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Craniovertebral Junction Realignment for the Treatment of Basilar Invagination with Syringomyelia: Preliminary Report of 12 Cases

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Craniovertebral Junction Realignment for the Treatment of Basilar Invagination with Syringomyelia: Preliminary Report of 12 Cases Neurol Med Chir (Tokyo) 45, 512¿518, 2005 Craniovertebral Junction Realignment for the Treatment of Basilar Invagination With Syringomyelia: Preliminary Report of 12 Cases Atul GOEL and Praveen SHARMA Department of Neurosurgery, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Parel, Mumbai, India Abstract Twelveselectedpatients,eightmalesandfourfemalesaged14to50years,withsyringomyeliaassoci- ated with congenital craniovertebral bony anomalies including basilar invagination and fixed atlan- toaxial dislocation, and associated Chiari I malformation in eight, were treated by atlantoaxial joint manipulation and restoration of the craniovertebral region alignment between October 2002 and March 2004. Three patients had a history of trauma prior to the onset of symptoms. Spastic quadriparesis and ataxia were the most prominent symptoms. The mean duration of symptoms was 11 months. The atlan- toaxial dislocation and basilar invagination were reduced by manual distraction of the facets of the atlas and axis, stabilization by placement of bone graft and metal spacers within the joint, and direct atlantoaxial fixation using an inter-articular plate and screw method technique. Following surgery all patients showed symptomatic improvement and restoration of craniovertebral alignment during follow up from 3 to 20 months (mean 7 months). Radiological improvement of the syrinx could not be evaluated as stainless steel metal plates, screws, and spacers were used for fixation. Manipulation of the atlantoaxial joints and restoring the anatomical craniovertebral alignments in selected cases of syringomyelia leads to remarkable and sustained clinical recovery, and is probably the optimum surgical treatment. Key words: atlantoaxial dislocation, basilar invagination, Chiari malformation, syringomyelia Introduction tiary response to reduction in the posterior cranial fossa volume resulting from the primary craniover- The complex of basilar invagination, Chiari I mal- tebral anomaly of basilar invagination and sec- formation, and syringomyelia is relatively common, ondary Chiari I malformation. Accordingly, whereas the association with fixed atlantoaxial posterior fossa bony decompression was recom- dislocationislesscommonbutnotrare.1,4,7,13,15–20) mended for this subgroup of patients. Such cases are generally treated by either anterior The present study included 12 patients with syrin- transoral or posterior foramen magnum bony gomyelia associated with bony abnormalities of the decompression. The indications for opening of the craniovertebral region including `fixed' atlantoaxial dura and manipulation of the arachnoid membrane, dislocation and basilar invagination, and with tonsils, and obex, and draining of the syrinx cavity Chiari I malformation in eight patients. All patients are currently under discussion. No specific fixation were treated by attempted reduction of the atlan- procedure has been recommended for fixed atlan- toaxial dislocation and basilar invagination, and by toaxial dislocation associated with basilar invagina- direct lateral mass plate and screw atlantoaxial tion in the presence of syringomyelia.6) fixation by our reported techniques.5,9,11) No bony or We previously classified cases of syringomyelia dural decompression or neural manipulation of any into three groups and suggested specific treatment kind was performed. protocols on the basis of the possible pathogenetic factors.7,8) We suggested that syringomyelia is a ter- Patients and Methods Received September 17, 2004; Accepted March 31, Twelve patients with syringomyelia associated with 2005 `fixed' atlantoaxial dislocation and basilar invagina- 512 Craniovertebral Realignment for Syringomyelia 513 Table 1 Principal presenting clinical features Number of patients Clinical features Preoperative Postoperative Neck pain 9 recovered in all 9 Torticollis 3 near complete correction of torticollis in all 3 Lower cranial nerve affection 1 improved Weakness all 12 patients improved and were able to walk unaided able to walk unaided 10 needed support to walk 2 Sensations all patients improved; residual affection of kinesthetic normal sensations 3 sensations in 3 patients, residual partial affection of kinesthetic sensations affected 9 spinothalamic sensations in 4 spinothalamic sensations affected 6 Sphincter disturbance 2 recovered Table 2 Preoperative radiological measurements and changes following surgery CL (mm) WL (mm) ADI (mm) Omega angle (degrees) Case Age/ Chiari No. Sex Pre Post Dt Pre Post Dt Pre Post Dt Pre Post Alt 122/M15123 6061376708010＋ 2 23/M 8 4 4 5 2 3 18 10 8 40 60 20 － 347/M844 5051257507020＋ 4 48/M 13 4 9 8 0 8 27 6 21 60 85 25 ＋ 5 14/M 9 6 3 15 9 6 20 11 9 60 80 20 － 630/M853 4041394458035－ 7 37/M 10 4 6 8 0 8 13 2 11 75 90 25 ＋ 8 50/F 14 8 6 11 0 11 18 6 12 75 80 5 － 9 35/F 8 3 5 9 2 7 18 5 13 70 80 10 ＋ 10 24/F 12 7 5 10 0 10 13 5 8 75 84 9 ＋ 11 26/M 18 7 11 7 2 5 13 6 7 