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LETTER TO EDITOR
Year : 2012  |  Volume : 60  |  Issue : 2  |  Page : 262-264

Management of upper cervical kyphosis in an adolescent with Larsen's syndrome


Department of Neurosurgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission13-Mar-2012
Date of Decision01-Apr-2012
Date of Acceptance04-Apr-2012
Date of Web Publication19-May-2012

Correspondence Address:
Sandeep Mohindra
Department of Neurosurgery, Postgraduate Institute of Medical Education and Research, Chandigarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.96438

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How to cite this article:
Mohindra S, Savardekar A. Management of upper cervical kyphosis in an adolescent with Larsen's syndrome. Neurol India 2012;60:262-4

How to cite this URL:
Mohindra S, Savardekar A. Management of upper cervical kyphosis in an adolescent with Larsen's syndrome. Neurol India [serial online] 2012 [cited 2019 Aug 21];60:262-4. Available from: http://www.neurologyindia.com/text.asp?2012/60/2/262/96438


Sir,

After a trivial fall, a 17-year-old adolescent was bedridden and presented with spastic quadriparesis. Before the fall, he was spasticitic and abmulatory, but could not run or walk briskly. Neurologic examination revealed severe spasticity in all the four limbs with an indwelling bladder catheter. Patient was not abnormally tall, but had thin long, slender fingers [Figure 1]a and b. He had been previously treated for right elbow dislocation and cubitus valgus deformity. His countenance was classical of "facies scaphoidea" or "Dish faces". [1] Radiological examination revealed anterior bending of axis over C3 and severe kyphotic deformity of upper cervical spine [Figure 1]c-e. Magnetic resonance imaging (MRI) of spine demonstrated severe cord compression and cord kinking. The clinical diagnosis was Larsen's syndrome. Patient underwent bony ventral decompression via the trans-oral route. After partial soft-palate splitting, lower third of C2 body and complete C3 body was drilled away, making the dura lax and pulsatile. Surgical exposure was made comfortable using 20 pounds of traction, intra-operatively. Anterior decompression was followed by rigid bony union, using screws in occipital squama, left pedicle of axis, and both the pedicles of the fourth cervical vertebra. Rib graft was used as on-lay graft. Postoperatively, patient was mechanically ventilated for three weeks. He improved to ambulatory state at discharge. At 18 months of follow-up [Figure 2]a-d, patient has normal power in all four limbs, minimal spasticity and normal bladder control. Radiology showed rigid bony union between the occiput, axis and fourth cervical vertebra and adequately decompressed cervical cord [Figure 3]a and b.
Figure 1: (a) Clinical picture of dorsal surface of both the hands. Fingers are thin, long and slender; (b) Plain radiograph showing the slender bony architecture of fingers; (c) Plain radiograph (lateral view) of the upper cervical spine showing kyphosis, with C3 as the apical body; (d) Sagittal section of CT scan showing upper cervical kyphotic deformity; (e) MRI scan (sagittal section) showing kinking of upper cervical cord

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Figure 2: (a) Clinical picture of patient's face showing "Dish facies" (b,d) Reconstructed CT image after 18 months of surgery showing rigid immobilization accomplished via pedicle screw placement. Both sided pedicle screws could be placed in C4, while only left pedicle of C2 could be screwed, because of hemi-vertebra of C2 (c) Plain radiograph (lateral view) of skull and upper cervical spine showing rigid fusion at 18 months of follow-up

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Figure 3: (a-b) Sagittal section of MRI (T1 and T2) scans showing adequately decompressed cervical cord

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Only 11 cases of Larsen syndrome, managed surgically for severe cervical kyphosis have been reported in the literature. [2],[3] All cases required long-segment fusion involving either the middle cervical spine and/or lower cervical spine. The present case had upper cervical kyphotic deformity, not described in literature earlier. Thus, the decision for anterior decompression via extended trans-oral route was adopted. Release of tight cartilaginous tissue, ventral to the cervical vertebrae followed by median corpectomies, and ventral dural decompression provides the best surgical outcome for severe cervical kyphosis. For pediatric patients, differential vertical growth of the spinal column should be taken into account. Anterior spinal fusion involves significant risk of spinal cord damage during decompression maneuvers. [3] Extremely hypoplastic vertebrae make the surgical technique highly demanding. Conventional anterior approach for anterior decompression has been described, where the apical vertebral body posterior to the adjacent vertebrae was excised. This surgical approach/procedure is potentially dangerous, keeping in view the (pediatric) age of patients. Lateral approach to mid-cervical spine/cord has been described as a safer method for cord decompression, [3] where direct visualization of the association between the vertebrae and the spinal cord is possible. This approach would be useful for patients having severe kyphotic deformity, even when retraction of the carotid sheath and release of the vertebral artery carry considerable surgical risks. [3] Anterior/lateral approach for anterior decompression in severe kyphotic cervical spine seems not only difficult, but potentially dangerous when isolated upper cervical spine is involved [Figure 3]. Extended trans-oral route provides better orientation and visualization, especially when the surgeon is familiar with cranio-vertebral junction surgeries. Posterior arthrodesis with segmental spinal instrumentation or pedicle screws' placement after anterior spinal decompression provides a rational solution. [3] All patients with Larsen's syndrome should be screened for spinal anomalies/deformities and surgical correction offered at an early symptomatic stage. Further, isolated posterior spinal fusion may provide a rather simpler and a safe solution, if patients are detected with less degree of kyphosis/spinal deformities.

 
  References Top

1.Larsen LJ, Schottstaedt ER, Bost FC. Multiple congenital dislocations associated with characteristic facial abnormality. J Pediatr 1950;37:574-81.  Back to cited text no. 1
[PUBMED]    
2.Madera M, Crawford A, Mangano FT. Management of severe cervical kyphosis in a patient with Larsen syndrome. Case report. J Neurosurg Pediatr 2008;1:320-4.  Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
3.Sakaura H, Matsuoka T, Iwasaki M, Yonenobu K, Yoshikawa H. Surgical treatment of cervical kyphosis in Larsen syndrome: Report of 3 cases and review of the literature. Spine (Phila Pa 1976) 2007;32:e39-44.  Back to cited text no. 3
    


    Figures

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