 Naif Bawazeer

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.

Physical Examination  Conscious, alert, with dysmorphic features including: protruded jaw, macroglossia, big hands and feet.  Otherwise unremarkable.

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.

MRI scan Measuring 2.8 x 3.6 x 1.7 cm

MRI scan Measuring 2.8 x 3.6 x 1.7 cm

CT scan

Diagnosis  Pituitary macro-adenoma with acromegaly

Management  Surgical removal of tumor by: Endoscopic Transsphenoidal Pituitary Surgery  Post- Operative:  no cerebrospinal fluid leak or seizure  Hormonal replacement therapy.  Follow up.

Anatomy 101

Phases of the Operation  Nasal stage (approaching tumor).  Sellar stage (resection of tumor).  Reconstruction phase (closure).

Video demonstration  See Attachment

Bone + Mucosal Graft

Navigation System

Definition  Excessive growth hormone (GH) after epiphyseal plate closure at puberty. Causing enlargement of hands, feet, and facial features

Epidemiology  Incidence about 3-4 per million per year.  Mean age at presentation 44 years.  Extrapolation : Incidence in Saudi Arabia Prevalence in Saudi Arabia

Epidemiology in Saudi Arabia:

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.

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.

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%)

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.

Treatment  After searching the literature through:

Treatment  American Association of Clinical Endocrinologists Medical Guidelines for Clinical Practice for the Diagnosis and Treatment of Acromegaly-2011 Update

Treatment  The Journal of Clinical Endocrinology & Metabolism May 1, 2009 vol. 94 no

Treatment Enclosed pituitary tumor:  Primary trans-sphenoidal surgery

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

Treatment In case of non-pituitary adenoma etiology:  Medical VS surgical for underlying pathology

Follow up:  Hormonal replacement therapy.  blood investigation for hormones levels.  MRI (evaluate recurrence).  Colonoscopy.

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: ]. Endocr Pract. 2004;10:  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),  2- Johnson N. New approaches to the development and use of treatment guidelines. Formulary. 1998;33:  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: ]. Endocr Pract. 2004;10:  4. Alexander L, Appleton D, Hall R, Ross WM, Wilkinson R. Epidemiology of acromegaly in the Newcastle region. Clin Endocrinol (Oxf). 1980;12:  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:  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: