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Context Duodenal dystrophy is a rare disease, characterized by the chronic inflammation of the aberrant pancreatic tissue in the duodenal wall. Case reports Two middle-aged men were admitted with upper abdominal pain of several months duration, periodic nausea and vomiting after meals, intermittent jaundice and weight loss. A diagnosis of cystic dystrophy of the vertical part of the duodenum without chronic inflammation of the orthotopic pancreas was established in both cases by multi-detector computed tomography, magnetic resonance imaging and endosonography. Both patients were successfully treated by two modifications of pancreas-preserving duodenal resections with reimplantation of the bile and pancreatic ducts into the neoduodenum. Conclusion These cases are a good example of a pancreas-preserving approach to duodenal dystrophy treatment and can be an alternative to the Whipple procedure in cases of mild changes of the orthotopic gland. Image: Resected specimen of the second part of the duodenum., JOP. Journal of the Pancreas, Vol 11, No 5 (2010): September - 423-497

Context It is important to recognize arterial variants in the preoperative planning of extended pancreatic resections. The absence of surgical confirmation of radiological data is a limitation of the majority of angiographic or CT-angiographic studies of celiac and mesenteric arterial anatomy. Objective The purpose of this study was to test the accuracy of CT angiography in delineating the arterial architecture by comparing the resultant 3D images with findings at surgery and determining the frequency of different celiac and mesenteric arterial anatomy variants. Methods Abdominal CT angiographies of 350 patients were performed on a 64- and 256-MDCT scanner prior to major pancreatic or hepatobiliary surgery. Variants of celiac and mesenteric arterial anatomy were documented as 3D reconstructions. Radiological data were compared to operative photographs during extended pancreaticoduodenectomies and extended distal pancreatectomies in 59 cases. Results Only 197 patients (56.3%) had the classic arterial anatomy identified at CT angiography. The most common variants were a replaced or accessory right hepatic artery originating from the superior mesenteric artery (62 cases, 17.7%) and a replaced or accessory left hepatic artery (43 cases, 12.3%) originating from the left gastric artery. According to a comparison with operative photographs, CT angiography demonstrated 100% accuracy in identifying celiac and mesenteric arterial anatomy variants, stenoses, obstructions and aneurysms of the celiac and mesenteric branches, including those which were hemodynamically significant and which influence the choice and sequence of operative procedures. Conclusion The celiac and mesenteric arterial anatomy variants are fairly common and are of great significance in planning extended pancreatic resections. Radiological findings were fully corroborated by operative data, which means that CT angiography is a reliable tool for identifying celiac and mesenteric arterial anatomy aberrations and arterial lesions. Image: Changes in functioning arterial collaterals after celiac trunk stenting., JOP. Journal of the Pancreas, Vol 11, No 4 (2010): July - p. 299-422

Key Points Question Is cortical-sparing adrenalectomy associated with increased pheochromocytoma-specific morbidity and mortality for patients with bilateral pheochromocytomas compared with total adrenalectomy? Findings In this cohort study of 625 patients with bilateral pheochromocytomas, most had hereditary syndromes, but 36% initially presented with unilateral pheochromocytoma. Bilateral total adrenalectomy resulted in a high rate of adverse effects from glucocorticoid replacement therapy, whereas cortical-sparing surgery was not associated with a worse outcome. Meaning These findings suggest that cortical-sparing surgery may be the preferred approach for patients at risk for, or diagnosed with, bilateral pheochromocytomas, especially those harboring a germline mutation in one of the known predisposition genes., This cohort study compares outcomes associated with cortical-sparing adrenalectomy vs total adrenalectomy for patients with bilateral pheochromocytomas., Importance Large studies investigating long-term outcomes of patients with bilateral pheochromocytomas treated with either total or cortical-sparing adrenalectomies are needed to inform clinical management. Objective To determine the association of total vs cortical-sparing adrenalectomy with pheochromocytoma-specific mortality, the burden of primary adrenal insufficiency after bilateral adrenalectomy, and the risk of pheochromocytoma recurrence. Design, Setting, and Participants This cohort study used data from a multicenter consortium-based registry for 625 patients treated for bilateral pheochromocytomas between 1950 and 2018. Data were analyzed from September 1, 2018, to June 1, 2019. Exposures Total or cortical-sparing adrenalectomy. Main Outcomes and Measures Primary adrenal insufficiency, recurrent pheochromocytoma, and mortality. Results Of 625 patients (300 [48%] female) with a median (interquartile range [IQR]) age of 30 (22-40) years at diagnosis, 401 (64%) were diagnosed with synchronous bilateral pheochromocytomas and 224 (36%) were diagnosed with metachronous pheochromocytomas (median [IQR] interval to second adrenalectomy, 6 [1-13] years). In 505 of 526 tested patients (96%), germline mutations were detected in the genes RET (282 patients [54%]), VHL (184 patients [35%]), and other genes (39 patients [7%]). Of 849 adrenalectomies performed in 625 patients, 324 (52%) were planned as cortical sparing and were successful in 248 of 324 patients (76.5%). Primary adrenal insufficiency occurred in all patients treated with total adrenalectomy but only in 23.5% of patients treated with attempted cortical-sparing adrenalectomy. A third of patients with adrenal insufficiency developed complications, such as adrenal crisis or iatrogenic Cushing syndrome. Of 377 patients who became steroid dependent, 67 (18%) developed at least 1 adrenal crisis and 50 (13%) developed iatrogenic Cushing syndrome during median (IQR) follow-up of 8 (3-25) years. Two patients developed recurrent pheochromocytoma in the adrenal bed despite total adrenalectomy. In contrast, 33 patients (13%) treated with successful cortical-sparing adrenalectomy developed another pheochromocytoma within the remnant adrenal after a median (IQR) of 8 (4-13) years, all of which were successfully treated with another surgery. Cortical-sparing surgery was not associated with survival. Overall survival was associated with comorbidities unrelated to pheochromocytoma: of 63 patients who died, only 3 (5%) died of metastatic pheochromocytoma. Conclusions and Relevance Patients undergoing cortical-sparing adrenalectomy did not demonstrate decreased survival, despite development of recurrent pheochromocytoma in 13%. Cortical-sparing adrenalectomy should be considered in all patients with hereditary pheochromocytoma.