Giant Frontotemporal Cavernous Malformation in a 2-Month-Old Infant: A Case Report and Review of the Literature.

INTRODUCTION

Cavernous malformations in the pediatric population are exceedingly rare, especially in infants. Giant cavernous malformations (GCM) are even more rare and have a diameter >4 cm. The onset of symptoms predominantly occurs in adulthood, but the rate of hemorrhage is significantly higher in the pediatric population. Similar to non-GCM, GCM can be misidentified as tumors on imaging due to their tumefactive pattern with edema. Here, we present a rare case of a right frontotemporal GCM in a 2-month-old girl, the youngest recorded case in the existing literature.

CASE PRESENTATION

A previously healthy 2-month-old girl presented to the emergency department following an increasing frequency of seizure-like activity that began 3 days prior to presentation. Magnetic resonance imaging of the brain with and without contrast characterized a large (5.8 × 4.3 × 4.2 cm) heterogeneous lesion of the right temporal lobe with diffuse scattered blood products of various ages seen throughout the lesion. She underwent a right-sided craniotomy where a gross total excision was achieved. Pathology confirmed the diagnoses of a GCM. The patient's seizures subsequently resolved, and she continues to do well postoperatively.

DISCUSSION/CONCLUSION: GCM can be mistaken for tumors due to their large size, cystic nature, and surrounding edema, but a vascular lesion should always remain in the differential diagnosis before operating, even in infants. Surgery is generally recommended in patients that present with a symptomatic hemorrhage, recurrent hemorrhages, persistent seizures despite medical management, or progressively worsening neurological deficits if the GCM is in a safe location. It has been shown that 70-99% of patients undergoing surgery with successful removal of the GCM can achieve seizure freedom 2 years postoperatively. Complete surgical excision of this infant's GCM was successful in treating her neurologic symptoms; therefore, pathological confirmation of this lesion is critical and should prompt a complete surgical excision.