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Endocrinology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Mehul Dattani, Catherine Peters
Adrenal and ectopic Cushing syndrome will not suppress with any dose of dexamethasone. Administration of CRH can be helpful in differentiating an adrenal and pituitary cause of the syndrome. Imaging of the pituitary and adrenals using MRI is usually indicated in order to locate the tumour, although the sensitivity is poor (70%). Inferior petrosal sinus sampling has a similar sensitivity and can be performed to locate a pituitary lesion and to lateralise a pituitary adenoma.
Head, neck and vertebral column
Published in David Heylings, Stephen Carmichael, Samuel Leinster, Janak Saada, Bari M. Logan, Ralph T. Hutchings, McMinn’s Concise Human Anatomy, 2017
David Heylings, Stephen Carmichael, Samuel Leinster, Janak Saada, Bari M. Logan, Ralph T. Hutchings
Internal jugular vein - main vein of the head and neck, continuous with the sigmoid sinus in the skull through the jugular foramen (Fig. 5.8). It runs down on the lateral side of the internal and common carotid arteries (Fig.3.38A) to join the subclavian vein deep to the sternoclavicular joint and form the brachiocephalic vein. It receives the inferior petrosal sinus and the pharyngeal, lingual, facial and superior and middle thyroid veins, in that order from above downwards. On the left, the thoracic duct (p. 134) joins the left side of the angle between the internal jugular and subclavian veins.
Endocrine problems in pregnancy
Published in Philip E. Harris, Pierre-Marc G. Bouloux, Endocrinology in Clinical Practice, 2014
Diagnosis can be difficult in pregnancy, because normal reference ranges vary considerably. There are wide trimester-specific changes in urine free cortisol in pregnancy. An overnight dexamethasone suppression test is not reliable in pregnancy, and false-positive values may occur in pregnancy. The low-dose dexamethasone suppression test is the definitive diagnostic test for the syndrome. In the differential diagnosis of Cushing’s syndrome, one should consider that adrenal adenoma is responsible for 40%–50% in pregnancy compared with 15% outwith pregnancy. ACTH suppression, usually diagnostic of an adrenal cause outwith pregnancy, may be unsuppressed in half of pregnant mothers with a primary adrenal cause. The high-dose dexamethasone suppression test has increased utility in pregnancy. Magnetic resonance imaging (MRI) of the adrenal or pituitary will be performed depending on results.66 Inferior petrosal sinus sampling is safe and useful in pregnancy when indicated.
Acute severe Cushing’s disease presenting as a hypercoagulable state
Published in Baylor University Medical Center Proceedings, 2021
Maria Mohammed Fariduddin, Wajihuddin Syed, Vidita Divan, Prashant Nadkarni, Ruban Dhaliwal
Approximately 40% of patients with CD have no visible tumor on standard MRI of the brain,12 as 90% to 95% of these tumors are microadenomas. Excessive ACTH production is followed by the loss of ACTH circadian rhythm, becoming independent of hypothalamic regulation and resistant to glucocorticoid feedback inhibition,13 causing adrenal hyperplasia and subsequent loss of circadian rhythm in cortisol secretion as well.14 The pituitary functions as if its threshold for glucocorticoid feedback inhibition is raised. Therefore, in most cases, there will be a positive response to high levels of glucocorticoids, defined by suppression of cortisol to <50% of the basal value. At other times, this response can be blunted15 and these intermediate values are not helpful in distinguishing a pituitary and ectopic source of ACTH. Adequate cortisol suppression was observed with high-dose DST in our patient. An unremarkable pituitary MRI added to the clinical dilemma. Corticotropin-releasing hormone stimulation and bilateral inferior petrosal sinus sampling confirmed the diagnosis of pituitary CD.
Treatment of low flow, indirect cavernous sinus dural arteriovenous fistulas with external manual carotid compression – the UK experience
Published in British Journal of Neurosurgery, 2020
Pratipal Kalsi, Rajeev Padmanabhan, Manjunath Prasad K. S., Nitin Mukerji
CS-DAVF usually occur in middle aged and elderly females but they can occur in any age group or sex. Clinical symptoms depend on whether the fistula drains anteriorly or posteriorly. Anteriorly draining CS-DAVF often present with visual symptoms, which include conjunctival injection, chemosis, extraocular nerve palsies leading to ohthalmoplegia, proptosis, retro-orbital pain and obtundation if an intracerebral hemorrhage occurs. Some patients may also have a bruit.4,5 Raised episcleral venous pressure may lead to an increase in intracocular pressure and visual loss. Visual loss is less common than in direct CS-DAVF but can occur in up to 30% of patients.6,7 CS-DAVF draining into the superior and inferior petrosal sinuses are usually asymptomatic. These patients don’t usually have ocular symptoms but can present with cranial nerve palsies.8–10 Infrequently posteriorly draining fistulas can cause brainstem congestion and neurological deficits.11 20-50% CS-DAVFs of will close spontaneously.12,13 Intracranial haemorrhage is an extremely rare complication.14
Severe ectopic Cushing’s syndrome
Published in Baylor University Medical Center Proceedings, 2020
Ankur Modi, Jamie Olejarski, Ashley Windham
Hypercortisolism must first be confirmed followed by a differentiation between an ACTH-dependent and -independent CS. In cases of CS with elevated ACTH levels, Cushing’s disease must be differentiated from EAS.2 Inferior petrosal sinus sampling is the most sensitive test for this purpose. It is the next step in evaluating ACTH-dependent CS when magnetic resonance imaging does not reveal a definite adenoma.3 A high-dose dexamethasone suppression test has limitations given that cortisol suppression is not present in 10% to 20% of patients with ACTH-secreting pituitary gland adenomas. Once EAS is diagnosed, visualization studies are required, usually with computed tomography of the neck, chest, abdomen, and pelvis; however, tumors are not identified in 19% of patients.4