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 fissure

R 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...

CT

Surgical 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