Belzutifan, a Potent HIF2α Inhibitor, in the Pacak-Zhuang Syndrome.

The integration of genomic testing into clinical care enables the use of individualized approaches to the management of rare diseases. We describe the use of belzutifan, a potent and selective small-molecule inhibitor of the protein hypoxia-inducible factor 2α (HIF2α), in a patient with polycythemia and multiple paragangliomas (the Pacak-Zhuang syndrome). The syndrome was caused in this patient by somatic mosaicism for an activating mutation in EPAS1. Treatment with belzutifan led to a rapid and sustained tumor response along with resolution of hypertension, headaches, and long-standing polycythemia. This case shows the application of a targeted therapy for the treatment of a patient with a rare tumor-predisposition syndrome. (Funded by the Morin Family Fund for Pediatric Cancer and Alex's Lemonade Stand Foundation.).

Beyond symptomatology: A comparative analysis of unilateral and bilateral macronodular mild autonomous cortisol secretion.

OBJECTIVE: To investigate the clinical, laboratory findings and signal intensity index (SII) on magnetic resonance imaging (MRI) of patients with bilateral and unilateral macronodular mild autonomous cortisol secretion (MACS). PATIENTS AND MEASUREMENTS: Clinical and laboratory findings of 81 patients with MACS were examined from retrospective records. SII of adenomas and internodular areas were evaluated by MRI. The unilateral group included patients with an adrenal macronodule (≥1 cm) in a single adrenal gland, while the bilateral group included patients with at least one macronodule in both adrenal glands. RESULTS: In total, 46 patients were in the unilateral (57%), while 35 (43%) patients were in the bilateral groups. The dehydroepiandrosterone sulphate (DHEA-S) level was lower in the unilateral than in the bilateral group (p < .001). The presence of type 2 diabetes mellitus (T2DM), glycosylated haemoglobin (HbA1c) and low-density lipoprotein (LDL) concentrations were higher in the bilateral group (p < .05). However, no significant difference was detected in terms of adrenocorticotropic hormone (ACTH) and overnight 1 mg dexamethasone suppression test (DST) between the two groups (p > .05). There was no difference in SII between adenomas within the same patient, as well as between the unilateral and bilateral groups (p > .05). Logistic regression analysis based on the differentiation between unilateral and bilateral macronodular MACS demonstrated that DHEA-S, HbA1c and LDL concentrations were associated factors. CONCLUSION: DHEA-S levels may not be as suppressed in patients with bilateral macronodular MACS as compared to those with unilateral adenoma. T2DM and hypercholesterolaemia have a higher frequency in bilateral patients. However, ACTH, overnight 1 mg DST and SII may not provide additional information for differentiation of bilaterality and unilaterality.

Feasibility Study of Single-Photon Emission Computed Tomography with Iodine-123 Labeled Metaiodobenzylguanidine for Preclinical Evaluation of Labetalol as a ß-Adrenergic Receptor Blocker.

Among clinically used radiopharmaceuticals, iodine-123 labeled metaiodobenzylguanidine ([123I]mIBG) serves for diagnosing neuroendocrine tumors and obtaining images of myocardial sympathetic innervation. mIBG, a structural analogue of norepinephrine (NE), a neurotransmitter acting in peripheral and central nerves, follows a pathway similar to NE, transmitting signals through the NE transporter (NET) located at synaptic terminals. It moves through the body without decomposing, enabling noninvasive image evaluation. In this study, we aimed to quantify [123I]mIBG uptake in the adrenal glands using small animal single-photon emission computed tomography/computed tomography (SPECT/CT) images post [123I]mIBG administration. We investigated the possibility of assessing the effectiveness of ß-adrenergic receptor blockers by quantifying SPECT/CT images and biodistribution results to determine the degree of [123I]mIBG uptake in the adrenal glands treated with labetalol, a known ß-adrenergic receptor blocker. Upon intravenous administration of [123I]mIBG to mice, SPECT/CT images were acquired over time to confirm the in vivo distribution pattern, revealing a clear uptake in the adrenal glands. Labetalol inhibited the uptake of [123I]mIBG in cell lines expressing NET. A decrease in [123I]mIBG uptake in the adrenal glands was observed in the labetalol-treated group compared with the normal group through SPECT/CT imaging and biodistribution studies. These results demonstrate that SPECT/CT imaging with [123I]mIBG could be applicable for evaluating the preclinical efficacy of new antihypertensive drug candidates such as labetalol, a ß-adrenergic receptor blocker.

