Clinical study Management of cervicomedullary compression in patients with congenital and acquired osseous–ligamentous pathologies Kadir Kotil a, * , Murat Kalayci b , Turgay Bilge a a Haseki Educational and Research Hospital, Department of Neurosurgery, Istanbul, Turkey b University of Karaelmas, Department of Neurosurgery, Zonguldak, Istanbul, Turkey Received 19 November 2005; accepted 7 March 2006 Abstract We present our experience in the diagnosis, surgical management and long-term follow-up of congenital and acquired osseous–liga- mentous abnormalities or pathologies of the craniovertebral junction. The purpose of this study was: (i) to determine the incidence and degree of cervicomedullary compression in pediatric and young adult patients with congenital and acquired abnormalities, and (ii) to correlate cervicomedullary compression with other imaging and clinical factors to determine to what extend cervicomedullary compres- sion is successfully treated with a posterior decompressive procedure, transoral decompression, and medical management. Between Jan- uary 1995 and December 2004, 26 cases were managed in our department. These patients had: rheumatoid arthritis (RA) (3); traumatic injury (2); congenital basilar impression (5, in 2 cases a posteriorly oriented or retroflexed odontoid); infection (10); craniovertebral junc- tion Pott’s disease (9); os odonteideum (3); condylus tertius (1); and tumor (2). Six of the patients (23.1%) had syringomyelia. Only three (11.3%) were in the pediatric age group. Symptoms and signs included headache (72%), ataxia (38%), lower cranial nerve dysfunction (54%), quadriparesis (44%), hyperreflexia (76%), Hoffman positivity (72%), achilles clonus (72%) nystagmus (33%) and dysphagia (22%). The mean follow-up time was 44 months (range 3–85). Twelve (46.2%) had undergone posterior fossa decompression; seven (26.6%) had ventral decompression. Seven of the patients (26.6%) had medical management. The major morbidity included pharyngeal wound sepsis leading to dehiscence (3.8%), valopharyngeal insufficiency (3.8%), cerebrospinal fluid leakage (3.8%), postoperative mac- roglossia (3.8%) and inadequate anterior decompression (3.8%). Transient neurological deterioration occurred in two patients (7.6%). Our management paradigm will result in some neurologic improvements and limit the progression of symptoms. Patients with these pathologies are likely to show a good neurologic outcome when treatment, whether with or without surgery, is administered early in the course of the disease. Ó 2006 Elsevier Ltd. All rights reserved. Keywords: Acquired osseous–ligamentous pathologies; Cervicomedullary compression; Congenital; Craniovertebral junction 1. Introduction The craniovertebral junction (CVJ) is a complex ana- tomical structure which is composed of the distal third of the clivus, occipital bone, atlas, axis and foramen magnum, and includes important neurovascular structures. 1–17 Con- genital or acquired inflammatory or traumatic pathologies and tumors of this region may cause serious neurological complications, including epidural ventral brain stem com- pression (or foramen magnum syndrome). It is necessary to undertake surgical decompression and fusion with a suitable surgical technique for patients with unstable ven- tral CVJ pathology, spinal cord compression and progres- sive clinical symptoms and which cannot be reduced with close reduction. 1,3,5,8–13,18–28 Patients suitable for closed reduction can be treated with external fixation without decompression. Optimal treatment of lesions with ventral epidural compression is transoral decompression. If the 0967-5868/$ - see front matter Ó 2006 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2006.03.005 * Corresponding author. Present address: Bag ˘dat cad. Hasan Ali yucel sok, senil apt. 34–14 ciftehavuzlar–istanbul 34728, Turkey. Tel.: +90 212 530 60 38; fax:+90 212 529 44 60. E-mail addresses: kadirkotil@superonline.com, kadirkotil@gmail.com (K. Kotil). www.elsevier.com/locate/jocn Journal of Clinical Neuroscience 14 (2007) 540–549