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Basilar Invagination and Atlantoaxial Dislocation: Reduction, Deformity Correction and Realignment Using the DCER (Distraction, Compression, Extension, and Reduction) Technique With Customized Instrumentation and Implants
Objective: The technique of distraction, compression, extension, and reduction (DCER) is effective to reduce, realign, and relieve cranio-spinal compression through posterior only approach. Methods: Study included all patients with atlantoaxial dislocation and basilar invagination (BI) with occipitalized C1 arch. Study techniques included Nurick grading, computed tomography scan to study atlanto-dental interval, BI, hyper-lordosis, and neck tilt. Sagittal inclination (SI), coronal inclination (CI), cranio-cervical tilt, presence of pseudo-joints, and anomalous vertebral artery were also noted. Patients underwent DCER with/without joint remodeling or extra-articular distraction (EAD) based on the SI being <100°, 100°–160°, or >160° respectively. In cases with pseudo-joints, joint remodeling was performed in type I and EAD in type II. Customized ‘bullet shaped’ PSC spacers (n=124) and prototype of the universal craniovertebral junction reducer (UCVJR, n=36) were useful. Results: A total of 148 patients with average age 27.25±17.43 years, ranging from 3 to 71 years (87 males) were operated. Nurick’s grading improved from 3.14±1.872 to 1.22±1.17 (p<0.0001). Fifty-two percent of total joints (n=154/296 joints) were either type I (19%)/type II (33%) pseudo-j oints. All traditional indices such as Chamberlein line, McRae line, atlanto-dental interval, and Ranawat line improved (p<at least 0.001). BI, SI, and CI values correlated with type of pseudo-joints (p<0.0001). Side of neck tilt correlated with the type of pseudo-joint (p<0.0001). Cervical hyperlordosis improved significantly (p<0.0001). Conclusion: Occipito-C2 pseudo-joints are important in determining the severity of BI. Asymmetrical pseudo-joint causes coronal/neck tilt. Type of pseudo-joint can strategize by DCER. Customized instruments and implants make technique safe, effective and easier.
Objective: Developmental bony craniovertebral junction (CVJ) anomalies seem to have a genetic basis and also abnormal joint morphology causing atlantoaxial dislocation (AAD) and basilar invagination (BI). Methods: DNA extracted polymerase chain reaction single-stranded conformation polymorphism (SSCP) performed for mutation screening of FBN1 gene (n=50 cases+ 50 age/sex-matched normal; total: 100). Samples with a deviated pattern of bands in SSCP were sequenced to detect the type of variation. Computed tomography (CT) scans of 100 patients (15–45 years old) compared with an equal number of age/sex-matched controls (21.9±8.2 years). Joint parameters studied: sagittal joint inclination (SI), craniocervical tilt (CCT), coronal joint inclination (CI). Results: Thirty-nine samples (78%) showed sequence variants. Exon 25, 26, 27, and 28 showed variable patterns of DNA bands in SSCP, which on sequencing gives various types of DNA sequence variations in intronic region of the FBN1 gene in 14%, 14%, 6%, and 44% respectively. CT radiology:SI and CCT correlated with both BI and AAD (p<0.01). The mean SI value in controls: 83.35°±8.65°, and in patients with BI and AAD:129°±24.05°. Mean CCT in controls: 60.2°±9.2°, and in patients with BI and AAD: 86.0°±18.1°. Mean CI in controls:110.3°±4.23°, and in cases: 125.15°±16.4°. Conclusion: The study showed mutations in FBN1 gene (reported in Marfan syndrome). There is also an alteration of joint morphology, correlating with AAD and BI severity. Hence, we propose a double-hit hypothesis: the presence of weak ligaments (due to FB1 gene alterations) and abnormal joint morphology may contribute to AAD and BI.