Infantile Tibia Vara (Blount Disease) with Iatrogenic Changes Causing Residual Tibial Deformities.

Treatment of infantile tibia vara or Blount disease (ITV/BD) in patients < 3 years old and Langenskiold stages I-III consists of orthosis and, in relapsing cases, proximal tibial osteotomy and/or proximal tibial guided growth laterally with a tension band plate. Our aim was to evaluate the results of treatments in a consecutive group. After Institutional Review Board approval, data from 2002 to 2018 were collected. Thirty-nine knees (average age 22.4 months) with ITV/BD were treated with orthoses, and 10 knees failed. Six knees showed hyperintense T2-weighted signal in the medial proximal tibial epiphyseal cartilage on magnetic resonance imaging. Three of six knees with tibial osteotomy failed and underwent guided growth. Tibial plateau slopes were abnormal medially from the ITV/BD and laterally from the guided growth (triangular physis and depressed plateau deformities) because of factors such as orthotic treatment, tibial osteotomy, magnetic resonance imaging "physis severity score," and guided growth. (Journal of Surgical Orthopaedic Advances 29(3):141-148, 2020).

Improved distribution of leg forces after fibular resection and synostosis.

PURPOSE: Genu varum- a common symptom in achondroplasia- may lead to ankle valgus in children. Ankle valgus may be mitigated by tibiofibular synostosis, but this is not always the case. The mechanical environment around the growth plates plays an important role in ankle valgus. The purpose of this project is 1) to determine the strain through the distal fibular growth plate before and after tibiofibular synostosis, and 2) postulate whether the change in strain affects ankle valgus. This project measured the distal fibular strain in a porcine hind leg model. METHODS: The lower legs of seven pigs were removed, instrumented with strain gauges, and loaded compressively in a material testing machine. Loads were applied at three phases: 1) the intact leg, 2) leg with fibula resected, and 3) leg with fibula resected and tibiofibular synostosis. Mean strains were compared between phases using a mixed affect model. The significance level was adjusted for multiple comparisons using the Bonferroni method. RESULTS: Phase 1, intact leg, had the highest strain value at 1247.9µÉ›. In phase 2, the mean strain was 106.2µÉ›. In phase 3, the compressive mean strain dropped to 477.4µÉ›, which is 38% of the strain in phase 1. Standard error was 86.8µÉ›; p < 0.001. CONCLUSION: Results indicate that more of the force through the leg is transmitted through the tibia after fibular resection and tibiofibular synostosis, which improves the balance of forces through the leg.

Tension band plate-guided growth of knee-flexion deformity in arthrogryposis multiplex congenita in which metaphyseal funnelization induced screw encroachment upon the neurovascular bundle.

Knee-flexion deformity in arthrogryposis multiplex congenita is treated by serial casting into extension, distal femoral osteotomies, distal femoral-guided growth, hemiepiphysiodesis, external fixation, capsulotomy, and soft-tissue releases. We are aware of four cases treated by distal anterior femoral-guided growth with tension band plates in which an unreported complication occurred: the screws of the tension band plates penetrated the posterior cortex of the femur during remodeling with metaphyseal funnelization risking the neurovascular bundle. Inclusion criteria were cases with arthrogryposis multiplex congenita and knee-flexion deformity, treatment at our institution by distal anterior femoral-guided growth with tension band plates, and radiographic evidence of posterior cortex screw penetration during remodeling from growth. Six knees (four cases) met the inclusion criteria. The average age at the distal anterior femoral-guided growth with tension band plate operation was 5.8 years. Radiographs after 6.6 years of follow-up showed that the screws of the tension band plates, which at surgery were intrametaphyseal, had penetrated the posterior cortex of the femur. Four knees (two cases) had diffuse pain around the knee to lower leg area, and instrumentation removal alleviated the symptoms. During distal anterior femoral-guided growth with tension band plate operation for knee-flexion deformity in arthrogryposis multiplex congenita, we found that the screws of the tension band plates, which were initially located inside the metaphysis, may protrude through the posterior bone cortex during metaphyseal funnelization with growth, and may encroach upon the neurovascular tissues. Level of evidence: Level IV - case series.

Orthopaedic manifestations of glutaric acidemia Type 1.

