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The aim of this report is to explain the historical differences between Klippel-Trenaunay (KT), Klippel-Trenaunay-Weber (KTW), and Klippel-Trenaunay-Servelle (KTS) syndromes. Furthermore, an attempt will be made to describe the different causes, symptoms, and consequences of the pathology, largely as a consequence of venous hypertension. The significance of these syndromes within the pediatric population is highlighted.

however, there are limited reports regarding EUS-CD in OLTs. We present a case of successful EUS-CD with lumen apposing metal stents (LAMS) in an OLT with recurrent cholangitis secondary to benign anastomotic stricture refractory to management with ERCP. Case Description/Methods: A 61-year old female with 3 OLTs (1996 and twice in 2008) for autoimmune hepatitis presented with recurrent cholangitis secondary to anastomotic biliary stricture in the setting of duct mismatch with a dilated common hepatic bile duct to 3 cm. She had recurrent choledocholithiasis measuring 3 cm despite prior mechanical and electrohydraulic lithotripsy with cholangioscopy confirmed clearance. As a result, she had undergone a total of 26 prior ERCP treatments (2012)(2013)(2014)(2015)(2016)(2017)(2018)(2019)(2020)(2021). She underwent an EUS-CD with LAMS for definitive treatment. 2-weeks later, an upper endoscope was traversed through the LAMS to perform lithotripsy of remaining choledocholithiasis. At this time, the patient remains free of cholangitis for the past 8 months. (Figure ) Discussion: Biliary complications, like anastomotic stricture and recurrent cholangitis, are a common occurrence in OLTs. With use of various ERCP techniques, obstructions can be removed or ameliorated. Timely treatment of cholangitis is imperative as the mortality rate is high (10-30%). With each cholangitis episode and subsequent ERCP, the cumulative procedural risk and mortality increases. EUS-CD has been demonstrated to be effective in treatment for malignant distal biliary obstruction after failed ERCP. Our patient had previously undergone 26 ERCPs and additionally was on Warfarin for pulmonary embolism putting her at very high risk for complications with additional future endoscopic procedures. This represents a case of successful use of EUS-CD with LAMS in a patient post-OLT with a significant distal common bile duct stricture which had been refractory to traditional management via ERCP. Long term studies are indicated to see if this can provide durable benefit.[2870] Figure 1. 1A. MRI imaging demonstrating a large 3 cm choledocholithiasis within the dilated CBD, which formed as a result of bile stasis. 1B. CT coronal view demonstrating successful deployment of LAMS to complete EUS-CD. 1C. Fluoroscopic view of the standard upper scope traversing through the LAMS for definitive lithotripsy and dredging of remaining choledocholithiasis. 1D. Endoscopic view of the final LAMS placement with double pigtail plastic stents.

Immune checkpoint inhibitors (ICI) are antibodies that block immune checkpoint proteins from binding with their partner proteins on cancer cells, subsequently allowing cytotoxic T-cell-associated enhancement of antitumor responses. Although ICIs have become the standard of care for various malignancies, their use is often limited by unique immune-related adverse events, including dermatologic, endocrine, inflammatory, hepatic, and gastrointestinal events. Diarrhea and colitis are common lower gastrointestinal tract immune-related adverse events, however, only a few cases have reported the association between celiac disease (CD) and ICIs. We report here a case of a 75-year-old man with new onset CD after exposure to the cytotoxic T-lymphocyte-associated antigen-4 ICI, ipilimumab. Although ICI-induced CD is relatively rare, it is essential to consider it in a genetically susceptible patient undergoing treatment with ICI. Patients with known high susceptibility to CD, such as a family history of CD, or with the ancestry of high celiac penetrance (eg, Northern Europe, North Africa, etc), dermatitis herpetiformis, or chronic bowel symptoms, we feel should have celiac panel testing before initiating ICI therapy.

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