Management of Basilar Invagination Tratamento De Invaginação Basilar Andrei Fernandes Joaquim1, MD, Phd
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53 Review Management of Basilar Invagination Tratamento de Invaginação Basilar Andrei Fernandes Joaquim1, MD, PhD. RESUMO A invaginação basilar (IB) constitui-se de uma anomalia do desenvolvimento da região crânio-cervical que resulta no prolapso da coluna cervical superior na base do crânio, comumente associada com outras anormalidades do neuro-eixo, tais como malformação de Chiari do tipo I e siringomielia. Neste artigo, revisamos os conceitos necessários para entender e tratar os pacientes com IB. O tratamento é discutido com base na classificação proposta por Goel, que divide a IB em dois grupos: grupo A - pacientes com elementos de instabilidade na junção crânio-cervical e grupo B - pacientes com IB secundária à hipoplasia do clivus. O tratamento no grupo A consiste no realinhamento e na estabilização da junção crânio-cervical, muitas vezes através de uma abordagem por via posterior isolada, evitando a morbidade inerente às descompressões por via anterior. No grupo B, a descompressão do forame magno é o tratamento de escolha. As técnicas cirúrgicas a serem utilizadas dependem da anatomia do paciente e da experiência do cirurgião. Resultados cirúrgicos adequados podem ser obtidos com o entendimento dos conceitos e formas de tratamento das diferentes apresentações da IB. Palavras Chave: invaginação basilar, classificação, tratamento. ABSTRACT Basilar invagination (BI) is a development anomaly of the craniocervical junction that results in a prolapsed of the upper cervical spine into the skull base, commonly associated to other bone and neural axis abnormalities, like Chiari I malformation and syringomyelia. In this paper, we review the concepts necessary to understand and treat BI. The most comprehensive and accepted classification system is the proposed by Goel, which divides patients with BI into two groups, as it follows: group A) patients with clear elements of instability; and group B) BI secondary to clivus hypoplasia. Treatment in group A includes craniocervical realignment and stabilization, most of the times using an isolated posterior approach, obviating an unnecessary and morbidity of the anterior decompression. In group B, foramen magnum decompression is the treatment of choice. Surgical techniques should be adequate according to patient’s anatomy and surgeon’s experience. Good surgical results can be obtained with the understanding of the main concepts and treatment options of BI. Keywords: basilar invagination, classification, diagnosis, surgical treatment. understand and treat BI based on its classification and the INTRODUCTION different surgical strategies adopted to treat this challenge and complex anomaly. Basilar invagination (BI) is a radiographic finding that consists in a development anomaly of the craniocervical junction in which the odontoid process is at least 2 mm above Chamberlain’s line (although up to 6.6 mm has been advocated as the normal MATERIAL AND METHODS limit), resulting in a prolapse of the upper cervical spine into the skull base6,10,24. Bone anomalies can cause or be associated A systematic review of the literature using the MedLine with BI. The most commonly findings are clivus hypoplasia, Database (National Library of Medicine), covering the period condyle hypoplasia, anomalies of the atlas formation and from 1980 to 2012, was performed. The search strategy atlanto-occipital assimilation24,25,26. In addition, about 30% of involved the keywords “basilar invagination treatment”. the patients with BI have also neural axis abnormalities, like Inclusion criteria included English language (or translated Chiari malformation, syringomyelia and hydrocephalus30. text), diagnosis of basilar invagination, discussion about management and surgical treatment of this entity. No age In this paper, we review the basic concepts necessary to 1Assistant Neurosurgeon – Neurosurgery Division, State University of Campinas (UNICAMP), Campinas-SP. Brazil and Centro Infantil Boldrini, Campinas-SP. Received, Apr 25, 2012. Accepted, Oct 08, 2013 Joaquim AF. - Management of Basilar Invagination J Bras Neurocirurg 24 (1): 53-59, 2013 54 Review restrictions were included. All case series, clinical studies and review articles were also included. In addition, a manual search of all referenced articles not found in the search was performed. 