Petrosal Sinus Sampling in diagnostic evaluation of ACTHdependent Cushing Syndrome: A Pictorial Review.

Transcription

1 Petrosal Sinus Sampling in diagnostic evaluation of ACTHdependent Cushing Syndrome: A Pictorial Review. Poster No.: C-2143 Congress: ECR 2014 Type: Educational Exhibit Authors: D. Rodriguez, L. Aja Rodriguez, J. J. Sanchez Fernandez, S Aixut Lorenzo, E. Montaño Claure, L. VALOYES GUERRERO ; El Vendrell/ES, Barcelona/ES, VALLIRANA (Barcelona)/ES, 4 L'Hospitalet de Llobregat/ES Keywords: Technical aspects, Sampling, Diagnostic procedure, MR, Catheter venography, Interventional vascular, CNS, Anatomy, Endocrine disorders DOI: /ecr2014/C-2143 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 16

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3 Learning objectives To describe the relevant anatomy and technical details of inferior petrosal sinus sampling (IPSS) in diagnosis of adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome (CS). Background CS is an uncommon endocrine disorder (1 per ), due to hypercortisolism that involves the hypothalamus-pituitary-adrenal axis (Fig. 1). Symptoms are nonspecific and include central obesity, moon facies, thin skin, purple striae, hirsutism, and hypertension, among others [1]. In most of cases, there is an ACTH-secreting pituitary adenoma causing this disorder. However, the features of pituitary ACTH-dependent CS are often undistinguishable from those of occult ectopic ACTH-dependent CS. Ordinary diagnostic tests like urinary free cortisol, late-night salivary cortisol and low-dose dexamethasone suppression are for the diagnosis of hypercortisolemia [2]. High levels of ACTH indicate ACTH-dependent CS of pituitary (secreting adenoma) or ectopic (e.g. carcinoid tumours, neuroendocrine tumours, gastrinomas, thyroid carcinomas, pheochromocytomas, etc.) origin [3]. The next step is to distinguish pituitary from ectopic ACTH secretion; that is made by other noninvasive tests, like high-dose suppression dexamethasone suppression, the corticotropin-releasing hormone (CRH) stimulation test and pituitary MRI [4]. This tests have a relative low sensitivity and therefore, their use is limited [2, 5-7]. Bilateral inferior petrosal sinus sampling (BIPSS) is highly sensitive and specific for accurately diagnosing pituitary CS and may be helpful in lateralizing the location of the adenoma. ACTH levels are sampled at petrosal sinuses and then compared with ACTH levels in peripheral blood, to determine whether a pituitary tumor is the source of ACTH-dependent CS. Therefore, bilateral IPSS is the gold standard for identifying the pituitary gland as the source of ACTH secretion in CS [1]. Anatomic considerations Knowledge of the pituitary venous drainage is essential for IPSS technique [1]. The cavernous sinuses are just lateral to the pituitary fossa and contain the carotid artery and cranial nerves [8] (Fig 2). They are interconnected by four intercavernous pathways (anterior, posterior, inferior and basilar plexus) [9]. Despite this huge communication, venous drainage from the pituitary in unilateral in normal, physiologic conditions; this makes necessary to perform a bilateral sampling, in order to avoid false-positive results and to lateralize the source of ACTH production [1]. Page 3 of 16

4 The IPS drains the cavernous sinus posteriorly and drains into internal jugular vein (IJV), receiving tributaries from pons, medulla, internal auditory meatus and anterior condylar vein (ACV) [1, 10]. There is some variability in the position of the junction of the IPS into IJV and in its anatomic form [10]. Shiu et al [11], describe four types of anatomic variants (Fig 3): Type I: IPS drains directly into internal jugular bulb without communication with ACV. Is the most common pattern. Type II: IPS anastomoses with ACV before draining into IJV. Type III: IPS drains into IJV as a venous plexus. Type IV: IPS drains into vertebral venous plexus, without connection between IPS and IJV. Later, Bonelli et al., and other authors [12], redefine this classification, combining types I and II in a new type A; type B is the type III and type C correspond to previous type IV [1] (Fig 4). Images for this section: Page 4 of 16

