Growth-Friendly Spine Surgery in Arthrogryposis Multiplex Congenita

Abstract

Background: Arthrogryposis multiplex congenita (AMC) is a condition that describes neonates born with ≥2 distinct congenital contractures. Despite spinal deformity in 3% to 69% of patients, inadequate data exist on growth-friendly instrumentation (GFI) in AMC. Our study objectives were to describe current GFI trends in children with AMC and early-onset scoliosis (EOS) and to compare long-term outcomes with a matched idiopathic EOS (IEOS) cohort to determine whether spinal rigidity or extremity contractures influenced outcomes. Methods: Children with AMC and spinal deformity of ≥30° who were treated with GFI for ≥24 months were identified from a multicenter EOS database (1993 to 2017). Propensity scoring matched 35 patients with AMC to 112 patients with IEOS with regard to age, sex, construct, and curve. Multivariable linear mixed modeling compared changes in spinal deformity and the 24-item Early Onset Scoliosis Questionnaire (EOSQ-24) across cohorts. Cohort complications and reoperations were analyzed using multivariable Poisson regression. Results: Preoperatively, groups did not differ with regard to age (p = 0.87), sex (p = 0.96), construct (p = 0.62), rate of nonoperative treatment (p = 0.54), and major coronal curve magnitude (p = 0.96). After the index GFI, patients with AMC had reduced percentage of coronal correction (35% compared with 44%; p = 0.01), larger residual coronal curves (49° compared with 42°; p = 0.03), and comparable percentage of kyphosis correction (17% compared with 21%; p = 0.52). In GFI graduates (n = 81), final coronal curve magnitude (55° compared with 43°; p = 0.22) and final sagittal curve magnitude (47° compared with 47°; p = 0.45) were not significantly different at the latest follow-up after definitive surgery. The patients with AMC had reduced T1-S1 length (p < 0.001), comparable T1-S1 growth velocity (0.66 compared with 0.85 mm/month; p = 0.05), and poorer EOSQ-24 scores at the time of the latest follow-up (64 compared with 83 points; p < 0.001). After adjusting for ambulatory status and GFI duration, patients with AMC developed 51% more complications (incidence rate ratio, 1.51 [95% confidence interval (CI), 1.11 to 2.04]; p = 0.009) and 0.2 more complications/year (95% CI, 0.02 to 0.33 more; p = 0.03) compared with patients with IEOS. Conclusions: Patients with AMC and EOS experienced less initial deformity correction after the index surgical procedure, but final GFI curve magnitudes and total T1-S1 growth during active treatment were statistically and clinically comparable with IEOS. Nonambulatory patients with AMC with longer GFI treatment durations developed the most complications. Multidisciplinary perioperative management is necessary to optimize GFI and to improve quality of life in this complex population. Level of Evidence: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

Perioperative Complications of Anterior Decompression with Fusion in Degenerative Cervical Myelopathy—A Comparative Study between Ossification of Posterior Longitudinal Ligament and Cervical Spondylotic Myelopathy Using a Nationwide Inpatient Database

“…Propensity scores were calculated using logistic regression analysis with the known preoperative items as explanatory variables [ 18 , 19 , 20 ]. Explanatory variables for logistic regression included age, sex, BMI, smoking, admission type, emergency, hospital type, ADL score, and preoperative comorbidities.…”

The Orthopaedic Management of Arthrogryposis Multiplex Congenita

“…In the older child, nonfusion spinal instrumentation with expandable implants is effective but limited to about 5 years of treatment time due to spine stiffness and autofusion limits. 51,54,55 Therefore, delaying surgery to after 6 years of age can allow for more overall growth.…”

Spinal Fusion in Patients With Classic Amyoplasia and General Arthrogryposis

“…Scoliotic curves in AMC are generally stiff, progressive, resistant to bracing, and frequently require surgery. 3,[5][6][7][8][9][10][11] Surgical results have noted modest curve correction and frequent complications. 3,[8][9][10]12,13 There are multiple classification schemes for arthrogryposis.…”

“…Although these conditions mostly affect the limbs, spine deformity has been reported to occur in 20% to 69% 3–7 of patients. Scoliotic curves in AMC are generally stiff, progressive, resistant to bracing, and frequently require surgery 3,5–11 . Surgical results have noted modest curve correction and frequent complications 3,8–10,12,13 …”