Genetic association and characterization of FSTL5 in isolated clubfoot.

Talipes equinovarus (clubfoot, TEV) is a congenital rotational foot deformity occurring in 1 per 1000 births with increased prevalence in males compared with females. The genetic etiology of isolated clubfoot (iTEV) remains unclear. Using a genome-wide association study, we identified a locus within FSTL5, encoding follistatin-like 5, significantly associated with iTEV. FSTL5 is an uncharacterized gene whose potential role in embryonic and postnatal development was previously unstudied. Utilizing multiple model systems, we found that Fstl5 was expressed during later stages of embryonic hindlimb development, and, in mice, expression was restricted to the condensing cartilage anlage destined to form the limb skeleton. In the postnatal growth plate, Fstl5 was specifically expressed in prehypertrophic chondrocytes. As Fstl5 knockout rats displayed no gross malformations, we engineered a conditional transgenic mouse line (Fstl5LSL) to overexpress Fstl5 in skeletal osteochondroprogenitors. We observed that hindlimbs were slightly shorter and that bone mineral density was reduced in adult male, but not female, Prrx1-cre;Fstl5LSL mice compared with control. No overt clubfoot-like deformity was observed in Prrx1-cre;Fstl5LSL mice, suggesting FSTL5 may function in other cell types to contribute to iTEV pathogenesis. Interrogating published mouse embryonic single-cell expression data showed that Fstl5 was expressed in cell lineage subclusters whose transcriptomes were associated with neural system development. Moreover, our results suggest that lineage-specific expression of the Fstl genes correlates with their divergent roles as modulators of transforming growth factor beta and bone morphogenetic protein signaling. Results from this study associate FSTL5 with iTEV and suggest a potential sexually dimorphic role for Fstl5 in vivo.

Predicting Satisfaction of Parents of Pediatric Patients: Perceived Quality of Providers' Communication Mitigates Negative Effects of Shorter than Desired Consultations.

This research investigated the predictors of satisfaction for parents of pediatric patients after a clinical consultation. Specifically, we assessed whether perceptions of their provider's communication quality influenced the degree to which their (dis)satisfaction with consultation length associated with their provider rating and intent to recommend the provider's office. Using patient satisfaction survey data collected after initial clinical visits to a pediatric hospital (N = 12,004), we found that communication quality was a stronger predictor for those who were dissatisfied with their consultation length, whereas communication quality made a relatively smaller difference for those who were satisfied with their consultation length. Put another way, parents' dissatisfaction with their child's consultation length mattered little when they perceived their provider to be high in communication quality, but it reduced their ratings and intentions to recommend when they perceived their provider to be low in communication quality. These results suggest that providers' communication behaviors have the capacity to buffer patients' negative evaluations otherwise incurred from shorter than desired consultations.

Are Some Randomized Clinical Trials Impossible?

Congenital tibial pseudarthrosis is a rare condition seen in neurofibromatosis type 1 (NF1), and treatment is complex. A randomized, placebo-controlled trial of bone morphogenetic protein (rhBMP-2; INFUSE bone graft) at time of tibial surgery was developed by the Neurofibromatosis Clinical Trials Consortium. Patients were randomized to receive rhBMP-2 that would, or would not, be added to the standard surgical procedure consisting of resection of pseudarthrosis tissue, insertion of a rigid intramedullary rod, and placement of autogenous iliac crest bone graft. Despite involvement of 16 centers with wide experience with NF1 orthopaedic management, only 5 patients (of 54 required) were able to be enrolled in the study during a 3-year time period. Because of the inability to recruit sufficient patients, this study was closed in June 2019, with plans to terminate. The obstacles that were encountered during the study are summarized. The authors question whether a randomized, placebo-controlled trial of a rare pediatric orthopaedic condition is possible to accomplish. Recommendations are provided to guide future studies of orthopaedic manifestations of NF1.Level of Evidence: Level V.

Successful Ponseti-treated Clubfeet at Age 2 Years: What Is the Rate of Surgical Intervention After This?

