Background and Aims
Walled-off pancreatic necrosis is a well-known serious adverse event of severe acute pancreatitis. EUS-guided transluminal access followed by direct endoscopic necrosectomy is increasingly used to remove necrosis, with good efficacy and a superior safety profile when compared with surgery. However, a percentage of patients is too critically ill to undergo this procedure or lack an appropriate transluminal window for access. Here we describe the use of percutaneous flexible endoscopic necrosectomy (PEN) with use of standard-sized upper endoscopes and accessories in a retrospective single-institution experience with a video demonstration of 1 patient in the series.
Methods
The authors present a 23-patient retrospective case series of PEN with standard-sized endoscopes. The series includes 12 patients from a previously published analysis in 2016 and 11 additional patients from 2013 to 2018. A representative case illustrates the described technique in a patient with severe acute pancreatitis complicated by multisystem organ failure who required immediate drainage of a pancreatic fluid collection and placement of a percutaneous drain into the collection for decompression. The drain was serially upsized to 28F, and its tract was used for PEN.
Results
A total of 23 patients have undergone PEN at our institution. On average, the size of the pancreatic fluid collection was 11.6 cm in cross-sectional diameter. Of those 23 patients, 11 presented with symptoms of severe disease. The median time from onset of symptoms to PEN was 84 days. The median number of procedures per patient was 2.1. The median time to complete resolution of symptoms and fluid collections was 67 days. In total, resolution was reached in 22 of 23 patients. Two patients died of unrelated causes.
Conclusion
PEN is a minimally invasive and effective treatment approach to walled-off pancreatic necrosis in patients who are not amenable to transluminal drainage and in whom percutaneous drains have been successfully placed. This case series demonstrates the efficacy and safety of this approach. A randomized prospective trial would be warranted to validate these results.
An accessory liver lobe is a congenital anomaly of hepatic tissue most commonly due to embryonic heteroplasia. Rarely, accessory liver lobes can undergo torsion and present as an acute surgical emergency. Although common in certain animals, there are only a few reported cases of accessory lobe torsion in humans. We report a multi-modality radiographic diagnosis of an acute torsion and subsequent infarct of an accessory liver lobe following minor trauma in a 29-year old male patient.
Hereditary hemorrhagic telangiectasia is an autosomal dominant disease caused by mutations in the ACVRL1 and ENG genes characterized by arterio-venous malformations and telangiectases. Over 700 mutations have been described in these two genes, and missense mutations are common. We describe 10 cases in which more than one potentially pathogenic mutation was identified. We report that 8 novel missense mutations , as well as previously reported pathogenic missense mutations , were seen in combination with a second mutation , which raises questions with regards to their respective pathogenicity. Our data and discussion indicate the challenges of classifying missense mutations as pathogenic or benign and the value of co-segregation studies , as well as suggest that there may be hereditary hemorrhagic telangiectasia gene mutations that have only mild phenotypic effects. We present evidence to suggest that four missense mutations (ENG p.G331S , ENG p.L8P , ENG p.P452L and ACVRL1 p.C344R) are pathogenic , two novel mutations (ACVRL1 p.A311T and ENG p.S576G) are neutral , and two previously reported disease-causing mutations are benign or have suspected benign variants (ACVRL1 p.A482V and ENG p.V504M). We conclude that for the purpose of establishing a causative hereditary hemorrhagic telangiectasia mutation in a family proband , all exons and intron/exon borders of both genes should be sequenced and deletion/duplication analysis should be performed unless a mutation that is well-proven to be pathogenic is identified. Telangiectases and arteriovenous malformations (AVMs) are the characteristic phenotypic manifestations of hereditary hemorrhagic telangiectasia (HHT), a heterogeneous autosomal dominant disorder. Recurring nosebleeds are the most common complication of telangiectases in patients with HHT, but can be mild and infrequent when they begin, typically in late childhood or early teens.
Perihilar cholangiocarcinoma (PHC) is a rare tumor that requires surgical resection for a potential cure. The role of preoperative biliary drainage has long been debated, given its treatment of biliary sepsis and decompression of the future liver remnant (FLR), but high procedure-specific morbidity. The indications, methods, and outcomes for preoperative biliary drainage are discussed to serve as a guide for perioperative management of patients with resectable PHC. Multiple studies from the literature related to perihilar cholangiocarcinoma, biliary drainage, and management of the FLR were reviewed. Commonly employed preoperative biliary drainage includes endoscopic biliary stenting and percutaneous transhepatic biliary drainage. Drainage of the FLR remains controversial, with most experts recommending drainage of the only in patients with an FLR <50%. Biliary drainage for resectable PHC requires a patient-specific approach with careful determination of the FLR and balancing of potential morbidity with the benefits of drainage.
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