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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 -
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 -
Translational Pediatrics Oct 2023Annular pancreas is a rare congenital disorder that requires surgical management once diagnosed. Diamond-shaped and side-to-side duodenoduodenostomy are both popular...
Diamond-shaped versus side-to-side anastomotic duodenoduodenostomy in laparoscopic management of annular pancreas in children: a single-center retrospective comparative study.
BACKGROUND
Annular pancreas is a rare congenital disorder that requires surgical management once diagnosed. Diamond-shaped and side-to-side duodenoduodenostomy are both popular worldwide nowadays in the surgical management of annular pancreas. Here we present our experience with laparoscopic management of annular pancreas in the last 5 years and compare the clinical results of the diamond-shaped versus side-to-side anastomotic techniques.
METHODS
Fifty-two patients diagnosed with annular pancreas who underwent duodenoduodenostomy at our medical center between January 2016 and April 2021 were included in the study. Forty-four patients underwent laparoscopic diamond-shaped duodenoduodenostomy (DS group) and eight underwent laparoscopic side-to-side duodenoduodenostomy (STS group). Clinical data, including surgical indices and early outcomes after surgery, with at least 19 months of follow-up, were collected and analyzed.
RESULTS
Of the 52 patients, 61.5% were prenatally diagnosed, and vomiting was the most common clinical manifestation after birth. The operative time and bleeding volume were 187.5 [interquartile range (IQR), 150-228)] min and 2 (IQR, 2-5) mL in the DS group, compared to 175 (IQR, 155-270) min and 2 (IQR, 2-4.25) mL in the STS group (P=0.89 and 0.32 respectively). The mean time from surgery to initial oral feeding and full oral feeding was 6 (IQR, 4-10) and 12 (IQR, 10-15) days in the DS group, compared to 8 (IQR, 4.75-11.25) and 14.5 (IQR, 13-16.75) days in the STS group (P=0.61 and 0.46 respectively). The mean hospital stay was 16 (IQR, 14-19) and 20 (IQR, 17.75-26) days in the DS and STS groups respectively (P=0.13). No severe complications such as anastomotic leakage, anastomotic stenosis, reoperation or unsuspected rehospitalization were noted in either group. Feeding intolerance was revealed in six cases in the DS group and two cases in the STS group, and there was no significant difference between the two groups (P=0.50).
CONCLUSIONS
Both laparoscopic diamond-shaped and side-to-side techniques showed good clinical results in treating annular pancreas. The surgical technique, trans-anastomotic tube and early feeding are not likely to increase the risk of postoperative feeding intolerance.
PubMed: 37969116
DOI: 10.21037/tp-23-156 -
Asian Journal of Surgery Jun 2019
Topics: Anatomic Variation; Female; Humans; Middle Aged; Pancreas; Pancreatic Diseases; Portal Vein; Tomography, X-Ray Computed
PubMed: 30852073
DOI: 10.1016/j.asjsur.2019.02.001 -
Turk Pediatri Arsivi Dec 2017Annular pancreas is a rare congenital anomaly that consists of a ring of pancreatic tissue partially or completely encircling the second part of the duodenum. It can...
Annular pancreas is a rare congenital anomaly that consists of a ring of pancreatic tissue partially or completely encircling the second part of the duodenum. It can affect anyone from neonates to adults, and is difficult to diagnose because it can present in a wide range of clinical conditions. Although cases have also been reported in adults, symptomatic cases are often referred in infancy or early childhood. A 17-year-old female patient who was diagnosed as having annular pancreas is reported. The patient had had non-bilious vomiting accompanied by abdominal pain, especially 5-10 minutes after meals, for seven years. Annular pancreas, which may be seen at any age, should be considered in the differential diagnosis of patients with non-bilious vomiting, particularly after meals, over a long period.
PubMed: 29483805
DOI: 10.5152/TurkPediatriArs.2017.3394 -
Acta Medica (Hradec Kralove) 2022The aim of our manuscript is to report of a successful perinatal outcome after treatment of acute polyhydramnios caused by duodenal atresia. A 34-year-old G3P1 was...