65 76 11 ＋ 12 32/F 14 8 6 12 2 10 14 6 8 65 80 15 ＋ Values show the distance between the tip of the odontoid process and the anatomical references. Chamberlain's line (CL): This line connects the posterior border of the hard palate to the opisthion. The tip of the odontoid process normally lies below this line.3,7) Wackenheim's line (WL): This line is the clivus baseline extended downwards. Normally, the tip of the odontoid process remains anterior to this line.7,21) Modified omega angle: A line is drawn traversing along the center of base of the axis parallel to the line of the hard palate. The inclination of the tip of the odontoid process to this line is defined as the modified omega angle.7,14) ADI: atlantodental interval, Alt: alteration, Dt: distraction, Pre: preoperative, Post: postoperative. tion were treated between October 2002 and March years (mean 11 months). 2004. Eight patients also had Chiari I malformation. All patients were investigated with magnetic Atlantoaxial dislocation was defined as `fixed' if the resonance imaging, computed tomography, and minimum distance between the tip of the odontoid dynamic radiography. Measurements of structures process and the posterior surface of the anterior based on the radiological studies are shown in arch of the atlas or clivus (in patients with assimilat- Table 2. Measurements were made on all available ed atlas) was more than 4 mm and there was no clear studies and were averaged. Partial or complete radiographic evidence of any reduction of the sub- occipitalization of the atlas was present in eight luxation with extension of the neck. patientsandfusionofC-2andC-3wasobservedin The clinical features of the patients are presented five. Os odontoideum was seen in three patients. in Table 1. Trauma of varying severity was the The surgical techniques employed were described principal precipitating factor in three patients. The previously.5,9–11) Cervical traction is progressively duration of symptoms ranged from 15 days to 5 increased to approximately one-fifth of the total Neurol Med Chir (Tokyo) 45,October,2005 514 A. Goel et al. Fig. 1 Case 4. A: Preoperative radiograph of the craniovertebral region with the neck in flexion showing occipitalization of the atlas, fusion of the C-2 and C-3 vertebrae, atlantoaxial dislocation, and basilar invagination. B: Preoperative radiograph of the craniovertebral region with the neck in extension showing persistent atlantoaxial dislocation. C: Preopera- tive T1-weighted magnetic resonance image showing basilar invagination, fixed atlantoaxial dislocation, Chiari I malformation, and syringomyelia. D: Preoperative computed tomography (CT) scan showing basilar invagination, fixed atlantoaxial dislocation, occipitalization of the atlas, and fusion of the C-2 and C-3 vertebrae. E: Postoperative radiograph with the neck in flexion showing the fixation with plates and screws, and the spacers within the joint cavity. F: Postoperative CT scan showing reduction of the basilar invagination and atlantoaxial dislocation. body weight prior to induction of anesthesia. The joint space. The average spacer measures 10 mm patient is placed prone with the head end of the table in length, 8 mm in breadth, and 4 mm in height. elevated to about 35 degrees. Use of the operating Titanium spacers have multiple small holes to per- microscope facilitates the dissection and screw mit bone fusion and are tapered at one end for easier implantation. The bilateral atlantoaxial facet joints placement in the joint cavity. Multiple bone graft are widely exposed after sectioning of the large C-2 chips are used to fill the distracted joint space ganglion. Exposure of the facet of the atlas was around the spacer. significantly difficult in the present group of Plate and screw fixation of the region is then car- patients because of assimilation of the atlas in most ried out by the interarticular technique.9,11) Atwo- cases and the rostral location of the basilar invagina- holed stainless steel plate measuring 15–20 mm in tion and the atlantoaxial joint. The joint capsule is length and screws of 2.4–2.6 mm diameter and excised and the articular cartilage widely removed 16–22 mm length are used. The screws are passed using the microdrill. The bilateral joints are distract- bilaterally through the holes in the plate into the ed using the intervertebral spreader used in anterior lateral mass of the atlas and axis (Figs. 1 and 2). The cervical disc surgery. The status of the dislocation site and angle of screw implantation is modified to and basilar invagination is evaluated by intraopera- suit the local anatomical situation. The preferred tive radiography. site of screw insertion is at the center of the The distraction of the C-1 and C-2 facets is posterior surface of the lateral mass of the atlas, 1 to maintained by titanium spacers, which are inserted 2 mm above the articular surface, but if there was into the joints (Figs. 1 and 2). The size of the spacers difficulty in exposure due to inadequate space, depends on the space available within the distracted screw insertion is done through the articular sur- Neurol Med Chir (Tokyo) 45, October, 2005 Craniovertebral Realignment for Syringomyelia 515 Fig.
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