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Hypothalamic Hamartoma With Infantile Spasms: Case Report With Surgical Treatment

Jordana Fox1, Shaun Hussain2, Raman Sankar2

  • 1Division of Pediatric Neurology, Hypothalamic Hamartoma Program, Barrow Neurological Institute at Phoenix Children's Hospital, Phoenix, AZ.

Seminars in Pediatric Neurology
|July 3, 2018
PubMed

Insights

Surgical treatment of hypothalamic hamartoma (HH) effectively resolved infantile spasms in a young boy. This highlights focal pathologies as a cause of infantile spasms, responsive to surgery.

Area of Science:

  • Pediatric Neurology
  • Neurosurgery
  • Epileptology

Background:

  • Infantile spasms (IS) are a severe epilepsy syndrome in infants.
  • Hypothalamic hamartoma (HH) is a rare congenital brain malformation.
  • Treatment-resistant IS poses significant management challenges.

Observation:

  • A 10-month-old boy presented with treatment-resistant infantile spasms and hypothalamic hamartoma (HH).
  • Preoperative EEG revealed modified hypsarrhythmia.
  • Transventricular endoscopic resection of the HH was performed.

Findings:

  • Immediate and sustained resolution of epileptic spasms post-surgery.
  • Postoperative EEG showed significant improvement.
  • Eight years later, the patient experiences only mild gelastic seizures with residual HH, off all antiepilepsy drugs.
  • Academic and social success was achieved.

Implications:

  • This case demonstrates HH as a focal pathology causing IS and hypsarrhythmia.
  • Surgical intervention for HH can effectively treat concurrent IS.
  • HH lesion is an active component in the epileptic network for IS.
  • Supports surgical consideration for focal epilepsy syndromes presenting with IS.

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