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6 Fig. 1: Hypothalamic pituitary-adrenal axis. Fig. 2: Anatomy of the venous sinuses of the skull base. CS: Cavernous sinus, IPS: Inferior petrosal sinus, SPS: Superior petrosal sinus, SS: Sigmoid sinus, JB: Jugular bulb, PV: Pharyngeal vein, IJV: Internal Jugular Vein, FV: Facial vein. Page 6 of 16

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8 Fig. 3: Classification of IPS anatomic variants. Page 8 of 16

9 Findings and procedure details Our standard protocol includes a bilateral femoral venous access (a 5F sheath in each femoral vein) for IPS catheterization. The procedure is performed at the angiosuite, with an anaesthesiologist providing sedation and pain control to the patient. Later, we advanced 5F diagnostic catheters (vertebral or similar) until they are correctly located. Optimal position is obtained when hand injection demonstrates ipsilateral IPS filling with contralateral reflux [8]. Assessing the anatomy is essential because of anatomic variants that can affect the interpretation of the results, as described above. Then, we started to sample ACTH from peripheral blood and from both IPS at baseline and after CRH stimulation (3, 5, 15 and 30 minutes). After the samples are obtained, both femoral sheaths are removed and manual compression is used to guarantee an optimal hemostasis. The patient is observed under strict bed rest for 24 hours before discharge the next day. To evaluate the results, we use the IPS to peripheral ACTH level ratio (IPS/P). Baseline IPS/P # 2 or CRH stimulated IPS/P # 3 confirms CS [13]. However, it has both falsenegative and false-positive results [3, 5, 7, 12, 14]. Also, the issue of lateralize the source of ACTH is still controversial. An intersinus ratio of 1.4 has been considered as evidence of ipsilateral localization of adenoma [14]; however, functional MRI and detailed surgical exploration may be useful for tumor localization in our institution. Bilateral IPSS it's a safe and accurate procedure, if it's done by experienced interventional neurorradiologists. However, there may be complications. The most frequent complication is groin hematoma; neurologic complications are extremely rare (venous thrombosis, thromboembolic events or pontine haemorrhage...).[1, 15, 16, 17]. Images for this section: Page 9 of 16

10 Fig. 4: 61 year-old woman with clinical hypercortisolism. Coronal T1-weighted (left) and T1-weighted gadolinium-enhanced (right) MRI shows a pituitary gland (red arrows) with normal morphology and intensity signal. Fig. 5: Sagital T1-weighted (left) and T1-weighted gadolinium-enhanced (right) pituitary MRI of the previous patient. Page 10 of 16

11 Fig. 6: Bilateral inferior petrosal sinus sampling technique. AP view. Note the catheters placed into the IPS bilaterally. This patient presented an intersinus ratio of 2 at the right IPS. Page 11 of 16

12 Fig. 7: Bilateral inferior petrosal sinus sampling technique. Lateral view. Page 12 of 16

13 Fig. 8: Bilateral inferior petrosal sinus sampling technique. Oblique view. The right IPS shows a discrete plexiform morphology. Page 13 of 16

14 Fig. 9: Bilateral inferior petrosal sinus sampling technique. Oblique view. Page 14 of 16

15 Conclusion Bilateral IPSS with CRH stimulation is the gold standard for diagnosing ACTH-dependent CS. An adequate knowledge of venous anatomy and a proper technique are essential to provide a safe procedure with interpretable results. Personal information Daniel Rodriguez Bejarano MD, Radiology Resident, Hospital Universitari de Bellvitge, L Hospitalet de Llobregat (Barcelona); daniel.rodriguez@bellvitgehospital.cat Lucia Aja Rodriguez MD, Department of Neuroradiology, Hospital Universitari de Bellvitge, L Hospitalet de Llobregat (Barcelona); lucia.aja@bellvitgehospital.cat Juan Jose Sanchez Fernandez MD, Radiologist. Institut de Diagnostic per la imatge (IDI), Hospital Universitari de Bellvitge, L Hospitalet de Llobregat (Barcelona); jsanchezfern@bellvitgehospital.cat Sonia Aixut Lorenzo MD, Department of Neuroradiology, Hospital Universitari de Bellvitge, L Hospitalet de Llobregat (Barcelona); saixut@bellvitgehospital.cat Evelyn Montaño Claure MD, Radiology Resident, Hospital Universitari de Bellvitge, L Hospitalet de Llobregat (Barcelona); evelynmontano@bellvitgehospital.cat Lisbeth Valoyes Guerrero MD, Radiology Resident, Hospital Universitari de Bellvitge, L Hospitalet de Llobregat (Barcelona); lisbeth.valoyes@bellvitgehospital.cat References 1. Deipolyi A, Karaosmanoglu A, Habito C, Brannan S, Wicky S, Hirsch J, Oklu R. The role of bilateral inferior petrosal sinus sampling in the diagnostic evaluation of Cushing syndrome. Diagn Interv Radiol 2012; 18: Page 15 of 16

