"Two-Birds-One-Stone" Approach for Treating an Infant with Chiari I Malformation and Hydrocephalus: Is Cerebrospinal Fluid Diversion as Sole Treatment Enough?
Arnold-Chiari Malformation
/ complications
Cervical Atlas
/ surgery
Cervical Vertebrae
Decompression, Surgical
Humans
Hydrocephalus
/ diagnostic imaging
Infant
Laminectomy
Magnetic Resonance Imaging
Male
Neurosurgical Procedures
Syringomyelia
/ complications
Thoracic Vertebrae
Treatment Outcome
Ventriculoperitoneal Shunt
/ methods
Chiari I malformation
Hydrocephalus
Infant
Ventriculoperitoneal shunting
Journal
World neurosurgery
ISSN: 1878-8769
Titre abrégé: World Neurosurg
Pays: United States
ID NLM: 101528275
Informations de publication
Date de publication:
05 2020
05 2020
Historique:
received:
24
11
2019
revised:
23
01
2020
accepted:
24
01
2020
pubmed:
7
2
2020
medline:
22
7
2020
entrez:
7
2
2020
Statut:
ppublish
Résumé
Chiari I malformation (CIM) is a disorder characterized by caudal displacement of the cerebellar tonsils below the foramen magnum. It is often associated with syringomyelia and occasionally with hydrocephalus. CIM is commonly treated by posterior fossa decompression with or without removal of the posterior arch of C1 and duraplasty, but the treatment for infants with symptomatic CIM is not well established. We present a case of symptomatic CIM in an infant that was successfully treated with a ventriculoperitoneal shunt (VPS) and discuss the importance of the pathophysiology in management decisions. A 6-month-old male with a CIM and a cervicothoracic syrinx presented with stridor, lower cranial nerve dysfunction, and increased tone that worsened with crying. Magnetic resonance imaging studies revealed cerebellar tonsillar displacement extending to the level of C3-C4 with a syrinx extending from C4 to T4. In addition, there was compression of the cervicomedullary junction, fourth ventricular outflow obstruction, and obstructive hydrocephalus. The decision was made to place a ventriculoperitoneal shunt (VPS) instead of performing decompressive surgery as the initial treatment intervention. The infant had significant symptomatic relief at 6-, 9-, and 12-month follow-ups. Postoperative magnetic resonance imaging at 6-month follow-up revealed resolution of the syrinx and ventriculomegaly and ascent of the cerebellar tonsils. Ventriculoperitoneal shunting alone was successfully used to treat an infant with concurrent CIM, syrinx, and hydrocephalus. This case underscores not only the importance of hydrocephalus as the pathogenesis of CIM in some cases but also the possibility of avoiding the morbidity of decompressive surgery in infants.
Sections du résumé
BACKGROUND
Chiari I malformation (CIM) is a disorder characterized by caudal displacement of the cerebellar tonsils below the foramen magnum. It is often associated with syringomyelia and occasionally with hydrocephalus. CIM is commonly treated by posterior fossa decompression with or without removal of the posterior arch of C1 and duraplasty, but the treatment for infants with symptomatic CIM is not well established. We present a case of symptomatic CIM in an infant that was successfully treated with a ventriculoperitoneal shunt (VPS) and discuss the importance of the pathophysiology in management decisions.
CASE DESCRIPTION
A 6-month-old male with a CIM and a cervicothoracic syrinx presented with stridor, lower cranial nerve dysfunction, and increased tone that worsened with crying. Magnetic resonance imaging studies revealed cerebellar tonsillar displacement extending to the level of C3-C4 with a syrinx extending from C4 to T4. In addition, there was compression of the cervicomedullary junction, fourth ventricular outflow obstruction, and obstructive hydrocephalus. The decision was made to place a ventriculoperitoneal shunt (VPS) instead of performing decompressive surgery as the initial treatment intervention. The infant had significant symptomatic relief at 6-, 9-, and 12-month follow-ups. Postoperative magnetic resonance imaging at 6-month follow-up revealed resolution of the syrinx and ventriculomegaly and ascent of the cerebellar tonsils.
CONCLUSIONS
Ventriculoperitoneal shunting alone was successfully used to treat an infant with concurrent CIM, syrinx, and hydrocephalus. This case underscores not only the importance of hydrocephalus as the pathogenesis of CIM in some cases but also the possibility of avoiding the morbidity of decompressive surgery in infants.
Identifiants
pubmed: 32028005
pii: S1878-8750(20)30206-0
doi: 10.1016/j.wneu.2020.01.188
pii:
doi:
Types de publication
Case Reports
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
174-177Commentaires et corrections
Type : CommentIn
Type : CommentIn
Informations de copyright
Copyright © 2020 Elsevier Inc. All rights reserved.