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The Korean Journal of Gastroenterology... May 2018
Topics: Betahistine; Endoscopy, Digestive System; Female; Humans; Meniere Disease; Pancreas; Pancreatic Diseases; Tomography, X-Ray Computed; Ultrasonography; Young Adult
PubMed: 29791990
DOI: 10.4166/kjg.2018.71.5.294 -
Medicine Apr 2018Annular pancreas (AP) is recognized as a cause of duodenal obstruction in children, while children with Meckel's diverticulum (MD) are usually asymptomatic. Here we...
RATIONALE
Annular pancreas (AP) is recognized as a cause of duodenal obstruction in children, while children with Meckel's diverticulum (MD) are usually asymptomatic. Here we present a rare case with both AP and MD, which was identified by abdominal exploration during diamond-shaped duodenoduodenostomy.
PATIENT CONCERNS
A "double-bubble" sign was found by ultrasound at 35 week of pregnancy. After 39 weeks of pregnancy, the male patient was transferred to the Department of General Surgery, Children's Hospital of Soochow University because of a suspected duodenal stenosis.
DIAGNOSES
Preoperative abdominal X-ray examination indicated "double-bubble" sign. AP was confirmed by exploratory surgery, with an MD located 30 cm above the ileocecal valve.
INTERVENTIONS
Diamond-shaped duodenoduodenostomy and a wedge resection of the intestine with end-to-end anastomosis were performed OUTCOMES:: The patient recovered and his appetite was good without vomiting.
LESSONS
Our experience demonstrates that abdominal exploration is essential for children with gastrointestinal malformations.
Topics: Anastomosis, Surgical; Diverticulitis; Duodenostomy; Humans; Infant, Newborn; Male; Meckel Diverticulum; Pancreas; Pancreatic Diseases
PubMed: 29703052
DOI: 10.1097/MD.0000000000010583 -
Journal of Minimal Access Surgery 2018Portal annular pancreas (PAP) is a pancreatic congenital anomaly consisting of pancreatic parenchyma encircling the portal vein and/or the superior mesenteric vein. It...
Portal annular pancreas (PAP) is a pancreatic congenital anomaly consisting of pancreatic parenchyma encircling the portal vein and/or the superior mesenteric vein. It has been reported that the risk of developing a post-operative pancreatic fistula is higher following pancreaticoduodenectomy in patients with PAP, probably because of the possibility of leaving undrained a portion of pancreatic parenchyma during the reconstructive phase. Few manuscripts have reported a surgical technique of pancreaticoduodenectomy in case of PAP, herein we report the first case of a patient with PAP undergoing laparoscopic pancreaticoduodenectomy.
PubMed: 29582803
DOI: 10.4103/jmas.JMAS_235_17 -
Case Reports in Medicine 2018Annular pancreas is a rare congenital abnormality characterized by a ring of pancreatic tissue surrounding the descending portion of the duodenum. Annular pancreas...
INTRODUCTION
Annular pancreas is a rare congenital abnormality characterized by a ring of pancreatic tissue surrounding the descending portion of the duodenum. Annular pancreas coexisting with replaced common hepatic artery which is also a rare anatomical variation has not been reported previously.
CASE PRESENTATION
A 53-year-old man visited our hospital complaining of epigastric pain. Based on radiological examinations, he was diagnosed as having pancreatitis, annular pancreas, and hepatomesenteric trunk. One month later, obstructive jaundice developed. Endoscopic examination revealed ampullary region carcinoma. We performed pancreaticoduodenectomy using the "artery-first" approach.
DISCUSSION
Both annular pancreas and common hepatic artery anomaly are rare. High-quality preoperative imaging and awareness of such rare conditions are necessary for operative safety. Although the embryological relationship between these anomalies is uncertain, the present case may suggest some relevance between the two.
CONCLUSION
The "artery-first" approach may be a useful method for pancreaticoduodenectomy in patients who have an anatomical abnormality.
PubMed: 29560005
DOI: 10.1155/2018/3258141 -
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 -
Indian Pediatrics Dec 2017Neonatal diabetes mellitus is a rare condition.
BACKGROUND
Neonatal diabetes mellitus is a rare condition.
