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Journal of Radiology Case Reports Mar 2020The underlying etiologies of paediatric bowel obstruction are wide ranging. It can be divided into proximal and distal bowel obstruction. Amongst the different...
The underlying etiologies of paediatric bowel obstruction are wide ranging. It can be divided into proximal and distal bowel obstruction. Amongst the different etiologies of the proximal bowel obstructions at the level of the duodenum, there are a few etiologies including duodenal atresia, internal hernias, intestinal malrotation, annular pancreas etc. Superior mesenteric artery syndrome is amongst one of these differential diagnoses which is more prevalent in the adolescent age group. We describe the imaging features of this entity and its demographics, imaging characteristics, treatment and prognosis.
Topics: Adolescent; Diagnosis, Differential; Duodenal Obstruction; Duodenum; Humans; Intestinal Atresia; Superior Mesenteric Artery Syndrome
PubMed: 33082917
DOI: 10.3941/jrcr.v14i3.3830 -
ANZ Journal of Surgery Jul 2021The aim of this report is to present our experience of the last 5 years with repairs of congenital annular pancreas in neonates to determine the efficacy and outcomes...
BACKGROUND
The aim of this report is to present our experience of the last 5 years with repairs of congenital annular pancreas in neonates to determine the efficacy and outcomes of laparoscopic side-to-side duodenoduodenostomy compared to laparoscopic diamond-shaped anastomosis.
METHODS
A total of 35 patients with congenital annular pancreas in neonates were operated by laparoscopic procedure at our hospital during January 2015 and September 2020. Twenty patients underwent laparoscopic side-to-side duodenoduodenostomy (group A), and 15 patients of the control group underwent laparoscopic diamond-shaped anastomosis (group B). The clinical data between the two groups were compared.
RESULTS
The operative time is 77.5 ± 18.7 min in group A (range 50 ~ 125 min), and 92.5 ± 20.2 min in group B (range 75-155 min) (P = 0.029). Feedings started on postoperative day 4.5 ± 0.9 (range 3-6 days) in group A, whereas 6.5 ± 0.8 (range 4-7 days) in group B (P = 0.013). The cases in group A were discharged uneventfully in a mean of 9.2 ± 2.3 (range 7-15 days) postoperative day, and 11.4 ± 3.7 days (range 8-20 days) in group B (P = 0.041). The cases of group A have been followed up for 33.1 ± 15.4 months (range 3-60 months), group B had been followed up for 32.0 ± 14.0 months (range 6-55 months) (P > 0.05), and all the cases were doing well at the last follow-up examination.
CONCLUSION
Laparoscopic side-to-side duodenoduodenostomy is beneficial to the recovery of intestinal function postoperatively in the neonate with annular pancreas.
Topics: Anastomosis, Surgical; Humans; Infant, Newborn; Laparoscopy; Pancreas; Pancreatic Diseases; Retrospective Studies; Treatment Outcome
PubMed: 34013592
DOI: 10.1111/ans.16959 -
Acta Medica Okayama Feb 2023An annular pancreas is a rare anomaly of the pancreas, defined as pancreatic tissue that totally or partly encircles the duodenum, usually the descending portion. A...
An annular pancreas is a rare anomaly of the pancreas, defined as pancreatic tissue that totally or partly encircles the duodenum, usually the descending portion. A 76-year-old man who was diagnosed with gastric cancer cT3N0M0 Stage IIB underwent laparoscopic distal gastrectomy with D2 lymph node dissection. Intraoperatively, the dorsal half of the duodenal bulb was seen to be half surrounded by the pancreas, and a non-typical annular pancreas was diagnosed. Because of the risk to the pancreas, it was considered impossible to perform anastomosis by a linear stapler as in the usual laparoscopic procedure. Therefore, we performed laparoscopically assisted distal gastrectomy and Billroth-I reconstruction using a circular stapler, and the surgery was completed without difficulties. His postoperative course was good despite the development of a pancreatic fistula, which was an International Study Group for Pancreas Fistula biochemical leak. Some APs can be diagnosed preoperatively, but the rarer subtypes such as ours are more difficult to visualize on imaging. In gastrectomy, it is both oncologically important and technically challenging to perform lymph node dissection around the pancreas. In this case with an especially proximal pancreas, a circular stapler was considered better suited for gastroduodenal anastomosis and required a broader field than that afforded by laparoscopy. A case of non-typical annular pancreas diagnosed during laparoscopic gastric surgery is described.
