The liver twist: A case of accessory liver lobe torsion presenting after mild trauma.

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.

Biliary Drainage Before and After Liver Resection for Perihilar Cholangiocarcinoma.

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.

Percutaneous debridement and washout of walled-off abdominal abscess and necrosis by the use of flexible endoscopy: an attractive clinical option when transluminal approaches are unsafe or not possible.

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.

Fractured Bard Recovery, G2, and G2 express inferior vena cava filters: incidence, clinical consequences, and outcomes of removal attempts.

PURPOSE: To increase the understanding of risks of inferior vena cava (IVC) filter fracture and embolization and the safety of removing fractured filters via retrospective review of a prospectively collected database of fractured IVC filters. MATERIALS AND METHODS: A total of 63 fractured IVC filters were discovered among 548 patients presenting for retrievable filter removal between April 2004 and November 2010 at a single institution. Device type, duration of implantation, component fracture, and embolization events were recorded. Success rates and techniques for removal of components were recorded. RESULTS: A total of 63 fractured Recovery, G2, and G2 Express IVC filters were identified, for an overall fracture rate of 12%. Excluding foot process fractures, the fracture rate for only filter arms and/or legs was 6%. The incidence of fracture increased with longer filter dwell times. Success rates for removal of the nonfractured component (ie, main body) and fractured components (ie, arm or leg) were 98.4% and 53.4%, respectively. The distal embolization rate of fractured filter components was 13%. There were no immediate clinically significant complications associated with fracture component embolization or filter removal. A single patient was encountered with symptoms related to their fractured filter. CONCLUSIONS: IVC filter fracture rates increase with longer dwell times; however, removal of fractured filters and fractured components (ie, arms and legs) can be achieved safely and effectively. Clinically significant complications of IVC filter fracture are rare, and there were no immediate clinical sequelae related to embolization of fracture components.

Multiple sequence variants in hereditary hemorrhagic telangiectasia cases: illustration of complexity in molecular diagnostic interpretation.

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.