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H EMISPHERECTOMY in a case of Sturge Weber Syndrome.

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Presentation on theme: "H EMISPHERECTOMY in a case of Sturge Weber Syndrome."— Presentation transcript:

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 ( )

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

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