BACKGROUND: In recent decades, nonoperative Ponseti casting has become the standard of care in the treatment of idiopathic clubfoot. However, the rate of recurrence, even after successful Ponseti treatment is not insignificant. The purpose of this study was to determine the future rate, timing, and type of surgery needed in patients whose idiopathic clubfeet treated by Ponseti casting were considered successful at the age of 2 years. METHODS: Inclusion criteria for this retrospective study were patients under 3 months with idiopathic clubfoot treated exclusively by Ponseti casting, who had successful outcomes at 2 years of age without surgery, and who had at least 5 years of follow-up. The total number of surgical interventions in the age range 2 to 5 and above 5 years, the number and type of procedures performed, and the timing of surgery were reviewed. RESULTS: Three hundred thirty-six patients with a total of 504 clubfeet fulfilled the inclusion criteria. One hundred twenty-two of these 336 patients (36.3%) eventually underwent surgical intervention. Between 2 and 5 years of age, 79 patients (23.5%) with 104 feet (20.6%) underwent surgery. The most common procedures performed between 2 and 5 years were limited (a la carte) in scope: tibialis anterior tendon transfer, posterior release, plantar fascia release, and repeat tendo-Achilles lengthening. At age above 5 years, 53 patients (20.1%) with 65 feet (16.9%) underwent surgery. Ten of these 53 patients had already undergone surgery between 2 and 5 years of age. The procedures most commonly performed were similar. CONCLUSIONS: In patients with idiopathic clubfoot who reached 2 years of age with successful outcomes from Ponseti cast treatment, ∼35% eventually underwent surgical intervention, mostly limited (a la carte), to regain or maintain a plantigrade foot. The most commonly performed procedures include tibialis anterior tendon transfer, posterior capsular release, plantar fascia release and repeat tendo-Achilles lengthening, either in isolation or in combination. However, before considering surgery, the need for these procedures can, and should, be minimized by recasting recurrent deformities using Ponseti method. LEVEL OF EVIDENCE: Level III.

Clubfoot Infants Initially Thought to be Idiopathic, But Later Found Not to Be. How do They do With Nonoperative Treatment?

BACKGROUND: Infants thought to be normal with idiopathic clubfeet when nonoperative treatment begins may later be found to have other complicating diagnoses. The purpose of this study was to determine the incidence of this occurrence, and to compare the clinical outcomes of these "nonidiopathic" patients with idiopathic clubfoot patients. METHODS: Infants below 3 months old with clubfeet who were thought to be normal (idiopathic) at presentation and had ≥2-year follow-up were studied. Treatment consisted of either the Ponseti method or the French physical therapy method. In total, 789 patients with 1174 clubfeet were identified. Those who were idiopathic (group 1) were compared with those later found to be nonidiopathic (group 2). The outcomes at 2 years were assessed as good (plantigrade foot±heelcord tenotomy only), fair (limited procedures), or poor (full-posteromedial release). For those feet rated good at age 2 years, outcomes were again assessed age 5 years and above. RESULTS: In total, 70 patients (8.9%) of the 789 enrolled patients were eventually found to have another disorder including neurological, syndromic, chromosomal, or spinal abnormalities. The remaining 719 idiopathic patients with 1062 clubfeet (group 1) were compared with these 70 nonidiopathic patients with 112 clubfeet (group 2). At age 2 years, in group 1 81% of the feet were rated good, 11% fair, and 8% poor, whereas in group 2 70% of the feet were rated good, 11% fair, and 19% poor (P=0.0004).With follow-up exceeding age 5 years in those rated good at age 2 years: in group 1, 73% continued to do well, but 22% rated fair, and 5% poor. In group 2, 59% continued to do well, but 31% rated fair, and 10% poor (P=0.046). CONCLUSIONS: For infants with clubfeet who were initially thought to be idiopathic, nearly 9% were later found to have a complicating disorder. Despite this, these patients' clubfeet can be expected to respond favorably to nonoperative treatment. However, they will require more surgical intervention early (by age 2 years) and later (age, 5 years and above) when compared with normal infants with idiopathic clubfeet. LEVEL OF EVIDENCE: Level IV-therapeutic, case series.

rhBMP-2 and Intramedullary Fixation in Congenital Pseudarthrosis of the Tibia.

