Alexa Semonche1, Ashish H Shah2, Daniel G Eichberg2, Sakir H Gultekin3, Ricardo J Komotar2, Michael E Ivan2. 1. Department of Neurological Surgery, University of Miami Miller School of Medicine, 1095 NW 14th Terrace, Room 2-06, Miami, FL, 33136, USA. ams757@rwjms.rutgers.edu. 2. Department of Neurological Surgery, University of Miami Miller School of Medicine, 1095 NW 14th Terrace, Room 2-06, Miami, FL, 33136, USA. 3. Department of Pathology, University of Miami Miller School of Medicine, 1120 NW 14th Street, 14th Floor Suite 1409, Miami, FL, 33136, USA.
Abstract
BACKGROUND: Chiari II malformation includes concomitant cerebellar tonsillar herniation, hydrocephalus, and myelomeningocele. Rarely, pediatric patients with persistent hindbrain herniation develop a new enhancing nodule at the cervicomedullary junction as adults. These new lesions may be suspicious for neoplastic growth, but it remains unclear if neurosurgical intervention is necessary. CASE REPORT: A 27-year-old female patient with history of Chiari II malformation and persistent hindbrain herniation presented with a 3-month history of headache and upper extremity weakness and numbness. Neuroimaging revealed a new enhancing nodule near the cervicomedullary junction suspicious for neoplasm. Following posterior fossa decompression and excision of the enhancing lesion, pathological analysis demonstrated only benign glioneural heterotopia. RESULTS: New enhancing nodules at the cervicomedullary junction in Chiari II malformation are exceedingly rare and are likely benign, reactive changes rather than a neoplastic process. Biopsy or surgical excision of these lesions is likely unnecessary for asymptomatic patients.
BACKGROUND: Chiari II malformation includes concomitant cerebellar tonsillar herniation, hydrocephalus, and myelomeningocele. Rarely, pediatric patients with persistent hindbrain herniation develop a new enhancing nodule at the cervicomedullary junction as adults. These new lesions may be suspicious for neoplastic growth, but it remains unclear if neurosurgical intervention is necessary. CASE REPORT: A 27-year-old female patient with history of Chiari II malformation and persistent hindbrain herniation presented with a 3-month history of headache and upper extremity weakness and numbness. Neuroimaging revealed a new enhancing nodule near the cervicomedullary junction suspicious for neoplasm. Following posterior fossa decompression and excision of the enhancing lesion, pathological analysis demonstrated only benign glioneural heterotopia. RESULTS: New enhancing nodules at the cervicomedullary junction in Chiari II malformation are exceedingly rare and are likely benign, reactive changes rather than a neoplastic process. Biopsy or surgical excision of these lesions is likely unnecessary for asymptomatic patients.
Entities:
Keywords:
Chiari II malformation; Neurodevelopmental disorders; Pediatrics