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Inferior petrosal sinus sampling

Kush Dev Jarial, Rama Walia, Santosh Kumar, Anil Bhansali
Cushing's syndrome (CS) can be classified as adrenocorticotropic hormone (ACTH)-dependent or ACTH-independent depending on the ACTH levels. However, 30% of the patients with CS have ACTH levels in the 'grey zone' (5-20 pg/mL), thereby posing a challenge in establishing the aetiological diagnosis. In a patient with full-blown features of Cushing's syndrome with equivocal ACTH levels, and a pituitary microadenoma on contrast-enhanced MRI sella, can falsely lead to a diagnosis of Cushing's disease. Pituitary microadenoma, if <6 mm in size, may be an incidental finding (incidentaloma) in this scenario and can be present in ∼3-27% of the healthy population...
March 29, 2017: BMJ Case Reports
Shivam Om Mittal, Junaid Siddiqui, Bashar Katirji
Isolated unilateral abducens nerve palsy is usually due to ischemia, trauma or neoplasm. Dorello's canal is the space between the petrous apex and superolateral portion of the clivus, bound superiorly by Gruber's ligament. The abducens nerve travels with inferior petrosal sinus (IPS) though the Dorello's canal before entering the cavernous sinus. A 31-year-old man presented with neck pain, and binocular horizontal diplopia, worse looking towards left and at distance. He had a history of intravenous drug abuse but no history of hypertension or diabetes...
February 24, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Esra Tutal, Demet Yılmazer, Taner Demirci, Evrim Cakır, Salih Sinan Gültekin, Bahadır Celep, Oya Topaloğlu, Erman Çakal
Ectopic adrenocorticotropic hormone (ACTH) syndrome is characterized by hypercortisolism due to the hypersecretion of a non-pituitary ACTH-secreting tumor leading to Cushing's syndrome. Only a few cases have been reported previously as causing ectopic ACTH related to paraganglioma. Herein, we present a case of Cushing's syndrome, in who was proved to be attributable to an ACTH-secreting renal malignant paraganglioma. A 40-year-old woman presented with a five-month history of newly diagnosed hypertension and diabetes, weakness, hyperpigmentation, oligomenorrhea, hirsutism, and acneiform lesions...
February 13, 2017: Archives of Endocrinology and Metabolism
Joseph M Pappachan, Christian Hariman, Mahamood Edavalath, Julian Waldron, Fahmy W Hanna
Diagnosis of Cushing's syndrome (CS) and identification of the aetiology of hypercortisolism can be challenging. The Endocrine Society clinical practice guidelines recommends one of the four tests for initial screening of CS, namely, urinary-free cortisol, late night salivary cortisol, overnight dexamethasone suppression test or a longer low-dose dexamethasone suppression test, for 48 hours. Confirmation and localisation of CS requires additional biochemical and radiological tests. Radiological evaluation involves different imaging modalities including MRI with or without different radio-nuclear imaging techniques...
April 2017: Journal of Clinical Pathology
Maria Koutourousiou, Welby I Winstead
BACKGROUND: Complete surgical resection of an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma is the gold standard of treatment of Cushing disease. Ectopic location of these adenomas is an extremely rare condition that may compromise the diagnosis and surgical success. We present the first case of an ectopic intracavernous ACTH-secreting macroadenoma totally resected with endoscopic endonasal surgery (EES). CASE DESCRIPTION: A 36-year-old woman presented with Cushing syndrome...
February 2017: World Neurosurgery
Alexander Faje, Nicholas A Tritos, Brooke Swearingen, Anne Klibanski
Significant advances in pituitary imaging have taken place in the past several decades, including the introduction of magnetic resonance imaging (MRI). This imaging modality has vastly improved our ability to detect and characterize sellar masses and more accurately characterize the extent and spread of lesions in and around the sella. Intraoperative MRI may help improve the completeness of resection of sellar masses. Other imaging modalities, including magnetic resonance angiography, computed tomography (CT), and CT angiography, have an important role in specific cases...
2016: Handbook of Clinical Neurology
Jon E F Diernaes, Anette Bygum, Per L Poulsen
We present a patient with Cushing disease apparently suppressing sarcoidosis, which was unmasked following surgical resection of a pituitary adrenocorticotropin (ACTH)-producing microadenoma. Case report and a short review of the literature published in this area. A 46-year-old Caucasian woman presented with symptoms of hypercortisolism such as progressive weight gain, Cushingoid appearance, proximal myopathy, easy bruising, and amenorrhea. Blood testing including inferior petrosal sinus sampling uncovered an ACTH-producing microadenoma in the right aspect of the anterior pituitary gland for which the patient underwent transphenoidal resection...