BACKGROUND: In congenital pseudarthrosis of the tibia, use of intramedullary (IM) fixation and autogenous bone graft has long been the standard of care. This study was undertaken to determine whether the addition of rhBMP-2 to this treatment method further enhances healing potential. METHODS: Twenty-one patients with congenital pseudarthrosis of the tibia were evaluated. Fifteen of these patients had neurofibromatosis type 1 (NF1). All had IM fixation and autogenous bone graft, followed by a BMP-soaked collagen sponge wrapped around both the fracture site and bone graft. A minimum 2 years' follow-up was required. RESULTS: Follow-up averaged 7.2 years (range, 2.1 to 12.8 y). Sixteen of 21 tibias achieved bone union following the index surgery, at an average 6.6 months postoperatively. The 5 persistent nonunions occurred in NF1 patients. Further surgery was undertaken in these 5 NF1 patients, including the use of BMP. One of the 5 healed, 1 had persistent nonunion, and 3 eventually had amputation. Of the 16 patients who healed initially following the index surgery, 5 refractured (3 had NF1). Of these 5 patients, the IM fixation at the index surgery did not cross the ankle joint, and refracture occurred at the rod tip in 4. Three of these 5 patients healed following further surgery, 1 had persistent nonunion, and 1 had amputation. All of those with eventual amputation had NF1. No deleterious effects related to the use of BMP-2 were recognized in any patient. CONCLUSIONS: The addition of rhBMP-2 appears to be helpful in shortening the time required to achieve fracture union in those who healed, but its use does not insure that healing will occur. LEVEL OF EVIDENCE: Level IV-therapeutic, case series.

Radiographic Assessment of Shoulder Position in 619 Idiopathic Scoliosis Patients: Can T1 Tilt Be Used as an Intraoperative Proxy to Determine Postoperative Shoulder Balance?

BACKGROUND: The purpose of this study is to assess radiographic shoulder measures from the preoperative to the postoperative time period, specifically to determine whether T1 tilt could be used as an intraoperative proxy for shoulder balance determination. This study focused on radiographic shoulder measures of 619 adolescent idiopathic scoliosis patients who underwent spinal deformity surgery. METHODS: A prospective, multicenter database of adolescent idiopathic scoliosis was queried to identify all patients who had undergone spinal deformity surgery with >2 years of follow-up postoperatively. Radiographic analysis focused on measures of shoulder balance: T1 tilt, clavicle angle, and radiographic shoulder height. RESULTS: A total of 619 patients were included in this analysis. Mean age at surgery was 14.8 years with 83% female. Mean preoperative curve size was 58.0 degrees. Mean T1 tilt preoperatively was -0.10 degrees and postoperatively 2.42 degrees. Mean clavicle angle preoperatively was -1.39 degrees and postoperatively 0.79 degrees. Mean radiographic shoulder height preoperatively was -7.04 mm and postoperatively 1.63 mm. All 3 radiographic parameters demonstrated reasonable correlation preoperatively and postoperatively to each other. To assess the viability of T1 tilt as an intraoperative proxy for shoulder balance, standardized ratios between the variables were created. Analysis of these ratios demonstrated little or no relationship preoperatively to postoperatively, hence the relationship of T1 tilt to radiographic shoulder height does not remain constant. CONCLUSIONS: Analysis of the relationship of T1 tilt to radiographic shoulder height from preoperative to postoperative did not demonstrate consistency. Lenke 3 and 6 curve patterns demonstrated preoperative to postoperative correlation, both with nonstructural proximal thoracic curves; however, for the remaining curve patterns T1 tilt cannot be used as an intraoperative proxy for shoulder balance. LEVEL OF EVIDENCE: Level IV.

Congenital band syndrome with pseudarthrosis of the radius and ulna and impending vascular compromise: a case report.

BACKGROUND: Although amniotic band syndrome is relatively rare, reports of pseudarthrosis in conjunction with amniotic band syndrome are even rarer, as are reports of impending vascular compromise in the neonatal period. Careful serial examinations and timely surgical intervention can successfully avoid the catastrophic event of limb loss. We report on a case of upper extremity amniotic band syndrome with pseudarthrosis of the radius and ulna that was complicated by vascular compromise in a neonate. METHODS: Chart and radiographic data for this single case were reviewed and reported retrospectively. RESULTS: A 1-day-old neonate born at 28 3/7 weeks of gestational age was transferred to our institution for increased swelling to the forearm distal to a congenital band associated with an underlying radius and ulna pseudarthrosis. Although the forearm and hand were soft and viable initially, severe edema and swelling occurred after fluid resuscitation, and on the fourth day of life, the patient underwent simple band releases at bedside with 2 longitudinal incisions over the radius and ulna. Circulation was restored, and the pseudarthrosis healed with no further surgical intervention. Successful delayed reconstruction of the band with Z-plasties was performed when the baby was 7 months of age. CONCLUSIONS: In this case, a relatively simple, straightforward procedure that is familiar to most pediatric orthopaedists salvaged a compromised neonatal limb with amniotic band syndrome and allowed healing of a pseudarthrosis, allowing more complex reconstruction to be performed in a delayed, elective manner. Careful observation is necessary in the neonatal period of the baby with a severe band; a viable, well-perfused, compressible extremity may still be at risk.

