Neurological outcome following delayed traction and fixation in severe tetraparesis consecutive to posterior decompression for Chiari malformation: a case report.

BACKGROUND: Chiari malformation type 1 (CM1) is a congenital hindbrain malformation characterized by herniation of the cerebellar tonsils below the foramen magnum. The term Chiari type 1.5 is used when herniation of the brainstem under the McRae line and anomalies of the craniovertebral junction are also present. These conditions are associated with several symptoms and signs, including headache, neck pain, and spinal cord syndrome. For symptomatic patients, surgical decompression is recommended. When radiographic indicators of craniovertebral junction (CVJ) instability or symptoms related to ventral brainstem compression are present, CVJ fixation should also be considered. CASE DESCRIPTION: We report the case of a 13-year-old girl who presented with severe tetraparesis after posterior decompression for Chiari malformation type 1.5, followed 5 days later by partial C2 laminectomy. Several months after the initial surgery, she underwent two fixations, first without and then with intraoperative cervical traction, leading to significant neurological improvement. DISCUSSION AND CONCLUSION: This case report underscores the importance of meticulous radiological analysis before CM surgery. For CM 1.5 patients with basilar invagination, CVJ fixation is recommended, and C2 laminectomy should be avoided. In the event of significant clinical deterioration due to nonadherence to these guidelines, our findings highlight the importance of traction with increased extension before fixation, even years after initial destabilizing surgery.

Risk Factors of Postoperative Cerebrospinal Fluid Leak After Craniovertebral Junction Anomalies Surgery: A Case-Control Study.

OBJECTIVE: To identify potential risk factors for cerebrospinal fluid (CSF) leakage after craniovertebral junction (CVJ) anomaly surgery and to provide a reference for clinical practice. METHODS: Sixty-six patients who underwent elective CVJ anomaly surgery during a 6-year period (April 2013 to September 2019) were retrospectively included. Research data were collected from the patients' medical records and imaging systems. Patients were divided into CSF leak and no CSF leak groups. Univariate tests were performed to identify potential risk factors. For statistically significant variables in the univariate tests, a logistic regression test was used to identify independent risk factors for CSF leakage. RESULTS: The overall prevalence of CSF leakage was 13.64%. Univariate tests showed that a basion-dental interval (BDI) > 10 mm and occipitalized atlas had significant intergroup differences (p < 0.05). Multivariate analysis indicated that a BDI > 10 mm was an independent risk factor for CSF leakage, and patients with CVJ anomalies with a BDI > 10 mm were more likely to have postoperative CSF leaks (odds ratio, 14.67; 95% confidence interval, 1.48-30.88; p = 0.004). CONCLUSION: It is necessary to maintain vigilance during CVJ anomaly surgery in patients with a preoperative BDI > 10 mm to avoid postoperative CSF leaks.

Morphological analysis and morphometry of the occipital condyle and its relationship to the foramen magnum, jugular foramen, and hypoglossal canal: implications for craniovertebral junction surgery.

Anatomical knowledge of the occipital condyle (OC) and its relationships to surrounding structures is important for avoiding injury during craniovertebral junction (CVJ) surgeries. This study was conducted to evaluate the morphology and morphometry of OC and its relationship to foramen magnum, jugular foramen (JF), and hypoglossal canal (HC). Morphometric parameters including length, width, height, and distances from the OC to surrounding structures were measured. The oval-like condyle was the most common OC shape, representing for 33.0% of all samples. The mean length, width and height of OC were 21.3±2.4, 10.5±1.4, and 7.4±1.1 mm, respectively. Moreover, OC was classified into three types based on its length. The most common OC length in both sexes was moderate length or type II (62.5%). The mean distance between anterior tips and posterior tips of OC to basion, and opisthion were 11.5±1.4, 39.1±3.3, 25.2±2.2, and 27.4±2.7 mm, respectively. The location of intracranial orifice of HC was commonly found related to middle 1/3 of OC in 45.0%. JF was related to the anterior 2/3 of OC in 81.0%, the anterior 1/3 of OC in 12.5%, and the entire OC length in 6.5%. These morphological analysis and morphometric data should be taken into consideration before performing surgical operation to avoid CVJ instability and neurovascular structure injury.

Anterior cervical meningocele with craniovertebral junction instability - A case report and literature review.

