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Published in: Child's Nervous System 7/2019

01-07-2019 | Laminectomy | Case Report

Enhancing nodular lesions in Chiari II malformations in the setting of persistent hindbrain herniation: case report and literature review

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

Published in: Child's Nervous System | Issue 7/2019

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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.
Literature
1.
go back to reference Browne TR, Adams RD, Roberson GH (1976) Hemangioblastoma of the spinal cord. Review and report of five cases. Arch Neruol 33:435–441CrossRef Browne TR, Adams RD, Roberson GH (1976) Hemangioblastoma of the spinal cord. Review and report of five cases. Arch Neruol 33:435–441CrossRef
2.
go back to reference Cesmebasi A, Loukas M, Hogan E, Kralovic S, Tubbs RS, Cohen-Gadol AA (2015) The Chiari malformations: a review with emphasis on anatomical traits. Clin Anat 28:184–194CrossRefPubMed Cesmebasi A, Loukas M, Hogan E, Kralovic S, Tubbs RS, Cohen-Gadol AA (2015) The Chiari malformations: a review with emphasis on anatomical traits. Clin Anat 28:184–194CrossRefPubMed
3.
go back to reference Dhandapani S, Srinivasan A (2016) Contiguous triple spinal dysraphism associated with Chiari malformation type II and hydrocephalus: an embryological conundrum between the unified theory of Peng and the unified theory of Malone. JNS Pediatrics 17:103–106 Dhandapani S, Srinivasan A (2016) Contiguous triple spinal dysraphism associated with Chiari malformation type II and hydrocephalus: an embryological conundrum between the unified theory of Peng and the unified theory of Malone. JNS Pediatrics 17:103–106
4.
go back to reference Hino-Shishikura A, Niwa T, Aida N, Okabe T, Nagaoka T, Shibasaki J (2012) Periventricular nodular heterotopia is related to the severity of hindbrain deformity in Chiari II malformation. Pediatr Radiol 42:1212–1217CrossRefPubMed Hino-Shishikura A, Niwa T, Aida N, Okabe T, Nagaoka T, Shibasaki J (2012) Periventricular nodular heterotopia is related to the severity of hindbrain deformity in Chiari II malformation. Pediatr Radiol 42:1212–1217CrossRefPubMed
5.
go back to reference Hirano S, Houdou S, Hasegawa M, Kamei A, Takashima S (1999) Clinicopathologic studies on leptomeningeal glioneuronal heterotopia in congenital anomalies. Pediatr Neurol 8:441–444CrossRef Hirano S, Houdou S, Hasegawa M, Kamei A, Takashima S (1999) Clinicopathologic studies on leptomeningeal glioneuronal heterotopia in congenital anomalies. Pediatr Neurol 8:441–444CrossRef
6.
go back to reference McAbee JH, Modica J, Thompson CJ, Broniscer M, Orr B, Choudhri AF et al (2015) Cervicomedullary tumors in children. J Neurosurg 16:357–366 McAbee JH, Modica J, Thompson CJ, Broniscer M, Orr B, Choudhri AF et al (2015) Cervicomedullary tumors in children. J Neurosurg 16:357–366
7.
go back to reference Messing-Junger M, Rohrig A (2013) Primary and secondary management of the Chiari II malformation in children with myelomeningocele. Childs Nerv Syst 29:1553–1562CrossRefPubMed Messing-Junger M, Rohrig A (2013) Primary and secondary management of the Chiari II malformation in children with myelomeningocele. Childs Nerv Syst 29:1553–1562CrossRefPubMed
8.
go back to reference Naidich TP, McLone DG, Fulling KH (1983) The Chiari II malformation: part IV. The hindbrain deformity. Neuroradiology 25:179–197CrossRefPubMed Naidich TP, McLone DG, Fulling KH (1983) The Chiari II malformation: part IV. The hindbrain deformity. Neuroradiology 25:179–197CrossRefPubMed
9.
go back to reference Oya S, Kawahara N, Aoki S, Hayashi N, Shibahara J, Izumi M et al (2005) Intracranial extracerebral glioneuronal heterotopia. JNS Pediatrics 102:105–112 Oya S, Kawahara N, Aoki S, Hayashi N, Shibahara J, Izumi M et al (2005) Intracranial extracerebral glioneuronal heterotopia. JNS Pediatrics 102:105–112
10.
go back to reference Peach B (1964) Cystic prolongation of fourth ventricle an anomaly associated with the Arnold Chiari malformation. JAMA Neurol 11:609–612 Peach B (1964) Cystic prolongation of fourth ventricle an anomaly associated with the Arnold Chiari malformation. JAMA Neurol 11:609–612
11.
go back to reference Piatt JH, D’Agostino A (1999) The Chiari II malformation: lesions discovered within the fourth ventricle. Pediatr Neurosurg 30:79–85CrossRefPubMed Piatt JH, D’Agostino A (1999) The Chiari II malformation: lesions discovered within the fourth ventricle. Pediatr Neurosurg 30:79–85CrossRefPubMed
12.
go back to reference Singla A, Silvera VM, Ciarlini P, Warf BC (2012) Dysplastic-reactive choroid plexus presenting as an intramedullary tumor of the cervicomedullary junction in a patient with myelomeningocele. JNS Pediatrics 10:406–410 Singla A, Silvera VM, Ciarlini P, Warf BC (2012) Dysplastic-reactive choroid plexus presenting as an intramedullary tumor of the cervicomedullary junction in a patient with myelomeningocele. JNS Pediatrics 10:406–410
13.
go back to reference Stark JE, Glasier CM (1993) MR demonstration of ectopic fourth ventricular choroid plexus in Chiari II malformation. AJNR Am J Neuroradiol 14:618–621PubMed Stark JE, Glasier CM (1993) MR demonstration of ectopic fourth ventricular choroid plexus in Chiari II malformation. AJNR Am J Neuroradiol 14:618–621PubMed
14.
go back to reference Stevenson KL (2004) Chiari type II malformation: past, present, and future. Neurosurg Focus 16:Article 5CrossRef Stevenson KL (2004) Chiari type II malformation: past, present, and future. Neurosurg Focus 16:Article 5CrossRef
15.
go back to reference Tubbs RS, Oakes WJ (2004) Treatment and management of the Chiari II malformation: an evidence-based review of the literature. Childs Nerv Syst 20:375–381CrossRefPubMed Tubbs RS, Oakes WJ (2004) Treatment and management of the Chiari II malformation: an evidence-based review of the literature. Childs Nerv Syst 20:375–381CrossRefPubMed
16.
go back to reference Wong SK, Barkovich AJ, Callen AL, Filly RA (2009) Supratentorial abnormalities in Chiari II malformation, III: the interhemispheric cyst. J Ultrasound Med 28:999–1006CrossRefPubMed Wong SK, Barkovich AJ, Callen AL, Filly RA (2009) Supratentorial abnormalities in Chiari II malformation, III: the interhemispheric cyst. J Ultrasound Med 28:999–1006CrossRefPubMed
Metadata
Title
Enhancing nodular lesions in Chiari II malformations in the setting of persistent hindbrain herniation: case report and literature review
Authors
Alexa Semonche
Ashish H. Shah
Daniel G. Eichberg
Sakir H. Gultekin
Ricardo J. Komotar
Michael E. Ivan
Publication date
01-07-2019
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 7/2019
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-019-04174-4

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