16 Arnaldi G, Angeli A, Atkinson AB, et al. Diagnosis and complications of Cushing's Syndrome: a consensus statement. J Clin Endocrinol Metab 2003; 88: Tomycz ND, Horowitz MB. Inferior petrosal sinus sampling in the diagnosis of sellar neuropathology. Neurosurg Clin N Am 2009; 20: Gross BA, Mindea SA, Pick AJ, Chandler JP, Batjer HH. Diagnostic approach to Cushing disease. Neurosurg Focus 2007; 23:E1. Aron DC, Raff H, Findling JW. Effectiveness versus efficacy: the limited value in clinical practice of high dose dexamethasone suppression testing in the differential diagnosis of adrenocorticotropin-dependent Cushing's syndrome. J Clin Endocrinol Metab 1997; 82: Ezzat S, Asa SL, Couldwell WT, et al. The prevalence of pituitary adenomas: a systematic review. Cancer 2004; 101: Kaskarelis IS, Tsatalou EG, Benakis SV, Malagari K, Komninos I, Vasiliadou D, Tsagakaris S, Thalassinos N. Bilateral Inferior Petrosal Sinuses Sampling in the Routine Investigation of Cushing's Syndrome: A Comparison with MRI. AJR 2006; 187: Doppman JL, Oldfield E, Krudy AG, et al. Petrosal sinus sampling for Cushing syndrome: anatomical and technical considerations. Work in progress. Radiology 1984; 150: Aquini MG, Marrone AC, Schneider FL. Intercavernous venous communications in the human skull base. Skull Base Surg 1994; 4: Gebarski SS, Gebraski KS, Inferior Petrosal Sinus: Imaging-anatomic Correlation. Radiology 1996; 194: Shiu PC, Hanafee WN, Wilson GH, Rand RW. Cavernous sinus venography. Am J Roentgenol Radium Ther Nucl Med 1968; 104: Bonelli FS, Huston J 3rd, Carpenter PC, Erickson D, Young WF Jr, Meyer FB. Adrenocorticotropic hormone-dependent Cushing's syndrome: sensitivity and specificity of inferior petrosal sinus sampling. AJNR 2000; 21: Javorsky BR, Findling JW. Inferior petrosal sampling for the differential diagnosis of ACTH-dependent Cushing's syndrome. In: Bronstein MD, ed. Cushing's syndrome: pathophysiology, diagnosis and treatment. New York: Humana Press, Jehle S, Walsh JE, Freda PU, Post KD. Selective Use of Bilateral Inferior Petrosal Sinus Sampling in Patients with Adrenocorticotropin-Dependent Cushing's Syndrome Prior to Transsphenoidal Surgery. J Clin Endocrinol Metab. December 2008, 93(12): Miller DL, Doppman JL. Petrosal Sinus Sampling: Technique and Rationale. Radiology 1991; 178: Gandhi CD, Meyer SA, Patel AB, Johnson DM, Post KD. Neurologic Complications of Inferior Petrosal Sinus Sampling. AJNR 2008; 29: Blevins LS Jr, Clark RV, Owens DS. Thromboembolic complications after inferior petrosal sinus sampling in patients with Cushing's syndrome. Endocr Pract 1998; 4: Page 16 of 16