The Effect of Implant Density on Adolescent Idiopathic Scoliosis Fusion: Results of the Minimize Implants Maximize Outcomes Randomized Clinical Trial.

BACKGROUND: Severe adolescent idiopathic scoliosis (AIS) can be treated with instrumented fusion, but the number of anchors needed for optimal correction is controversial. METHODS: We conducted a multicenter, randomized study that included patients undergoing spinal fusion for single thoracic curves between 45° and 65°, the most common form of operatively treated AIS. Of the 211 patients randomized, 108 were assigned to a high-density screw pattern and 103, to a low-density screw pattern. Surgeons were instructed to use ≥1.8 implants per spinal level fused for patients in the high-implant-density group or ≤1.4 implants per spinal level fused for patients in the low-implant-density group. The primary outcome measure was the percent correction of the coronal curve at the 2-year follow-up. The power analysis for this trial required 174 patients to show equivalence, defined as a 95% confidence interval (CI) within a ±10% correction margin with a probability of 90%. RESULTS: In the intention-to-treat analysis, the mean percent correction of the coronal curve was equivalent between the high-density and low-density groups at the 2-year follow-up (67.6% versus 65.7%; difference, -1.9% [95% CI: -6.1%, 2.2%]). In the per-protocol cohorts, the mean percent correction of the coronal curve was also equivalent between the 2 groups at the 2-year follow-up (65.0% versus 66.1%; difference, 1.1% [95% CI: -3.0%, 5.2%]). A total of 6 patients in the low-density group and 5 patients in the high-density group required reoperation (p = 1.0). CONCLUSIONS: In the setting of spinal fusion for primary thoracic AIS curves between 45° and 65°, the percent coronal curve correction obtained with use of a low-implant-density construct and that obtained with use of a high-implant-density construct were equivalent. LEVEL OF EVIDENCE: Therapeutic Level I . See Instructions for Authors for a complete description of levels of evidence.

Approaches to treating NF1 tibial pseudarthrosis: consensus from the Children's Tumor Foundation NF1 Bone Abnormalities Consortium.

BACKGROUND: Neurofibromatosis 1 (NF1) is an autosomal dominant disorder with various skeletal abnormalities occurring as part of a complex phenotype. Tibial dysplasia, which typically presents as anterolateral bowing of the leg with subsequent fracture and nonunion (pseudarthrosis), is a serious but infrequent osseous manifestation of NF1. Over the past several years, results from clinical and experimental studies have advanced our knowledge of the role of NF1 in bone. On the basis of current knowledge, we propose a number of concepts to consider as a theoretical approach to the optimal management of tibial pseudarthrosis. METHODS: A literature review for both clinical treatment and preclinical models for tibial dysplasia in NF1 was performed. Concepts were discussed and developed by experts who participated in the Children's Tumor Foundation sponsored International Bone Abnormalities Consortium meeting in 2011. RESULTS: Concepts for a theoretical approach to treating tibial pseudarthrosis include: bone fixation appropriate to achieve stability in any given case; debridement of the "fibrous pseudarthrosis tissue" between the bone segments associated with the pseudarthrosis; creating a healthy vascular bed for bone repair; promoting osteogenesis; controlling overactive bone resorption (catabolism); prevention of recurrence of the "fibrous pseudarthrosis tissue"; and achievement of long-term bone health to prevent recurrence. CONCLUSIONS: Clinical trials are needed to assess effectiveness of the wide variation of surgical and pharmacologic approaches currently in practice for the treatment of tibial pseudarthrosis in NF1. LEVEL OF EVIDENCE: Level V, expert opinion.

Building consensus: development of a Best Practice Guideline (BPG) for surgical site infection (SSI) prevention in high-risk pediatric spine surgery.

