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Captopril challenge test (CCT) is a simple and safe confirmatory test for primary aldosteronism (PA). We investigated the effectiveness of the indices after captopril administration for prediction of unilateral hyperaldosteronism (UHA) on adrenal vein sampling (AVS). We studied 238 patients with PA who had CCT and successful AVS between July 2007 and December 2019 in Sapporo City General Hospital. Receiver operating characteristic (ROC) curve analysis showed that the diagnostic performance for prediction of UHA on AVS in regard to the reduction rate of plasma aldosterone concentration (PAC) after captopril administration was inferior to aldosterone to renin ratio (ARR) and PAC (area under the ROC curve 0.72 vs. 0.84, 0.72 vs. 0.89, respectively, both p < 0.01). Based on the optimal cut-off values in ARR (897 pg/mL/ng/mL/h, sensitivity 64.6%, specificity 93.0%) and PAC (203 pg/mL, sensitivity 73.9%, specificity 93.0%) after captopril administration, the patients were divided into three groups: (1) both positive, (2) one positive, and (3) both negative. The prevalence of UHA on AVS in the three groups were 90.0%, 52.9%, and 7.3%, respectively. In the first group, 31 of 32 patients with unilateral nodular lesion on CT had an ipsilateral unilateral AVS. In conclusion, the combination of post-captopril ARR and PAC is useful for prediction of laterality diagnosis on AVS. AVS is strongly recommended in patients with both positive or one positive results for the optimal cut-off values of post-captopril ARR and PAC and is weakly recommended in patients with both negative results.

Summary A 31-year-old man with Williams syndrome (WS) was referred to our hospital because of a 9-year history of hypertension, hypokalemia, and high plasma aldosterone concentration to renin activity ratio. A diagnosis of primary aldosteronism (PA) was clinically confirmed but an abdominal CT scan showed no abnormal findings in his adrenal glands. However, a 13-mm hypervascular tumor in the posterosuperior segment of the right hepatic lobe was detected. Adrenal venous sampling (AVS) subsequently revealed the presence of an extended tributary of the right adrenal vein to the liver surrounding the tumor. Segmental AVS further demonstrated a high plasma aldosterone concentration (PAC) in the right superior tributary vein draining the tumor. Laparoscopic partial hepatectomy was performed. The resected tumor histologically separated from the liver was composed of clear cells, immunohistochemically positive for aldesterone synthase (CYP11B2), and subsequently diagnosed as aldosterone-producing adrenal adenoma. After surgery, his blood pressure, serum potassium level, plasma renin activity and PAC were normalized. To the best of our knowledge, this is the first report of WS associated with PA. WS harbors a high prevalence of hypertension and therefore PA should be considered when managing the patients with WS and hypertension. In this case, the CT findings alone could not differentiate the adrenal rest tumor. Our case, therefore, highlights the usefulness of segmental AVS to distinguish adrenal tumors from hepatic adrenal rest tumors. Learning points: Williams syndrome (WS) is a rare genetic disorder, characterized by a constellation of medical and cognitive findings, with a hallmark feature of generalized arteriopathy presenting as stenoses of elastic arteries and hypertension. WS is a disease with a high frequency of hypertension but the renin-aldosterone system in WS cases has not been studied at all. If a patient with WS had hypertension and severe hypokalemia, low PRA and high ARR, the coexistence of primary aldosteronism (PA) should be considered. Adrenal rest tumors are thought to arise from aberrant adrenal tissues and are a rare cause of PA. Hepatic adrenal rest tumor (HART) should be considered in the differential diagnosis when detecting a mass in the right hepatic lobe. Segmental adrenal venous sampling could contribute to distinguish adrenal tumors from HART.

In adrenal venous sampling (AVS) for patients with primary aldosteronism (PA), adrenocorticotropic hormone (ACTH) stimulation generally increased the success rate. The effect of ACTH stimulation on the left-right differences of laterality diagnosis in AVS remains unclear. A total of 167 patients with PA underwent successful AVS were examined. Patients with autonomous cortisol secretion were excluded. The proportion of dominant side in AVS was compared before and after ACTH stimulation. Unilateral disease on AVS was defined as a lateralization index of more than 4, both before and after ACTH stimulation. Before ACTH stimulation, unilateral disease was more frequently observed on the right side than the left side (right 33.5% vs. left 13.8%, p < 0.01). After ACTH stimulation, unilateral disease was more frequently observed on the left side than the right side, without statistical significance (left 15.6% vs. right 10.8%, p = 0.20). Among the 56 patients who had right unilateral disease before ACTH stimulation, 17 patients (30.0%) also had right unilateral disease after ACTH stimulation. The affected side of AVS was changed from right unilateral to bilateral after ACTH stimulation in 34 (60.7%) out of 56 patients. These patients had milder PA and CT scans showed no nodular lesions on the right side. In AVS, ACTH stimulation not only decreased unilateral results but also shifted to the dominant side. Overestimation should be carefully considered when the surgical indication for the right adrenal gland was decided based on AVS results without ACTH stimulation.