Feasibility of spectral CT-derived extracellular volume fraction for differentiating aldosterone-producing from nonfunctioning adrenal nodules.

OBJECTIVE: To assess the feasibility of spectral CT-derived extracellular volume (ECV) for differentiating aldosterone-producing nodules (APN) from nonfunctioning adrenal nodules (NFN). METHODS: Sixty-nine patients with biochemically and histologically confirmed unilateral APN (34) and NFN (35) as well as 23 patients with bilateral APN (19) and NFN (27) confirmed biochemically and by adrenal vein sampling (AVS) were enrolled in this retrospective study from October 2020 to April 2022. All patients underwent contrast-enhanced spectral CT of the adrenal glands with a 10-min delayed phase. The haematocrit level was measured within 2 days of CT. An iodine density map was derived from the delayed CT. The ECV fractions of the APN and NFN were calculated and compared in the test cohort of 69 patients with unilateral adrenal nodules. The optimal cut-off value was determined to evaluate the diagnostic efficacy of the ECV fraction for differentiating APN from NFN in the validation cohort of 23 patients with bilateral adrenal nodules. RESULTS: The ECV fractions of the APN (11.17 ± 4.57%) were significantly lower (p < 0.001) than that of the NFN (24.79 ± 6.01%) in the test cohort. At cut-off ECV value of 17.16%, the optimal area under the receiver operating characteristic curve was 0.974 (95% confidence interval: 0.942-1) with 91.4% sensitivity, 93.9% specificity, and 92.8% accuracy in the test cohort and 89.5% sensitivity, 96.3% specificity, and 93.5% accuracy in the validation cohort for differentiating APN from NFN. CONCLUSION: The spectral CT-derived ECV fraction can differentiate APN from NFN with high diagnostic performance. CLINICAL RELEVANCE STATEMENT: Spectral CT-derived extracellular volume fraction could accurately differentiate between adrenal aldosterone-producing nodules and nonfunctioning nodules. It might serve as a noninvasive alternative to adrenal vein sampling in primary aldosteronism patients with bilateral adrenal nodules. KEY POINTS: • Conventional CT cannot differentiate aldosterone-producing adrenal nodules from nonfunctioning nodules. • Extracellular volume of adrenal aldosterone-producing nodules was significantly lower than that of nonfunctioning nodules and normal adrenal glands. It can accurately differentiate between aldosterone-producing and nonfunctioning adrenal nodules. • Extracellular volume may be a novel, noninvasive biomarker alternative to adrenal vein sampling for determining the functional status of bilateral adrenal nodules in patients with primary aldosteronism.

Imaging Concordance With Vein Sampling for Primary Aldosteronism: A Cohort Study and Literature Review.

INTRODUCTION: Adrenal venous sampling (AVS) is used to distinguish unilateral from bilateral aldosterone hypersecretion as a cause of primary aldosteronism (PA). Unilateral disease is treated with adrenalectomy and bilateral hypersecretion managed medically. METHODS: We performed a single institution retrospective cohort study of adult patients undergoing adrenalectomy for PA from July 2013 to June 2022. Concordance of imaging findings with AVS was evaluated. Statistical analysis was performed with Mann-Whitney U and chi-squared Fisher's exact. Literature review performed via triple method search strategy. RESULTS: Twenty-one patients underwent AVS and adrenalectomy for PA. Two patients did not have imaging findings and 19 were localized with an adenoma. For patients with image localization, AVS was concordant in nine, discordant in four, and nondiagnostic in six. For patients with discordant findings, age range was 35.8 to 72.4 y compared with concordant patient age range of 49.8 to 71.7 y. Overall discordance between imaging results and AVS was 40%. The aldosterone level was associated with concordance with a median of 52 ng/dL compared with 26 ng/dL if discordant (P = 0.002). There was a significant reduction in antihypertensive medications for the entire cohort from a median of three medications (interquartile range 2-4) to 1 medication (interquartile range 1-2), P < 0.001. CONCLUSIONS: In this cohort, 40% of patients with selective AVS had discordant imaging and AVS results. Aldosterone level was associated with concordance. Hypertension was significantly improved with a median decrease of two antihypertensives. Our results support performance of AVS on all candidates for adrenalectomy for PA.

