Successful endoscopic sclerotherapy with bile duct stenting for a vascular malformation neighboring the duodenal papilla in blue rubber bleb nevus syndrome.
Ontology highlight
ABSTRACT:
SUBMITTER: Unoura S
PROVIDER: S-EPMC9302048 | biostudies-literature |
REPOSITORIES: biostudies-literature
Similar Datasets
Project description:Duodenal obstruction may prevent performance of endoscopic retrograde cholangiopancreatography (ERCP). Percutaneous transhepatic biliary drainage (PTBD) or Endoscopic ultrasonograhy-guided biliary access (EUS-BD) are alternative treatments but are associated with a higher morbidity and mortality rate. The aim of the study is to report overall technical success rate and clinical outcome with deployment of temporary fully or partially covered self-expanding duodenal stent (pc/fcSEMS) as a bridge to ERCP in case of inaccessible papilla due to duodenal strictures.This retrospective study included 66 consecutive patients presenting with a duodenal stricture impeding the ability to perform an ERCP. Provisional duodenal stenting was performed as a bridge to ERCP. A second endoscopic session was performed to remove the provisional stent and to perform an ERCP. Afterward, a permanent duodenal stent was delivered if necessary.Sixty-six duodenal stents (17 pcSEMS and 49 fcSEMS) were delivered with a median indwelling time of 3.15 (1 - 7) days. Two migrations occurred in the pcSEMS group, 1 of which required lower endoscopy for retrieval. No other procedure-related complications were observed. At second endoscopy a successful ERCP was performed in 56 patients (85 %); 10 patients (15 %) with endoscopic failure underwent PTBD or EUS-BD. Forty patients needed permanent duodenal stenting.Provisional removable covered duodenal stenting as a bridge to ERCP for duodenal obstruction is safe procedure and in most cases allows successful performance of therapeutic ERCP. This technique could be a sound option as a step up approach before referring such cases for more complex techniques such as EUS-BD or PTBD.
| S-EPMC5025317 | biostudies-other
Project description:Blue rubber bleb nevus syndrome (BRBNS), also called Bean's syndrome, is a rare disease associated with multiple venous malformations in the skin and gastrointestinal (GI) tract. Dermatological lesions, which are the first clinically visible manifestations, appear as skin-colored compressible protuberances or as dark-blue venous nodules, rubbery in consistency. Central nervous system (CNS) manifestations are rare, variable, non-specific, and tend to occur late in the disease, mainly reported as seizures and focal neurological deficits secondary to compression. Most cases occur sporadically, however, an autosomal dominant inheritance pattern has been reported. A 74-year-old male with history of focal epilepsy secondary to possible neurocysticercosis presented at the emergency department due to sudden onset of aphasia, left central facial paralysis, and dysphagia secondary to catastrophic intracerebral hemorrhage. Cerebral MRI showed multiple cerebral cavernous malformations (CCM)-like lesions and, on the general exploration, multiple dark-blue nodules, rubbery in consistency. One week later he died due to complicated pneumonia; a brain autopsy was performed showing multiple vascular malformations. His son had a history of focal epilepsy presumed to be related to neurocysticercosis. He had the same skin lesions and brain MRI pattern. Histological analysis of the skin lesions of the two cases showed venous vascular malformations. A non-systematic review was carried out, in which all case reports of blue nevus syndrome with neurological manifestations in adults were included.
| S-EPMC7154106 | biostudies-literature
Project description: Not available
| S-EPMC7482362 | biostudies-literature
Project description:Blue rubber bleb nevus syndrome (BRBNS) is a rare disease in which venous malformations (VMs) involve any body organ, most commonly the skin and the gastrointestinal (GI) tract. Treatment of BRBNS aims at preserving the GI tract as much as possible. Although there are several dozen case reports about BRBNS that describe short-term clinical courses, a few provide an account of long-term clinical course. Here, we report a case of BRBNS in a girl that required multiple abdominal surgeries due to the GI VMs and a recurrence at an interval of 14 years. The preferred approach for gastrointestinal VMs involves the complete resection of all lesions without residual VMs. It is important to bear in mind the possibility of delayed recurrence of GI VMs after surgical or endoscopic treatment.
| S-EPMC6286777 | biostudies-other
Project description:To study the clinical effect of oral sirolimus in the treatment of children with blue rubber bleb nevus syndrome (BRBNS) in the gastrointestinal tract, a retrospective analysis was performed on the clinical data and follow-up results of two children with BRBNS treated by sirolimus. The two children with BRBNS had gastrointestinal bleeding and anemia and were treated with sirolimus at a dose of 1 mg/day as part of treatment. The plasma concentration of the drug was maintained between 2.5-12.0?ng/mL. The children showed disappearance of gastrointestinal bleeding and improvements in anemia and coagulation function, and blood transfusion could be stopped during treatment, with no obvious adverse drug reactions. PubMed, Wanfang Data, and CNKI were searched for related articles on sirolimus in the treatment of BRBNS. A total of 26 cases of children with BRBNS, aged 0-18 years, were obtained. With the addition of the 2 cases in this study, sirolimus treatment achieved a satisfactory clinical effect in all 28 cases. Sirolimus may be effective and safe in the treatment of children with BRBNS, and further prospective studies are needed to evaluate the long-term efficacy of this drug.
