Walking status and spinopelvic parameters in young children with achondroplasia: 10-year follow-up.

PURPOSE: Thoracolumbar kyphosis (TLK) is common in children with achondroplasia and resolves in 90% by 10 years of age. Our purpose was to describe the natural progression of TLK in a cohort of pre-walking children with achondroplasia. METHODS: A single-center, retrospective review identified 62 children (32 male, 30 female) with achondroplasia. Clinical information and sagittal spinopelvic parameters were collected. The children were divided into positive pelvic tilt (PT) and negative PT. All parents were routinely counseled about unsupported sitting. RESULTS: Spontaneous resolution rate was 64.5% at 1-year post-walking, 74.2% at 5 years of age, and 88.7% at 10 years of age. None of the children required posterior spinal decompression and fusion for progressive deformity or symptomatic spinal stenosis. At 1-year post-walking, the negative PT group had a higher sacral slope (p = 0.006), higher lumbar lordosis (p < 0.001), and lower pelvic incidence (p < 0.001). This relationship remained constant up to 10 years of age, and there was no association with TLK. CONCLUSION: In this largest series to date, spontaneous resolution of TLK in children with achondroplasia was 64.5% at 1-year post-walking, 74.2% at 5 years of age, and 88.7% in children followed to 10 years of age. With early identification and regular follow-up with patient education, no patient in this series required treatment or developed symptomatic spinal stenosis. While not predictive of resolution of TLK, the dichotomous presentation of PT in young children with achondroplasia persists at 5 and 10 years of age and reliably predicts the spinopelvic parameters. LEVEL OF EVIDENCE: III-retrospective comparative study.

Achondroplasia natural history study (CLARITY): 60-year experience in orthopedic surgery from four skeletal dysplasia centers.

BACKGROUND: The purpose of this study was to describe the frequency and risk factors for orthopedic surgery in patients with achondroplasia. CLARITY (The Achondroplasia Natural History Study) includes clinical data from achondroplasia patients receiving treatment at four skeletal dysplasia centers in the United States from 1957 to 2018. Data were entered and stored in a Research Electronic Data Capture (REDCap) database. RESULTS: Information from one thousand three hundred and seventy-four patients with achondroplasia were included in this study. Four hundred and eight (29.7%) patients had at least one orthopedic surgery during their lifetime and 299 (21.8%) patients underwent multiple procedures. 12.7% (n = 175) of patients underwent spine surgery at a mean age at first surgery of 22.4 ± 15.3 years old. The median age was 16.7 years old (0.1-67.4). 21.2% (n = 291) of patients underwent lower extremity surgery at a mean age at first surgery of 9.9 ± 8.3 years old with a median age of 8.2 years (0.2-57.8). The most common spinal procedure was decompression (152 patients underwent 271 laminectomy procedures), while the most common lower extremity procedure was osteotomy (200 patients underwent 434 procedures). Fifty-eight (4.2%) patients had both a spine and lower extremity surgery. Specific risk factors increasing the likelihood of orthopedic surgery included: patients with hydrocephalus requiring shunt placement having higher odds of undergoing spine surgery (OR 1.97, 95% CI 1.14-3.26); patients having a cervicomedullary decompression also had higher odds of undergoing spine surgery (OR 1.85, 95% CI 1.30-2.63); and having lower extremity surgery increased the odds of spine surgery (OR 2.05, 95% CI 1.45-2.90). CONCLUSIONS: Orthopedic surgery was a common occurrence in achondroplasia with 29.7% of patients undergoing at least one orthopedic procedure. Spine surgery (12.7%) was less common and occurred at a later age than lower extremity surgery (21.2%). Cervicomedullary decompression and hydrocephalus with shunt placement were associated with an increased risk for spine surgery. The results from CLARITY, the largest natural history study of achondroplasia, should aid clinicians in counseling patients and families about orthopedic surgery.

Growth Modulation by Tension Band Plate in Achondroplasia With Varus Knee Deformity: Comparison of Gait Analysis Measurements.