CASE CHARACTERISTICS
A small for gestational age male, presented with neonatal onset diabetes mellitus, duodenal atresia, annular pancreas and gall bladder hypoplasia.
OBSERVATION
Observation: A novel homozygous mutation p.K163R (c.488A>G) in the PDX1 gene was found. Parents were heterozygous for the same.
MESSAGE
This case highlights the importance of establishing the genetic diagnosis in all cases of neonatal diabetes mellitus.
Topics: Diabetes Mellitus; Duodenal Obstruction; Exocrine Pancreatic Insufficiency; Gallbladder; Homeodomain Proteins; Humans; Infant, Newborn; Intestinal Atresia; Male; Mutation; Pancreas; Pancreatic Diseases; Trans-Activators
PubMed: 29317564
DOI: 10.1007/s13312-017-1211-2 -
Medicine Dec 2017Portal annular pancreas is a rare anatomic variation, where the uncinated process of the pancreas connects with the dorsal pancreas and the pancreas tissue encases the...
Radical antegrade modular pancreatosplenectomy for adenocarcinomaof the body of the pancreas in a patient with portal annular pancreas, aberrant hepatic artery, and absence of the celiac trunk: A case report.
RATIONALE
Portal annular pancreas is a rare anatomic variation, where the uncinated process of the pancreas connects with the dorsal pancreas and the pancreas tissue encases the portal vein (PV), superior mesenteric vein (SMV) or splenic vein (SV). Malignancies are quite uncommon in the patients, who have an annular pancreas especially portal annular pancreas. Ectopic common hepatic artery and absence of the celiac trunk (CT) are the other infrequent abnormalities.
PATIENT CONCERNS
A 74-year-old man suffered from upper abdominal and back pain.
DIAGNOSES AND INTERVENTIONS
Contrast enhanced computed tomography indicated a low-density mass in the body of the pancreas. Pathological report showed adenocarcinoma of the body of pancreas after radical antegrade modular pancreatosplenectomy (RAMPS).
OUTCOMES
In the operation, we found the superior vein and portal vein was surrounded by the pancreatic tissue. The left gastric artery and splenic artery originated respectively from abdominal aorta, and celiac trunk was not viewed. In addition, the common hepatic artery was a branch from the superior mesenteric artery.
LESSONS
In general, this is a novel clinical case of pancreatic carcinoma happening in the portal annular pancreas which was accompanied with aberrant hepatic artery and absence of the celiac trunk at the same time. Confronted with the pancreatic neoplasms, the possibility of coexistent annular pancreas and arterial variations should be considered.
Topics: Adenocarcinoma; Aged; Celiac Artery; Contrast Media; Hepatic Artery; Humans; Male; Pancreas; Pancreatectomy; Pancreatic Neoplasms; Portal Vein; Splenectomy; Tomography, X-Ray Computed
PubMed: 29310347
DOI: 10.1097/MD.0000000000008738 -
European Journal of Medical Genetics May 2018Craniofacial Microsomia (CFM) also known as Oculo-auriculo-vertebral Spectrum (OAVS) or Goldenhar Syndrome, presents wide phenotypic and etiological heterogeneity. It... (Review)
Review
Craniofacial Microsomia (CFM) also known as Oculo-auriculo-vertebral Spectrum (OAVS) or Goldenhar Syndrome, presents wide phenotypic and etiological heterogeneity. It affects mainly the structures originated from the first and second pharyngeal arches. In addition, other major anomalies may also be found, including congenital heart diseases. In this study, we report a patient with distal deletion in the 22q11.2 region and a phenotype which resembles CFM. The proband is a girl, who presented bilateral preauricular tags, left auditory canal stenosis, malar hypoplasia, cleft lip and palate, mild asymmetry of soft tissue in face, congenital heart disease, intestinal atresia, annular pancreas and hydronephrosis. The genomic imbalances investigation by Multiplex Ligation-dependent Probe Amplification (MLPA) and Chromosomal Microarray Analysis (CMA) revealed a distal deletion of 1,048 kb at 22q11.2 encompassing the region from Low Copy Repeats (LCRs) D to E. We did review of the literature and genotype-phenotype correlation. This is the sixth case of distal 22q11.2 deletion resembling CFM and the second encompassing the region between LCRs D to E. All cases share some phenotypic signs, such as preauricular tags, facial asymmetry, cleft lip and palate, and congenital heart diseases. Candidate genes in this region have been studied by having an important role in pharyngeal arches developmental and in congenital heart diseases, such as HIC2, YPEL1and MAPK1/ERK2. This case corroborates the phenotypic similarity between 22q11.2 distal deletion and CFM/OAVS. It also contributes to genotype-phenotype correlation and reinforces that candidate genes for CFM, in the 22q11.2 region, might be located between LCRs D and E.
