The pediatric lower extremity has well known growth patterns. When deformities or growth disturbances occur, there are several methods to measure and predict the resulting discrepancy, including the Green-Anderson, Moseley, and Multiplier methods. Many techniques exist to correct leg length discrepancy and deformity such and temporary epiphysiodesis, permanent epiphysiodesis, external fixators, and internal lengthening devices. All of these methods have numerous complications and limitations; however, with careful planning and patient selection, length and alignment can be improved with high patient satisfaction.

Background

The Ponseti method is the standard of care for managing idiopathic congenital talipes equinovarus (clubfoot) in the outpatient setting, but there are no clinical guidelines for inpatient treatment. Children in the neonatal intensive care unit (NICU) with clubfoot often delay treatment initiation due to medical reasons.

Methods

We systematically reviewed literature related to the treatment of clubfoot in the NICU, non-idiopathic clubfoot, and older infants, as well as barriers to care.

Results

In a mixed NICU population of syndromic and idiopathic clubfoot, the Ponseti method has good functional outcomes with minimal interference with medical management. The Ponseti method has good functional outcomes with reduced need for extensive surgical procedures in non-idiopathic clubfoot and idiopathic clubfoot with delayed presentation (under one year of age).

Conclusions

It is possible to begin Ponseti treatment in the NICU without compromising medical management. It is not clear if this confers an advantage over waiting for outpatient casting.

Rationale

The sciatic nerve runs a predictable course combining L4-S3 nerve roots through the true pelvis and under the greater sciatic notch. There are reports of bony protuberances from the sacrum and ilium in cases of spinal dysraphism; however advanced imaging, treatment, or outcomes are not described. There are no cases with associated fibular hemimelia in the current literature.

Patient concerns

This is a 4-year-old girl with tethered cord, acetabular dysplasia with hip subluxation, congenital short femur, anterior cruciate ligament (ACL) deficiency, and fibular hemimelia with her sciatic nerve coursing through the ilium.

Diagnosis

Aberrant course of the sciatic nerve through the ilium in the setting of spinal dysraphism.

Outcomes

The hip subluxation was treated with a femoral varus derotation osteotomy and Salter osteotomy with transposition of the sciatic nerve into the greater sciatic notch resulting in a stable hip with no sciatic nerve symptoms at last follow-up.

Lessons

The combination of spinal dysraphism with acetabular dysplasia should be a warning for anomalous sciatic nerveanatomy, possibly through the ilium. Preoperative imaging (MRI, CT scan) may be obtained and carefully reviewed for the course of the sciatic nerve prior to pelvic or femoral osteotomy. Decompressing the sciatic nerve from the aberrant foramen may be considered as part of the procedure.

Smad4 is an intracellular effector of the TGFβ family that has been implicated in Myhre syndrome, a skeletal dysplasia characterized by short stature, brachydactyly and stiff joints. The TGFβ pathway also plays a critical role in the development, organization and proliferation of the growth plate, although the exact mechanisms remain unclear. Skeletal phenotypes in Myhre syndrome overlap with processes regulated by the TGFβ pathway, including organization and proliferation of the growth plate and polarity of the chondrocyte. We used in vitro and in vivo models of Smad4 deficiency in chondrocytes to test the hypothesis that deregulated TGFβ signaling leads to aberrant extracellular matrix production and loss of chondrocyte polarity. Specifically, we evaluated growth plate chondrocyte polarity in tibiae of Col2-Cre^+/-;Smad4^fl/fl mice and in chondrocyte pellet cultures. In vitro and in vivo, Smad4 deficiency decreased aggrecan expression and increased MMP13 expression. Smad4 deficiency disrupted the balance of cartilage matrix synthesis and degradation, even though the sequential expression of growth plate chondrocyte markers was intact. Chondrocytes in Smad4-deficient growth plates also showed evidence of polarity defects, with impaired proliferation and ability to undergo the characteristic changes in shape, size and orientation as they differentiated from resting to hypertrophic chondrocytes. Therefore, we show that Smad4 controls chondrocyte proliferation, orientation, and hypertrophy and is important in regulating the extracellular matrix composition of the growth plate.

Purpose

Cerebral palsy (CP) is the most common motor disorder in childhood. Scoliosis is a common complication of CP that can reach clinically severe levels, but predictors for scoliosis in CP are not well understood. Some variables identified in the literature involve the severity of the brain injury and the presence of hip deformity. We aimed to identify associations with developing severe scoliosis in a prospective cohort of patients with cerebral palsy at higher risk for severe curve progression.

Methods

This study reviewed a prospectively collected database at a tertiary children's hospital. We evaluated a panel of potential associations with severe scoliosis-including age, sex, Gross Motor Function Classification System (GMFCS) class, history of hip surgery, epilepsy, and feeding tube presence-in a population of children with limited ambulatory ability defined as GMFCS level IV or V CP. Univariate analysis and multivariate logistic regression with stepwise selection was used for analysis.