BACKGROUND: Perioperative surgical site infection (SSI) after pediatric spine fusion is a recognized complication with rates between 0.5% and 1.6% in adolescent idiopathic scoliosis and up to 22% in "high risk" patients. Significant variation in the approach to infection prophylaxis has been well documented. The purpose of this initiative is to develop a consensus-based "Best Practice" Guideline (BPG), informed by both the available evidence in the literature and expert opinion, for high-risk pediatric patients undergoing spine fusion. For the purpose of this effort, high risk was defined as anything other than a primary fusion in a patient with idiopathic scoliosis without significant comorbidities. The ultimate goal of this initiative is to decrease the wide variability in SSI prevention strategies in this area, ultimately leading to improved patient outcomes and reduced health care costs. METHODS: An expert panel composed of 20 pediatric spine surgeons and 3 infectious disease specialists from North America, selected for their extensive experience in the field of pediatric spine surgery, was developed. Using the Delphi process and iterative rounds using a nominal group technique, participants in this panel were as follows: (1) surveyed for current practices; (2) presented with a detailed systematic review of the relevant literature; (3) given the opportunity to voice opinion collectively; and (4) asked to vote regarding preferences privately. Round 1 was conducted using an electronic survey. Initial results were compiled and discussed face-to-face. Round 2 was conducted using the Audience Response System, allowing participants to vote for (strongly support or support) or against inclusion of each intervention. Agreement >80% was considered consensus. Interventions without consensus were discussed and revised, if feasible. Repeat voting for consensus was performed. RESULTS: Consensus was reached to support 14 SSI prevention strategies and all participants agreed to implement the BPG in their practices. All agreed to participate in further studies assessing implementation and effectiveness of the BPG. The final consensus driven BPG for high-risk pediatric spine surgery patients includes: (1) patients should have a chlorhexidine skin wash the night before surgery; (2) patients should have preoperative urine cultures obtained; (3) patients should receive a preoperative Patient Education Sheet; (4) patients should have a preoperative nutritional assessment; (5) if removing hair, clipping is preferred to shaving; (6) patients should receive perioperative intravenous cefazolin; (7) patients should receive perioperative intravenous prophylaxis for gram-negative bacilli; (8) adherence to perioperative antimicrobial regimens should be monitored; (9) operating room access should be limited during scoliosis surgery (whenever practical); (10) UV lights need NOT be used in the operating room; (11) patients should have intraoperative wound irrigation; (12) vancomycin powder should be used in the bone graft and/or the surgical site; (13) impervious dressings are preferred postoperatively; (14) postoperative dressing changes should be minimized before discharge to the extent possible. CONCLUSIONS: In conclusion, we present a consensus-based BPG consisting of 14 recommendations for the prevention of SSIs after spine surgery in high-risk pediatric patients. This can serve as a tool to reduce the variability in practice in this area and help guide research priorities in the future. Pending such data, it is the unsubstantiated opinion of the authors of the current paper that adherence to recommendations in the BPG will not only decrease variability in practice but also result in fewer SSI in high-risk children undergoing spinal fusion. LEVEL OF EVIDENCE: Not applicable.

Correction of moderate (<70 degrees) Lenke 1A and 2A curve patterns: comparison of hybrid and all-pedicle screw systems at 2-year follow-up.

BACKGROUND: Multiple reports have demonstrated the superiority of all-pedicle screw constructs, over hybrid and hook constructs, for severe sagittal and coronal deformities (>70 degrees) and have made anterior spinal releasing unnecessary for almost all spinal deformities. However, for lesser deformities, specifically coronal deformities <70 degrees, published studies have not been able to consistently demonstrate clinical superiority of all-pedicle screw constructs over hook or hybrid constructs. METHODS: A prospective, multicenter database on AIS identified patients with <70 degrees main thoracic (MT) curves surgically treated with a posterior spinal fusion. Inclusion criteria were: Lenke 1A and 2A curve patterns, neurologically normal, primary surgery only, and at least 13 years of age at surgery or be Risser 3 or greater and a construct consisting of all-pedicle screws (PS) or hybrid instrumentation. Minimum follow-up was 2 years postoperative. Patients were excluded if surgeries included any releases (laminectomies, ligament releases, or osteotomies), which may increase curve flexibility. RESULTS: A total of 101 patients satisfied the criteria for inclusion: PS (n=53) and hybrid (n=48). Preoperative patient data, preoperative curve characteristics, and operative data were similar between the 2 groups. Postoperative thoracic coronal Cobb demonstrated PS had better proximal thoracic (PT) and MT correction, MT Cobb correction % and correction index than hybrid. Interestingly there were no differences in correction index/fixation point between the 2 groups, indicating PS constructs achieved better correction due, at least in part, to the greater number of spine fixation points. Lower instrumented vertebrae tilt and rotational correction was better in the PS group than hybrid. At 2-year follow-up PS had better absolute forced expiratory volume in 1 second values, trunk shift, and total scoliosis appearance questionnaire than hybrids. T5-T12 sagittal alignment was unchanged at 2-year follow-up for PS versus increased kyphosis in hybrids. CONCLUSIONS: All-pedicle screw systems had better coronal correction, lower instrumented vertebrae tilt, MT scoliometer measurements and scoliosis appearance questionnaire total measures than hybrid constructs. The improved coronal correction in the PS group is likely due, in part, to the higher number of spine fixation points than used in the hybrid groups.

