Disconnective procedures

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Status
Done

Multiple subpial transections of cortex

  • Done when epileptogenic zone is in the eloquent cortex
  • Cortex is instead disrupted by parallel subpial cuts transecting the gray matter every 3-5 mm.
  • Intention is to preserve centripetal axonal outflow while isolating silos of epileptogenic neurons by disrupting lateral dendritic communication across gyri, thereby preventing Jacksonian patterns of spreading cortical propagation of seizures.
  • The benefit and indications of this technique remain controversial.

Hemispherectomy

General

  • Incomplete disconnection may predispose young children to less seizure control.
  • Most patients will end up with deficit but child can typically ambulate with an orthotic device and use the hand as a helper hand while receiving special education
    • This is eminently better than being developmentally frozen in a stupor or coma from continued daily seizures.

Indication (more for paeds) better neuroplasticity

  • Infants and children AND
  • Intractable epilepsy from an epilepsy involving an entire hemisphere (poor prognosis)
    • West syndrome with malformation of cortical development,
    • Rasmussen’s encephalitis,
    • Sturge-Weber syndrome
    • Chronic encephalitis
    • Hemimegalencephaly

Anatomic hemispherectomy

  • Often used for children with hemimegalencephaly.
  • Involves the removal of a cerebral hemisphere,
  • Complications
    • Siderosis
      • The dura of the cavity following a hemispherectomy may have multiple bleeding points that have direct access to the ventricular system.
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Functional hemispherectomy

  • Leaves the hemisphere physically intact but functionally disconnected
  • Multiple modifications of hemispherectomy have been developed to address various types of complication associated with anatomical hemispherectomy
  • Includes
    • Hemicorticectomy
      • Resection
        • Of the cortical Gray matter in all accessible affected regions AND
          • The incidence of unexpected neurologic deficits after cortical resection is rare.
            • 50% of a presumed eloquent area such as the motor strip can be removed without causing a permanent deficit if the underlying white matter tracts are spared.
            • The children often experience a transient deficit that resolves over several weeks.
            • Even in children with some developmental delay with a curtailed ability to rehabilitate due to limited cognition, they tend to have a better quality of life without seizures even with a new deficit such as a hemiparesis.
        • Temporal lobe.
    • Complete corpus callostomy
      • See below
    • Temporal lobectomy
    • Transection of the parietal lobe from the occipital and frontal lobes
    • Keyhole hemispherectomy
      • Recently developed and may offer adequate seizure control with less surgical morbidity.
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Posterior quadrantectomy

  • The posterior quadrant consists of the parietal, posterior temporal, and occipital lobes

Partial or complete section of the corpus callosum (Corpus callostomy)

Indication

  • Atonic seizures with loss of postural tone leading to falls
    • 70% of patients
    • Aim is to reduce drop attack resultant injury
  • Generalized major motor seizures without an identifiable resectable epileptogenic zone
  • Generalized seizures with unilateral hemispheric damage if not deemed candidates for functional or anatomic hemispherectomy (e.g. preserved motor function)

Complications

  • Postoperative sequelae of decreased vocalization or akinetic mutism will usually resolve within weeks to months.
  • Transient hemiparesis
    • Present for about a week
    • Due to the retraction on the hemisphere.
  • Disconnection syndrome
    • Occurs with complete callosotomy
    • To avoid
      • The anterior 2/3 of the corpus callosum is sectioned, often sparing the anterior commissure;
      • In left-handed patients shown by Wada testing to have crossed dominance with speech and handedness in opposite hemispheres,
        • Even partial corpus callosotomy is contraindicated to avoid major behavioural and language deficits postoperatively.
    • Includes
      • Left tactile anomia
      • Left dyspraxia
      • Pseudohemianopsia
      • Right anomia for smell
      • Right hand-impaired spatial synthesis and difficulty copying complex figures
      • Decreased spontaneity of speech
      • Incontinence.

Outcome

  • See Graham 2019
      • Findings
        • 55 patients
        • 11 ant 2/3 CC
        • 43 complete CC
        • 47% good outcome for drops
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  • Corpus callosotomy rarely results in complete seizure control
    • About 80% of children will experience a significant improvement in seizure control and associated quality of life.