PURPOSE: Glutaric acidemia type 1 (GA1), a rare hereditary metabolic disease caused by biallelic mutations of GCDH, can result in acute or insidious striatal degeneration within the first few years of life. We reviewed the orthopaedic sequelae and management of 114 neurologically injured patients with a confirmed molecular diagnosis of GA1. METHODS: We performed a retrospective chart review spanning 28 years identifying 114 GA1 patients, most from the Old Order Amish population of Lancaster County, Pennsylvania, who were homozygous for a pathogenic founder variant of GCDH (c.1262C>T). We collected demographics, medical comorbidities, muscle tone patterns, Gross Motor Function Classification System level, gastrostomy tube status, seizure history, inpatient events, orthopaedic diagnoses and operative characteristics. RESULTS: Over an average follow-up of 4.7 ± 3.4 years, 24 (21%) of 114 patients had musculoskeletal problems requiring orthopaedic consultation. Scoliosis (n = 14), hip dislocation (n = 8/15 hips), hip subluxation (n = 2/three hips), and windswept hip deformity (n = 2) in the spine and hip joint were most common. In total, 35 orthopaedic surgeries were performed in 17 (71%) patients. The most common primary operations were one-stage procedures with proximal femoral varus derotation osteotomy and/or pelvic osteotomy (n = 8/14 hips) for subluxation or dislocation. In all, 11 patients had posterior spinal fusion for severe scoliosis. With the recommended metabolic management, there were no disease-specific complications in this cohort. CONCLUSIONS: Children with GA1 who have static striatal lesions are at risk for musculoskeletal complications, especially scoliosis and hip dislocation, and appropriate operative management requires consultation with a metabolic specialist with specific considerations for fluid management and nutrition. LEVEL OF EVIDENCE: IV.

Anterior Hip Dislocation from Post-injection Gluteal Muscular Fibrosis: Case Report.

CASE: A female with 24 years of follow-up who received multiple intramuscular injections of penicillin in the buttock at age 2 years. By age 6 years, she had left hip subluxation and underwent operative soft-tissue release and femoral plating. At age 12 years, she developed progressive anterior hip dislocation and had release of the gluteal contracture, open reduction, and femoral osteotomy. Currently, she is aged 26 years, fully employed, and active at home. CONCLUSIONS: This is the first reported case associated with anterior hip dislocation after progressive post-injection gluteus muscle fibrosis with a follow-up of 24 years.

Developmental dysplasia of the hip and clubfoot treated by Pavlik and Ponseti methods.

Children having both typical developmental dysplasia of the hip (DDH) and clubfoot are rare, and early treatments of both conditions are recommended. The aim of this study was to evaluate the effects of the Ponseti method of clubfoot treatment on hips with DDH. After institutional review board approval, we identified children treated by the Ponseti and Pavlik methods between 2003 and 2016. During the Ponseti method treatment, the duration of manipulations, number of casts, tenotomies performed, and days in clubfoot orthosis were recorded. During DDH treatment, we registered duration for Pavlik and hip brace usage. Hips had dynamic sonography and radiographic evaluations. The cases were grouped according to the combination of DDH and clubfoot treatments: (a) concurrent, (b) sequential, and (c) hip observation. Seven cases of DDH and clubfoot were identified. The average number of Ponseti casts was 5.8 (range: 4-8 casts). The average number of days following the post-Achilles-tenotomy casting to the end of clubfoot bracing was 870 days (range: 90-1605 days). Eleven (four bilateral cases and three unilateral) clubfeet were corrected initially by the Ponseti method. The average number of days for Pavlik harness treatment was 74 (range: 10-126 days). Additionally, a hip orthosis was utilized in three children for an average of 131 days. At follow-up, all children had a high femoral neck-shaft angle averaging 152° (range: 144°-164°). One child (case 5) developed avascular necrosis of the femoral head, Kalamchi type I. Children with typical and nonsyndromic DDH and clubfoot treated by Pavlik harness and Ponseti methods are associated with abnormal hip development (coxa valga).

Orthopedic Manifestations of Bruck Syndrome: A Case Series with Intermediate to Long-term Follow-Up.

The aim of this study was to evaluate the association of contractures, fractures, and deformities in four patients with Bruck syndrome treated in our facility. Data were collected from medical records, radiographs, dual-energy X-ray absorptiometry (DEXA) scans, genetic tests, and gait analysis. All had contractures at birth and genotypic findings including mutations in PLOD2 or FPKB10. Three cases were treated with bisphosphonates with improvement in bone density verified by DEXA. In Bruck syndrome, orthopedic deformities include the following sequential aspects: contractures, characterized by upper and lower extremity contractures such as clubfeet; fractures, characterized by multiple diaphyseal fractures in the long bones of the extremities; and deformities, characterized by malalignment of extremities and the spine. Physical therapy and bracing proved helpful for the contractures to try to stop progression. Bone fragility needs to be considered when deciding to attempt cast correction. Surgeries in the soft tissues can be performed to retain joint movement. In fractures with angulation, intramedullary nail fixation was useful, and in cases without deformity, casting alone was successful. We suggest monitoring the bone density with DEXA, nutrition support with vitamin D and calcium, and treatment with bisphosphonates. Spine deformities were successfully treated by spinal fusion and instrumentation.