Kyphectomy improves sitting and skin problems in patients with myelomeningocele.

BACKGROUND: Progressive kyphosis occurs in up to 20% of patients with myelomeningocele. Severely affected patients can develop recurrent skin breakdown, osteomyelitis, sitting imbalance, and poor cosmetic appearance. QUESTIONS/PURPOSES: We (1) assessed the ability of kyphectomy to restore an intact skin envelope and allow comfortable seating in a wheelchair; (2) reviewed the complications of kyphectomy and spinal fusion in myelomeningocele; and (3) determined whether patients requiring unexpected reoperation had worse correction or more ulceration compared with those patients treated with a single surgery. METHODS: We retrospectively reviewed the records of 23 children with thoracic-level myelomeningocele who were treated with kyphectomy and spinal fusion since 1980. Indications for surgery included recurrent skin breakdown (15 patients) and poor sitting balance or unacceptable cosmetic deformity (three patients). We evaluated operative technique, type of sacropelvic fixation, surgical complications, radiographic correction, and skin condition at followup. The minimum followup was 2 years (median, 4.1 years; range, 2.1-10 years); 18 of the 23 children had greater than 2 years followup and are reported here. RESULTS: Kyphectomy achieved a sitting balance and resolved in skin ulceration in 17 of 18 patients. Seven patients had complications requiring reoperation. Three patients had multiple reoperations for early deep infection and one patient each had reoperation for late infection, pseudarthrosis, implant-related sacral pressure sore, and planned extension of proximal fusion after growth. Patients requiring multiple operations had similar correction and relief of ulceration to those treated with a single procedure. CONCLUSIONS: Complications after kyphectomy are frequent and many children with myelomeningocele and severe hyperkyphosis require multiple procedures and lengthy hospital stays. Nonetheless, improved seating balance and resolution of skin problems was achieved in 17 of 18 patients.

Current treatment preferences for early onset scoliosis: a survey of POSNA members.

BACKGROUND: Surgical options for the management of idiopathic early onset scoliosis (EOS) have increased over the past decade, perhaps surpassing traditional nonoperative methods. We sought to assess current treatment preferences in the management of EOS among pediatric orthopaedic surgeons. We hypothesized that practitioner access to casting tables and halo traction would be significantly associated with treatment choices. METHODS: A web-based survey was distributed to the members of the Pediatric Orthopaedic Society of North America. Information with regard to practice type, access to casting tables and halo traction, and management of patients with EOS was obtained. Clinical vignettes were used to assess current physician preferences in the treatment of EOS, including the use of bracing, casting, halo-gravity traction, fusionless spine techniques, definitive fusion, and chest wall devices. RESULTS: Members of Pediatric Orthopaedic Society of North America (19.8%) completed the survey with the vast majority of respondents (93.8%) treating children with EOS. Sixty-six percent of respondents had access to a casting table and 77% reported access to halo-gravity traction. Access to casting tables and access to halo-gravity traction was associated with the use of casting and traction (P<0.0001). Equal numbers of surgeons currently use casting (62%) and growing spine techniques (64.1%). Chest wall expansion was offered as a treatment option by 39.1% of surgeons, and 27% of surgeons reported the use of halo-gravity traction. Ninety-three percent of respondents chose nonoperative management of a 2-year-old child with a 50° progressive scoliosis. In contrast, 63% of surgeons would offer surgery as the initial management to a 5-year-old child with a progressive 70° idiopathic scoliosis. CONCLUSIONS: The majority of respondents had access to halo traction and casting tables at their hospitals. There was a statistically significant association between access to equipment and use of casting and halo traction. Nonoperative management was the preferred treatment option in the very young (2 year-old). Two-thirds of surgeons report initial surgical management of the 5-year-old child with a large idiopathic curve.

Narrative Review of the objective analysis of long-term outcome of the Ponseti technique: experience from Dallas.