Calculated Right Adrenal Vein Aldosterone Levels in Patients Undergoing Adrenal Vein Sampling: The Potential to Lateralize Despite Unsuccessful Selection of the Right Adrenal Vein.

PURPOSE: The aim of this study was to use calculated aldosterone level in the right adrenal vein (RAV) (cAldoRAV) rather than measured level for identifying the dominant side of aldosterone secretion in patients with primary aldosteronism undergoing adrenocorticotropic hormone-stimulated adrenal venous sampling (AVS). MATERIALS AND METHODS: Patients with primary aldosteronism who had successful AVS (selectivity index, >3) were studied. Based on the assumption that cortisol production from both adrenal glands is equal, aldosterone level in the RAV was calculated using the data from the left adrenal vein and inferior vena cava. The aldosterone level in the left adrenal vein (AldoLAV) compared with the cAldoRAV (AldoLAV:cAldoRAV ratio) was then used to determine the dominant side of aldosterone secretion compared with standard AVS interpretation using lateralization index (LI). LI ≥4 indicated unilateral disease, and LI ≤3 indicated bilateral disease. The LI between 3 and 4 was diagnosed as indeterminate. RESULTS: Sixty-eight patients with concordant results between AVS and adrenal imaging study (32 were left-sided, 22 were right-sided, and 14 were bilateral) were selected for studying diagnostic performance. The AldoLAV:cAldoRAV ratio with the cutoff values of ≥3 and ≤0.33 could identify unilateral diseases (left-sided and right-sided disease, respectively) with 92.6% sensitivity and 100% specificity. CONCLUSIONS: The calculated AldoLAV:cAldoRAV ratio can determine the dominant side of aldosterone secretion with high sensitivity and specificity when compared with standard AVS interpretation of measured levels. It provides an option for identification of unilateral and bilateral disease in select patients in whom right adrenal vein selection is unsuccessful.

Adrenal Vein Sampling: Tips and Tricks.

Adrenal vein sampling (AVS) is the standard method for distinguishing unilateral from bilateral sources of autonomous aldosterone production in patients with primary aldosteronism. This procedure has been performed at limited specialized centers due to its technical complexity. With recent advances in imaging technology and knowledge of adrenal vein anatomy in parallel with the development of adjunctive techniques, AVS has become easier to perform, even at nonspecialized centers. Although rare, anatomic variants of the adrenal veins can cause sampling failure or misinterpretation of the sampling results. The inferior accessory hepatic vein and the inferior emissary vein are useful anatomic landmarks for right adrenal vein cannulation, which is the most difficult and crucial step in AVS. Meticulous assessment of adrenal vein anatomy on multidetector CT images and the use of a catheter suitable for the anatomy are crucial for adrenal vein cannulation. Adjunctive techniques such as intraprocedural cortisol assay, cone-beam CT, and coaxial guidewire-catheter techniques are useful tools to confirm right adrenal vein cannulation or to troubleshoot difficult blood sampling. Interventional radiologists should be involved in interpreting the sampling results because technical factors may affect the results. In rare instances, bilateral adrenal suppression, in which aldosterone-to-cortisol ratios of both adrenal glands are lower than that of the inferior vena cava, can be encountered. Repeat sampling may be necessary in this situation. Collaboration with endocrinology and laboratory medicine services is of great importance to optimize the quality of the samples and for smooth and successful operation. ©RSNA, 2024 Test Your Knowledge questions for this article are available in the supplemental material.

