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

Fingerprint

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.
@article{3d0b650867cb4fd88ca67779ecfc9197,
title = "Enhancing nodular lesions in Chiari II malformations in the setting of persistent hindbrain herniation: case report and literature review",
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.",
keywords = "Chiari II malformation, Neurodevelopmental disorders, Pediatrics",
author = "Alexa Semonche and Shah, {Ashish H.} and Eichberg, {Daniel G.} and Sakir Gultekin and Komotar, {Ricardo J.} and Ivan, {Michael E.}",
year = "2019",
month = "1",
day = "1",
doi = "10.1007/s00381-019-04174-4",
language = "English (US)",
journal = "Child's Nervous System",
issn = "0256-7040",
publisher = "Springer Verlag",

}

TY - JOUR

T1 - Enhancing nodular lesions in Chiari II malformations in the setting of persistent hindbrain herniation

T2 - case report and literature review

AU - Semonche, Alexa

AU - Shah, Ashish H.

AU - Eichberg, Daniel G.

AU - Gultekin, Sakir

AU - Komotar, Ricardo J.

AU - Ivan, Michael E.

PY - 2019/1/1

Y1 - 2019/1/1

N2 - 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.

AB - 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.

KW - Chiari II malformation

KW - Neurodevelopmental disorders

KW - Pediatrics

UR - http://www.scopus.com/inward/record.url?scp=85065249296&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85065249296&partnerID=8YFLogxK

U2 - 10.1007/s00381-019-04174-4

DO - 10.1007/s00381-019-04174-4

M3 - Article

C2 - 31062141

AN - SCOPUS:85065249296

JO - Child's Nervous System

JF - Child's Nervous System

SN - 0256-7040

ER -