摘要 Objective: To describe the different pathological characteristics of congenital basilar invaginations and discuss the surgical treatment of such cases. Methods: A total of 139 patients diagnosed with basilar invaginations underwent surgical treatment from 2008 to 2015. Based on Atul Goel's classification and simultaneous consideration of atlantoaxial dislocation or syringomyelia, the cases were subdivided into four groups. Individualized posterior surgical decompression and/or atlantoaxial reset procedures were performed to correct atlantoaxial dislocation, decompress the brain stem, or resolve syringomyelia. The indications and critical points of each procedure were documented. Results: All 139 patients were surgically treated; 27 patients (19.4%) had underwent at least one decompression surgery previously. On an average, there was gratifying clinical improvement based on the Japanese Orthopaedic Association score analysis. One patient exhibited severe post-operative infection, and the fusion instrument was removed. One patient experienced fracture of internal fixation. Two patients exhibited persistent respiratory symptoms at early stages after the surgery. Four patients felt worse at the latest follow-up. There was no surgical mortality. The poor outcome/ morbidity in this series was 5.8% (8/139). Conclusion: The different pathological image characteristics of congenital basilar invaginations based on the presence or absence of syringomyelia and/or atlantoaxial dislocation, reflected the pathological features of complicated basilar invaginations more accurately. Based on these features, different posterior decompression and/or reset procedures, combined with occipitocervical fusion and C1-2 fusion, could be tailored to different patients. These individualized surgeries could reduce surgical complications, decrease morbidity and mortality, and further promote positive outcomes.
Abstract: Objective: To describe the different pathological characteristics of congenital basilar invaginations and discuss the surgical treatment of such cases. Methods: A total of 139 patients diagnosed with basilar invaginations underwent surgical treatment from 2008 to 2015. Based on Atul Goel's classification and simultaneous consideration of atlantoaxial dislocation or syringomyelia, the cases were subdivided into four groups. Individualized posterior surgical decompression and/or atlantoaxial reset procedures were performed to correct atlantoaxial dislocation, decompress the brain stem, or resolve syringomyelia. The indications and critical points of each procedure were documented. Results: All 139 patients were surgically treated; 27 patients (19.4%) had underwent at least one decompression surgery previously. On an average, there was gratifying clinical improvement based on the Japanese Orthopaedic Association score analysis. One patient exhibited severe post-operative infection, and the fusion instrument was removed. One patient experienced fracture of internal fixation. Two patients exhibited persistent respiratory symptoms at early stages after the surgery. Four patients felt worse at the latest follow-up. There was no surgical mortality. The poor outcome/ morbidity in this series was 5.8% (8/139). Conclusion: The different pathological image characteristics of congenital basilar invaginations based on the presence or absence of syringomyelia and/or atlantoaxial dislocation, reflected the pathological features of complicated basilar invaginations more accurately. Based on these features, different posterior decompression and/or reset procedures, combined with occipitocervical fusion and C1-2 fusion, could be tailored to different patients. These individualized surgeries could reduce surgical complications, decrease morbidity and mortality, and further promote positive outcomes.
20180119184052 Figure 1 Different pathological image characteristics of congenital basilar invagination: (a) Type Ia which is BI with ADI increase and without syringomelia; (b) Type Ib which is BI with ADI increase with syringomelia; (c) Type IIa which is BI without ADI increase and without syringomelia; (d) Type IIb which is BI without ADI increase but with syringomelia.
20180119184140 Figure 2 36 yrs male, presented with left finger numbness and left leg weakness for 2 months. The image characteristics of this patient is Type Ia (BI with ADI increase and without syringomelia). (a) Sagittal T2 sequence magnetic resonance imaging showing brain stem compression without syringomyelia. (b) CT scan showing odontoid process position moved upward. (c and e) Postoperattory MRI and CT showing atlantoaxial dislocation reliefed. (d) CT scan 3D reconstruction of occipito-C2 fusion. JOA score increased from preoperative value of 14 to 16 at the latest follow-up time point.
20180119184204 Figure 3 26 yrs female, presented with neck pain and limb numbness for six years. Her symptom aggravated for 3 months. The image characteristics of this patient is Type Ib (BI with ADI increase and with syringomelia). (a) Sagittal T2 sequence magnetic resonance imaging showing tonsillar herniation with syringomyelia. (b) CT scan showing odontoid process position moved upward. (c) Intraoperative view of subarachnoid manuplation. (d) Intraoperative view of final construction. (e and f) Postoperattory MRI and CT showing atlantoaxial dislocation reliefed and syringomyelia significantly diminish. JOA score increased from preoperative value of 11 to 14 at the latest follow-up time point.
20180119184230 Figure 4 15 yrs, female, clinical symptom includes walking unstable, intermittent headache. Physical exam reveals cerebellar ataxia. The image characteristics of this patient is Type IIa (BI without ADI increase and without syringomelia). (a) Cervical CT scan before surgery showing basilar invagination, normal atlantodental interval, without syringomyelia. (b) Sagittal T2 sequence magnetic resonance imaging showing odontoid compression over the brainstem and upper spinal cord. (c) MRI scan after surgery. (d) CT scan after surgery. 1 years after the surgery, the JOA score was 16. JOA score increased from preoperative value of 15 to 16 at the latest follow up time point.
20180119184300 Figure 5 26 yrs, male. The patient underwent suboccipital decompression surgery 2 yrs ago. And one year after his surgery, he felt progression of right hand numbness, and intermittent right upper limb pain. The image characteristics of this patient is Type IIb (BI without ADI increase but with syringomelia). (a) CT scan 3D reconstruction showing the range of resection. (b) CT scan showing basilar invagination, normal atlantodental interval. (c) MRI imaging showing tonsillar hemiation with syringomyelia. (d) 10 days after the surgery, MRI showing the syringomyelia significantly diminish. (e and f) Postoperative sagittal CT scan showing a C1-C2 fusion. JOA score increased from preoperative value of 13 to 15 at the latest follow up time point.
20180119184310 Table 2 Clinical data and surgical management. POC: Pure occipital craniotomy; SMD: Subarachnoid manuplation and decompression.
20180119184318 Table 3 The outcome of patients evaluated by JOA scoring system. Improved: most of the clinical symptoms improved. Unchanged: some clinical symptoms still exist. Worse: symtoms increased.
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