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.

Glioneuronal tumors of cerebral hemisphere in children: correlation of surgical resection with seizure outcomes and tumor recurrences.

OBJECT: Glioneuronal tumors are common neoplasms among the cerebral hemisphere during childhood. They consist of several histological types, of which gangliogliomas (GGs) and dysembryoplastic neuroepithelial tumors (DNTs) are most common and often present with seizures. A great majority of glioneuronal tumors are benign. However, there are conflict reports regarding postoperative tumor recurrence rates and seizure control. The authors analyzed and compared these tumors for their locations and histology and the tumor and seizure control following resection. METHODS: The authors conducted a retrospective analysis of patients with pediatric glioneuronal tumors in the cerebral hemisphere. All histology reports and neuroimaging are reviewed. Seizure group and non-seizure group were compared with their tumor types and locations. The extent of tumor resections were divided into gross total resection (GTR) and subtotal resection (STR). Postoperative tumor recurrence-free survival (RFS) and seizure-free survival for patients who had the initial surgery done at our institution were calculated using Kaplan-Meier method. RESULTS: There were 90 glioneuronal tumors including 58 GGs, 22 DNTs, 3 papillary glioneuronal tumor, 3 desmoplastic infantile gangliogliomas, 3 anaplastic GGs, and 1 central neurocytoma. Seventy-one patients (seizure group) presented with seizures. The temporal lobe is the most common location, 50 % in this series. GTR was attained in 79 patients and STR in 11. All of the patients with GTR had lesionectomy, and only six of them had extended corticectomy or partial lobectomy. Postoperative seizure outcome showed that 64 (90 %) of seizure group had Engel's class I, but five patients subsequently developed recurrent seizures. Patients with DNTs had a higher seizure recurrence rate. Tumor RFS was 87 % at 5 years and 75.5 % at 10 years. There are no significant difference in tumor recurrences between GGs and DNTs (p = 0.876). Comparison between GRT (67) and STR (9) showed that in spite of the better 5-year tumor RFSs among GRT group (94 %) than STR group (66 %), the 10-year RFSs showed no significant difference between GRT and STR groups (p = 0.719). Recurrent seizures are often related to recurrent tumor. CONCLUSION: Lesionectomy alone often provides a high-rate seizure freedom. GGs and DNTs are benign tumor, but recurrences of GGs and DNTs are not uncommon. They may show late recurrences in spite of GTR. These patients need longer follow-up for 10 years. Recurrent seizures are often related to a tumor recurrence.

Cochlear implantation via middle fossa approach - a case report.

Objective: Cochlear implantation is routinely performed all over the world via the post-auricular, facial recess approach. Our case study describes the middle fossa approach for the management of bilateral profound sensorineural hearing loss in a female child with multiple external, middle and inner ear malformations. The middle fossa approach has been reported sporadically and has been used inconsistently in patients with chronic otitis media and inner ear malformations. Case study: A 3-year-old female child presented with bilateral profound sensorineural hearing loss, bilateral anotia, right cochlear nerve agenesis, right facial paralysis, bilateral mild inner ear dysplasia and an aberrant left facial nerve covering the left round window. Further tests indicated that she was a suitable candidate for cochlear implantation and was medically cleared for surgery. In view of the multiple malformations and high risk of injury to her only functioning left facial nerve on the side with a cochlear nerve, cochlear implantation via a middle fossa approach was performed. Conclusion: Middle fossa approach to cochlear implantation is challenging but can be safely performed even in children and offers an option in patients when a routine mastoidectomy, facial recess and round window approach cannot be undertaken.