Anterior meningocele involves herniation of meninges through an abnormal defect in the anterior vertebral column. The pathogenesis, natural history, and management strategy of anterior cervical meningocele (ACM) are uncertain. We report a case of ACM with high cervical instability in a case of neurofibromatosis 1. Unlike other reported cases, torticollis and instability due to ACM were the major concerns in this case. We aim to discuss the management strategy and surgical nuances of such cases.

Cerebral and occipito-atlanto-axial involvement in mucopolysaccharidosis patients: clinical, radiological, and neurosurgical features.

BACKGROUND: Neurosurgical features of mucopolysaccharidosis (MPS) patients mainly involve the presence of cranio-vertebral junction (CVJ) abnormalities and the development of communicating hydrocephalus. CVJ pathology is a critical aspect that severely influences the morbidity and mortality of MPS patients. Hydrocephalus is slowly progressing; it must be differentiated from cerebral atrophy, and rarely requires treatment. The aim of this paper was to review the literature concerning these conditions, highlighting their clinical, radiological, and surgical aspects to provide a practical point of view for clinicians. RESULTS: CVJ involvement may present with cervical pain, unsteady gait, frequent falls, and progressive impairment of autonomous ambulation, an acute tetraplegia even after minor trauma. Magnetic resonance imaging (MRI) of the cervical spine, including active dynamic flexion and extension scans, is the most powerful imaging technique for detecting spinal cord compression at the CVJ in MPS patients. The main radiological features include atlanto-axial subluxation, odontoid hypoplasia, periodontoid soft tissue masses, spinal canal narrowing, and spinal cord compression. Together with MRI, fine-cut computed tomography (CT) scans with coronal and sagittal three-dimensional reconstructions are important diagnostic tools in the preoperative workup thanks to the information gleaned about bone structure conformation and angles. Finally, angio-CT slices are equally useful in preoperative planning, defining vertebral artery position in relation to bony structures. Surgery of the CVJ is proposed both to treat cord compression with MRI signs of myelopathy or as a preventive treatment in patients at high risk of cord damage. Among different surgical options, we always suggest performing decompression and instrumented stabilization. Hydrocephalus may occasionally present clinically with intracranial hypertension symptoms such as headache, vomiting, and high sight impairment. Neurocognitive symptoms may be hidden by the constitutive cognitive impairment. MRI with a study of dynamic cerebrospinal fluid (CSF) flow is helpful to differentiate from ventriculomegaly, which does not require treatment. Ventriculo-peritoneal shunt placement is the gold standard to treat hydrocephalus, although endoscopic third ventriculostomy has recently shown good results in some patients. CONCLUSION: Early recognition of CVJ pathology and hydrocephalus is critical to avoid the development of severe complications. A multidisciplinary approach involving physicians, neuroradiologists, and neurosurgeons is needed to detect such conditions and to select patients eligible for surgery.

Craniovertebral junction instability as an extension of cocaine-induced midline destructive lesions: case report.

With the increasingly widespread illicit use of cocaine, a broad spectrum of clinical pathologies related to this form of drug abuse is emerging. The most frequently used method of administration of powdered cocaine is intranasal inhalation, or "snorting." Consequently, adverse effects of cocaine on the nasal tract are common. Habitual nasal insufflations of cocaine can cause mucosal lesions. If cocaine use becomes chronic and compulsive, progressive damage of the mucosa and perichondrium leads to ischemic necrosis of the septal cartilage and perforation of the nasal septum. Occasionally, cocaine-induced lesions cause extensive destruction of the osteocartilaginous structures of the nose, sinuses, and palate and can mimic other diseases such as tumors, infections, and immunological diseases. In the literature currently available, involvement of the craniovertebral junction in the cocaine-induced midline destructive lesions (CIMDLs) has never been reported. The present case concerns a 44-year-old man who presented with long-standing symptoms including nasal obstruction, epistaxis, dysphagia, nasal reflux, and severe neck pain. A diagnosis of CIMDL was made in light of the patient's history and the findings on physical and endoscopic examinations, imaging studies, and laboratory testing. Involvement of the craniovertebral junction in the destructive process was evident. For neurosurgical treatment, the authors considered the high grade of atlantoaxial instability, the poorly understood cocaine-induced lesions of the spine and their potential evolution overtime, as well as cocaine abusers' poor compliance. The patient underwent posterior craniovertebral fixation. Understanding, classifying, and treating cocaine-induced lesions involving the craniovertebral junction are a challenge.