BACKGROUND: Limited evidence exists concerning growth modulation by tension band plate (TBP) to correct varus deformity in patients with achondroplasia with limited growth due to FGFR3 gene mutation. We evaluated the efficacy of TBP in children with achondroplasia with genu varum and reported the static radiographic and dynamic motion data to determine parameters that impact the rate of deformity correction. METHODS: Patients with achondroplasia with genu varum who underwent TBP surgery for growth modulation were studied. Those with at least 1 year of follow-up with TBP were included. Radiographic parameters were measured. Growth velocity of femoral/tibial length was calculated separately. Patients were deemed successful or unsuccessful. Spearman correlation analysis and Student t test were used to describe statistical results. RESULTS: Twenty-two patients (41 limbs; 12 girls) fulfilled our criteria. Mean age at TBP surgery was 7.6±2.4 years. Thirty-six femoral TBP and 41 tibial TBP were in place for 24.5±9.7 months. Mean mechanical axis deviation, mechanical lateral distal femoral angle, and medial proximal tibial angle preoperatively were 30.1±7.6 mm, 97.2±6.4, and 80.3±4.3 degrees, and 11±15.6 mm, 87.4±5.9, and 84.7±5.3 degrees at last follow-up ( P <0.001). Fifteen limbs were successfully straightened; 4 limbs were in more varus than the initial deformity. Twenty-four limbs with TBP were still undergoing correction. In successful limbs, mean age at surgery was 6.5±1.7 years and duration of TBP was 29.9±7.8 months. In 4 unsuccessful limbs, mean age at surgery was 11.7±1.2 years. Analysis in the gait laboratory included physical examination with the measurement of knee varus and kinematic varus based on a posterior view static standing photograph. Photographic measurement of varus was higher than the radiographic measurement. CONCLUSIONS: Growth modulation by TBP surgery is a reliable and simple technique to correct genu varum in achondroplasia. An early age at TBP implementation (mean: 6.5 y) is crucial to successfully correct the varus knee deformity. Furthermore, we recommend early and regular surveillance of achondroplasia for progressive varus knee deformity. LEVEL OF EVIDENCE: Level IV-cohort study.

Evaluation of Gait Pattern and Lower Extremity Kinematics of Children with Morquio Syndrome (MPS IV).

Morquio syndrome (mucopolysaccharidosis IV/MPS IV) is a genetic disorder leading to skeletal abnormalities and gait deviations. Research on the gait patterns and lower extremity physical characteristics associated with skeletal dysplasia in children with MPS IV is currently limited. This research aimed to provide baseline gait patterns and lower limb skeletal alignment of children with MPS IV utilizing three-dimensional instrumented gait analysis. This Institutional Review Board-approved retrospective study evaluates the kinematics of the lower extremities of children with MPS IV during gait, comparing them with an age-matched group of typically developing children. Thirty-three children with MPS IV were included (8.6 ± 4.0 years old). Children with MPS IV walk with increased anterior pelvic tilt, knee valgus, knee flexion, external tibial torsion, and reduced walking speed and stride length (p < 0.001). Multiplanar abnormal alignment results in abnormal knee moments (p < 0.001). Limited correlations exist (r = 0.69-0.28) between dynamic three-dimensional measurements of knee varus/valgus and rotational alignment and traditional static two-dimensional measures (physical examination or radiographs) suggesting the possibility of knee instability during gait and the benefits of dynamic assessment.

What Should I Wear to Clinic? A National Survey of Pediatric Orthopaedic Patients and Parents.

