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Naif Bawazeer
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History This 40 yr old female, past medically free, para 2+0. patient cant speak or hear. She complain of headache especially in the morning mainly frontal associated with blurred vision, amenorrhea and enlargement in hand and foot since 7 years. Past 6 month symptoms progressed out of proportion and associated with lower limb weakness. Otherwise unremarkable.
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Physical Examination Conscious, alert, with dysmorphic features including: protruded jaw, macroglossia, big hands and feet. Otherwise unremarkable.
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Investigation CBC and chemistry: with normal range Glucose: 126 mg/dl Alk pho:154 U/L (high) Urea and creatinie: with normal range. Growth hormone: 40mcg/L (very high) Prolactin : 23.6 ng/mL (high) FSH: 6.3 mIU/ml ECG: Sinus rhythm.
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MRI scan Measuring 2.8 x 3.6 x 1.7 cm
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MRI scan Measuring 2.8 x 3.6 x 1.7 cm
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CT scan
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Diagnosis Pituitary macro-adenoma with acromegaly
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Management Surgical removal of tumor by: Endoscopic Transsphenoidal Pituitary Surgery Post- Operative: no cerebrospinal fluid leak or seizure Hormonal replacement therapy. Follow up.
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Anatomy 101
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Phases of the Operation Nasal stage (approaching tumor). Sellar stage (resection of tumor). Reconstruction phase (closure).
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Video demonstration See Attachment
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Bone + Mucosal Graft
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Navigation System
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Definition Excessive growth hormone (GH) after epiphyseal plate closure at puberty. Causing enlargement of hands, feet, and facial features
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Epidemiology Incidence about 3-4 per million per year. Mean age at presentation 44 years. Extrapolation : Incidence in Saudi Arabia Prevalence in Saudi Arabia
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Epidemiology in Saudi Arabia:
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Causes Endogenous sources: 1- pituitary adenoma (90%). 2- familial syndromes: multiple endocrine neoplasia (MEN) type I, McCune-Albright syndrome, Carney's syndrome. 3- pituitary carcinoma. 4- peripheral neuroendocrine tumors. Exogenous sources.
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History and Physical In case of pituitary adenoma: Mass effect: headaches, cranial nerve palsy, visual field defects + Hormonal effect: GH: increase in soft tissue, CTS (60%) Prolactin: Amenorrhoea. TSH: Hyperthyroidism. ACTH: Cushing's syndrome Apoplexy: sudden deterioration.
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History and Physical Other causes of acromegaly: according on underlying cause will give us the clinical picture. + Hormonal effect: GH: increase in soft tissue, CTS (60%)
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Diagnosis Clinical suspicion Biochemical marker: elevated insulin-like growth factor 1 (IGF-1) elevated growth hormone (GH < 1 ng/mL ) Radiology: MRI of pituitary gland to detect tumor and evaluation.
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Treatment After searching the literature through:
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Treatment American Association of Clinical Endocrinologists Medical Guidelines for Clinical Practice for the Diagnosis and Treatment of Acromegaly-2011 Update http://aace.metapress.com/content/5h1427154k550851/?id=5H1427154K550851
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Treatment The Journal of Clinical Endocrinology & Metabolism May 1, 2009 vol. 94 no. 5 1509-1517
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Treatment Enclosed pituitary tumor: Primary trans-sphenoidal surgery
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Treatment In case of unresectable pituitary tumour (with neural or vascular impingement/invasion): Medical therapy: somatostatin analogs dopamine agonists growth hormone-receptor antagonist (GHRA) Debulking surgery Radiotherapy
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Treatment In case of non-pituitary adenoma etiology: Medical VS surgical for underlying pathology
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Follow up: Hormonal replacement therapy. blood investigation for hormones levels. MRI (evaluate recurrence). Colonoscopy.
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References 1- Cook DM, Ezzat S, Katznelson L, et al (AACE Acromegaly Guidelines Task Force). AACE medical guidelines for clinical practice for the diagnosis And treatment of acromegaly [published corrections Appear in Endocr Pract. 2005;11:144 and Endocr Pract. 2008;14:802-803]. Endocr Pract. 2004;10:213-225. Javer, A., Marglani, O., Lee, A., Matishak, M., & Genoway, K. (2008). Image-guided endoscopic transsphenoidal removal of pituitary tumours. Journal Of Otolaryngology - Head & Neck Surgery = Le Journal D'oto-Rhino-Laryngologie Et De Chirurgie Cervico- Faciale, 37(4), 474-480. 2- Johnson N. New approaches to the development and use of treatment guidelines. Formulary. 1998;33:665-678. 3. Mechanick JI, Bergman DA, Braithwaite SS, Palumbo PJ (American Association of Clinical Endocrinologists Ad Hoc Task Force for Standardized Production of Clinical Practice Guidelines). American Association of Clinical Endocrinologists protocol for standardized production of clinical practice guidelines [published correction appears in Endocr Pract. 2008;14:802-803]. Endocr Pract. 2004;10:353-361. 4. Alexander L, Appleton D, Hall R, Ross WM, Wilkinson R. Epidemiology of acromegaly in the Newcastle region. Clin Endocrinol (Oxf). 1980;12:71-79. 5. Daly AF, Rixhon M, Adam C, Dempegioti A, Tichomirowa MA, Beckers A. High prevalence of pituitary adenomas: a cross-sectional study in the province of Liege, Belgium. J Clin Endocrinol Metab. 2006;91:47694775. 6. Avagnina P, Martini M, Terzolo M, et al. Assessment of functional liver mass and plasma flow in acromegaly before and after long-term treatment with octreotide. Metabolism. 1996;45:109-113.
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