This article is part of the supplement: Malformations of the craniocervical junction chiari type I and syringomyelia: classification, diagnosis and treatment

Open Access Highly Accessed Review

Malformations of the craniocervical junction (chiari type I and syringomyelia: classification, diagnosis and treatment)

Alfredo Avellaneda Fernández12*, Alberto Isla Guerrero3, Maravillas Izquierdo Martínez4, María Eugenia Amado Vázquez5, Javier Barrón Fernández6, Ester Chesa i Octavio7, Javier De la Cruz Labrado8, Mercedes Escribano Silva5, Marta Fernández de Gamboa Fernández de Araoz9, Rocío García-Ramos10, Miguel García Ribes11, Carmen Gómez12, Joaquín Insausti Valdivia13, Ramón Navarro Valbuena14 and José R Ramón1

Author Affiliations

1 Carlos III Health Institute. Sinesio Delgado n. 6 (pavilion 12), 28029, Madrid, Spain

2 Spanish Society of Primary Care. c/ Narváez, 15 1º Izda 28009, Madrid, Spain

3 Spanish Society of Neurosurgery, Spain

4 Public Health and Health Management Chair, European University of Madrid Villaviciosa de Odón, Spain

5 Spanish Society of Physiotherapy, Madrid, Spain

6 Osteopathy, Cantabrian Health Service, Spain

7 Association of Syringomyelia Sufferers, Barcelona, Spain

8 Spanish Society of Psychology, Pozuelo de Alarcón, Madrid, Spain

9 National Association of Friends of Arnold Chiari, Burgos, Spain

10 Spanish Society of Neurology, Barcelona, Spain

11 Spanish Society of Family and Community Medicine, Barcelona, Spain

12 Spanish Society of Medical-Physical Rehabilitation of Madrid, Spain

13 Spanish Society of Pain, Madrid, Spain

14 Spanish Society of Pediatric Neurosurgery, Spain

For all author emails, please log on.

BMC Musculoskeletal Disorders 2009, 10(Suppl 1):S1  doi:10.1186/1471-2474-10-S1-S1

Published: 17 December 2009


Chiari disease (or malformation) is in general a congenital condition characterized by an anatomic defect of the base of the skull, in which the cerebellum and brain stem herniate through the foramen magnum into the cervical spinal canal. The onset of Chiari syndrome symptoms usually occurs in the second or third decade (age 25 to 45 years). Symptoms may vary between periods of exacerbation and remission. The diagnosis of Chiari type I malformation in patients with or without symptoms is established with neuroimaging techniques. The most effective therapy for patients with Chiari type I malformation/syringomyelia is surgical decompression of the foramen magnum, however there are non-surgical therapy to relieve neurophatic pain: either pharmacological and non-pharmacological. Pharmacological therapy use drugs that act on different components of pain. Non-pharmacological therapies are primarly based on spinal or peripheral electrical stimulation.

It is important to determine the needs of the patients in terms of health-care, social, educational, occupational, and relationship issues, in addition to those derived from information aspects, particularly at onset of symptoms.

Currently, there is no consensus among the specialists regarding the etiology of the disease or how to approach, monitor, follow-up, and treat the condition.

It is necessary that the physicians involved in the care of people with this condition comprehensively approach the management and follow-up of the patients, and that they organize interdisciplinary teams including all the professionals that can help to increase the quality of life of patients.