1. H EMISPHERECTOMY in a case of Sturge Weber Syndrome

2. Introduction Sturge Weber Syndrome – Pathophysiology, diagnosis and management Hemispherectomy – Indications and outcomes Case – day 3 post hemispherectomy

3. Sturge Weber syndrome Frederick Parkes Weber (1863 – 1962)William Allen Sturge (1850 – 1919)

4. Sturge Weber syndrome Port wine stain Intracranial angiomas – Seizures – Focal deficits – Headaches – Developmental disorders Ocular manifestations

5. Pathophysiology Spontaneous mutation in GNAQ gene Failure of regression of part of neural tube vascular plexus, usually unilateral Vascular steal + seizures > hypoxic injury Progressive

6. Diagnosis Clinical – PWS, deficits, visual problems CSF analysis – protein (microhaemorrhage) EEG – evaluate and localise seizures CT, MRI

7. Treatment Glaucoma medications +/- surgery Seizure control with anti-convulsants Neurosurgery for refractive seizures Endocrinology, dermatology, psychology, psychiatry, MDT

8. Hemispherectomy First described 1923 for GBM Craniotomy and removal of all cerebral cortex Children with intractable epilepsy, hemiplegia and normal contralateral cortex Walter Edward Dandy (1886 - 1946)

9. Outcomes Contralateral hemiparesis, visual loss, speech disturbance Brain plasticity: maximum in younger patients 91% seizure free, rare seizures or > 75% improvement in frequency at 4 years (2003 UK review)

10. CASE diagnosed at birth Glaucoma surgery in infancy Modified hemispherectomy 2002 Doing well with moderate left hemiparesis and developmental delay In past 3 months: Intractable epilepsy. Simple partial seizures uncontrolled with levetiracetam, phenytoin, clobazam

11. Axial CT L frontal lobe L lateral ventricle L parietal lobe R hemisphere resection cavity Residual R hemisphere Calcification Septum Pellucidum

12. T2 MRI L frontal sinus L lateral ventricle L Sylvian fissure R hemisphere resection cavity Residual R frontal lobe Residual R occipital lobe Septum Pellucidum

13. Hemispherectomy Right craniotomy opened Insular cortex, residual frontal lobe resected. Occipital lobe disconnected Haemostasis and closure Post-operatively...

14. CT Surgical clips Resection cavity Craniotomy site Calcification in Disconnected occipital lobe Pneumocephalus Midline shift

15. Summary Up to 33% Port wine stains associated with Sturge Weber Syndrome Ocular and neurologic complications Consider surgery for intractable epilepsy Hemispherectomy: radical and extremely invasive surgery, can be effective in rare select cases