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Pediatric Surgery International Mar 2022We aimed to evaluate possible positive and negative effects of postoperative use of transanastomotic feeding tube (TAFT) in neonates operated for congenital duodenal...
PURPOSE
We aimed to evaluate possible positive and negative effects of postoperative use of transanastomotic feeding tube (TAFT) in neonates operated for congenital duodenal obstruction (CDO).
METHODS
This is a retrospective study reviewing medical records of neonates operated for CDO during 2003-2020 and comparing postoperative feeding outcomes and complications in patients with and without TAFT. Approval from the hospital's data protection officer was obtained.
RESULTS
One hundred patients, 59% girls, were included, and 37% received TAFT. Mean birth weight and gestational age were 2628 (675.1) grams and 36.6 (2.4) weeks, respectively. Furthermore, 45% had no other malformations, and 36% had Down syndrome. Patient demographics were similar for TAFT and not-TAFT patients, except that not-TAFT neonates weighed median 335 g less (p = 0.013). The TAFT group got parenteral nutrition 2 days shorter (p < 0.001) and started enteral feeds 1.5 days earlier (p < 0.001) than the not-TAFT group. Fewer neonates with TAFT got a central venous catheter [65 vs 89%, (p = 0.008)]. In the TAFT group, 67% were breast fed at discharge compared to 49% in the not-TAFT group (p = 0.096).
CONCLUSION
Neonates with TAFT had earlier first enteral feed, fewer days with parenteral nutrition and fewer placements of central venous catheters.
Topics: Duodenal Obstruction; Enteral Nutrition; Female; Humans; Infant, Newborn; Intubation, Gastrointestinal; Male; Parenteral Nutrition; Retrospective Studies
PubMed: 34910223
DOI: 10.1007/s00383-021-05053-3 -
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 -
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 -
Radiology Case Reports Jan 2024Congenital duodenal obstruction is a common cause of bowel obstruction. It is relatively easy to diagnose in the neonatal period. However, if the obstruction is due to a...
Congenital duodenal obstruction is a common cause of bowel obstruction. It is relatively easy to diagnose in the neonatal period. However, if the obstruction is due to a duodenal diaphragm, diagnosis may be delayed until later in infancy or even adulthood, depending on the size of the aperture in the diaphragm. Congenital duodenal obstruction may be associated with other gastrointestinal and biliary anomalies. The association of a duodenal diaphragm and a partial annular pancreas is a rare clinical entity. We present an unusual case of late presentation of duodenal diaphragm with partial annular pancreas in a 10-year-old girl with a 3-month history of abdominal distension and vomiting. The plain abdominal X-ray showed the classic picture of a double bubble. The CT images showed narrowing of the third duodenal portion and dilatation of the stomach and duodenum. The pancreatic tissue incompletely surrounded the second part of the duodenum. Intraoperatively, both the duodenal diaphragm and the partial annular pancreas were confirmed. Excision of the diaphragm and duodenoplasty were performed. A duodenal diaphragm should be suspected in patients with a history of abdominal distension and vomiting, even in late childhood. Associated gastrointestinal and biliary anomalies should always be excluded.
PubMed: 38028306
DOI: 10.1016/j.radcr.2023.10.003 -
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 -
Endoscopy Dec 2023
Topics: Humans; Pancreas Divisum; Pancreas; Pancreatic Diseases; Cholangiopancreatography, Endoscopic Retrograde; Pancreatic Ducts
PubMed: 36958350
DOI: 10.1055/a-2032-3462 -
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 -
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 -
Radiologia Brasileira 2019
PubMed: 31435094
DOI: 10.1590/0100-3984.2017.0196 -
Journal of Surgical Case Reports Jul 2023The annular pancreas (AP) is an uncommon congenital anomaly, characterised by a circumferential envelope in the second portion of the duodenum. In recent years, some...
The annular pancreas (AP) is an uncommon congenital anomaly, characterised by a circumferential envelope in the second portion of the duodenum. In recent years, some genetic component has been found in the etiology. A newborn full-term male, weighing at 1910 g at birth, had a history of intrauterine growth restriction and diagnosis of tetralogy of Fallot, Down syndrome and congenital hypothyroidism. Duodenal membrane is suspected after persistent postprandial vomiting and abdominal distension; his abdomen was distended, hyperresonant and soft. The gastroduodenal series showed data compatible with a duodenal membrane so exploratory laparotomy was performed, finding the pancreas completely wrapping the second portion of the duodenum, so a diamond-shaped-duodenoduodenostomy anastomosis was performed. The AP should be considered, especially in male neonates with postprandial vomiting, abdominal distension, who show some other congenital anomaly, and in the abdominal X-ray, the sign of the double bubble is observed.
PubMed: 37489161
DOI: 10.1093/jscr/rjad407