January 2016: Dermato-endocrinology
Benedetta Zampetti, Erika Grossrubatscher, Paolo Dalino Ciaramella, Edoardo Boccardi, Paola Loli
Simultaneous bilateral inferior petrosal sinus sampling (BIPSS) plays a crucial role in the diagnostic work-up of Cushing's syndrome. It is the most accurate procedure in the differential diagnosis of hypercortisolism of pituitary or ectopic origin, as compared with clinical, biochemical and imaging analyses, with a sensitivity and specificity of 88-100% and 67-100%, respectively. In the setting of hypercortisolemia, ACTH levels obtained from venous drainage of the pituitary are expected to be higher than the levels of peripheral blood, thus suggesting pituitary ACTH excess as the cause of hypercortisolism...
July 2016: Endocrine Connections
Martin G Radvany, Alfredo Quinones-Hinojosa, Gary L Gallia, Gary S Wand, Roberto Salvatori
OBJECTIVE: Because magnetic resonance imaging (MRI) fails to detect many adrenocorticotropic hormone (ACTH)-secreting pituitary adenomas, inferior petrosal sinus sampling (IPSS) is considered the gold standard to differentiate Cushing disease (CD) from ectopic ACTH secretion syndrome (EAS). Some authors have suggested internal jugular vein sampling (IJVS) as an alternative to IPSS. METHODS: We simultaneously compared IJVS to IPSS in 30 consecutive patients referred for ACTH-dependent Cushing syndrome and equivocal MRI exams...
September 2016: Endocrine Practice
Narendra Kotwal, Yogesh Kumar, Vimal Upreti, Amandeep Singh, M K Garg
CONTEXT: Anatomical localization of pituitary adenoma can be challenging in adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome, and bilateral inferior petrosal sinus sampling (BIPSS) is considered gold standard in this regard. Stimulation using corticotrophin-releasing hormone (CRH) improves the sensitivity of BIPSS, however, same is not easily available in India. Therefore, we undertook this study of BIPPS using vasopressin as agent for stimulation owing to its ability to stimulate V3 receptors present on corticotrophs...
May 2016: Indian Journal of Endocrinology and Metabolism
Maria Güemes, Philip G Murray, Caroline E Brain, Helen A Spoudeas, Catherine J Peters, Peter C Hindmarsh, Mehul T Dattani
UNLABELLED: The diagnosis and management of paediatric Cushing syndrome (CS) is highly challenging. This study aims to characterise its presentation, diagnosis, management and outcome by a retrospective case review of 30 patients (14 females) followed at a single tertiary paediatric endocrinology centre over a 30-year period. At presentation, median age was 8.9 years (0.2-15.5) and the commonest manifestations were weight gain (23/30), hirsutism (17/30), acne (15/30) and hypertension (15/30)...
July 2016: European Journal of Pediatrics
Joo Hee Lim, Soo Jung Kim, Mo Kyung Jung, Ki Eun Kim, Ah Reum Kwon, Hyun Wook Chae, Duk Hee Kim, Ho-Seong Kim
A 14-year-old girl was referred for evaluation of the etiology of Cushing syndrome. During the previous 2 years, she had experienced weight gain, secondary amenorrhea, growth retardation, and back pain. Random serum cortisol level, 24-hour urinary free cortisol excretion, and overnight and low-dose dexamethasone suppression tests suggested Cushing syndrome. Midnight adrenocorticotropic hormone (ACTH) level and high-dose dexamethasone suppression test confirmed Cushing disease. Pituitary magnetic resonance imaging was suspicious for microadenoma...
March 2016: Annals of Pediatric Endocrinology & Metabolism
Ming Feng, Lin Lu, Xiaohai Liu, Congxin Dai, Xinjie Bao, Kan Deng, Bing Xing, Feng Feng, Huijuan Zhu, Yong Yao, Renzhi Wang
OBJECTIVE: To analyze inferior petrosal sinus sampling (IPSS) in diagnosis and treatment of Cushing's disease with negative magnetic resonance imaging (MRI). METHODS: We retrospectively reviewed 79 cases of Cushing's disease with negative MRI in the Department of Neurosurgery of Peking Union Medical College Hospital from August 2012 to August 2014. Of the 79 patients, 58 underwent IPSS. There were 16 males and 42 females in this study, with age 12 to 65 years old...
