Background: Thoracolumbar kyphosis (TLK) is common in infants with achondroplasia. Our goals were to examine the natural history of TLK and identify factors associated with persistent TLK. Methods: We reviewed records of patients with achondroplasia seen by a board-certified orthopaedic surgeon at a tertiary care medical center between 1997 and 2013. Inclusion criteria were minimum 2-year follow-up and radiographs taken at time of presentation, within 6 months of walking age, and within 6 months of the first anniversary of walking age. We defined TLK as kyphosis of ≥20 degrees centered at T12 and L1. We assessed patient demographic characteristics, radiographic parameters (Cobb angle, apical vertebral translation, and apical vertebral wedging for vertebral height and width), and clinical parameters (developmental motor delay, hydrocephalus, presence of a ventriculoperitoneal shunt, and foramen magnum decompression). Developmental motor delay was defined as the inability to sit or ambulate independently by age 14 or 30 months, respectively. Associations between these factors and persistent TLK (ie, unresolved at final follow-up) were evaluated using logistic regression and χ2, Fisher exact, and independent t tests. Significance was set at P<0.05. Results: A total of 60 patients were included. Mean values were as follows: age at presentation, 10.9±7.0 months; length of follow-up, 5.7±3.6 years; initial curve, 43.8±11.0 degrees; independent sitting age, 12.6±5.5 months; and independent walking age, 21.1±7.8 months. At walking age and 1 year after walking age, 15% and 58% of patients, respectively, had spontaneous TLK resolution. In total, 30% of patients had persistent TLK at final follow-up. Apical vertebral translation (P=0.001), percentage of apical vertebral wedging for vertebral height (P=0.031), and developmental motor delay (P=0.043) were associated with unresolved TLK. Conclusions: In patients with achondroplasia, TLK resolved at walking age in 15% of patients and after a year of walking in 58% of patients. Earlier bracing may slow TLK progression in patients with achondroplasia and developmental motor delay. Patients with kyphotic curves between 20 and 40 degrees should be examined intermittently for progressive deformity or worsening symptoms of spinal cord compression. Level of Evidence: Level II.

Background Guidelines suggest referral for scoliosis when rib slope (scoliometer measurement, angle of trunk rotation [ATR]) is ≥7 degrees. We hypothesized that overweight and obese patients would have lower scoliometer measurements compared with normal-weight and underweight patients for a given spinal curvature, causing overweight and obese patients with adolescent idiopathic scoliosis to present for treatment later and with larger curves. Our goal was to determine the association between scoliometer readings and major curve magnitudes in relation to body mass index (BMI). Methods This retrospective cohort study at a tertiary referral center included 483 patients (420 girls) aged 10 to 18 years (mean age, 14±1.6 years) with thoracic adolescent idiopathic scoliosis who presented to 1 orthopaedic surgeon for initial evaluation of spinal deformity from 2010 to 2015. Records were reviewed for BMI percentile for age and sex (underweight, ≤4th percentile; normal weight, 5th to 84th percentile; overweight, 85th to 94th percentile; obese, ≥95th percentile), patient characteristics, thoracic scoliometer measurements, and thoracic major curves. Results Of the 483 patients, 23 were underweight, 372 were normal weight, 52 were overweight, and 36 were obese. Obese patients had a larger mean major curve (44 degrees) than normal-weight patients (34 degrees) (P = 0.004). The odds of presenting with a major curve ≥20 degrees were 4.9 (95% confidence interval, 1.1-22, P = 0.037) times higher for obese vs normal-weight patients. Receiver operating characteristic analysis of major curves (≥20 vs <20 degrees) estimated the scoliometer values with the greatest sensitivity and specificity to be 8 degrees for underweight patients, 7 degrees for normal-weight patients, 6 degrees for overweight patients, and 5 degrees for obese patients. Conclusions Obese patients presented with larger thoracic curves vs normal-weight patients. Differences in chest-wall thickness in patients with different BMI values may alter scoliometer measurements for a given rotational deformity. Our data suggest new referral criteria for the scoliometer test based on BMI values. Specifically, obese patients should be referred at an ATR of 5 degrees.

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Objective: Congenital heart disease (CHD) is associated with the development of scoliosis. Improvements in cardiac care have extended survival of children with cyanotic CHD which possess a need for correction of scoliosis. There is limited information on spinal care for these patients. We present 3 patients with CHD who underwent surgical correction of scoliosis. Materials and Methods: We reviewed demographic and clinical data on patients with cyanotic CHD. Results: Patient 1 underwent posterior spinal fusion T3–L3 at the age of 16 years. He had a double inlet left ventricle and was treated with completion of a Fontan circulation. Hypotensive anaesthesia was used but he lost 3,000 mL of blood. The operative time was 370 min and most of the blood loss occurred in the second half of the procedure. Patient 2 underwent posterior spinal fusion T5–T12 when aged 14 years. She had transposition of the great vessels corrected over multiple surgeries. Hypotensive anaesthesia was used, she had blood loss of 300 mL, and the surgical time was 282 min. Patient 3 underwent posterior spinal fusion extending from T5–T12 when he was 17 years old. He had a double inlet left ventricle and was treated with completion of a Fontan circulation. Hypotensive anaesthesia was used, he had blood loss of 1,021 mL, and a surgical time of 342 min. Conclusion: Scoliosis surgery in patients with complex cardiac disease may be indicated to treat progressive deformities which produce severe symptoms. A multidisciplinary approach including a spinal surgeon as well as a cardiologist, haematologist, respiratory paediatrician, and spinal anaesthetist can evaluate the general medical condition and weigh the benefits and risks of surgery. Deformity correction can be performed using a meticulous technique and has produced a series of satisfactory outcomes.

All individual domains and total SRS-22 scores improved significantly with incremental change during post-operative follow-up. Patient satisfaction was very high for all individual diagnosis. 2-year post-operative outcomes compared favorably to reported SRS-22 scores in healthy adolescents.