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.

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.

Intra-Operative

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

Acromegaly

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

http://aace.metapress.com/content/5h1427154k550851/?id=5H1427154K550851

Treatment

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

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;14:802-803]. 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.