315 articles were initially found. Cross references of classic historical articles were also included. We excluded some articles based on repeated information for the purpose of our review. After all abstracts were screened, 31 articles were evaluated and included in our review. Basic craniocervical craniometry (figure 1) Some basic craniocervical craniometrical concepts are necessary to diagnosis, understand and treat BI. A brief review Figure 1: A – In B, we have the Basion (full arrow) and in O the opisthion (dash arrow). B – The Chamberlain Line, C- The McRae line defining the plane of the of the main craniometrical points is exposed, as it follows: foramen magnum and D- The Wackenheim Clivus Baseline or Basilar line (full line), that forms part of the clivus-canal angle if we add an additional line (dash line) that Basion – it refers to the anterior margin of the foramen passes along the posterior body of the axis. magnum24. Opisthion – it refers to the posterior margin of the foramen Craniocervical junction – anatomic anomalies of clinical magnum24. importance in the treatment of bi McRae line – defines the plan of the foramen magnum. It is Some anatomic development anomalies are associated with BI. formed by a line from the basion to the opisthion. The tip of the The most important are presented below: odontoid process should be fall below this line24. Atlantooccipital Assimilation - assimilation of the atlas is Platybasia – this term refers to a flattening of the skull base. caused by a failure of segmentation between the skull and It can be associated to BI but also may present in isolation23. the first cervical vertebra, completely or partially. Although The diagnosis of platybasia is made based on the measurement the Wackenheim clivus baseline may be normal, the clivus- of the Welcher Basal Angle24. This angle is defined by a line canal angle may be decreased. It’s commonly associated with extended from the nasium to the anterior tubercullum sellae fusion of the axis and third cervical vertebra. This association and other from this last to the basion. In normal subjects, it (atlantooccipital assimilation and axis-C3 fusion) is associated 26 should be less than 140o. Patients with increasing in this angle with atlantoaxial subluxation in about 50% of the cases . had the diagnosis of platybasia. Basilar impression – it refers to the acquired form of BI Wackenheim Clivus Baseline or Basilar line – this is a line secondary to softening of the bone and joints at the base of the along the clivus extending inferiorly into the upper cervical skull. The main etiologies include rheumatoid arthritis, Paget 24,25 spine canal. It should fall tangent to the posterior aspect disease, infection, tumors, among others . This term was of the tip of the odontoid process. The angle formed by the proposed by Virchow, in 1876, 19 years later of the introduction 27,28 intersection of the line constructed along the posterior surface of the term platybasia . of the axis body and the Wackenhein clivus baseline is called as Basiocciput (clivus) Hypoplasia – represents the hypoplasia of craniovertebral or clivus-canal angle. It normally ranges from the basiocciput that results in shortening of the clivus and BI 150o to 180o, changing about 30o with flexion and extension (violation of the Chamberlain line by the tip of the odontoid), positions24,26,29. Craniocervical kyphosis can be attributed when decreasing the clivus-canal angle and producing cranio-cervical the clivus-canal angle is less than 150o. kyphosis, resulting in deformity at the cervicomedullary junction26. Basion – it refers to the anterior margin of the foramen Joaquim AF. - Management of Basilar Invagination J Bras Neurocirurg 24 (1): 53-59, 2013 55 Review magnum24. Condylar Hypoplasia - The underdevelopment of the occipital CLASSIFICATION OF BI condyles, with a flattened appearance, leads to basilar Goel proposed a classification of BI based on the presence invagination, secondary to violation of the Chamberlain line or absence of clinical and radiological instability12. This and widening of the atlantooccipital joint axis angle. The tip classification is important once it can help surgeons in to of the odontoid process and the lateral masses of the atlas lie decide the best form of surgical treatment: below the McRae line. The lateral mass of the atlas may be fused to the hypoplastic condyles, accentuating the BI and Group A – This group main characteristic is the presence of limiting the movements of the atlantoccipital joint24. a clear instability of the region manifested by the tip of the odontoid process distancing itself from the anterior arch of the Chiari Type I Malformation – a caudal rhombencephalon atlas or the lower end of the clivus. A case example of BI of this abnormality that it is represented by a downward displacement group is presented in Figure 2 and 4. of the cerebellar tonsils and the medial portions of the inferior cerebellar lobes into the cervical spinal canal7,8. Symptoms Diagnostic criteria: are secondary to valve effect by the protruding tonsils at the - atlantoaxial dislocation (generally with C1-2 subluxation) foramen magnum or direct compression of nervous tissue. - tip of the odontoid process