Surgical decompression for lumbar stenosis in pediatric achondroplasia.

BACKGROUND: Spinal stenosis is a common complication of achondroplasia. To our knowledge, no study has evaluated a greater than 2-year outcome after surgical intervention for spinal stenosis in such children or compared decompression with and without instrumentation in relation to revision surgery. Our purpose was to assess the efficacy of lumbar decompression and instrumentation for symptomatic stenosis in children with achondroplasia. METHODS: We retrospectively reviewed our institution's database to identify children (< or =18 y old) with achondroplasia undergoing initial spinal decompression for lumbar stenosis from 1995 through 2003. We identified 18 such patients and reviewed their medical records for demographic data, presenting signs and symptoms, and treatment and outcome data. Mean follow-up was 72.0+/-27.6 months. We determined each patient's symptom score (SS) based on presence of leg weakness, numbness, or pain; abnormal reflexes; incontinence; and walking intolerance (unable to walk > or =5 blocks). Each finding was scored 1 point (6 points maximum). Nine patients requiring revision surgery were assigned a revision postoperative SS. All patients were contacted at the end of data collection and assigned a final follow-up SS. Baseline SS values were compared with postoperative, revision postoperative, and final follow-up scores using a paired t test (alpha=0.05). RESULTS: The mean preoperative and final SS values were significantly different: 4.0+/-0.9 (most common symptoms, leg weakness and incontinence) and 1.6+/-1.7 (most common symptom, leg weakness), respectively. Nine patients underwent decompression with instrumentation initially; 9 did not; 7 of the latter required instrumentation during revision; and 2 of the former also required revision. Those without initial instrumentation were 3.5 times more likely (odds ratio=12.3) to require revision. CONCLUSIONS: Surgical decompression with instrumentation significantly reduced the symptoms of lumbar stenosis and the likelihood of revision surgery in children with achondroplasia. LEVEL OF EVIDENCE: Level III therapeutic study.

Laminectomy in patients with achondroplasia: the impact of time to surgery on long-term function.

STUDY DESIGN: Retrospective analysis, survey. OBJECTIVE: To describe a cohort of individuals with achondroplasia undergoing thoracolumbar laminectomy and to examine if shorter time to surgery was related to improvement in long-term functional outcome. SUMMARY OF BACKGROUND DATA: Data on the long-term benefits of laminectomy are mixed for such patients. Earlier intervention may be associated with greater likelihood of long-term benefit, but quantified data are lacking. METHODS: We retrospectively studied 49 patients with achondroplasia who underwent primary laminectomy for spinal stenosis. Patients completed a questionnaire to assess symptoms, walking distance, and independence (per Modified Rankin Scale), before surgery and currently. Responses were analyzed for the likelihood of improved walking distance or Rankin level. RESULTS: Our patients had the following mean values: age, 37.7 ± 10.6 years; body mass index, 31.8 ± 5.5; symptom duration, 74.0 ± 100.1 months; preoperative symptom severity score, 2.7 ± 1.0 points; mean changes in blocks walked, +0.39 ± 2.0; and Rankin level, +0.08 ± 1.47. Patients with a time-to-surgery interval of <6 months were 7.13 times (95% confidence interval [CI], 1.39-36.66) more likely to experience improvement in walking distance and 4.00 times (95% CI, 1.05-15.21) more likely to experience Rankin level improvement than patients whose interval was >6 months. Intervals of up to 12 and 24 months were associated with increased likelihoods of 4.95 (95% CI, 1.41-17.41) and 3.43 (95% CI, 1.05-11.22), respectively, of improved walking distance compared with those with longer time-to surgery intervals, but those Rankin level improvements were not statistically significant. CONCLUSION: Time from symptom onset to surgery in patients with achondroplasia is an important predictor of long-term functional outcome. For sustained long-term postsurgical improvement, the window of opportunity might be relatively narrow. Patients with achondroplasia should seek medical advice for spinal stenotic symptoms as soon as possible.

Progression of low back and lower extremity pain in a cohort of patients with achondroplasia.

OBJECT: The aim of this study was to assess the natural history of pain associated with spinal stenosis in individuals with achondroplasia and to characterize pain patterns and associated functional and psychological effects. METHODS: The authors measured pain severity, spatial distribution of pain, functional disability, psychological distress, physical symptoms other than pain, and healthcare utilization in 181 individuals with achondroplasia. They also assessed low back and/or lower extremity pain at the initial visit and 1-year follow-up via self-rated patient questionnaires, calculated composite scores from responses via component analyses, and used repeated measures linear regression analyses for score changes (significance, p < or = 0.05). RESULTS: At the follow-up, back pain severity was unchanged. Patients reported significant progression of pain toward involvement of the lower extremities and significant increases in lower extremity pain severity overall. There were also significant increases in healthcare utilization overall. Compared with patients with back pain only, those with back pain and proximal or distal leg pain had higher self-rated pain severity; higher functional disability; and more bowel and bladder dysfunction symptoms, sleep disturbances, extremity numbness, and psychological distress. CONCLUSIONS: Individuals with achondroplasia and symptomatic spinal stenosis often experience back pain, which may progress to lower extremity pain and debilitating consequences. A more thorough understanding of the progression of spatial pain characteristics and pain severity may aid clinical decision making regarding the optimal timing for intervention.