Success Rate of Adrenal Venous Sampling and its Determining Factors: Experience of a Single Center in Malaysia.

BACKGROUND: Primary hyperaldosteronism has been regarded as the commonest and potentially curable cause of secondary hypertension in up to 80% of cases if this condition is detected early. Laparoscopic adrenalectomy proved to be a promising curable method for primary hyperaldosteronism secondary to aldosterone producing adenoma, while primary hyperaldosteronism secondary to bilateral adrenal hyperplasia requires optimization of medical treatment. Adrenal venous sampling (AVS) has been recommended by the Endocrine Society's guideline in addressing the subtypes of primary hyperaldosteronism. Therefore, determining success rate of AVS in our center is crucial in the management of primary hyperaldosteronism and to prevent redundant procedures. The objectives of this study were to evaluate the success rate of AVS in our center and the associated factors that correlate with the success rate. METHODS: A retrospective study in a single center, all adult patients who were diagnosed with primary hyperaldosteronism and underwent AVS in our center between 2014 until 2022 were included. Successful samples defined by a selectivity index of ≥2, that is, the ratio of adrenal vein cortisol level to the peripheral vein cortisol level, were evaluated. The baseline demographic characteristics of each patient, technical parameters of AVS, and anatomical parameters of right adrenal vein from the venographic images were evaluated and analyzed to correlate with the outcome of AVS. A P-value of <0.05 was considered statistically significant. RESULTS: The overall success rate of AVS in our center was 61.3%. The success rate significantly increased to 80.6% in the trained interventional radiologist (IR) group, which represents a single IR who had completed specific training in AVS, compared to non-trained IR group which consisted of a total of 10 IRs without specific AVS training (P = 0.046). The right AVS had lower success rate than the left AVS (64% vs. 94.6%). The type of right adrenal vein pattern showed significant association with the outcome of AVS (P = 0.014). There were 6 types of right adrenal vein patterns observed in our study; Type 1 - gland-like pattern with numerous branches, Type 2 - delta pattern, Type 3 - triangular pattern with central "blush", Type 4 - no discernible pattern and Type 5 - spidery or stellate pattern. The sixth pattern was the hepatic radicles blush with hepatic drainage, for which blood sampling were also collected due to its appearance was frequently indistinguishable from the Type 1 pattern. The results showed Type 2 pattern had the highest incidence (30.5%; 32/105 samples) and Type 5 had the highest success rate (100%; 11/11 samples), while the hepatic radicles blush pattern had the highest incidence in fail samples resulting in the highest failure rate (94.7%; 18/19 samples). The visualization of the inferior emissary vein (IEV) from the venographic images during right AVS had a significant association with the successful AVS (97.5% success rate; 39/40 samples; P = 0.003). Contrariwise, blood sampling withdrawn from the right adrenal vein in the presence of hepatic drainage communication associated with the failure AVS (86.7% failure rate; 26/30 samples; P = 0.001). CONCLUSIONS: The overall success rate of both AVS in our center was 61.3%, and the success rate was higher in the trained IR group than the non-trained IR group. The visualization of IEV and the type of right adrenal vein patterns were the key determining factors with significant association to the successful right AVS. Conversely, blood sampling withdrawn from the hepatic radicles and hepatic drainage had significant association with the failure right AVS.

Brain tissue heterotopic in the adrenal gland in a child: a scarce case report.

Heterotopic brain tissue is rare and has not been reported. Our center made the first report. 4 years and 2 months old Girl presented with a cystic mass in the right adrenal gland 2 weeks after right upper abdominal pain. The operation was successful, and the diagnosis was confirmed by postoperative pathology. 6 months after the procedure, the incision healed well without recurrence. This case report has a detailed diagnosis and treatment process and satisfactory examination results. It can provide a reference for diagnosing and treating clinical HBT and reduce the risk of misdiagnosis and mistreatment.

