Hemispherotomy - Indications
and Techniques
Syed Yasin Shahtaz Emanee
Hemispheric disconnection procedures
• Group of surgical interventions for
chronic epilepsy that are used as alternatives
to anatomic hemispherectomy.
• Disconnection of the cortex of
one hemisphere from the contralateral
hemisphere and from the deeper structures
of the basal ganglia
• Hemispherotomy
• Hemispheric Deafferentiation
• Hemispherectomy
• Hemidecortication
• Hemicorticectomy
• 1928 – Dandy – Anatomic hemispherectomy
for Glioma
• 1950 – Kyrnauw – For drug resistant epilepsy
• High rate of complications following anatomic
resections – need for functional resections
Less Resection—More Disconnection
• 1970 – Rasmussen’s Functional
Hemispherectomy
“Removal of two larger brain
segments (the temporal lobe and
an en bloc resection of the central
cortex in the suprasylvian location),
combined with callosotomy and
disconnection of the frontal,
parietal, and occipital lobe”
Less Resection—More Disconnection
• 1992 – Transition to exclusive disconnective
techniques
• Schramm, Delande, Villemure, Shimizu,
Maehara
• Fewer complications
Indications and Patient Selection
• To treat drug resistant epilepsy resulting from
diffuse damage to one hemispehre
• Usually associated with hemiparesis,
hemianopia, delayed cognitive milestones
Why do Hemispheric Epilepsies occur?
• Inborn, Perinatal or Acquired conditions
Sturge Weber Syndrome
• Seizures in 80% - usually by end of 1st year
• Often Drug resistant
• Changes remain restricted to one hemisphere
• Classic Candidates – Children with widespread
hemispheric involvement
• Seizure Free rates – 100 to 82% in various
studies
Hemimegalencephaly
• Neuronal Migration Disorder
• Hemiparesis, mental retaration
• Most commonly involved syndromes are epidermal nevus
syndrome , Proteus syndrome, and Klippel–Trenaunay syndrome
Rasmussen Encephalitis
• Intractable epilepsy, progressive hemiparesis -.
Hemiplegia, mental decline, hemispheric
atrophy
Rasmussen Encephalitis
• Epilepsia partialis continua
• Perisylvian atrophy and encephalomalacia
• Histology – Perivascular infiltrate of T
lymphocytes
• Progressive atrophy on serial MRI
Generalised indications
• “Unihemispheric lesions that are either inborn
or occurred around the time of birth and
manifested during infancy or early childhood
as frequent and intractable seizures
• Small infants with catastrophic epilepsy
How is it possible to have a
functional existence after
such a procedure?
• Disconnection mostly in a nonfunctional or
partly destroyed hemisphere
• Nearly always results in loss of fine motor
control of the hand and occasional gait
imbalance, but most persons are able to walk
When to operate?
• HME with severe seizures – early (around 4
months)
• Dilemma – in children more than 7 or 8 years,
with language dominant side affected
• Acceptance of motor loss earlier – bad
outcome may be averted (avoiding epileptic
encephalopathy)
When NOT to operate
• If the presurgical evaluation cannot
demonstrate that all typical ictal activity
originates from the affected hemisphere.
• Independently arising seizures from the
healthy hemisphere
• Incomplete hemianopia – in older children
• Mental retardation is not a contraindication
Presurgical evaluation
• Clinical history, MRI, video EEEG of seizures
• Neuropsychological testing
• Wada test – if contralateral ictal activity
disappears when amobarb is injected to
affected hemisphere ->conducted
phenomenon
• fMRI
• If Rasmussens suspected – brain biopsy
Presurgical evaluation
• Intracranial electrodes rarely needed
• Dural recordings may be used to identify
patients for multilobar resections (vs complete
hemispherectomies.
• Customised Stereo EEG preferred in some
centres
• Nuclear imaging – unilateral hypometabolism
on FDG PET predicts a favourable seizure
outcome postop
Goals of Surgery
• Cessation of Seizures
• Relief of neurological deterioration
• Improvement of cognitive development and
behavioral disturbances
Side Effects and Complications
• UNAVOIDABLE:
– complete hemianopia
– Loss of fine motor movements
• Pincer grasp
• Inversion, Eversion and Fanning of toes
• Gait Imbalance
• More adverse affects when disease develops
late and hemiparesis is mild
• Prediction of post operative good motor
function – when unilateral stimulation of the
motor cortex results in bilateral responses
• Consent must include:
– Lack of success of seizure cessation
– Need for postop shunting
– Need for transfusions, wound infection,
meningitis, dysphagia, movement disorders, death
Less Resection—More Disconnection
Transsylvian keyhole approach
• Exposure of circular sulcus
• Temporomesial disconnection
• Complete opening of the lateral ventricle
• Mesial disconnnection and callosotomy
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Vertical Parasaggital Hemispherotomy
(dorsal transcortical subinsular hemispherotomy)
• 5 steps
– Parasaggital frontal craniotomy
– Transcortical access to lateral ventricle
– Paramedian callosotomy
– Lateral transection between thalamus and striatum
– Resection of posterior part of gyrus rectus
• Parasagittal craniotomy and posterior frontal
resection
Vertical Parasagittal Hemispherotomy
• Posterior callosotomy
Vertical Parasagittal Hemispherotomy
• Laterothalamic disconnection
Vertical Parasagittal Hemispherotomy
• Anterior callosotomy and gyrus rectus
resection
Vertical Parasagittal Hemispherotomy
• Frontal disconnection
VPH vs PIH
• In small children, a VPH more useful - smaller
incision.
• The structures required to be disconnected
being at a greater depth are more easily
accessed in smaller heads.
• The presence of ventriculomegaly makes this
technique easier.
VPH vs PIH
• A distance of less than 10 cm between the
vertex (point of entry) and the temporal horn
has been used as a rough cutoff point in
deciding to perform VPH.
• (upper limit of comfortably working under the
microscope through a cortical incision)
VPH vs PIH
• PIH - larger scalp incision and the structures to
be disconnected are more easily accessed -
more convenient for older children.
• Better for pathologies like
hemimegelencephaly with large hemisphere,
abnormal tissue and 'chinked' ventricles.
Combined resection and Deafferentiation
techniques
• Peri insular hemispherotomy
– Schramms peri insular transcortical approach
– Vilemure’s peri insular hemispherotomy -
– Japanese peri insular modification – insular cortex
partly removed
• Perisylvian window technique – FP, T opercula
resected
Techniques – Transsylvian and
modifications
Alternative Techniques
• Anatomic hemispherectomy
• Hemicorticectomy – only ictogenic cortex is
removed
Post operative Management
• Compare pre and post op seizures
• Continue same AEDs
• Post operative MRI using blood as contrast
Choice of surgical Procedure
• Safety considerations
• Surgeon’s preference
• In HME, preoperative embolisation may be
required
Video - PIH Video - VPH
Outcome Measures
• Seizure free rate
– More than 5 accute postop seizures = worse
seizure control at 1 year
• Transfusion rate
• Shunt rate
• Cognitive outcome
• Poor outcome – HME
• Better outcome – SWS, porencephalic cysts
Complications
• Intraop
• Early postop
– Electrolyte imbalances, DI, SIADH
– Transient rise in temperature (versus bacterial
meningitis)
– Hematoma, Subdural Hygroma
• Late
– Bone flap infections
– Hydrocephalus
Key Messages
• Hemispherotomies provide a definitive cure
for otherwise untreatable structural
epilepsies
• Evolving techniques for better outcomes –
trends towards minimally invasive
• Balance between acceptable functional loss,
seizure free outcome, minimising
complications
THANK YOU