In 2001, Texas Scottish Rite Hospital for Children (TSRH) prospectively began a clubfoot database that included all of our patients with clubfeet who were willing to enroll. Nonoperative treatment, primarily the Ponseti method, was utilized. This article summarizes the experience from Dallas treating idiopathic clubfeet using the Ponseti technique, and is based on previously published studies utilizing information from the database. Patient clinical outcomes were defined as "good" (plantigrade foot achieved either with or without a percutaneous heel-cord tenotomy), "fair" (a plantigrade foot that required a limited procedure, such as tibialis anterior tendon transfer or posterior release), or "poor" (a plantigrade foot that required posteromedial release). Nearly 95% of idiopathic clubfeet obtained initial correction using the Ponseti technique, but relapses occurred and by age two years 24% needed some surgical intervention, usually limited procedures. Use of Dimeglio's rating system before treatment strongly correlated with the probability of a "good" outcome at two years. Objective measurements of brace wear compliance (iButton) in those who reached age two years with "good" outcomes demonstrated an unexpected pattern of diminishing use of the foot abduction orthoses over the first two years of brace wear. By the 18-month period of brace wear, 1/3 patients wore the orthoses less than 6 hours per day, and nearly half of the patients wore the orthoses less than 8 hours per day. Between ages 2-5 years, nearly 21% of the corrected clubfeet at age two years needed limited procedures to maintain/regain plantigrade positioning. Lateral weight-bearing radiographs between 18-24 months were not helpful in predicting future relapse in these patients, and are no longer routinely obtained. Following these patients for normal development is important, as nearly 9% of infants initially presenting as idiopathic clubfeet were eventually found to have another disorder including neurological, syndromic, chromosomal, or spinal abnormalities. We continue to emphasize the need to devote great attention to detail when using the Ponseti method in an effort to optimize the clinical outcomes.

Spinal balance and lumbar curve stability after selective thoracic fusion in idiopathic scoliosis.

PURPOSE: Selective thoracic fusion (STF) for double curve patterns in idiopathic scoliosis is an attractive treatment option. However, short-term coronal decompensation and truncal imbalance are known findings. Previous studies with hook constructs showed that eventually balance is achieved via an increase in lumbar curve magnitude, as the lumbosacral obliquity did not change following surgery. Our aim is to investigate patients with idiopathic curves who underwent STF using all-pedicle screw constructs to determine if the uninstrumented lumbar curve and lumbosacral obliquity responded in the same manner as was previously reported with all-hook constructs. METHODS: 102 consecutive patients with Lenke 1B, 1C or 3C curves who underwent STF using all-screw constructs at a single institution were included in this study. Radiographic assessment was performed, and patient reported outcomes were obtained. Subgroup analyses were performed based on preoperative thoracic: lumbar curve ratio as well as lumbar curve magnitude. RESULTS: Overall, the patients showed statistically significant improvement in both trunk shift and L4-pelvis obliquity at final follow-up. The uninstrumented lumbar curves trended toward improvement over time, but did not reach statistical significance (p = 0.107). SRS-30 scores were statistically significantly improved in multiple domains. CONCLUSION: Selective thoracic fusion is an excellent treatment option in most double curve patterns. Balance in the coronal plane is predictably achieved at 2-year follow-up. The lumbosacral obliquity improves more with screw technology than was previously found with hook constructs; therefore, the improvement in balance over time does not depend upon an increase in the uninstrumented lumbar curve. LEVEL OF EVIDENCE: IV.

Complications associated with the use of bone morphogenetic protein in pediatric patients.

BACKGROUND: Recombinant human bone morphogenetic protein (rhBMP) has been found to be a powerful adjunct to healing nonunions and obtaining fusions. Despite the apparent clinical efficacy and good safety profile reported with rhBMP use in adults, there is little data regarding the safety of this product in pediatric patients. We evaluated the use of rhBMP-2 in pediatric patients to determine if any complications were associated with its use. METHODS: We performed a retrospective review of 81 patients, all less then 18 years old, in whom rhBMP-2 was used. Theoretical complications associated with rhBMP-2 use were compiled based on a review of the published literature on rhBMP-2. A review of each patient's chart and radiographs was performed to record the occurrences of complications, which may have been associated with the use of rhBMP-2. RESULTS: A total of 16 complications were found, which may have been attributed to the use of rhBMP-2. There were no incidences of systemic toxicity associated with rhBMP-2 use. Nine patients were noted to have some local operative site problem, 3 deep infections were noted, 1 patient was found to have a postoperative compartment syndrome, 2 patients were found to have neurologic complications (1 with progressive myelopathy and 1 with weakness and dural fibrosis), and 1 patient with neurofibromatosis and previously diagnosed intracranial gliomas was found to have subsequent enlargement of these gliomas. In reviewing these complications, only the case of dural fibrosis and subsequent weakness was thought to possibly be directly related to the use of rhBMP-2. CONCLUSION: We found few complications in pediatric patients, which were felt to be directly attributable to the use of rhBMP-2. As such, rhBMP-2 use seems to be relatively safe in this young patient population. Due to the current use of this product in pediatric patients in an "off-label" fashion, we recommend a thorough discussion of the possible risks and benefits of this product with the family before its use. LEVEL OF EVIDENCE: Therapeutic studies-Level IV.

