Combined two-centre experience of single-entry telescopic rods identifies characteristic modes of failure.

AIMS: The Fassier Duval (FD) rod is a third-generation telescopic implant for children with osteogenesis imperfecta (OI). Threaded fixation enables proximal insertion without opening the knee or ankle joint. We have reviewed our combined two-centre experience with this implant. METHODS: In total, 34 children with a mean age of five years (1 to 14) with severe OI have undergone rodding of 72 lower limb long bones (27 tibial, 45 femoral) for recurrent fractures with progressive deformity despite optimized bone health and bisphosphonate therapy. Data were collected prospectively, with 1.5 to 11 years follow-up. RESULTS: A total of 24 patients (33%) required exchange of implants (14 femora and ten tibiae) including 11 rods bending with refracture. Four (5%) required reoperation with implant retention. Loss of proximal fixation in the femur and distal fixation in the tibia were common. Four patients developed coxa vara requiring surgical correction. In total, 13 patients experienced further fractures without rod bending; eight required implant revision. There was one deep infection. The five-year survival rate, with rod revision as the endpoint, was 63% (95% confidence interval (CI) 44% to 77%) for femoral rods, with a mean age at implantation of 4.8 years (1.3 to 14.8), and 64% (95% CI 36% to 82%) for tibial rods, with a mean age at implantation of 5.2 years (2.0 to 13.8). CONCLUSION: FD rods are easier to implant but do not improve on the revision rates reported for second generation T-piece rods. Proximal femoral fixation is problematic in younger children with a partially ossified greater trochanter. Distal tibial fixation typically fails after two years. Future generation implants should address proximal femoral and distal tibial fixation to avoid the majority of complications in this series. Cite this article: Bone Joint J 2020;102-B(8):1048-1055.

A Modification of the Dunn Osteotomy With Preservation of the Ligamentum Teres.

BACKGROUND: We aimed to determine if a modified Dunn osteotomy could be safely performed without surgical dislocation and consequent preservation of the ligamentum teres. METHODS: All patients undergoing a modified Dunn osteotomy for a slipped capital femoral epiphysis over an 8-year period were included in this study, and all had a severe slip with an open physis. RESULTS: The modified Dunn procedure was performed on 34 hips in 34 patients. The mean age was 13.1 years (range, 11 to 16 y) with a mean follow-up time of 54 months (range, 15 to 102 mo). All slips were severe (grade 3) with a mean slip angle of 73.2 degrees (range, 60 to 90 degrees). Nineteen slips were stable and 15 were unstable. Of the unstable slips, the average time from initial presentation to the emergency department until surgery was 9.4 days (range, 2 to 42 d). Excluding 1 patient who developed complete collapse of the femoral head (NAHS 56), the average Nonarthritic Hip score was 98 (range, 93.7 to 100). Four (11.8%) patients developed avascular necrosis of the femoral head, of which 3 were unstable slips. CONCLUSIONS: A modified Dunn osteotomy with preservation of the ligamentum teres allows an excellent restoration of the anatomic alignment of the femoral head and neck. Rates of AVN are not increased compared with other techniques of subcapital osteotomy but this complication cannot be eliminated particularly in patients with unstable slips. LEVEL OF EVIDENCE: Level III.

Lower Extremity Deformity Management in MPS IVA, Morquio-Brailsford Syndrome: Preliminary Report of Hemiepiphysiodesis Correction of Genu Valgum.

BACKGROUND: Morquio-Brailsford syndrome or mucopolysaccharidosis type IV-A (MPS IV-A) is an inherited metabolic disease in which skeletal deformities can be extreme. An international registry has shown that 46% of patients are unable to walk 200 m and 30% use a wheelchair. Lower limb surgery is performed to restore alignment with the expectation of maintaining mobility. We are, however, not aware of reports correlating function with alignment. This study assesses the role of orthopaedic intervention with reference to anatomic and functional outcome in MPS IV-A: specifically hemiepiphysiodesis correction of genu valgum. METHODS: Interrogation of the MPS patient database identified 63 patients. Patient demographic and radiographic data were collected along with information on deformity, correction, mobility, and genotype. Genu valgum was assessed by intermalleolar distance and mobility by a standardized 6-minute walk test. Sequential hip anatomy was assessed radiographically. RESULTS: Twenty-six surgical hemiepiphysiodesis episodes were performed on 23 patients. There were no cases of implant failure, loosening, or infection. The average intermalleolar distance reduction was 6.12 cm (paired t test, P=0.0001) (95% confidence interval, 7.6-4.7 cm) and the mean change in 6-minute walk test was +69.5 m (P=0.0339). There was no correlation between hip subluxation/dislocation and mobility (Fisher exact test, P=1.000), although hip parameters deteriorated over time. Three patients required repeat 8-plate insertion and 1 a femoral osteotomy. CONCLUSIONS: Eight-plate hemiepiphysiodesis correction of genu valgum in MPS IV-A is an effective treatment with low complication rates. The insult of surgery is minimal compared with that of osteotomy which can reduce mobility further. Our outcomes suggest that mobility is maintained or improved even in severe genotypes where mobility typically worsens. We have found hip pathology to be less debilitating, but further studies are needed to assess the effect of genu valgum correction upon femoral head subluxation and collapse.We suggest hemiepiphysiodesis should be considered as part of the treatment strategy for limb alignment in MPS IV-A patients, depending on remaining growth, mobility status, and genotype. LEVEL OF EVIDENCE: Level IV-therapeutic case series.

Orthopedic management of the extremities in patients with Morquio A syndrome.

BACKGROUND: Musculoskeletal involvement in Morquio A syndrome (mucopolysaccharidosis IVA; MPS IVA) contributes significantly to morbidity and mortality. While the spinal manifestations of the disorder have received considerable attention in the literature, there have been few reported studies to date to guide the management of the orthopedic problems associated with the lower and upper extremities. PURPOSE: The objective was to develop recommendations for the management of the extremities in patients with Morquio A syndrome. METHODS: A group of specialists in orthopedics, pediatrics and genetics with experience in the management of Morquio A patients convened to review and discuss current clinical practices and to develop preliminary recommendations. Evidence from the literature was retrieved. Recommendations were further refined until consensus was reached. RESULTS AND CONCLUSIONS: This present article provides a detailed review and discussion of the lower and upper extremity deformities in Morquio A syndrome and presents recommendations for the assessment and treatment of these complications. Key issues, including the importance of early diagnosis and the implications of medical therapy, are also addressed. The recommendations herein represent an attempt to develop a uniform and practical approach to managing patients with Morquio A syndrome and improving their outcomes.