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Published on: April 12, 2024
Fourth ventricular hamartoma presenting with status epilepticus treated with emergency surgery in an infant
P Sarat Chandra1, Sheffali Gulati, Veena Kalra
1Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India.
Insights
A rare case of a 4-week-old infant with severe hemifacial spasms and status epilepticus due to a fourth ventricular hamartoma was successfully treated with surgery. This youngest patient reported achieved a complete recovery after tumor excision.
Area of Science:
- Pediatric Neurology
- Neurosurgery
- Neuro-oncology
Background:
- Infantile hemifacial spasms can be a symptom of underlying neurological conditions.
- Refractory status epilepticus in neonates requires urgent intervention.
Observation:
- A 4-week-old infant presented with progressive hemifacial spasms starting on day 4 postpartum.
- The infant developed refractory status epilepticus, necessitating mechanical ventilation and emergency surgery.
- MRI revealed a fourth ventricular hamartoma, and video EEG correlated facial twitches with epileptic spikes.
Findings:
- Surgical excision of the fourth ventricular hamartoma led to initial seizure freedom.
- Recurrence of spasms occurred due to residual tumor, which was completely removed in a second surgery.
- The patient achieved complete seizure recovery and maintained it over a 5-year follow-up.
Implications:
- This case highlights fourth ventricular hamartomas as a treatable cause of infantile spasms and status epilepticus.
- Successful surgical management in neonates is feasible and can lead to long-term positive outcomes.
- It establishes a new youngest age record for successful surgical intervention of this condition.
Abstract:
A 4-week-old infant presented with hemifacial spasms noticed from the 4th day after delivery. These progressed in severity, with generalization every 3-4 h. On admission, the infant went into refractory status epilepticus and had to be electively ventilated and taken for surgery on a semi-emergency basis. MRI showed a fourth ventricular hamartoma and video EEG showed spikes that were synchronous with the facial 'twitches' with generalization. Following the first surgery, the infant had an initial complete recovery, but developed recurrence of facial twitches after 2 weeks. Repeat MRI showed a small residual tumor which was re-operated and completely excised (at 8 weeks). Following this, the patient had complete recovery from seizures (5-year follow-up). This is the youngest patient reported presenting with status epilepticus with a fourth ventricular hamartoma operated successfully.