| S-EPMC7499453 | biostudies-literature
Project description:Introduction Treatment of recurrent severe gastrointestinal bleeding due to arteriovenous malformations may require complex resections. In some particular locations, extensive surgery is the only way out, as shown in this report. Case Report A 2.5-year-old child suffered repeated episodes of upper gastrointestinal bleeding since the first month of life. After an extensive diagnostic workout, the diagnosis of duodenal arteriovenous malformation was established. Cephalic pancreaticoduodenectomy with pyloric preservation was performed and no further episodes of bleeding occurred in the ensuing 2 years. Conclusion Bleeding malformations located in the pancreaticoduodenal area can be effectively treated in children by pylorus-preserving cephalic pancreaticoduodenectomy.
| S-EPMC4336108 | biostudies-other
Project description:Background and aimsIn recent years, with the development of endoscopic techniques, endoscopic resection is widely used for duodenal papillary adenomas, but conventional endoscopic resection has a high rate of incomplete resection and recurrence. On this basis, we have employed a novel modified endoscopic papillectomy (ESP). In this study, we evaluated the feasibility and advantages of this ESP for the treatment of duodenal major papilla adenoma.MethodsA total of 56 patients with duodenal major papilla adenoma confirmed by endoscopic ultrasonography, intraluminal ultrasound and gastroscopic biopsy from October 2007 to June 2017 were collected in the Department of Gastroenterology, Nanjing Drum Tower Hospital. The diameter of the adenoma ranged from 1.41 to 2.02 cm. 16 cases were given the conventional method and 40 cases underwent the modified ESP procedure in which a small incision was made by cutting current when anchoring the snare tip on the distal side of the adenoma.ResultsEn bloc resection rate was significantly higher in the modified group (100%, 40/40) than that in the conventional group (81.3%, 13/16; P = 0.02). However, no significance was seen between the modified group and the conventional group in complete resection rate (92.5%, 37/40 vs 93.8%, 15/16; P = 1.00). There was no significant difference in the number and difficulty of postoperative pancreatic and biliary stents placement between the two groups (P = 0.20). Total bleeding occurrence was much lower in the modified group (37.5%, 15/40 vs 87.5%, 14/16; P = 0.001), and no significant differences were found in other short-term complications and the 3, 6, 12 and 24 months recurrences rate between the conventional and modified ESP groups.ConclusionsThe modified ESP improves the treatment outcome of duodenal major papilla adenoma with higher en bloc resection rate and lowering bleeding rate.
| S-EPMC7572334 | biostudies-literature
Project description:Video 1Rubber band-assisted closure of a mucosal defect after duodenal EMR.
| S-EPMC8573376 | biostudies-literature
Project description:BackgroundDuodenal papilla carcinoma (DPC) is a rare malignancy of the gastrointestinal tract with high recurrence rate, and the pathogenesis of this highly malignant neoplasm is yet to be fully elucidated. This study aims to identify key genes to further understand the biology and pathogenesis underlying the molecular alterations driving DPC, which could be potential diagnostic or therapeutic targets.MethodsTumor samples of three DPC patients were collected and integrating RNA-seq analysis of tumor tissues and matched normal tissues were performed to discover differentially expressed genes (DEGs). Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) enrichment analysis were carried out to understand the potential bio-functions of the DPC differentially expressed genes (DEGs). Protein-protein interaction (PPI) network was constructed for functional modules analysis and identification of hub genes. qRT-PCR of clinical samples was conducted to validate the expression level of the hub genes.ResultsA total of 110 DEGs were identified from our RNA-seq data, GO and KEGG analyses showed that the DEGs were mainly enriched in multiple cancer-related functions and pathways, such as cell proliferation, IL-17signaling pathway, Jak-STAT signaling pathway, PPAR signaling pathway. The PPI network screened out five hub genes including IL-6, LCN2, FABP4, LEP and MMP1, which were identified as core genes in the network and the expression value were validated by qRT-PCR. The hub genes identified in this work were suggested to be potential therapeutic targets of DPC.DiscussionThe current study may provide new insight into the exploration of DPC pathogenesis and the screened hub genes may serve as potential diagnostic indicator and novel therapeutic target.
| S-EPMC7789135 | biostudies-literature
Project description:Background We investigated whether duodenal major papilla morphology could be a risk factor for failure of selective biliary cannulation (SBC) and post endoscopic retrograde cholangiography and pancreatography (ERCP) complications. Methods A prospectively recorded database was reviewed retrospectively. Patients were included if they received therapeutic ERCP and had naïve major duodenal papilla. We used Haraldsson’s classification for papilla morphology, as follows: Regular (Type 1), Small (Type 2), Protruding or Pendulous (Type 3) and Creased or Ridged (Type 4). Risk factors for failing SBC and post-ERCP complications were analyzed by multivariate analysis. Results A total of 286 cases were included. Age, gender, indications and therapeutic procedures were not different among the four types of papillae. The failure rates of SBC with Type 3 papilla and Type 4 papilla were 11.11% and 6.25%, respectively. In the multivariate analysis, Type 2 papilla (odd ratio 7.18, p?=?0.045) and Type 3 papilla (odd ratio 7.44, p?=?0.016) were associated with greater SBC failure compared with Type 1 papilla. Malignant obstruction compared to stone (odds ratio 4.45, p?=?0.014) and age (odd ratio?=?1.06, p?=?0.010) were also risk factors for cannulation failure. Type 2 papilla was correlated with a higher rate of post-ERCP pancreatitis (20%, p?=?0.020) compared to the other types of papilla However, papilla morphology was not a significant risk factor for any complications in the multivariate analysis. Conclusion Small papilla and protruding or pendulous papilla are more difficult to cannulate compared to regular papilla. Small papilla is associated with a higher rate of post-ERCP pancreatitis.
| S-EPMC7520951 | biostudies-literature