Prenatal diagnosis of intrathoracic kidney and adrenal gland in left-sided congenital diaphragmatic hernia.

A 29-year-old primigravida at 31 weeks of gestation was referred for intrathoracic kidney (ITK). Ultrasound revealed left kidney intrathoracic placement with an anteriorly positioned ectopic adrenal gland. Magnetic resonance imaging confirmed diaphragmatic interruption and colon herniation. A female neonate, delivered at 37 weeks, underwent successful thoracoscopic repair for a left Bochdalek hernia. Despite compression of the left lung, notably optimistic lung-to-head ratio (LHR) values were observed, correlating with favorable outcomes. This case underscores the rare occurrence of ITK, its association with Bochdalek hernia, and the importance of comprehensive prenatal evaluations.

Ultrasonographic measurements of the normal adrenal glands in guinea pigs (Cavia Porcellus).

Hyperadrenocorticism is an uncommon but important endocrine disease in guinea pigs, but due to its subtle clinical signs and the limited information in veterinary literature, it can be underdiagnosed or misdiagnosed. Ultrasound of the adrenal glands in patients with suspected hyperadrenocorticism can help in identifying adrenomegaly. The purpose of this prospective study was to identify ultrasonographic adrenal gland dimensions in presumed healthy guinea pigs using the same standardized method described for dogs and cats. A conscious ultrasound scan was conducted on twenty client-owned, presumed healthy guinea pigs, and their adrenal glands were measured. A possible correlation between adrenal dimensions with age, sex, and body weight was investigated. The mean length, cranial and caudal pole thickness for the left and right adrenal glands were, respectively, 12.64 ± 2.11 mm and 11.55 ± 1.52 mm; 4.83 mm ± 1.03 mm and 4.69 ± 1.34 mm; 4.8 ± 1.23 mm and 4.04 ± 0.75 mm. The thickness of the left caudal pole was significantly higher than the right (P = 0.02). A significant positive correlation was found between the length of the left adrenal gland and both age (r = 0.46; P = .03) and weight (r = 0.59; P = .01). Statistical correlation between the thickness of each cranial and caudal pole, with age, sex, or weight, was not found. The dimensions provided could prove a useful tool in the clinical evaluation of guinea pigs with suspected hyperadrenocorticism.

Super Mario sign at somatostatin receptor PET/CT.

68Ga-DOTA NOC PET-CT imaging has been shown to have high accuracy for the evaluation of neuroendocrine tumours. We present the case of a 59-year-old male with well differentiated gastric neuroendocrine tumour (grade II) treated with surgery. 68Ga-DOTA NOC PET/CT was performed to rule out metastasis. 68Ga-DOTA NOC showed physiological uptake in the bilateral adrenal and horseshoe kidney appearing as the famous character Super Mario. There is no evidence of any abnormal somatostatin avid lesion.

Successful Conservative Treatment of Maternal Spontaneous Unilateral Adrenal Hemorrhage Causing Severe Anemia in the Third Trimester of Pregnancy-A Case Report.

We present the case of a 32-year-old pregnant woman in the 32 + 3 weeks of pregnancy who presented to the hospital with an exacerbation of pain in the right flank. The diagnostic evaluation revealed the presence of severe anemia and a spontaneous adrenal hemorrhage (SAH) in the right adrenal gland. The patient was transferred to the Perinatology, Obstetrics and Gynecology Clinic with the intention of undergoing preterm childbirth. However, the doctors made a risky decision to wait until week 37 and to terminate the pregnancy at that point. The decision was right, as a cesarean section was performed without complications, and the patient gave birth to a healthy child. Spontaneous adrenal hemorrhage (SAH) is a rare condition, defined as spontaneous hemorrhage without trauma or anticoagulant therapy. Due to bleeding and damage to the adrenal cortex, SAH can lead to adrenal insufficiency. Because of its non-specific symptoms and potentially fatal outcomes for the patient and fetus, it should be considered during diagnostics.

