1. Transcranial orbital exenteration: An extended approach for intraorbital malignancies Lip Teh Matthew Hansen Royal Perth Hospital

2. Introduction Intraorbital tumours Intraorbital tumours Pseudotumours Pseudotumours Hemangiomas – capillary/cavernous Hemangiomas – capillary/cavernous Meningiomas Meningiomas Optic nerve gliomas Optic nerve gliomas Lymphomas Lymphomas Sarcomas Sarcomas Epithelial malignancies Local Invasion Eyelids, paranasal sinuses Perineural spread Adenoid cystic carcinoma of lacrimal gland Cutaneous malignancies

3. Perineural spread SCC, melanoma, BCC, sclerosing sweat duct carcinoma SCC, melanoma, BCC, sclerosing sweat duct carcinoma Direct invasion, skip lesions, antegrade/ retrograde spread Direct invasion, skip lesions, antegrade/ retrograde spread Poor prognosis despite radical surgery Poor prognosis despite radical surgery Clinically Clinically Opthalmoplegia Opthalmoplegia Palpable nodule Palpable nodule Cranial nerve involvement Cranial nerve involvement Pain, hypo/dysaesthesia, formication - usually V 1 Pain, hypo/dysaesthesia, formication - usually V 1 Cavernous sinus syndrome Cavernous sinus syndrome

4. Indications for exenteration Postseptal spread of high grade malignancy

5. Transcranial orbital exenteration AIM Extended clearance of cranial nerve branches to cavernous sinus

6. Technique Coronal flap Coronal flap Temporalis reflected inferiorly Temporalis reflected inferiorly Disassembly of orbit, zygoma Disassembly of orbit, zygoma Frontotemporal craniotomy Frontotemporal craniotomy Neurosurgery involvement Neurosurgery involvement Reconstruction Reconstruction

7. Methods Retrospective review of our experience in transcranial orbital exenteration Retrospective review of our experience in transcranial orbital exenteration Performed since 1999 Performed since 1999 Two Institutions – RPH, Mount Hospital Two Institutions – RPH, Mount Hospital Single plastic surgeon Single plastic surgeon Single neurosurgeon involved for ligation of orbital adnexae structures at level of cavernous sinus Single neurosurgeon involved for ligation of orbital adnexae structures at level of cavernous sinus

8. Results 11 patients 11 patients All males All males 10 Caucasians (cutaneous), 1 Asian (sarcoma) 10 Caucasians (cutaneous), 1 Asian (sarcoma) Average age: 67yrs old (range: 52-82) Average age: 67yrs old (range: 52-82) Average followup period – 21months (range: 3-52months) Average followup period – 21months (range: 3-52months)

9. Results 4 local extension of periorbital tumors 4 local extension of periorbital tumors 1 melanoma 1 melanoma 2 SCC 2 SCC 1 malignant fibrous histiocytoma 1 malignant fibrous histiocytoma 7 perineural spread 7 perineural spread 6 SCC 6 SCC 1 sclerosing sweat duct carcinoma 1 sclerosing sweat duct carcinoma

10. Results Primary reconstructions Primary reconstructions 7 Temporalis flap 7 Temporalis flap 3 Free rectus 3 Free rectus 1 Free gracilis flap 1 Free gracilis flap 8 underwent post op radiotherapy 8 underwent post op radiotherapy 2 had necrotic temporalis flaps 2 had necrotic temporalis flaps 1 requiring free gracilis 1 requiring free gracilis 3 required re-excision of incomplete excision at skin margins 3 required re-excision of incomplete excision at skin margins

11. Results 3 had local recurrence(27%) 3 had local recurrence(27%) Case 8 – recurrence Lt zygoma at 13 months Case 8 – recurrence Lt zygoma at 13 months Case 9 – recurrence Rt zygoma, parotid at 10 months Case 9 – recurrence Rt zygoma, parotid at 10 months Case 10 – recurrence involving CN V, VII, VIII and pons at 15 months Case 10 – recurrence involving CN V, VII, VIII and pons at 15 months 3 deaths (27%) 3 deaths (27%) Case 5 – died at 5 months, radiation necrosis and abscess right frontal lobe Case 5 – died at 5 months, radiation necrosis and abscess right frontal lobe Case 8 – died at 40 months from recurrence Case 8 – died at 40 months from recurrence Case 9 – died at 20 months from recurrence Case 9 – died at 20 months from recurrence

12. Conclusion Often detected at advanced stages where surgical extirpation with preservation of orbit is not possible Often detected at advanced stages where surgical extirpation with preservation of orbit is not possible Poor prognosis Poor prognosis Despite multimodality treatment, 20-60% mortality in 3 years Despite multimodality treatment, 20-60% mortality in 3 years Transcranial approach allows greater clearance of the trigeminal nerve branches to the cavernous sinus Transcranial approach allows greater clearance of the trigeminal nerve branches to the cavernous sinus