- GeneReviews(®) [BOOK]
- BOOKUniversity of Washington, Seattle: Seattle (WA)
- Myhre syndrome is a connective tissue disorder with multisystem involvement, progressive and proliferative fibrosis that may occur spontaneously or following trauma or surgery, mild-to-moderate intel...
Myhre syndrome is a connective tissue disorder with multisystem involvement, progressive and proliferative fibrosis that may occur spontaneously or following trauma or surgery, mild-to-moderate intellectual disability, and in some instances, autistic-like behaviors. Organ systems primarily involved include: cardiovascular (congenital heart defects, long- and short-segment stenosis of the aorta and peripheral arteries, pericardial effusion, constrictive pericarditis, restrictive cardiomyopathy, and hypertension); respiratory (choanal stenosis, laryngotracheal narrowing, obstructive airway disease, or restrictive pulmonary disease), gastrointestinal (pyloric stenosis, duodenal strictures, severe constipation); and skin (thickened particularly on the hands and extensor surfaces). Additional findings include distinctive craniofacial features and skeletal involvement (intrauterine growth restriction, short stature, limited joint range of motion). To date, 55 individuals with molecularly confirmed Myhre syndrome have been reported.
- Use of a Lumen-Apposing Metal Stent to Treat Pyloric Stenosis. [Journal Article]
- GEGastrointest Endosc 2017 Apr 04
- Perinatal risk factors for infantile hypertrophic pyloric stenosis: A meta-analysis. [Review]
- JPJ Pediatr Surg 2017 Mar 15
- CONCLUSIONS: First-born, cesarean section delivery, preterm birth, and bottle-feed are associated with the development of IHPS. Well-designed future studies are needed to help understand the etiology of IHPS.
- Excessive Bright Echoes Sign for Hypertrophic Pyloric Stenosis Suggest the Diagnosis: Gastric Pneumatosis and Portal Venous Gas in Infants Suggest HPS. [Journal Article]
- JUJ Ultrasound Med 2017; 36(5):1059-1063
- We describe a new finding, the "excessive bright echoes" sign, for the diagnosis of hypertrophic pyloric stenosis (HPS). Portal venous gas and gastric wall pneumatosis were noted in 4 vomiting infant...
We describe a new finding, the "excessive bright echoes" sign, for the diagnosis of hypertrophic pyloric stenosis (HPS). Portal venous gas and gastric wall pneumatosis were noted in 4 vomiting infants proven to have HPS. Portal venous gas can be concerning for ischemic bowel. Gastric wall pneumatosis can be seen in association with necrotizing enterocolitis and has been associated with increased gastric pressure from severe, usually proximal, bowel obstruction. Our HPS cases had prominent bright punctate echoes on sonography of the liver, portal vein lumen, and gastric wall. Knowledge of this excessive bright echoes sign suggests the need for sonography of the antropyloric area. One should consider HPS as a differential diagnostic possibility when the combination of bright echoes within the liver parenchyma, consistent with portal venous gas, and bright echoes in the gastric wall, consistent with gastric pneumatosis, are seen.
- Comparing pyloromyotomy outcomes across Canada. [Journal Article]
- JPJ Pediatr Surg 2017 Jan 28
- CONCLUSIONS: This study found no difference in complication rate or LOS stay between hospital type and surgeon type across Canada. This may reflect a previously identified referral trend in the United States towards pediatric centers. Several regions of higher HPS incidence were identified, and may aid in identifying genetic elements causing HPS.
- Corrected to uncorrected? The metabolic conundrum of hypertrophic pyloric stenosis. [Journal Article]
- JPJ Pediatr Surg 2017 Jan 28
- CONCLUSIONS: There is little evidence to support need for repeat serum chemistry testing in cases of HPS once normality has been established. Development of clinical pathways to reduce the use of unnecessary serum testing may improve efficiency of patient care and limit unnecessary resource utilization.
- Idiopathic pyloric stenosis. [Case Reports]
- PAPan Afr Med J 2016; 25:23
- [A Long-Surviving Patient with Unresectable Gastric Cancer Treated with Multiple Rounds of Chemotherapyafter Gastrojejunostomyand Ileocolonostomy]. [Journal Article]
- GTGan To Kagaku Ryoho 2016; 43(12):1926-1928
- We report a long-surviving patient with unresectable gastric cancer treated with multiple rounds of chemotherapy after gastrojejunostomy and ileocolonostomy. The patient was a 79-year-old man. Endosc...
We report a long-surviving patient with unresectable gastric cancer treated with multiple rounds of chemotherapy after gastrojejunostomy and ileocolonostomy. The patient was a 79-year-old man. Endoscopic examination revealed a type 3 gastric cancer in the antrum and showed pyloric stenosis. We observed that the gastric tumor had invaded the transverse colon, pancreas, and duodenum, and was associated with peritoneal metastasis on the surface of the hepatoduodenal ligament and the posterior leaf of the transverse mesocolon. We performed gastrojejunostomy and ileocolonostomy to allow oral intake and to prevent ileus. The final diagnosis was fT4b(SI), N2, H0, P1, M0, CY0, Stage IV . The patient's oral intake was not sufficient following the operation, and therefore, he was treated with 8 courses of 5-FU plus PTX intravenously until disease progression was observed. Bi-weekly administration of CPT-11 and CDDP was chosen as the second-line chemotherapy. Grade 2 renal dysfunction was observed after 13 courses of this therapy, and therefore, we changed the regimen to CPT-11 monotherapy, repeating 18 courses until disease progression was observed. The patient was treated with 18 courses of S-1 monotherapy as the fourth-line chemotherapy, and then 2 courses of S-1 plus DTX. The patient died of progressive disease 4 years and 5 months after surgery. During chemotherapy, he maintained a good performance status and could be treated as an outpatient.
- Atropine: A Cure for Persistent Post Laparoscopic Pyloromyotomy Emesis? [Journal Article]
- JNJ Neonatal Surg 2017 Jan-Mar; 6(1):2
- CONCLUSIONS: Oral atropine is a viable treatment for persistent emesis after a pyloromyotomy and reduces the need for a second operation due to incomplete pyloromyotomy.
New Search Next
- Roux-en-Y augmented gastric advancement: An alternative technique for concurrent esophageal and pyloric stenosis secondary to corrosive intake. [Journal Article]
- WJWorld J Gastrointest Surg 2016 Dec 27; 8(12):766-769
- Select group of patients with concurrent esophageal and gastric stricturing secondary to corrosive intake requires colonic or free jejunal transfer. These technically demanding reconstructions are as...
Select group of patients with concurrent esophageal and gastric stricturing secondary to corrosive intake requires colonic or free jejunal transfer. These technically demanding reconstructions are associated with significant complications and have up to 18% ischemic conduit necrosis. Following corrosive intake, up to 30% of such patients have stricturing at the pyloro-duodenal canal area only and rest of the stomach is available for rather less complex and better perfused gastrointestinal reconstruction. Here we describe an alternative technique where we utilize stomach following distal gastric resection along with Roux-en-Y reconstruction instead of colonic or jejunal interposition. This neo-conduit is potentially superior in terms of perfusion, lower risk of gastro-esophageal anastomotic leakage and technical ease as opposed to colonic and jejunal counterparts. We have utilized the said technique in three patients with acceptable postoperative outcome. In addition this technique offers a feasible reconstruction plan in patients where colon is not available for reconstruction due to concomitant pathology. Utility of this technique may also merit consideration for gastroesophageal junction tumors.