[Evaluation of the anatomical morphology of the adrenal veins in patients with primary aldosteronism by adrenal venous sampling].

Objective: To analyze the anatomical characteristics of the adrenal veins through adrenal venography to improve the success rate of adrenal venography (AVS). Methods: This study was a cross-sectional study. Patients who were diagnosed with primary aldosteronism and underwent AVS from January 2019 to October 2023 at the First Affiliated Hospital of Dalian Medical University were included. Adrenal vein imaging was collected from the enrolled patients. We performed statistical analysis on the adrenal vein orifice position, inflow angle, and adrenal venography morphology. The adrenal venous orifice was defined as the location where the catheter was placed at the end of the calm inhalation. Spearman correlation analysis was used to explore the relationship between the positions of bilateral adrenal vein orifices and body mass index (BMI). Results: A total of 282 patients with successful bilateral AVS and complete bilateral adrenal vein imaging were enrolled, of whom 57.1% (161/282) were male and the age was (53.3±10.7) years old. The orifice of the left adrenal vein was located between the middle segment of the 11th thoracic vertebra and the upper segment of the 2nd lumbar vertebra. The inflow angle relative to the position of the orifice was all leftward and upward. The orifice of the right adrenal vein was located between the upper segment of the 11th thoracic vertebra and the lower segment of the 1st lumbar vertebra, and 91.1% (257/282) had a rightward and downward angle of inflow relative to the position of the orifice. The position of the adrenal vein orifices on both the left (r=0.211, P<0.001) and right (r=0.196, P=0.001) showed positive correlation with BMI. The position of the right adrenal vein orifice also increased with the position of the left adrenal orifice (r=0.530, P<0.001). The most common adrenal venography morphology on the right side was triangular (36.5%, 103/282), while the most common venography morphology on the left side was glandular (66.3%, 187/282). Conclusions: The anatomical morphology of adrenal veins are diverse. Being familiar with the morphological characteristics of the adrenal vein and identifying the adrenal vein accurately during surgery has important clinical value in improving the success rate of AVS.

Fully automatic volume measurement of the adrenal gland on CT using deep learning to classify adrenal hyperplasia.

OBJECTIVES: To develop a fully automated deep learning model for adrenal segmentation and to evaluate its performance in classifying adrenal hyperplasia. METHODS: This retrospective study evaluated automated adrenal segmentation in 308 abdominal CT scans from 48 patients with adrenal hyperplasia and 260 patients with normal glands from 2010 to 2021 (mean age, 42 years; 156 women). The dataset was split into training, validation, and test sets at a ratio of 6:2:2. Contrast-enhanced CT images and manually drawn adrenal gland masks were used to develop a U-Net-based segmentation model. Predicted adrenal volumes were obtained by fivefold splitting of the dataset without overlapping the test set. Adrenal volumes and anthropometric parameters (height, weight, and sex) were utilized to develop an algorithm to classify adrenal hyperplasia, using multilayer perceptron, support vector classification, a random forest classifier, and a decision tree classifier. To measure the performance of the developed model, the dice coefficient and intraclass correlation coefficient (ICC) were used for segmentation, and area under the receiver operating characteristic curve (AUC), accuracy, sensitivity, and specificity were used for classification. RESULTS: The model for segmenting adrenal glands achieved a Dice coefficient of 0.7009 for 308 cases and an ICC of 0.91 (95% CI, 0.90-0.93) for adrenal volume. The models for classifying hyperplasia had the following results: AUC, 0.98-0.99; accuracy, 0.948-0.961; sensitivity, 0.750-0.813; and specificity, 0.973-1.000. CONCLUSION: The proposed segmentation algorithm can accurately segment the adrenal glands on CT scans and may help clinicians identify possible cases of adrenal hyperplasia. KEY POINTS: • A deep learning segmentation method can accurately segment the adrenal gland, which is a small organ, on CT scans. • The machine learning algorithm to classify adrenal hyperplasia using adrenal volume and anthropometric parameters (height, weight, and sex) showed good performance. • The proposed segmentation algorithm may help clinicians identify possible cases of adrenal hyperplasia.

