BACKGROUND:Nephropathic cystinosis is an autosomal recessive disease resulting from intracellular accumulation of cystine leading to multiple organ failure.CASE REPORT:We describe the clinical course of a patient managed from the age of six until his death at the age of 33 years. He underwent multiple surgery, including two renal transplants, developed transplant renal artery stenosis that was managed medically, and progressive heart failure at the age of 33 years. His death from a ruptured pseudoaneurysm associated with a restrictive cardiomyopathy is noteworthy. A limited cardiac autopsy revealed the presence of cystine crystals in interstitial cardiac histiocytes and one myocardial cell, along with 1000-fold higher tissue cystine content of the left ventricular myocardium compared to patients without cystinosis, suggesting the possibility of direct cystine mediated metabolic injury.

Pancreatic pseudocyst is a common complication of pancreatitis. Pseudocysts may require decompression when they become painful, infected, or start compressing surrounding organs. Decompression is achieved by endoscopic cystogastrostomy. Recently, the use of lumen-apposing metal stent (LAMS) for cystogastrostomy has gained popularity due to ease of use and high technical success. LAMS has a wider lumen, which allows for direct endoscopic necrosectomy in the cases of walled-off necrosis. Our patient is a 30-year-old male who presented with massive hematemesis and dizziness. He had a history of chronic alcohol-induced pancreatitis. Three weeks before the presentation, he underwent a cystogastrostomy with LAMS placement to treat a 10-cm walled-off necrosis. Urgent computed tomography (CT) scan did not reveal any acute finding suggestive of bleeding. Esophagogastroduodenoscopy showed blood protruding from the LAMS with a large clot formation. Attempts to stop bleeding were unsuccessful. He underwent CT angiography of the abdomen. CT angiography showed a bleeding pseudoaneurysm (PA) believed to be a complication of the LAMS. Subsequently, multiple coils were placed in the splenic artery near the PA. The patient continued to improve without a further drop in hemoglobin and was eventually discharged. PA formation and subsequent rupture is a rare delayed complication of LAMS. It may lead to massive gastrointestinal bleeding with a high mortality rate. Diagnostic delays have resulted in increased mortality by 60%. In this article, we present a case of massive gastrointestinal bleeding due to a ruptured splenic artery PA presenting as a delayed complication of LAMS.

This study sought to evaluate a new method that uses injection of fibrin sealant under simultaneous balloon occlusion for the treatment of postinterventional access site bleeding complications. With the rising complexity of interventional procedures, iatrogenic false aneurysms and active bleeding has become more common. In general, these complications are associated with increased morbidity and mortality, especially if surgical repair is required. Although high success rates are reported for ultrasound‐guided compression and ultrasound‐guided thrombin injection, these methods are not always feasible. All procedures of fibrin sealant injection under simultaneous balloon occlusion for the treatment of postinterventional access site bleeding complications or pseudoaneurysm were prospectively collected. Additional data were retrospectively obtained and analyzed for all patients treated by this new method. In total, 53 patients were included from 2018 to 2021. Most of the access site complications were related to transcatheter aortic valve replacement (40%) or percutaneous coronary intervention (21%), but also to a wide variety of other procedures. Of the 53 patients, 30 had to be treated for false aneurysms and 23 for active bleeding. A high primary success rate of 94% was achieved. Recurrences of false aneurysms occurred in six patients, of which only one needed open surgical repair. Regarding complications, two peripheral embolisms, thereof one requiring additional stent implantation occurred. Balloon‐assisted thrombin injection seems to be feasible and safe. It provides a new alternative to prevent surgery for patients where common techniques are unavailable or have failed.