Objective Measurement of Brace Wear in Successfully Ponseti-Treated Clubfeet: Pattern of Decreasing Use in the First 2 Years.

PURPOSE: Once Ponseti correction of a clubfoot is achieved and 3-month full-time bracing treatment is completed, part-time bracing treatment for 12 hours at night for 2 to 4 years is considered necessary to maintain a successful outcome. This study objectively documents the amount of daily orthosis wear time in those who maintained correction at age 2 years and, in so doing, determines how well patients' caretakers comply with the prescribed brace program. METHODS: Patients <3 months old with idiopathic clubfeet when Ponseti treatment was initiated, who successfully maintained correction at age 2 years without surgery and who had complete objective brace wear data, were included. The foot abduction orthoses had a temperature data logger embedded in a shoe. Six 3-month time intervals were monitored in every patient as follows: full time: 0 to 3; night time: 4 to 6, 7 to 9, 10 to 12, 13 to 15, and 16 to 18 months. The families were not informed that hours of brace wear were being measured. RESULTS: One hundred twenty-four patients with 187 clubfeet were included. During the 0- to 3-month interval, wear time averaged 19.8 hr/d. After this period of full-time use, the night-time brace wear decreased over each of the subsequent five intervals: 11.9, 9.6, 8.6, 7.9, and 7.7 hours. By the 18-month period of brace wear, 1 of 3 patients wore the orthoses less than 6 hours per day, and nearly 1 of 2 patients wore the orthoses less than 8 hours per day. DISCUSSION: In patients evaluated at age 2 years whose clubfeet had successful nonsurgical treatment, night-time brace wear varied greatly and decreased over each 3-month period measured. By the second year of bracing treatment, nearly half of the patients wore them 8 hours or less. LEVEL OF EVIDENCE: Level IV case series.

Clubfoot treatment: Ponseti and French functional methods are equally effective.

UNLABELLED: Over the past 15 years, the reemergence of nonoperative treatment of clubfeet throughout the world has been profound. Two methods have been utilized-the Ponseti method and, to a lesser extent, the French functional method. Our review presents one institution's experience using both methods. Satisfactory initial correction was achieved in 95% of idiopathic clubfeet, regardless of method. However, maintenance of the correction was challenging as relapses occurred in 37% of feet treated by the Ponseti method and 29% of feet treated by the French functional method. At an average 4.3 year followup, using either method, posteromedial releases were avoided in 84% of our patients. Using gait analysis to evaluate the function of children treated with these techniques, there was no difference in cadence parameters between the two groups. More of the children treated with the French method walked with knee hyperextension, a mild equinus gait, and mild footdrop. In contrast, more of the patients in the Ponseti group demonstrated mildly increased stance-phase dorsiflexion and a calcaneus gait. LEVEL OF EVIDENCE: Level V, therapeutic study. See the Guidelines for Authors for a complete description of levels of evidence.

Infantile tibia vara: correction of recurrent varus deformity following epiphyseolysis.

Infantile tibia vara (infantile Blount's disease) is a condition in which progressive varus of the knee develops due to diminished physeal growth of the medial aspect of the proximal tibia. A 6-year-old boy with severe bilateral infantile tibia vara (Langenskiold stage V) underwent operative correction of his deformities. Surgery consisted of epiphyseolyses of the medial proximal tibiae and valgus-producing corrective tibial osteotomies. Nearly 5 years after symmetric growth, he again developed progressive varus involving his left knee. Usually, a repeat proximal tibial osteotomy is required along with consideration for completion of epiphyseodesis. In this case report, a simpler approach using staples was taken to successfully regain correction in a patient with recurrent deformity who was treated previously by a combination of epiphyseolysis and proximal tibial osteotomy. Stapling of the lateral aspect of the physis allowed the limited remaining growth potential within the abnormal medial physeal region to correct the angular deformity. A small overcorrection into valgus corrected after subsequent removal of the staple. At 14 years, with clinical and radiologic alignment normal, bilateral proximal tibial epiphysiodeses were performed. The patient returned to playing football at a high school level and had no functional limitations. To our knowledge, this is the first report of growth modulation (physeal stapling) resulting in successful correction of recurring genu varum in a knee that had previously undergone epiphyseolysis of the medial proximal tibial physis in advanced infantile tibia vara.