Topics: 22q11 Deletion Syndrome; Child; Diagnosis, Differential; Female; Genetic Loci; Genotype; Goldenhar Syndrome; Humans; Phenotype
PubMed: 29288792
DOI: 10.1016/j.ejmg.2017.12.013 -
Medicine Oct 2017This study is to prospectively evaluate the efficiency of sonography for volvulus diagnosis in neonates with clinically suspected intestinal malrotation.A total of 83... (Observational Study)
Observational Study
This study is to prospectively evaluate the efficiency of sonography for volvulus diagnosis in neonates with clinically suspected intestinal malrotation.A total of 83 patients with suspected intestinal malrotation who underwent detailed abdominal sonography and upper gastrointestinal contrast study were included. Malrotation was characterized by inversion of the superior mesenteric artery (SMA) and superior mesenteric vein (SMV) in sonographic examination. The "whirlpool sign" of Color Doppler Sonography was recognized as a characteristic for malrotation with volvulus. The degrees of rotation of the SMV winding around SMA were also detected by sonography. Surgery was performed in patients with sonography diagnosed malrotation.A total of 39 patients were sonographically diagnosed as malrotation which was subsequently confirmed by surgery. The sensitivity and positive predictive value of the sonographic diagnosis were both 100% (39/39). The sensitivity, specificity and accuracy of "whirlpool sign" for the detection of midgut volvulus were 95.2% (20/21), 88.9% (16/18), and 92.3% (36/39), respectively. Greater degrees of rotation (equal or greater than 720°) showed higher risk (odds ratio, 5.0; P < .01) for intestinal necrosis occurrence.Sonography is more accurate in diagnosing suspected malrotation than upper gastrointestinal contrast study. Specific sonographic "whirlpool sign" related to volvulus may be used as a potential indicator for intestinal necrosis. In addition, sonography can exclude malrotation and may help the diagnosis of other diseases, such as annular pancreas and duodenal atresia.
Topics: Digestive System Abnormalities; Female; Humans; Infant, Newborn; Intestinal Volvulus; Male; Mesenteric Artery, Superior; Mesenteric Veins; Sensitivity and Specificity; Ultrasonography, Doppler, Color
PubMed: 29049228
DOI: 10.1097/MD.0000000000008287 -
Frontiers in Pediatrics 2017The aim of this report was to review our early experience of the last 7 years with repairs of congenital duodenal obstruction (CDO) to determine the efficacy and...
BACKGROUND
The aim of this report was to review our early experience of the last 7 years with repairs of congenital duodenal obstruction (CDO) to determine the efficacy and outcomes of laparoscopic repairs compared to laparotomy.
METHODS
A retrospective review was conducted on all neonate (<30 days) with CDO between 2009 and 2015. Patients with duodenal atresia, stenosis, web, and annular pancreas were included. Patients with only malrotation or delayed presentation were excluded.
RESULTS
Twenty-six neonates underwent laparoscopy and 30 underwent traditional laparotomy. The operative time was longer in the laparoscopic group ( = 0.001), but time to initiation of feeds and time to full feeds were similar for the laparoscopic and open groups. There was no mortality, anastomosis leakage, or stenosis in the laparoscopic group. Six laparoscopic cases required conversion to an open procedure (23%). In the earlier cases, the open conversion rate was high, but it decreased over time ( = 0.003).
CONCLUSION
Laparoscopic repair is safe and effective for repair of CDO in neonates. Despite operative time was slightly longer in the laparoscopic group, clinical outcomes remained similar to the open group. For pediatric surgeon with experience in laparoscopic techniques, laparoscopic duodenoduodenostomy is a sufficient available procedure.
PubMed: 29018788
DOI: 10.3389/fped.2017.00203