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Treatment of Spinal Deformities in Neurofibromatosis Type 1 with Segmental Pedicle Screw System in One-stage Posterior Procedure

Author: WangZhenYu
Tutor: LiuYi
School: Jilin University
Course: Clinical
Keywords: Neurofibromatosis spinal deformities segmental pedicle screws system
CLC: R687.3
Type: Master's thesis
Year: 2009
Downloads: 27
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Abstract


Background In recent years, Hook-Rod System were most frequently used to correct scoliosis in NF-1. Meanwhile, the combining of anterior and posterior procedure, even multistage procedure is required. However, the literatures about treatment of spinal deformities in NF-1 with one stage posterior segmental pedicle screws system are rare. The treatment of spinal deformities in NF-1 with one stage posterior segmental pedicle screws system was performed in 15 patients of NF-1 by the chairman LiuYi MD. of the Spinal division of the first hospital of JiLin University. All of these patients were dystrophic. In this report, this 15 patients of neurofibromatosis-induced spinal deformities treated surgically with one stage posterior segmental pedicle screws system were reviewed. The minimum period of follow-up visiting is 4 months ranging from 4-64 months.Objective To value the curative effect of the treatment of scoliosis in NF-1 with one stage posterior segmental pedicle screws system.Method Fifteen patients with the diagnosis of socoliosis in NF-1 treated with one stage posterior segmental pedicle screw system were reviewed in this research. There were 8 male patients and 7 female patients, whose initial mean age at the time of operation was 12.9 years (range, 8 years -16 years). All of these patients were dystrophic scoliosis. There were 8 patients with right main thoracic curve, 5 patients with left main thoracic curve, and 2 patients with right main lumbar curve. The apex vertebrae were T5-T9 in 7 paients, T10-T11 in 6 patients, and L1-L3 in 2 patients. The main curves range included from 3 vertebraes to 6 vertebraes (mean 4.9 vertebrae). In this retrospective research, all patients had imaging features of scalloping vertebrae and vertebral wedging, 11 patients had penciling of the ribs, and 7 patients had widening distance between pedicles. The mean main curves cobb angle in coronal plane of preoperative patients is 79.5°ranging from 45°to 142°, and the corresponding value in sagittal plane is 53.9°ranging from 13°to 160°. 2 methods of surgical fusion segment were applied in this research. One kind of fusion segment was carried out in 9 patients from one level proximal to the upper end vertebra to one level distal to the lower end vertebra (EV+1), and the selection of the 6 other patients were fusion to the stable vertebrae (SV). All the patients were treated by one stage posterior segmental pedicle screw instrumentation and spinal fusion. If the flexibility rate less than 35%, including 7 cases, posterior vertebral column resection (PVCR) were assumed to performed, while actually, owing to greater actual flexibility than expecting in 3 case,and severe hemorrhage during operation in the other 1 case, PVCR were performed in 3 cases finally. If the flexibility rate more than 35%, including 8 patients, single posterior segmental pedicle screw instrumentation and fusion were performed.Results The average blood transfusion was 1163ml (range, 200ml-4100ml). The mean operation time 296 minutes (range, 170min–585min). They were followed for an average of 26.3months (range from 4 months to 64 months). 11 cases in this research were followed for more than 24 months. The initial average cobb angle of scoliosis was 79.5°(range from 45°-142°), whereas the corresponding value of kyphosis was 53.9°(range from 13°-160°). After surgery, these values were reduced to 26.3°(scoliosis ranging from 4°-58°) and 25.2°(kyphosis ranging from 5°-55°), and the scoliosis correction rates were 68.5% (ranging from 39.7%-92.5%). At the most recent follow-up the mean value of scoliosis was 27.3°(range from 4°to 62°), showing a mean correction of 67.6% (range from 33.3%-92.5%); while the corresponding value of kyphosis was 25.2°(range from 15°to 55°). During the follow-up, 2 cases show adding on without significant trunk imbalance in the group of fusion segment to one level beyond upper and lower end vertebra. There was no decompensation in fusion segment to stable vertebrae.Conclusion: 1. The spinal deformities of neurofibromatosis in dystrophic type may be treated with one stage posterior segmental pedicle screw instrumentation, and posterior vertebrae column resection is recommended if the flexibility is less than 35%. 2. Fusing to the stable vertebraes during surgery is recommended in those patients of dystrophic scoliosis in NF-1. 3. The perfect assessment of curative effect of the spinal deformity in NF-1 should contain the correction rate of the Cobb angle, the spinal balance, and the spinal aesthetic evaluation.

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CLC: > Medicine, health > Surgery > Orthopaedic Surgery ( movement system diseases,orthopedic surgery ) > Orthopedic surgery and surgery > Bone surgery
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