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Cervical neurofibromatosis with quadriparesis: Management by fibular strut graft.

Laohacharoensombat W, Wajanavisit W, Woratanarat P - Indian J Orthop (2010)

Bottom Line: This is a case report of an eight-year old boy with neurofibromatosis and a 120 degrees dystrophic kyphosis of the cervical spine.He presented with progressive quadriparesis caused by spondyloptosis of the C2/C3, and was successfully treated by skull traction and one-stage anterior fibular strut graft lying between the tubercle of the atlas through the C2 body slot and lower vertebrae.At seven years follow-up there was, loosening of lower vertebral screws which allowed growth and residual mobility of lower vertebral joints while the fusion of upper cervical spines was still solid.

View Article: PubMed Central - PubMed

Affiliation: Department of Orthopaedics, Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand.

ABSTRACT
This is a case report of an eight-year old boy with neurofibromatosis and a 120 degrees dystrophic kyphosis of the cervical spine. He presented with progressive quadriparesis caused by spondyloptosis of the C2/C3, and was successfully treated by skull traction and one-stage anterior fibular strut graft lying between the tubercle of the atlas through the C2 body slot and lower vertebrae. At seven years follow-up there was, loosening of lower vertebral screws which allowed growth and residual mobility of lower vertebral joints while the fusion of upper cervical spines was still solid.

No MeSH data available.


Related in: MedlinePlus

After surgery, the cast was maintained for 4 months. The radiograph show the evidence of bony healing
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Figure 0004: After surgery, the cast was maintained for 4 months. The radiograph show the evidence of bony healing

Mentions: Neurological examination revealed weakness on all extremities (left side grade 2-3/5, right side grade 3-4/5) with obvious long tract signs. Radiological examination revealed spondyloloptosis at C2/C3 with kyphotic angle between C2/4 of 120° [Figure 1]. The left clavicle, humerus, and the first four ribs were found to be dysplastic [Figure 2]. Mild scoliosis of the thoracic spine was noted. The magnetic resonance scan of the C-spines showed a spondyloptotic-kyphotic deformity of the spine of more than 120° at C2-C4 vertebral levels. The cervical cord was stretched and compressed at the apex of the kyphosis [Figure 3]. Mild-to-moderate cord atrophy was noted. Dural ectasia of upper cervical spine was also detected. Skull traction with one-kilogram weight was applied on the second day of admission. The motor power of all extremities improved one grade on the third day. The kyphotic angle was improved to 90°. The patient underwent surgery on the 12th day of admission. The skull traction was maintained during intra- and post-operative period. The left anterior para-sternocleidomastoid incision was used, extending from the sub-mandibular to supra-clavicular region. After blunt dissection, the lower part of vertebral bodies, C2 to C7, were exposed. Discectomy of the C3/C4 disc facilitated the removal of C3 body. The position of the spine was re-adjusted to reduce kypho-scoliosis deformity under 5 SEP-monitoring. A small slot was created at the anterior port of C2, and C4 – C7 bodies. A 10-centimeter fibular graft was taken sub-periosteally from the middle-third of left leg. Its cephalous end was embedded in the C2 slot and was then hammered proximally to support tubercle of the atlas. The caudal end of the graft was subsequently pushed into the slot of lower vertebral bodies (C4-C7). Four lag screws were used to fix the distal end of fibular graft to the C4-C7 bodies respectively. Post-operatively, the neurological status of the patient was stable. The kyphotic angle between C2-C4 was reduced to 55°. The traction was securely maintained for one week. A Minerva cast was applied on the 10th day after surgery. The patient was discharged two weeks after surgery. The cast was maintained for four months and removed when bone healing was roengentnographically evident [Figure 4]. The neurological status gradually improved.


Cervical neurofibromatosis with quadriparesis: Management by fibular strut graft.

Laohacharoensombat W, Wajanavisit W, Woratanarat P - Indian J Orthop (2010)

After surgery, the cast was maintained for 4 months. The radiograph show the evidence of bony healing
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2822428&req=5

Figure 0004: After surgery, the cast was maintained for 4 months. The radiograph show the evidence of bony healing
Mentions: Neurological examination revealed weakness on all extremities (left side grade 2-3/5, right side grade 3-4/5) with obvious long tract signs. Radiological examination revealed spondyloloptosis at C2/C3 with kyphotic angle between C2/4 of 120° [Figure 1]. The left clavicle, humerus, and the first four ribs were found to be dysplastic [Figure 2]. Mild scoliosis of the thoracic spine was noted. The magnetic resonance scan of the C-spines showed a spondyloptotic-kyphotic deformity of the spine of more than 120° at C2-C4 vertebral levels. The cervical cord was stretched and compressed at the apex of the kyphosis [Figure 3]. Mild-to-moderate cord atrophy was noted. Dural ectasia of upper cervical spine was also detected. Skull traction with one-kilogram weight was applied on the second day of admission. The motor power of all extremities improved one grade on the third day. The kyphotic angle was improved to 90°. The patient underwent surgery on the 12th day of admission. The skull traction was maintained during intra- and post-operative period. The left anterior para-sternocleidomastoid incision was used, extending from the sub-mandibular to supra-clavicular region. After blunt dissection, the lower part of vertebral bodies, C2 to C7, were exposed. Discectomy of the C3/C4 disc facilitated the removal of C3 body. The position of the spine was re-adjusted to reduce kypho-scoliosis deformity under 5 SEP-monitoring. A small slot was created at the anterior port of C2, and C4 – C7 bodies. A 10-centimeter fibular graft was taken sub-periosteally from the middle-third of left leg. Its cephalous end was embedded in the C2 slot and was then hammered proximally to support tubercle of the atlas. The caudal end of the graft was subsequently pushed into the slot of lower vertebral bodies (C4-C7). Four lag screws were used to fix the distal end of fibular graft to the C4-C7 bodies respectively. Post-operatively, the neurological status of the patient was stable. The kyphotic angle between C2-C4 was reduced to 55°. The traction was securely maintained for one week. A Minerva cast was applied on the 10th day after surgery. The patient was discharged two weeks after surgery. The cast was maintained for four months and removed when bone healing was roengentnographically evident [Figure 4]. The neurological status gradually improved.

Bottom Line: This is a case report of an eight-year old boy with neurofibromatosis and a 120 degrees dystrophic kyphosis of the cervical spine.He presented with progressive quadriparesis caused by spondyloptosis of the C2/C3, and was successfully treated by skull traction and one-stage anterior fibular strut graft lying between the tubercle of the atlas through the C2 body slot and lower vertebrae.At seven years follow-up there was, loosening of lower vertebral screws which allowed growth and residual mobility of lower vertebral joints while the fusion of upper cervical spines was still solid.

View Article: PubMed Central - PubMed

Affiliation: Department of Orthopaedics, Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand.

ABSTRACT
This is a case report of an eight-year old boy with neurofibromatosis and a 120 degrees dystrophic kyphosis of the cervical spine. He presented with progressive quadriparesis caused by spondyloptosis of the C2/C3, and was successfully treated by skull traction and one-stage anterior fibular strut graft lying between the tubercle of the atlas through the C2 body slot and lower vertebrae. At seven years follow-up there was, loosening of lower vertebral screws which allowed growth and residual mobility of lower vertebral joints while the fusion of upper cervical spines was still solid.

No MeSH data available.


Related in: MedlinePlus