March 22, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Amy Deipolyi, Alexander Bailin, Joshua A Hirsch, T Gregory Walker, Rahmi Oklu
OBJECTIVE: To describe findings and outcomes of 331 bilateral inferior petrosal sinus sampling (BIPSS) procedures performed in 327 patients evaluated for Cushing disease (CD). MATERIALS AND METHODS: The radiology department's electronic database was searched to identify all BIPSS procedures (1990-2013). Electronic medical records were used to identify demographics, laboratory, procedural, surgical and pathologic findings. RESULTS: A total of 331 BIPSS procedures were performed in 327 patients (254 F, 73 M), mean age 41 (range 7-81) years...
February 2017: Journal of Neurointerventional Surgery
Kharis Burns, Darshika Christie-David, Jenny E Gunton
UNLABELLED: Ketoconazole was a first-line agent for suppressing steroidogenesis in Cushing's disease. It now has limited availability. Fluconazole, another azole antifungal, is an alternative, although its in vivo efficacy is unclear. A 61-year-old female presented with weight gain, abdominal striae and worsening depression. HbA1c increased to 76 mmol/mol despite increasing insulin. Investigations confirmed cortisol excess; afternoon serum cortisol was 552 nmol/l with an inappropriate ACTH of 9...
2016: Endocrinology, Diabetes & Metabolism Case Reports
Harish Venugopal, Katherine Griffin, Saima Amer
UNLABELLED: Resection of primary tumour is the management of choice in patients with ectopic ACTH syndrome. However, tumours may remain unidentified or occult in spite of extensive efforts at trying to locate them. This can, therefore, pose a major management issue as uncontrolled hypercortisolaemia can lead to life-threatening infections. We present the case of a 66-year-old gentleman with ectopic ACTH syndrome from an occult primary tumour with multiple significant complications from hypercortisolaemia...
2015: Endocrinology, Diabetes & Metabolism Case Reports
Sonam Tashi, Keng Sin Ng
Cushing's syndrome is known to have a wide spectrum of clinical presentation with debilitating consequences and morbidity if not diagnosed and treated in time. Sometimes the diagnosis of Cushing's syndrome can be challenging to the endocrinologist, especially when the usual battery of biochemical tests and advanced cross-sectional imaging is negative or inconclusive. We described a case in which the use of bilateral inferior petrosal sinus sampling (BIPSS) was conclusive albeit being technically challenging (due to a rare incidental finding of double superior vena cava) and invasive in nature...
August 2015: Journal of Radiology Case Reports
Sauradeep Sarkar, Simon Rajaratnam, Geeta Chacko, Sunithi Mani, Asha S Hesargatta, Ari George Chacko
BACKGROUND: This study was performed to examine patient outcomes following pure endoscopic transsphenoidal surgery (ETS) for Cushing's disease (CD). METHOD: We studied 64 consecutive patients who underwent 69 endoscopic transsphenoidal procedures. Radiological evaluation comprised detailed examination of preoperative magnetic resonance images (MRI), including positron emission tomography (PET) for select cases. Inferior petrosal sinus sampling (IPSS) was not performed for any patient...
January 2016: Acta Neurochirurgica
William F Chandler, Ariel L Barkan, Todd Hollon, Alla Sakharova, Jayson Sack, Barunashish Brahma, David E Schteingart
BACKGROUND: Transsphenoidal surgery is the standard approach for treating Cushing disease. Evidence is needed to document effectiveness. OBJECTIVE: To analyze results of transsphenoidal surgery in 276 consecutive patients, including 19 children. METHODS: Medical records were reviewed for patients treated initially with surgery for Cushing disease from 1980 to 2012. Radiographic features, pathology, remissions, recurrences, and complications were recorded...
February 2016: Neurosurgery
Yuichi Takashi, Yuka Kinoshita, Noriko Makita, Manabu Taguchi, Katsutoshi Takahashi, Masaomi Nangaku, Seiji Fukumoto
We describe a 30-year-old man with ectopic adrenocorticotropic hormone (ACTH) syndrome. Before the operation, there was no diurnal variation of ACTH, and ACTH did not respond to CRH or dexamethasone suppression tests. These abnormalities disappeared after the removal of a neuroendocrine tumor in the lung. In addition, plasma ACTH was measureable at as early as postoperative day 3 with ACTH levels increasing thereafter. Furthermore, an insulin tolerance test and inferior petrosal sinus sampling indicated that ACTH was secreted from the pituitary...
2015: Internal Medicine
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