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Meena Thatikunta, Ian Mutchnick, Jennifer Elster, Matthew P. Thompson, Michael A. Huang, Aaron C. Spalding and Thomas Moriarty

Atypical teratoid rhabdoid tumors (ATRTs) are a rare pediatric brain tumor with high mortality rate. Several large series have reported achieving gross-total resection (GTR) in less than 50% of patients due to the lesions' large size, vascularity, and limited blood volume in young patients. While neoadjuvant chemotherapy for choroid plexus carcinomas in pediatric patients has become widely accepted, it has not been used as widely for other pediatric brain tumors. To the best of the authors' knowledge, there are only 3 published cases of neoadjuvant chemotherapy for ATRTs. In the present report, the authors present a fourth case of neoadjuvant chemotherapy for ATRT and review the available literature on this strategy. A 17-month-old child presented with a left ventricular ATRT for which imaging raised concern for a highly vascularized tumor. The authors undertook neoadjuvant chemotherapy with 2 cycles of Head Start II therapy, which reduced the size of the ventricular tumor by 35% and decreased the vascularity of the lesion on imaging. The estimated blood loss during resection was 425 ml and GTR was achieved. The patient continued with postoperative chemotherapy but suffered an on-therapy recurrence. While higher-quality data are necessary, available evidence suggests that neoadjuvant chemotherapy can reduce the size and vascularity of ATRTs and facilitate a surgical avenue for large or “inoperable” tumors.

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Jacob Archer, Meena Thatikunta and Andrew Jea

The transoral transpharyngeal approach is the standard approach to resect the odontoid process and decompress the cervicomedullary spinal cord. There are some significant risks associated with this approach, however, including infection, CSF leak, prolonged intubation or tracheostomy, need for nasogastric tube feeding, extended hospitalization, and possible effects of phonation. Other ventral approaches, such as transmandibular and circumglossal, endoscopic transcervical, and endoscopic transnasal, are also viable alternatives but are technically challenging or may still traverse the nasopharyngeal cavity. Far-lateral and posterior extradural approaches to the craniocervical junction require extensive soft-tissue dissection. Recently, a posterior transdural approach was used to resect retro-odontoid cysts in 3 adult patients. The authors present the case of a 12-year-old girl with Down syndrome and significant spinal cord compression due to basilar invagination and a retro-flexed odontoid process. A posterior transdural odontoidectomy prior to occiptocervical fusion was performed. At 12 months after surgery, the authors report satisfactory clinical and radiographic outcomes with this approach.

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Meena Thatikunta, Jeffrey Bumpous, Jarrod Little, Samir Karia, Nicole R. Herring, Mohammed Nuru, Nitin Engineer and Ian Mutchnick

Primary encephaloceles (PEs) present only rarely in the temporal region; in the rare instance that they project through the floor of the middle fossa they are secondary. In this case report the authors report on the management of a giant PE extending through the floor of the middle fossa.

An 8-month-old boy presented to the authors’ service with a large PE projecting into his neck through a missing left middle fossa floor; the lesion was causing significant meta-, dys-, and hypoplasia of the structures of the anterolateral neck on that side. Surgical goals for this patient included the following: 1) removal of potentially epileptogenic and dysfunctional tissue; 2) preservation of cranial nerves; 3) prevention of cognitive decline or iatrogenic deficit; 4) prevention of CSF leak; 5) reconstruction of skull base; 6) prevention of airway and swallowing compromise; and 7) cosmesis. After a multidisciplinary evaluation with ENT, plastic surgery, and neurology, an operation was performed using a preauricular infratemporal approach when the patient was 3 years old. Gliotic tissue was resected and amygdala, hippocampus, and middle cerebral artery were preserved.

The immediate results of the operation showed good immediate outcome. Seizure freedom and neurodevelopment outcomes remain to be seen at follow-up.