Results

Descriptive analysis showed that female sex, higher GMFCS class, history of hip surgery, non-upright seating, pelvic obliquity, presence of epilepsy, and presence of a feeding tube were associated with an increased risk for scoliosis. Multivariate logistic regression analysis revealed that the presence of a feeding tube was associated with severe scoliosis even when controlling for GMFCS and age.

Conclusions

Feeding tube use may stratify risk for severe scoliosis progression in patients with GMFCS IV or V CP.

Purpose

There are over 500 medically related applications (apps) for mobile devices. Very few of these applications undergo testing and peer-review for accuracy. The purpose of this study is to assess the accuracy of limb deformity measurements on the Bone Ninja app compared to PACS and to determine the intra- and inter-observer variability among different orthopaedic practitioners.

Methods

Four participants (attending, senior and junior resident, and physician assistant) measured the leg length (LL), the lateral distal femoral angle (LDFA), and the medial proximal tibial angle (MPTA) of 48 limbs (24 patients), twice with both Bone Ninja and PACS. The difference between the measurements obtained with the Bone Ninja app and PACS were measured. We determined the consistency of the intra-observer intra-class correlation coefficient (ICC) for both systems.

Results

There were no statistical differences in leg length discrepancy (LLD), MPTA, or LDFA measurements between Bone Ninja and PACS (p = 0.96, 0.87, and 0.97, respectively). The intra-observer ICC for the LL, LDFA, and MPTA was similar between Bone Ninja and PACS (0.83, 0.89, and 0.96 vs. 0.96, 0.93, and 0.95, respectively). The inter-observer ICC was similar between Bone Ninja and PACS (0.95, 0.96, and 0.99 vs. 0.99, 0.98, and 0.98, respectively).

Conclusions

This study demonstrates that Bone Ninja is an accurate educational tool for measuring LLD, LDFA, and MPTA. Both systems are reliable instruments for evaluating limb length differences and angles on standing radiographs for pre-operative deformity planning and education. This is the first study to evaluate the accuracy of Bone Ninja compared to the gold standard of PACS.

Spinal anesthesia (SA) has been used relatively sparingly in the pediatric population, as it is typically reserved for patients in whom the perceived risk of general anesthesia is high due to comorbid conditions. Recently, concern has been expressed regarding the potential long-term neurocognitive effects of general anesthesia during the early stages of life. In view of this, our center has developed a program in which SA may be used as the sole agent for applicable surgical procedures. While this approach in children is commonly used for urologic or abdominal surgical procedures, there have been a limited number of reports of its use for orthopedic procedures in this population. We present the use of SA for 6 infants undergoing tendon Achilles lengthening, review the use of SA in orthopedic surgery, describe our protocols and dosing regimens, and discuss the potential adverse effects related to this technique.

Background

Juvenile idiopathic scoliosis (JIS) outcomes with brace treatment are limited with poorly described bracing protocols. Between 49 and 100% of children with JIS will progress to surgery, however, young age, long follow-up, and varying treatment methods make studying this population difficult. The purpose of this study is to report the outcomes of bracing in JIS treated with a Boston brace™ and identify risk factors for progression and surgical intervention.

Methods

This is a single-center retrospective review of 175 patients with JIS who initiated brace treatment between the age of 4 and 9 years. A cohort of 140 children reached skeletal maturity; 91 children had surgery or at least 2 year follow-up after brace completion. Standard in-brace protocol for scoliosis ³20° was a Boston brace for 18-20 h/day after MRI (n = 82). Family history, MRI abnormalities, comorbidities, curve type, curve magnitude, bracing duration, number of braces, compliance by report, and surgical interventions were recorded.

Results

Children were average 7.9 years old (range 4.1-9.8) at the initiation of bracing. The Boston brace™ was prescribed in 82 patients and nine used night bending brace. Mid-thoracic curves (53%) was the most frequent deformity. Maximum curve at presentation was on average 30 ± 9 degrees, in-brace curve angle was 16 ± 8 degrees, and in-brace correction was 58 ± 24 percent. Patients were braced an average of 4.6 ± 1.9 years. 61/91 (67%) went on to posterior spinal fusion at 13.3 ± 2.1 (range 9.3-20.9) years and curve magnitude of 61 ± 12 degrees. Of those that underwent surgery, 49/55 (86%) progressed > 10°, 6/55 (11%) stabilized within 10°, and 0/55 (0%) improved > 10° with brace wear. No children underwent growth-friendly posterior instrumentation. Of the 28 who did not have surgical correction, 3 (11%) progressed > 10°, 13/28 (46%) stabilized within 10°, and 12/28 (43%) improved > 10° with brace wear.

Conclusions

This large series of JIS patients with bracing followed to skeletal maturity with long-term follow-up. Surgery was avoided in 33% of children with minimal to no progression, and no child underwent posterior growth-friendly constructs. Risk factors of needing surgery were noncompliance and larger curves at presentation.