The aim of our manuscript is to report of a successful perinatal outcome after treatment of acute polyhydramnios caused by duodenal atresia. A 34-year-old G3P1 was referred due to polyhydramnios in the 30th week of pregnancy. Ultrasound revealed polyhydramnios, amniotic fluid index (AFI) 28, and a double bubble sign that indicated duodenal atresia and dilatated oesophagus. In the 32nd week of gestation, the volume of amniotic fluid increases, AFI 35, along with symptoms of dyspnea and abdominal pain. Due to the clinical picture and the early gestational age, it was decided to perform an amnioreduction. In the 36th week of gestation cesarean section was performed. The baby was taken for exploratory laparotomy and found to have a simultaneous complete duodenal atresia and annular pancreas with associated dilated the first portion of the duodenum and the stomach. A side-to-side duodenoduodenostomy via single-layer hand-sewn anastomosis was performed over a transanastamotic feeding tube (TAFT). The postoperative course was uneventful. Amnioreduction is useful and safe in the treatment of acute polyhydramnios caused by duodenal atresia and thus has a significant role in prolonging gestation until fetal maturity.
Topics: Pregnancy; Humans; Female; Adult; Polyhydramnios; Pregnancy Outcome; Cesarean Section; Amniotic Fluid
PubMed: 36942706
DOI: 10.14712/18059694.2023.6 -
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 the Korean Society of... Jan 2024Malrotation and midgut volvulus are surgical emergencies that commonly occur within the first month of life. The classic symptom is acute bilious vomiting, while... (Review)
Review
Malrotation and midgut volvulus are surgical emergencies that commonly occur within the first month of life. The classic symptom is acute bilious vomiting, while nonspecific symptoms such as recurrent abdominal pain may be present in older children. Malrotation can be associated with duodenal obstruction caused by an abnormal peritoneal fibrous band or congenital anomalies, such as an annular pancreas or a preduodenal portal vein. Volvulus can lead to bowel ischemia and a life-threatening condition, thus prompt and accurate diagnosis is crucial. Diagnosis can be made through upper gastrointestinal series, ultrasonography, and CT, with ultrasonography being preferred as a screening tool due to its rapid and accurate diagnosis, without radiation exposure, in children. This pictorial essay discusses the key imaging findings and diagnostic approaches for malrotation and midgut volvulus, as well as diagnostic pitfalls based on actual cases.
PubMed: 38362395
DOI: 10.3348/jksr.2023.0002 -
International Journal of Surgery Case... Dec 2022Annular pancreas is an uncommon congenital abnormality characterized by a ring of pancreatic tissue around the duodenum descending part. It is exceptionally rare in...
INTRODUCTION
Annular pancreas is an uncommon congenital abnormality characterized by a ring of pancreatic tissue around the duodenum descending part. It is exceptionally rare in adults and is increasingly detected after the investigation of symptoms caused by its consequences, which include recurrent pancreatitis, duodenal stenosis at the site of the annulus, or duodenal or gastric ulcers. In this study, we present a case report of symptomatic annular pancreas in an adult patient.
PRESENTATION OF CASE
A 23-year-old woman hospitalized for epigastric pain and recurrent vomiting. Radiological examination was consistent with an annular pancreas. At operation, a partial 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. A gastrojejunostomy was performed and she was discharged after 8 days with good recovery.
CONCLUSION
Because annular pancreas in adults is a rare clinical scenario, it should be included in the differential diagnosis of adult patients with gastrointestinal obstructive symptoms for a prolonged period of time, given that surgical intervention has a reliable outcome.
PubMed: 36455345
DOI: 10.1016/j.ijscr.2022.107804 -
World Journal of Clinical Cases Nov 2020Annular pancreas (AP) is a rare congenital abnormal rotation of the pancreas. AP rarely occurs in adults. Pancreatic tumors and ampullary tumors are related to AP, so...
BACKGROUND
Annular pancreas (AP) is a rare congenital abnormal rotation of the pancreas. AP rarely occurs in adults. Pancreatic tumors and ampullary tumors are related to AP, so the discovery and treatment of AP are essential.
CASE SUMMARY
This study investigated the clinical manifestations, imaging features, complications, and treatment of six patients diagnosed with AP at the Department of Hepatobiliary and Pancreatic Surgery, First Hospital of Jilin University from January 2010 to June 2020. There were four males and two females, with an average age of 56.00 ± 9.86 years old. In this study, abdominal pain and jaundice were the main clinical manifestations. Imaging can show the "crocodile jaw sign" or "double bubble sign".
CONCLUSION
For patients with duodenal or biliary obstruction, physicians should give priority to AP when imaging examinations suggest that the duodenum is wrapped with tissue similar to the density of the pancreas. Symptomatic patients should actively undergo surgical treatment.
PubMed: 33344566
DOI: 10.12998/wjcc.v8.i22.5722