To Use or Not to Use? Quantifying the Benefit of Microcatheter Use in Adrenal Vein Sampling.

Adrenal vein sampling is a technically difficult procedure with reported sampling success rates as low as 41%. Routine microcatheter use has been suggested by some to improve sampling adequacy. This study is a single-institution retrospective review of adrenal vein sampling procedures performed from 2014 to 2021 to quantify sample selectivity and adequacy with and without the use of a microcatheter. Microcatheter sampling was performed in 43 (47%) of 92 cases on the right adrenal gland and 44 (48%) of 92 cases on the left adrenal gland. Mean selectivity index was significantly higher bilaterally with microcatheters (right, 36.8 vs 27.7; P = .05; left, 33.9 vs 19.9 left; P < .001). However, sampling adequacy rates did not significantly differ between microcatheter and 5-F sampling bilaterally (right, 91% vs 90%; P = .88; left, 96% vs 98%; P = .51). Adrenal hemorrhage occurred exclusively with right-sided microcatheter sampling (n = 6, 13%). In conclusion, although microcatheter sampling increases mean selectivity index, it does not change sampling adequacy rate and may increase the risk of right adrenal hemorrhage.

Utility of Carbon Dioxide Venography and Intraprocedural CT for Adrenal Venous Sampling in Patients with an Allergy to Iodinated Contrast Media.

PURPOSE: To assess the diagnostic performance of carbon dioxide (CO2) and intraprocedural unenhanced computed tomography (CT) for adrenal venous sampling (AVS) (CO2-intraprocedural unenhanced CT-AVS) in patients with primary aldosteronism (PA) and a history of iodine contrast medium allergy. MATERIALS AND METHODS: CO2-intraprocedural unenhanced CT-AVS was performed in 18 patients with iodine contrast media allergies at the authors' hospital between December 2015 and January 2021. CT and noncontrast magnetic resonance angiography were used to evaluate the preoperative adrenal vein anatomy. CO2 venography was performed to confirm adrenal vein catheterization. Additionally, intraprocedural unenhanced CT was also performed to confirm catheter position in the right adrenal gland. RESULTS: In all cases in which CO2-intraprocedural unenhanced CT-AVS was performed, the right and left adrenal veins were catheterized appropriately, leading to a localized diagnosis. Catheterization of the left adrenal vein was confirmed using CO2 venography in all cases. In 7 of the 18 cases, CO2 venography demonstrated selection of the right adrenal vein. In 15 of 18 cases, intraprocedural unenhanced CT demonstrated selection of the right adrenal vein. CONCLUSIONS: CO2-intraprocedural unenhanced CT-AVS demonstrated the same diagnostic ability for PA localization as conventional AVS with iodine contrast media. The proposed method is clinically feasible for AVS, in which iodine contrast media use is restricted.

Best Practices: Indications and Procedural Controversies of Adrenal Vein Sampling for Primary Aldosteronism.

Primary hyperaldosteronism (PA) is the most common secondary form of hyper-tension in middle-aged adults. Its harmful effects exceed those of essential hyper-tension. Once PA is diagnosed, treatment hinges on whether aldosterone secretion is unilateral or bilateral, as the former can be effectively treated with adrenalectomy but the latter is treated medically with mineralocorticoid receptor antagonists such as spironolactone or eplerenone. Adrenal vein sampling (AVS) is critical in this determination. There is wide variation in how AVS is performed, including whether to use synthetic adrenocorticotropic hormone stimulation and where the catheter tip should be placed during left adrenal gland sampling. In addition, some institutions and guidelines omit AVS in young patients (i.e., those younger than an age threshold ranging from 35 to 40 years old) who have unilateral adrenal findings on cross-sectional imaging. In this article, we provide background on PA and performance of AVS and then summarize the evidence supporting best practices for these three areas of controversy regarding how and when to perform AVS.