Background: Williams syndrome (WS) is a microdeletion disorder caused by heterozygous loss of approximately 1.5-Mb pairs of DNA from chromosome 7. Patients with WS have a characteristic constellation of medical and cognitive findings, with a hallmark feature of generalized arteriopathy presenting as stenoses of elastic arteries and hypertension. We present here a case of WS associated with primary aldosteronism (PA), whose source of aldosterone excess was difficult to locate. Clinical case: A 31-year-old man suspected having PA was referred to our Hospital. He was diagnosed as having Williams syndrome at 2 months of age. He had been hypertensive for 9 years. At the age of 31 years old, low serum potassium (K) and high aldosterone to renin ratio were pointed out. His plasma renin activity (PRA) was 0.2 ng/ml/h, plasma aldosterone concentration (PAC) was 456 pg/ml, serum K level was 3.1 mEq/l at admission. Diagnosis of PA was confirmed according to positive captopril challenge test. CT scan revealed a 13mm tumor with low density in posterosuperior segment of right hepatic lobe, but adrenal tumor was not detected. Venography of adrenal vein sampling (AVS) showed an extended tributary of the right adrenal vein to the liver that surrounded the tumor. Segmental AVS was performed for right adrenal vein and showed a high value of PAC (377000 pg/ml) in superior tributary which was drained from the tumor. PAC in other tributaries of right adrenal vein and left adrenal vein was suppressed. Laparoscopic partial hepatectomy was performed as a liver tumor. After surgery, serum K level was normalized, PAC decreased to 67 pg/ml. Pathological examination revealed a golden-yellow tumor separated from the liver by fibrous septum. The tumor was attached to an adrenal cortex embedded in the liver. The tumor was composed of clear cells, positive for 3βHSD2 and CYP11B2 using immunohistochemistry, diagnosed as aldosterone producing adrenal adenoma. Conclusion: To the best of our knowledge, this is the first report of WS associated with PA. Since WS is a disease with high prevalence of hypertension, measurement of PRA and PAC for screening of PA is recommended for patients with WS associated with hypertension. According to CT findings, hepatic adrenal rest tumor was suspected because the tumor was not continuous with the right adrenal gland, but pathological findings demonstrated that an adrenal adenoma was originated from right adrenal cortex rather embedded in the liver. Segmental AVS was useful to distinguish adrenal tumor from hepatic adrenal rest tumor.

To assess the safety and effectiveness of transcatheter arterial embolization (TAE) with tris-acryl gelatin microspheres for patients with symptomatic enlarged polycystic liver disease (PCLD). This prospective study was approved by our hospital’s institutional review board and planned for patients with symptoms related to enlarged PCLD, such as distended abdomen, gastrointestinal obstruction and abdominal pain. Hemi-hepatic embolization with tris-acryl gelatin microspheres was performed in the hepatic artery supplying the hepatic lobe that showed the predominant presence of cysts. Each patient underwent an assessment of liver function, a questionnaire survey about symptoms, measurement of the estimated volume of the whole liver before and after TAE, and an assessment of complications associated with TAE. Five patients (four females, one male; mean age 52.6 ± 9.1 years) were treated. All five patients successfully completed TAE. The left lobe was treated in three patients and the right in two. After TAE, post-embolization syndrome and transient elevation of white blood cells, aspartate aminotransferase, and alanine aminotransferase occurred in all patients, but none developed hepatic insufficiency or severe complications. The mean whole liver volume was 7406 ± 2323 mL before TAE, and 6995 ± 2139 mL (95.1 ± 5.2% of the pre-therapeutic value) at 3 months and 6855 ± 2246 mL (93.3 ± 9.7%) at 12 months after TAE. Three of the five patients reported an improvement of clinical symptoms within 12 months after TAE. TAE with microspheres can be a safe and effective treatment for symptomatic enlarged PCLD.

To determine whether the preoperative pancreatic apparent diffusion coefficient (ADC) can be used to predict the development of postoperative pancreatic anastomotic failure (PAF).We retrospectively examined the cases of 79 patients who underwent pancreatic head resection between January 2010 and October 2013. The patients underwent 1.5-T MR imaging including diffusion-weighted imaging before surgery. The main pancreatic duct diameter (MPD), the pancreatic parenchymal thickness (PT), and the ADC of the pancreatic remnant parenchyma were measured. Two radiologists blinded to the patients' outcomes performed the measurements. The imaging parameters were compared between the patients who developed PAF and those who did not. The cut-off ADC for the development of PAF was calculated with a receiver operating characteristic analysis.The imaging parameters were highly correlated between the two observers. The MPD and PT did not differ significantly among the patients. The mean pancreatic ADCs were significantly higher in the patients with PAF than in those without PAF. An ADC higher than 1.50 × 10(-3) mm(2)/s (Az = 0.719, observer-1) or 1.35 × 10(-3) mm(2)/s (Az = 0.752, observer-2) was optimal for predicting the development of postoperative PAF.Measuring the preoperative non-tumorous pancreatic ADC may be useful for the prediction of a postoperative PAF.