BACKGROUND: A number of factors have been shown to affect how surgeons are subjectively viewed, including their appearance in clinic. Patient preference for pediatric orthopaedic surgeon attire has not previously been studied, nor has its influence on initial perception of the surgeon. METHODS: At 5 pediatric regional sites across the United States, parents and patients presenting to the pediatric orthopaedic clinic were given anonymous surveys showing 8 photos of surgeons in different clinical dress including in business or scrub dress, each with or without white coat (WC). Subjects reported their demographics, single preferred photo, rated characteristics of the surgeons in each photo on a 5-point Likert scale, and answered specific questions on scrubs and WCs. The first consecutively completed 100 parent and 100 patient surveys were included from each site. RESULTS: One thousand surveys were collected from patients (500) and parents (500). The majority felt a surgeon's clinical dress would not affect how they are cared for (83%), and that it was ok to wear scrubs in clinic (90%). Overall, WC was preferred to those without no matter the age, but there was no difference between scrub and business choices. Of those stating a preference, woman in business and WC (24%) and woman in scrubs and WC (21%) were most selected, with the only geographic difference being the midwest's preference for man in business and WC. Females were more likely to prefer women photographs (P<0.0001). CONCLUSIONS: In general, pediatric orthopaedic patients and parents do not have a strong specific preference toward what their surgeon wears to clinic, including whether or not we are dressed in scrubs, but some initial biases exist. When asked to choose, the traditional WC worn over any attire is preferred, and female patients and parents uncover a preference for a surgeon of their own sex. LEVEL OF EVIDENCE: Level III.

Surgical site infection following spinal instrumentation for scoliosis: a multicenter analysis of rates, risk factors, and pathogens.

BACKGROUND: Surgical site infection following correction of pediatric scoliosis is well described. However, we are aware of no recent multicenter study describing the rates of surgical site infection, and associated pathogens, among patients with different etiologies for scoliosis. METHODS: A multicenter, retrospective review of surgical site infections among pediatric patients undergoing spinal instrumentation to correct scoliosis was performed at three children's hospitals in the United States. Study subjects included all patients undergoing posterior spinal instrumentation from January 2006 to December 2008. Surgical site infections were defined according to the Centers for Disease Control and Prevention's National Healthcare Safety Network case definition, with infections occurring within one year after surgery. RESULTS: Following the analysis of 1347 procedures performed in 946 patients, surgical site infection rates varied among procedures performed in patients with different scoliosis etiologies. Procedures performed in patients with neuromuscular scoliosis had the highest surgical site infection rates (9.2%), followed by those performed in patients with syndromic scoliosis (8.8%), those performed in patients with other scoliosis (8.4%), those performed in patients with congenital scoliosis (3.9%), and those performed in patients with idiopathic scoliosis (2.6%). Surgical site infection rates varied among procedures in patients undergoing primary spinal arthrodesis based on etiology, ranging from 1.2% (95% confidence interval, 0.1% to 1.3%) in patients with idiopathic scoliosis to 13.1% (95% confidence interval, 8.4% to 17.8%) in patients with neuromuscular scoliosis. Surgical site infection rates following primary and revision procedures were similar among patients with different etiologies. In distraction-based growing constructs, rates were significantly lower for lengthening procedures than for revision procedures (p = 0.012). Multivariate analysis demonstrated that non-idiopathic scoliosis and extension of instrumentation to the pelvis were risk factors for surgical site infections. The three most common pathogens were Staphylococcus aureus (25.0% [95% confidence interval, 17.8% to 32.2%]), coagulase-negative staphylococci (17.1% [95% confidence interval, 10.9% to 23.3%]), and Pseudomonas aeruginosa (10.7% [95% confidence interval, 5.6% to 15.8%]). Overall, 46.5% (95% confidence interval, 35.5% to 57.5%) of surgical site infections contained at least one gram-negative organism; 97.0% (95% confidence interval, 90.8% to 100.0%) of these infections were in patients with non-idiopathic scoliosis. CONCLUSIONS: Surgical site infection rates were significantly higher following procedures in patients with non-idiopathic scoliosis (p < 0.001). Lengthening procedures had the lowest rate of surgical site infection among patients with early onset scoliosis who had undergone instrumentation with growing constructs. Gram-negative pathogens were common and were most common following procedures in patients with non-idiopathic scoliosis. These findings suggest a role for targeted perioperative antibiotic prophylaxis to prevent surgical site infection following pediatric scoliosis instrumentation procedures.

Percutaneous achilles tendon repair using ring forceps.

In this article, we describe a method of percutaneous repair of acute Achilles tendon ruptures. We suggest an inexpensive and practical technique, using standard ring forceps, that produces results comparable with those obtained with Dr. Richard Stern's Achillon apparatus.