Post on 24-Feb-2016
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HEMISPHERECTOMYin 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
Frederick Parkes Weber (1863 – 1962)William Allen Sturge (1850 – 1919)
Sturge Weber syndrome• Port wine stain
• Intracranial angiomas– Seizures– Focal deficits– Headaches– Developmental disorders
• Ocular manifestations
Pathophysiology• Spontaneous mutation in GNAQ gene
• Failure of regression of part of neural tube vascular plexus, usually unilateral
• Vascular steal + seizures > hypoxic injury
• Progressive
Diagnosis• Clinical – PWS, deficits, visual
problems
• CSF analysis – protein (microhaemorrhage)
• EEG – evaluate and localise seizures
• CT, MRI
Treatment• Glaucoma medications +/- surgery
• Seizure control with anti-convulsants
• Neurosurgery for refractive seizures
• Endocrinology, dermatology, psychology, psychiatry, MDT
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)
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)
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
Axial CT
L frontal lobe
L lateral ventricle
L parietal lobeR hemisphereresection cavity
ResidualR hemisphere
Calcification
SeptumPellucidum
T2 MRI
L frontal sinus
L lateral ventricle
L Sylvian fissureR hemisphereresection cavity
ResidualR frontal lobe
ResidualR occipital lobe
SeptumPellucidum
Hemispherectomy• Right craniotomy opened
• Insular cortex, residual frontal lobe resected. Occipital lobe disconnected
• Haemostasis and closure
• Post-operatively...
CTSurgical clips
Resection cavity
Craniotomy site
Calcification inDisconnectedoccipital lobe
Pneumocephalus
Midline shift
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