This document discusses acromegaly, a rare disorder caused by excess growth hormone secretion from the pituitary gland after epiphyseal closure. It presents in adults with coarse facial features, joint pain, and organ enlargement. Diagnosis involves blood tests and imaging of the pituitary. Treatment options include surgery, radiation, or medication to reduce growth hormone levels. The trans-sphenoidal surgical approach is described which involves accessing the pituitary through the nasal cavity and sphenoid sinus. Risks of surgery include bleeding, infection, and damage to nearby structures.

1. DR.MUMTAZ ALI
JPMC KARACHI

2. Anatomy

3. Clinical Features
 five cases per one million individuals
 fourth and fifth decades of life
 before epiphyseal closure : gigantism
 after puberty : acromegaly
 connective tissue and bone overgrowth
 impaired glucose tolerance
 Musculoskeletal
 Cardiovascular
 respiratory derangements
 premature mortality

4. Acromegaly
 Coarsening of facial features
 frontal skull bossing
 Prognathism
 Malocclusion
 Macroglossia
 skin thickening
 increased shoe and ring size
 Visceromegaly
 painful osteoarthritis
 spinal kyphoscoliosis
 diffuse skeletal hyperostosis
 barrel chest deformity
 low and deeply resonant pitch
 snoring and sleep apnea

6. multitiered classification : WHO
 Clinical presentation and secretory activity : GH
Densely granulated :7.1 , 1 : 0.7
sparsely granulated :6.2 , 1 : 1.6

7.  Histopathologic features, including
immunohistochemical and ultrastructural profile :
 cellular origin
 hormonal content
 ultrastructure
 mitotic figures
 pleomorphism
 (CSF) spread

8.  Molecular biologic and genetic features :
 X-chromosome inactivation
 proliferation of a single
 Mutation
 AIP, MEN1
 CDKN1B, PRKAR1A
 (MEN1)
 familial isolated pituitary adenomas
 multiple endocrine neoplasia type 4
 Carney’s complex

9.  Neuroimaging:
 Size
 Hardy
 Wilson

12. Presentation
 Harmonal excess state
 Pituitary insufficiency
 Pituitary apoplexy
 Mass effect:
 Headache
 visual loss
 bitemporal hemianopia
 hydrocephalus
 cavernous sinus
 mesial temporal lobe
 hypothalamus

13. D/D

14. Workup
 Routine
 Specific
Radiology
Endocrine
 Relevant
CT scan
Visual perimetry
Fundoscopy
EEG

15. Treatment Options
 Medical
 Somatostatin analogue
 Dopamine agonist
 GHRA :Pegvisomant
 Surgery
 Radiotherapy :
 refractory to medical
 refractory to surgery
 Neurovascular invasion
 frequent recurrence

16. Pre-op evaluation
 medical condition
 Co-morbids
 Prognathism ,soft tissue hypertrophy
 macroglossia
 obstructive sleep apnea
 Visual field & acuity
 MRI ,CT scans & Angiograms :
 nasal septal deviations
 sphenoid septations
 osseous defects in the carotid canal
 degree of sphenoid pneumatization
 extent of bony expansion
 erosion from an aggressive lesion
 Encrochment of cavernous sinus & circle

17. Trans-sphenoidal Approach
 O.T setup
 Patient preparation :
Nasal decongestion ; HTN ,IHD
oxymetazoline (Afrin) 0.05%
1% lidocaine
 1 : 200,000
chlorhexidine

18.  Nasal Phase
 Sphenoid Phase
 Sellar Phase
 Closure

19. Complications

20. THANKYOU