Background Hyperammonemia secondary to liver disease is a very common cause of hepatic encephalopathy (HE) and it is easily recognized in patients with advanced liver disease. Non-cirrhotic causes of hyperammonemia are rare, particularly extrahepatic portosystemic venous shunts (EPS). The majority of these shunts are between a mesenteric vein and the inferior vena cava. We report a case of a non-cirrhotic hyperammonemia secondary to a shunt between the superior mesenteric vein (SMV) and the right renal vein (RRV) that presented with encephalopathy. Diagnosis was delayed due to lack of awareness of non-cirrhotic hyperammonemia underscoring the importance of measuring ammonia in all patients presenting with encephalopathic symptoms irrespective of their liver function. Aims To report our experience with a patient with unexplained cognitive dysfunction that was eventually attributed to hyperammonemia secondary to a rare non-cirrhotic portosystemic shunt. Also, we discuss the differential diagnoses of non-cirrhotic hyperammonemia and the pathophysiology, classification and diagnosis of spontaneous portosystemic shunts. Methods A retrospective chart review Results A 57-year-old woman with longstanding essential tremors on topiramate presented with a 30-month history of recurrent disabling episodes of unexplained “zoning out”. Her neurologists undertook extensive investigations which excluded primary neurological conditions. These episodes persisted despite discontinuation of topiramate, treatment of urinary tract infection (UTI) and continuing daily prophylactic antibiotics for recurrent UTIs as presumed etiologies. Due to her unexplained and disabling symptoms she was referred to an internist. During further evaluation, ammonia level was measured for first time, in the absence of any obvious features of chronic liver disease, and the level was strikingly elevated 152uml/l. Hence, an abdominal CT was obtained and revealed a prominent shunt between SMV and RRV. The patient was diagnosed with non-cirrhotic hyperammonemia secondary to EPS. She is currently stable on lactulose and rifaximin with a drop in her ammonia level to 63uml/l, and an interventional radiological procedure is being considered. Conclusions While hyperammonemia is most commonly related to liver failure, our case highlights the importance of awareness of non-cirrhotic hyperammonemia. Any unexplained change in level of consciousness, cognition and/or behavior merits measurement of serum ammonia irrespective of clinical liver status. Funding Agencies None
Background Pancreatic intraductal papillary mucinous neoplasms (IPMNs) carry malignant risk and they account for up to 7% of pancreatic neoplasms, hence it is crucial to identify high-risk lesions to determine timely therapy Distinction between main duct-IPMN (MD-IPMN) and other pancreatic disorders often relies on endoscopic ultrasound, MRI, and CT findings. These findings are very accurate in advanced cases, yet they are less sensitive for the diagnosis of early lesions Direct endoscopic visualization of the biliary and pancreatic ducts has gained an increased interest due to the development of single-operator scopes with a better image resolution. Several case series and a retrospective single-center cohort study report the beneficial role of single operator pancreatoscopy (SOP) in the evaluation and sampling of indeterminate PD lesions Aims Evaluate our experience with SOP and determine its 1.Efficacy and safety in diagnosing MD-IPMN and PD neoplasia 2.Sensitivity (Sn) and specificity (Sp) in diagnosing high risk PD lesions Methods All patients (age ≥18 year old) who had attempted SOP using the SpyGlass (Boston Scientific Corp, Natick, MA) between June 2013 and November 2019 for the evaluation of indeterminate main PD strictures, dilatation, or with suspected or known MD-IPMN were included The primary outcome was the efficacy and safety of SOP in diagnosing high-risk PD lesions A minimum of 12 months follow-up (F/U) from index SOP was required to define a true negative for patients (pts) with benign SOP findings Results A total of 43 pts underwent SOP; 18 (42%) males and 25 (58%) females, with a mean age of 66.1 years. Technical success was (n=35/43; 81.4%) In the diagnosed MD-IPMN group (n=14/35; 40%), all underwent surgery except three; one is awaiting surgery, the second had an aborted Whipple’s and the last was lost to F/U. The remaining eleven pts (78.6%) had confirmed diagnosis of MD-IPMN with moderate or high-grade dysplasia, or pancreatic cancer Of those who had benign SOP findings (n=21), nineteen (90.5%) did not develop pancreatic neoplasm during a mean F/U period of 59.3 months (range 13 to 84 months). One Pt developed mild pancreatitis Conclusions This study suggests that SOP is a safe, effective and feasible procedure that should be considered in the diagnostic algorithm of indeterminate main PD strictures, dilatation, or those with suspected or known MD-IPMN with or without side branch involvement. We demonstrate a high technical success rate, Sn, and Sp of 81.4%, 100%, and 100%, respectively If all pts with incomplete outcomes data are considered to have be diagnostically inaccurate, the Sn and Sp remain high at 81.4% and 86.4% respectively. The adverse events seem similar to ERCP with only one Pt. (2.7%) developed mild pancreatitis, although this sample has insufficient numbers to evaluate rare events Funding Agencies None
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