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Hemispherectomy in a case of Sturge Weber Syndrome

Hemispherectomy in a case of Sturge Weber Syndrome. Introduction. Sturge Weber Syndrome Pathophysiology , diagnosis and management Hemispherectomy Indications and outcomes Case day 3 post hemispherectomy. Sturge Weber syndrome. William Allen Sturge (1850 – 1919).

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Hemispherectomy in a case of Sturge Weber Syndrome

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  1. Hemispherectomyin 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 William Allen Sturge (1850 – 1919) Frederick Parkes Weber (1863 – 1962)

  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 Residual R hemisphere L lateral ventricle Septum Pellucidum R hemisphere resection cavity L parietal lobe Calcification

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

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

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

  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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