portal annular pancreas (PAP) is a congenital anomaly resulting from aberrant fusion of the ventral and dorsal pancreatic buds around the portal vein (PV). PAP was classified into three types by Joseph et al., based on the location of the main pancreatic duct around the PV. The presence of PAP is important for the surgical procedure because it is associated with the postoperative pancreatic fistula. There are no standardized surgical procedures of resection and reconstruction for PAP.
CASE PRESENTATION
We report 2 cases of subtotal stomach-preserving pancreatoduodenectomy in patients with PAP. One case of PAP was discovered coincidentally intraoperatively, and the other case was diagnosed before surgery. The first case was an 84-year-old male patient who underwent surgery for distal bile duct cancer. PAP was noticed intraoperatively when the uncinate process of the pancreas was detached from behind the PV. The second case was an 84-year-old female patient who also underwent surgery for distal bile duct cancer. We recognized PAP from preoperative computed tomography images. In both cases, the ductal anatomy was consistent with type IIIA PAP, and the dorsal pancreas was resected using a stapling device. During the postoperative period, there was no clinically relevant postoperative pancreatic fistula.
CONCLUSION
PAP is rarely encountered intraoperatively; however, it is important to recognize it before surgery and take it into consideration when deciding upon the procedures for resection and reconstruction.
PubMed: 38628808
DOI: 10.1159/000538370 -
African Journal of Paediatric Surgery :... 2023Annular 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...
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.
Topics: Female; Infant, Newborn; Humans; Child; Infant; Duodenal Obstruction; Duodenum; Pancreas; Vomiting
PubMed: 36722572
DOI: 10.4103/ajps.ajps_86_21 -
Medicine Dec 2021Portal annular pancreas (PAP) is a rare pancreatic anomaly characterized by portal vein encasement in the pancreatic parenchyma. Due to its rarity, PAP may often be...
RATIONALE
Portal annular pancreas (PAP) is a rare pancreatic anomaly characterized by portal vein encasement in the pancreatic parenchyma. Due to its rarity, PAP may often be missed on preoperative computed tomography (CT) review, and surgeons may face challenges in dealing with an unexpected intraoperative encounter with PAP. We documented 2 such intraoperatively diagnosed cases and illustrated their surgical management.
PATIENTS CONCERNS
In case 1, a 70-year-old man was found to have a 15-mm mass in the pancreatic body and dilatation of the peripheral main pancreatic duct on enhanced CT. Case 2 involved a 46-year-old woman with a history of familial adenomatous polyposis, and rectal cancer with a mass in the duodenal papilla.
DIAGNOSES
The patient in case 1 was diagnosed with resectable pancreatic cancer. In case 2, the patient was diagnosed with duodenal papillary carcinoma.
INTERVENTIONS
In case 1, the patient underwent distal pancreatectomy with lymph node dissection. In case 2, the patient underwent pancreaticoduodenectomy. Intraoperatively, PAP was observed in both cases. In case 1, after the usual transection at the right border of the portal vein, an additional dissection was performed on the dorsal pancreas using a powered linear stapler. In case 2, an additional section was made in the pancreatic body caudal to the cricoid pancreatic junction so that the pancreatic cross-section was oriented in 1 plane.
OUTCOMES
The patient in case 1 was discharged without complications. In case 2, although the patient had a grade-B pancreatic fistula (International Study Group of Pancreatic Fistula Classification), the patient recovered conservatively and was discharged without significant complications. In both cases, a retrospective review identified PAP in patients' preoperative CT images.
LESSONS
Both cases required ingenuity during pancreatectomy. Awareness about PAP and its management will enable surgeons to prepare for unexpected encounters with the condition. Moreover, surgeons (especially pancreatic surgeons) should consider the possibility of PAP while managing pancreatic anomalies to make appropriate treatment decisions.
Topics: Aged; Female; Humans; Lymph Node Excision; Male; Middle Aged; Pancreas; Pancreatectomy; Pancreatic Diseases; Pancreatic Fistula; Pancreatic Neoplasms; Pancreaticoduodenectomy; Portal Vein; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 34918681
DOI: 10.1097/MD.0000000000028204 -
Surgical Case Reports May 2020Portal annular pancreas (PAP) is a rare pancreatic anomaly in which the uncinate process wraps annularly around the portal vein and fuses to the body of the pancreas....