Topics: Male; Humans; Aged; Laparoscopy; Pancreas; Gastrectomy; Stomach Neoplasms
PubMed: 36849152
DOI: 10.18926/AMO/64368 -
Surgical Oncology Jun 2022Portal annular pancreas (PAP), also called circumportal pancreas, is a congenital pancreatic anomaly in which the portal and/or mesenteric veins are surrounded by...
BACKGROUND
Portal annular pancreas (PAP), also called circumportal pancreas, is a congenital pancreatic anomaly in which the portal and/or mesenteric veins are surrounded by pancreatic parenchyma [1,2]. Joseph et al. classified PAP into three types (according to the fusion pattern of the pancreatic parenchyma and ductal system [1]), each of which they subdivided (based on the relation to the portal confluence) into the suprasplenic, infrasplenic, and mixed type [1,3]. The most common type is IIIa [1,4], where the portal vein (suprasplenic) is encased by the uncinate process with an anteportal main pancreatic duct.
METHODS
The patient was a 78-year-old woman who had undergone left nephrectomy for renal cell carcinoma five years prior. We performed laparoscopic pancreatoduodenectomy for a metastatic tumor of the head of a type IIIa PAP (Fig. 1). The anteportal pancreas was transected, and dissection was performed around the superior mesenteric artery using a right approach. The retroportal pancreas was transected using a linear stapler with bioabsorbable polyglycolic acid felt. We performed pancreatojejunostomy for the anteportal stump of the pancreas containing a main pancreatic duct; the retroportal stump was not reconstructed, because it had no major pancreatic ducts on preoperative imaging.
RESULTS
The operative time was 505 minutes, and the blood loss was 70 ml. The postoperative course was uneventful, and the patients was discharged on postoperative day 12.
CONCLUSION
Laparoscopic pancreatoduodenectomy was performed successfully in a patient with a type IIIa PAP. The retroportal pancreas can be transected using a linear stapler, without reconstruction.
Topics: Aged; Carcinoma, Renal Cell; Female; Humans; Kidney Neoplasms; Laparoscopy; Neoplasms, Second Primary; Pancreas; Pancreatic Diseases; Pancreatic Neoplasms; Pancreaticoduodenectomy; Portal Vein
PubMed: 35462237
DOI: 10.1016/j.suronc.2022.101772 -
Current Medical Imaging Nov 2023Fetal small bowel obstruction (SBO) is a serious condition with high morbidity and mortality rates. Prenatal ultrasound is an important tool for detecting SBO, but the...
BACKGROUND
Fetal small bowel obstruction (SBO) is a serious condition with high morbidity and mortality rates. Prenatal ultrasound is an important tool for detecting SBO, but the optimal cutoff value for intestinal diameter remains undefined.
OBJECTIVE
This study aimed to investigate the ultrasonic characteristics of fetal SBO and determine the optimal cutoff value for intestinal diameter to enhance prenatal ultrasound diagnosis.
METHODS
We retrospectively analyzed the ultrasonic characteristics and postpartum data of 76 cases diagnosed with SBO. Receiver operating characteristic (ROC) curve analysis was performed to identify the optimal cutoff value for dilated intestinal diameter.
RESULTS
Among the 76 cases, 31 displayed the "double bubble sign" on ultrasound, with 20 cases identified as annular pancreas, 6 as duodenal atresia, and 5 as duodenal membranous stenosis. In 45 cases, the lesions were located in the jejunal or ileal segment and exhibited intestinal dilatation above the lesion site, including 27 cases of small bowel atresia, 7 cases of membranous jejunal stenosis, and 11 cases of small bowel volvulus. Out of the 76 cases, 9 showed no abnormalities after birth. ROC curve analysis determined optimal cutoff values of 17.5mm and 10.5mm for predicting "double bubble sign" lesions in the gastric and duodenal widths. For predicting small intestinal dilatation, the optimal cutoff values for dilated width and length of the intestinal tube were 11.5mm and 21.5mm, respectively, with high sensitivity and specificity.
CONCLUSION
Ultrasonic imaging and changes in intestinal diameter provide valuable information for prenatal diagnosis and management of SBO. Establishing these cutoff values can improve the accuracy of prenatal ultrasound diagnosis for SBO.
