Enhancing nodular lesions in Chiari II malformations in the setting of persistent hindbrain herniation

case report and literature review

Alexa Semonche, Ashish H. Shah, Daniel G. Eichberg, Sakir Gultekin, Ricardo J. Komotar, Michael E. Ivan

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
JournalChild's Nervous System
DOIs
StatePublished - Jan 1 2019
Externally publishedYes

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Rhombencephalon
Encephalocele
Neoplastic Processes
Meningomyelocele
Hypesthesia
Hydrocephalus
Decompression
Neuroimaging
Upper Extremity
Headache
Pediatrics
Biopsy
Growth
Neoplasms

Keywords

  • Chiari II malformation
  • Neurodevelopmental disorders
  • Pediatrics

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

Cite this

Enhancing nodular lesions in Chiari II malformations in the setting of persistent hindbrain herniation : case report and literature review. / Semonche, Alexa; Shah, Ashish H.; Eichberg, Daniel G.; Gultekin, Sakir; Komotar, Ricardo J.; Ivan, Michael E.

In: Child's Nervous System, 01.01.2019.

Research output: Contribution to journalArticle

Semonche, Alexa ; Shah, Ashish H. ; Eichberg, Daniel G. ; Gultekin, Sakir ; Komotar, Ricardo J. ; Ivan, Michael E. / Enhancing nodular lesions in Chiari II malformations in the setting of persistent hindbrain herniation : case report and literature review. In: Child's Nervous System. 2019.
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