BACKGROUND
Portal annular pancreas (PAP) is a rare pancreatic anomaly in which the uncinate process wraps annularly around the portal vein and fuses to the body of the pancreas. PAP is highly relevant to the development of postoperative pancreatic fistula (POPF) in pancreatic surgery. Here, we describe our experience and surgical technique of laparoscopic spleen-preserving distal pancreatectomy using Warshaw's procedure for patients with the PAP.
CASE PRESENTATION
A 49-year-old woman with PAP was referred to our hospital for a suspicious mucinous cystic neoplasms 1.5 cm in diameter in the pancreatic tail. Laparoscopic spleen-preserving distal pancreatectomy using Warshaw's procedure was performed. Mobilization of the pancreatic tail was first performed, and then, the splenic artery was cut. After dividing the pancreatic tail from the splenic hilum, the ventral pancreatic parenchyma was divided using a stapler. After cutting the splenic vein, complete mobilization of the pancreatic body and tail enabled easy division of the PAP. Finally, the PAP was also divided using the stapler. Although grade B POPF occurred, she was discharged on the 9th postoperative day.
CONCLUSIONS
Surgeons should understand the anatomical characteristics of PAP and be aware of the possibility of POPF.
PubMed: 32358636
DOI: 10.1186/s40792-020-00852-7 -
Journal of Indian Association of... 2021
PubMed: 34728929
DOI: 10.4103/jiaps.JIAPS_7_21 -
Cureus Mar 2024Lymphatic malformations frequently present as benign masses in the neck and clavicle region among infants and young children. Cystic hygroma represents an...
Lymphatic malformations frequently present as benign masses in the neck and clavicle region among infants and young children. Cystic hygroma represents an often-encountered form of lymphatic malformation. This case report details the medical history of a one-year-old girl characterized by a multifaceted medical background, initially exhibiting symptoms of persistent cough, cold, and neck swelling. Further investigations revealed more severe conditions: complex congenital heart defects, including large atrial septal defect (ASD), large ventricular septal defect (VSD), and aorta arising from the right ventricle with cystic hygroma and annular pancreas. The patient underwent various diagnostic tests, including chest X-rays, ultrasound, magnetic resonance imaging (MRI), and computed tomography pulmonary angiogram (CTPA), leading to multidisciplinary treatment involving sclerotherapy for cystic hygroma and supportive therapies. The case underscores the challenges in diagnosing and managing pediatric patients with overlapping conditions and the critical need for continuous follow-up.
PubMed: 38659571
DOI: 10.7759/cureus.56852 -
International Journal of Surgery Case... 2020Pancreatitis is a dire clinical diagnosis with variable presentation in the paediatric population. Moreover, neonatal pancreatitis has been rarely reported in the...
INTRODUCTION
Pancreatitis is a dire clinical diagnosis with variable presentation in the paediatric population. Moreover, neonatal pancreatitis has been rarely reported in the English literature.
PRESENTATION OF CASE
A newborn, product of a poorly supervised, pre-term gestation with pre-natally diagnosed intestinal obstruction, and post-natal clinical features of jaundice, vomiting, abdominal distension, aphonation and suspected chromosomal abnormalities. There was maternal hepatitis which was untreated. Diagnosed as duodenal atresia, the baby was investigated, resuscitated and had surgery. Intra-operative findings were of an omental bubble, duodenal stenosis with annular pancreas, coagulative necrosis of the pancreas and multiple intra peritoneal cheesy deposits. Following an unfortunate demise, autopsy confirmed pancreatitis and multiple congenital abnormalities.
DISCUSSION
Paediatric caregivers should be aware of the possibility of neonatal pancreatitis in jaundiced newborns with intestinal obstruction especially with a background of maternal viraemia.
CONCLUSION
A constellation of unusual presentations as highlighted could be a pointer to an emerging syndrome. All paediatric caregivers should entertain a high index of suspicion of pancreatitis in such a case, investigate and expedite appropriate interventions to prevent mortality.
PubMed: 33395874
DOI: 10.1016/j.ijscr.2020.11.114