Cervical disc arthroplasty for Klippel-Feil syndrome.

OBJECTIVE: Klippel-Feil syndrome (KFS) is a congenital musculoskeletal condition characterized by improper segmentation of the cervical spine. This study aimed to evaluate outcomes of KFS patients who underwent cervical disc arthroplasty (CDA). METHODS: Consecutive patients who underwent anterior cervical surgery were retrospectively reviewed. Those patients with KFS who received discectomy adjacent to the congenitally fused vertebral segments were extracted and grouped into either the fusion or the CDA group. Clinical and radiological evaluations included visual analog scales, Neck Disability Index (NDI), Japanese Orthopedic Association (JOA) scores, C2-7 range of motion (ROM), C2-7 Cobb angle, C2-7 sagittal vertical axis (SVA), and T1-slope. RESULTS: Among 2320 patients, there were 41 with KFS (prevalence = 1.77%), who were younger than the entire cohort (53.3 vs 56.4 years). Thirty KFS patients had adjacent discs and were grouped into the CDA and fusion groups (14 vs 16). Type-I KFS with C3-4 involvement was the most common for both groups (92.8% vs 81.2% with 57% vs 50%, respectively). Post-operation, both groups demonstrated improvement of all the patient reported outcomes. The C2-7 ROM significantly decreased in the fusion group than that of pre-operation (12.8 ± 6° vs 28.1 ± 11.5°). In contrast, the CDA group successfully preserved C2-7 and segmental ROM without additional complications. CONCLUSIONS: KFS is rare (prevalence = 1.77%) among cervical spine surgery patients, and it rarely affects the overall cervical spinal alignment, except that it decreases segmental mobility. CDA is a feasible option for KFS because it not only avoids long-segment fusion but also preserves segmental and global mobility.

Cervical disc arthroplasty for less-mobile discs.

OBJECTIVE: The published clinical trials of cervical disc arthroplasty (CDA) have unanimously demonstrated the success of preservation of motion (average 7°-9°) at the index level for up to 10 years postoperatively. The inclusion criteria in these trials usually required patients to have evident mobility at the level to be treated (≥ 2° on lateral flexion-extension radiographs) prior to the surgery. Although the mean range of motion (ROM) remained similar after CDA, it was unclear in these trials if patients with less preoperative ROM would have different outcomes than patients with more ROM. METHODS: A series of consecutive patients who underwent CDA at the level of C5-6 were followed up and retrospectively reviewed. The indications for surgery were medically refractory cervical radiculopathy, myelopathy, or both, caused by cervical disc herniation or spondylosis. All patients were assigned to 1 of 2 groups: a less-mobile group, which consisted of those patients who had an ROM of ≤ 5° at C5-6 preoperatively, or a more-mobile group, which consisted of patients whose ROM at C5-6 was > 5° preoperatively. Clinical outcomes, including visual analog scale, Neck Disability Index, and Japanese Orthopaedic Association Scale scores, were evaluated at each time point. Radiological outcomes were also assessed. RESULTS: A total of 60 patients who had follow-up for more than 2 years were analyzed. There were 27 patients in the less-mobile group (mean preoperative ROM 3.0°) and 33 in the more-mobile group (mean ROM 11.7°). The 2 groups were similar in demographics, including age, sex, diabetes, and cigarette smoking. Both groups had significant improvements in clinical outcomes, with no significant differences between the 2 groups. However, the radiological evaluations demonstrated remarkable differences. The less-mobile group had a greater increase in ΔROM than the more-mobile group (ΔROM 5.5° vs 0.1°, p = 0.001), though the less-mobile group still had less segmental mobility (ROM 8.5° vs 11.7°, p = 0.04). The rates of complications were similar in both groups. CONCLUSIONS: Preoperative segmental mobility did not alter the clinical outcomes of CDA. The preoperatively less-mobile (ROM ≤ 5°) discs had similar clinical improvements and greater increase of segmental mobility (ΔROM), but remained less mobile, than the preoperatively more-mobile (ROM > 5°) discs at 2 years postoperatively.

Irradiation-Induced Secondary Tumors following Pediatric Central Nervous System Tumors: Experiences of a Single Institute in Taiwan (1975-2013).

PURPOSE: Complications can occur following a prolonged latency period after radiation therapy for cancer, and this is a growing concern because secondary tumors are potentially fatal. Few studies have examined secondary tumors in patients who received radiation therapy as children. METHODS AND MATERIALS: This retrospective study examined 1697 pediatric patients with central nervous system tumors who received treatment at Taipei Veterans General Hospital from January 1, 1975, to December 31, 2013. Secondary tumors developed in 27 of 681 patients who received cranial irradiation. Overall survival was estimated using the Kaplan-Meier method, and the significance of differences was determined by the log-rank test. RESULTS: The overall cumulative incidence of secondary tumors at 25 years was 3.96%, and there were similar numbers of male patients (n = 16) and female patients (n = 11). The mean age at diagnosis was 8.8 years (range, 3-16.5 years), the median dose of cranial irradiation was 52.5 Gy (mean, 53.4 Gy), the mean latency period was 14.6 years (range, 2-33 years), and the mean age at diagnosis of a secondary tumor was 23.1 years. The secondary tumors were mainly meningiomas (n = 13), sarcomas (n = 7), and high-grade gliomas (n = 6), and the mean latency periods were 19.66, 8.00, and 10.83 years, respectively. The overall survival rate from these secondary tumors was significantly different (P < .05). Age at irradiation of <7 years and craniospinal irradiation significantly increased the risk of a secondary tumor (P < .05). Secondary tumors developed in 11 of 128 patients (8.6%) with primary medulloblastomas, which was higher than the overall cumulative incidence. CONCLUSIONS: Clinicians should consider the increased risk of secondary tumors in long-term cancer survivors who received craniospinal irradiation as children. Using a selective dose de-escalation strategy or deferring radiation therapy for young patients at highest risk of secondary cancers should be studied.