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Naif Bawazeer

Naif Bawazeer. Case presentation. 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.

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Naif Bawazeer

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  1. NaifBawazeer

  2. Case presentation

  3. 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.

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

  5. 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.

  6. MRI scan Measuring 2.8 x 3.6 x 1.7 cm

  7. MRI scan Measuring 2.8 x 3.6 x 1.7 cm

  8. CT scan

  9. Diagnosis • Pituitary macro-adenoma with acromegaly

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

  11. Intra-Operative

  12. Anatomy 101

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

  14. Video demonstration • See Attachment

  15. Bone + Mucosal Graft

  16. Navigation System

  17. Acromegaly

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

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

  20. Epidemiology in Saudi Arabia:

  21. 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.

  22. 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.

  23. 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%)

  24. 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.

  25. Treatment • After searching the literature through:

  26. 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

  27. Treatment • The Journal of Clinical Endocrinology & Metabolism May 1, 2009 vol. 94 no. 5 1509-1517

  28. Treatment Enclosed pituitary tumor: • Primary trans-sphenoidalsurgery

  29. Treatment In case of unresectablepituitary tumour (with neural or vascular impingement/invasion): • Medical therapy: • somatostatin analogs • dopamine agonists • growth hormone-receptor antagonist (GHRA) • Debulking surgery • Radiotherapy

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

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

  32. References • 1- Cook DM, Ezzat S, Katznelson L, et al (AACE Acromegaly Guidelines Task Force). AACE medical guidelines for clinical practice for the diagnosisAnd treatment of acromegaly [published correctionsAppear in EndocrPract. 2005;11:144 and EndocrPract. 2008;14:802-803]. EndocrPract. 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 ChirurgieCervico-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 EndocrPract. 2008;14:802-803]. EndocrPract. 2004;10:353-361. • 4. Alexander L, Appleton D, Hall R, Ross WM, Wilkinson R. Epidemiology of acromegaly in the Newcastle region. ClinEndocrinol (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 ClinEndocrinolMetab. 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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