PubMed: 37957877
DOI: 10.2174/0115734056262425231031171130 -
Frontiers in Pediatrics 2024A congenital duodenal diaphragm (CDD) is a rare cause of duodenal obstruction in infants and children. The traditional approach to treatment has been surgical...
BACKGROUND
A congenital duodenal diaphragm (CDD) is a rare cause of duodenal obstruction in infants and children. The traditional approach to treatment has been surgical intervention either with excision and duodenoplasty or with bypass through a duodenoduodenostomy, which is invasive and carries the risk of anastomotic leakage.
AIMS
To summarize the key points of differential diagnosis and treatment of a CDD under gastroscopy and to evaluate its efficacy and safety.
METHODS
The clinical data of patients with a duodenal obstruction diagnosed and treated by gastroscopy in our hospital between January 2019 and December 2022 were retrospectively analyzed. The gastroscopic features of the CDD were summarized. The clinical outcomes of patients with CDD treated by gastroscopy were collected and investigated.
RESULTS
A total of 13 children with a duodenal obstruction underwent a gastroscopic examination and/or treatment, and of these, 10 were diagnosed as having a CDD and 3 were finally diagnosed as having an annular pancreas. A dome-shaped structure during inspiration (9/10) and a guidewire that could be inserted through the opening into the distal lumen (6/10) were the gastroscopic features of the CDD, while the annular pancreas had none of these features. The 10 patients, 4 men and 6 women with the CDD, were treated through endoscopic diaphragmotomy and dilation. The age and weight at operation was 15 days to 7 years (M: 2.25 years) and 2.85-22 kg (M: 13.6 kg), respectively. A total of 11 endoscopic operations were performed in the 10 patients. The time of single operation was 15-55 min (M: 38 min). The patients did not experience complications such as bleeding, pneumoperitoneum, and duodenal papilla injury during the operation. Feeding was started 12-24 h after surgery, and the patients were discharged 2-10 days (M: 7 days) postoperatively. The patients were followed up for 3-36 months (M: 17 months), and none of them had a recurrence of vomiting. However, three showed a recurrence of stenosis in upper gastrointestinal imaging, one of whom underwent a partial diaphragm resection again 2 months after the primary treatment.
CONCLUSIONS
A CDD can be confirmed by endoscopy based on its characteristic features. Endoscopic diaphragmotomy and balloon dilation are safe and effective, which can be a new option for minimally invasive treatment of a CDD.
PubMed: 38445081
DOI: 10.3389/fped.2024.1298748 -
Journal of Korean Medical Science Oct 2023In Korea, there have been no reports comparing the prevalence of major congenital anomalies with other countries and no reports on surgical treatment and long-term...
BACKGROUND
In Korea, there have been no reports comparing the prevalence of major congenital anomalies with other countries and no reports on surgical treatment and long-term mortality. We investigated the prevalence of 67 major congenital anomalies in Korea and compared the prevalence with that of the European network of population-based registries for the epidemiological surveillance of congenital anomalies (EUROCAT). We also investigated the mortality and age at death, the proportion of preterm births, and the surgical rate for the 67 major congenital anomalies.
METHODS
Korean National Health Insurance claim data were obtained for neonates born in 2013-2014 and admitted within one-year-old. Sixty-seven major congenital anomalies were defined by medical diagnoses classified by International Classification of Diseases-10 codes according to the EUROCAT definition version 2014. Mortality and surgery were defined if any death or surgery claim code was confirmed until 2020. Poisson distribution was used to calculate the 95% confidence interval of the congenital anomaly prevalence.
RESULTS
The total prevalence of the 67 major anomalies was 433.5/10,000 livebirths. When compared with the prevalence of each major anomaly in EUROCAT, the prevalence of spina bifida, atrial septal defect (ASD), congenital megacolon, hip dislocation and/or dysplasia and skeletal dysplasia were more than five times higher in Korea. In contrast, the prevalence of aortic atresia/interrupted aortic arch and gastroschisis was less than one-fifth in Korea. The proportion of preterm births was 15.7%; however, more than 40% of infants with anencephaly, annular pancreas and gastroschisis were preterm infants. Additionally, 29.2% of the major anomalies were admitted to the neonatal intensive care units at birth, and 25.6% received surgical operation. The mortality rate was 1.7%, and 78.2% of the deaths occurred within the first year of life. However, in neonates with tricuspid valve atresia and stenosis, duodenal atresia or stenosis, and diaphragmatic hernia, more than half died within their first month of life. ASD and ventricular septal defect were the most common anomalies, and trisomy 18 and hypoplastic left heart syndrome were the most fatal anomalies. All infants with aortic atresia/interrupted aortic arch and conjoined twins received surgery.
CONCLUSION
The proportion of surgeries, preterm births and mortality was high in infants with major congenital anomalies. The establishment of a national registry of congenital anomalies and systematic support by national medical policies are needed for infants with major congenital anomalies in Korea.
Topics: Infant; Female; Infant, Newborn; Humans; Child; Gastroschisis; Premature Birth; Constriction, Pathologic; Infant, Premature; Republic of Korea; Aortic Diseases; Congenital Abnormalities; Registries; Prevalence
PubMed: 37821084
DOI: 10.3346/jkms.2023.38.e304 -
Frontiers in Pediatrics 2022Primary ciliary dyskinesia (PCD) is a rare genetic disorder characterized by motile ciliary dysfunction and impaired ultrastructure. Despite numerous studies, the...
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 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 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 splicing disruption. Thus, in summary, we identified two mutations of the gene that can be considered pathogenic compound heterozygous mutations in a case of familial PCD, thereby expanding the known mutational spectrum of the gene in this disease.
PubMed: 36245716
DOI: 10.3389/fped.2022.996332 -
Radiology Case Reports Jun 2022VACTERL Syndrome affects multiple body systems and can include various anomalies of the Vertebral column, Anus and/or rectum, heart (Cardiac), Tracheo-Esophagus, kidneys...
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.
PubMed: 35401909
DOI: 10.1016/j.radcr.2022.02.062 -
Surgical Oncology Jun 2021A fundamental aspect of oncosurgical planning in organ resections is the identification of feeder vessel details to preserve healthy organ tissue while fully resecting...
BACKGROUND
A fundamental aspect of oncosurgical planning in organ resections is the identification of feeder vessel details to preserve healthy organ tissue while fully resecting the tumors. The purpose of this study was to determine whether three-dimensional (3D) cancer case models of computed tomography (CT) images will assist resident-level trainees in making appropriate operative plans for organ resection surgery.
METHODS
This study was based on the perception of surgery residents who were presented with 5 different oncosurgical scenarios. A five-station carousel including cases of liver mass, stomach mass, annular pancreas, pelvic mass and mediastinal mass was formed for the study. The residents were required to compare their perception level of the cases with their CT images, and 3D models in terms of identifying the invasion of the mass, making differential diagnosis and preoperative planning stage.
RESULTS
All residents have given higher scores for models. 3D models provided better understanding of oncopathological anatomy and improved surgical planning. In all scenarios, 70-80% of the residents preferred the model for preoperative planning. For surgical choice, compared to the CT, the model provided a statistically significant difference in terms of visual assessment, such as tumor location, distal or proximal organotomy (p:0.009). In the evaluation of presacral mass, the perception of model was significantly better than the CT in terms of bone-foramen relationship of chondrosarcoma, its origin, geometric shape, localization, invasion, and surgical preference (p:0.004). The model statistically significantly provided help to evaluate and prepare the case together with the colleagues performing surgery (p:0.007). Commenting on the open-ended question, they stated that the tumor-vessel relationship was clearly demonstrated in the 3D model, which has been very useful.
CONCLUSIONS
With the help of 3D printing technology in this study, it is possible to implement and evaluate a well-structured real patient scenario setup in cancer surgery training. It can be used to improve the understanding of pathoanatomical changes of multidisciplinary oncologic cases. Namely, it is used in guiding the surgical strategy and determining whether patient-specific 3D models change pre-operative planning decisions made by surgeons in complex cancer mass surgical procedures.
Topics: Humans; Imaging, Three-Dimensional; Internship and Residency; Liver Neoplasms; Mediastinal Neoplasms; Models, Anatomic; Pancreas; Pancreatic Diseases; Printing, Three-Dimensional; Sacrum; Spinal Neoplasms; Stomach Neoplasms; Surgical Oncology; Tomography, X-Ray Computed
PubMed: 33711767
DOI: 10.1016/j.suronc.2021.101537