Significance of computed tomography combined with postural stimulation test in predicting laterality of primary aldosteronism.

BACKGROUNDS: Adrenal venous sampling (AVS) represents the gold standard for classifying primary aldosteronism (PA). However, AVS is a technically demanding, expensive and invasive procedure. Computed tomography (CT) scans is recommended as the initial study of classification diagnosis by the current guidelines. In addition, postural stimulation test (PST) has been used to provide additional subtype diagnostic information. OBJECTIVE: This work aimed to evaluate the diagnostic utility of the adrenal CT combined with PST in the classification diagnosis of PA. METHODS: We analyzed PA patients who underwent AVS from November 2017 to February 2022 at a single center. Subtype classification of PA was determined by AVS. We analyzed the concordance rate between AVS outcomes, adrenal CT, and PST, and explored the value of adrenal CT combined with PST for predicting laterality of PA. RESULTS: Total 531 PA patients were included in the present study. The concordance rate between AVS and the adrenal CT was 51.0%(271/531). Receiver operating characteristic (ROC) curve of PST showed that the area under curve (AUC) was 0.604 [95% confidence interval (CI): 0.556, 0.652], the optimal cut-off value was 30%. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), positive likelihood ratio (+LR), and negative likelihood ratio (-LR) of PST for diagnosis bilateral PA on AVS was 72.8, 46.2%, 0.48, 0.71, 1.35, and 0.59, respectively. The prevalence of unilateral PA on AVS in patients with unilateral lesion on CT and negative PST, unilateral lesion on CT and positive PST, bilateral normal or lesions on CT and negative PST, and bilateral normal or lesions on CT and positive PST was 82.4% (108/131), 59.9% (91/152), 50.7% (37/73), and 44.6% (78/175), respectively. The sensitivity, specificity, PPV, NPV, +LR, and -LR of adrenal CT combined with PST for the diagnosis of unilateral PA were 34.4, 89.4%, 0.82, 0.49, 3.25, and 0.73, respectively. CONCLUSIONS: The combination of CT findings and PST can improve the accuracy of predicting laterality of PA.

Label-Free Enhancement of Adrenal Gland Visualization Using Near-Infrared Autofluorescence for Surgical Guidance.

BACKGROUND: During adrenalectomy, surgeons have traditionally relied on their subjective visual skills to distinguish adrenal glands (AGs) from retroperitoneal fat and surrounding structures, while ultrasound and exogenous contrast agents have been employed for intraoperative AG visualization, all of which have their limitations. We present a novel label-free approach that uses near-infrared autofluorescence (NIRAF) detection, which demonstrates potential for enhanced intraoperative AG visualization and efficient tumor resection during adrenalectomies. METHODS: Patients undergoing adrenalectomy or nephrectomy were enrolled for this feasibility study. NIRAF emitted beyond 800 nm was detected in vivo from AGs and surrounding tissues during open adrenalectomies or nephrectomies. NIRAF was also measured ex vivo in excised AGs following robotic adrenalectomies. NIRAF images of tissues were captured using near-infrared (NIR) camera systems, whereas NIRAF intensities were recorded concurrently using fiber-optic probe-based NIR devices. Normalized NIRAF intensities (expressed as mean ± standard error) were analyzed and compared. RESULTS: Among the 55 enrolled patients, NIRAF intensity was elevated significantly for AGs versus retroperitoneal fat and other structures. NIR images of AGs also revealed a distinct demarcation of NIRAF between adrenal cortex and other periadrenal structures. NIRAF intensity in AGs was decreased markedly in malignant adrenal tumors, while benign adrenal cortical tumors and healthy adrenal cortex exhibited the strongest NIRAF levels. CONCLUSIONS: Our preliminary findings indicate that NIRAF detection could be a promising label-free technology to enhance intraoperative AG visualization and holds immense potential for effective tumor demarcation during cortical-sparing adrenalectomies or adrenal-conserving surgeries.