An original preoperative orthopaedic management of a complex spinal deformity in a 16-year-old patient with spondyloepiphyseal dysplasia congenita.

PURPOSE: Spinal deformities in patients with Spondyloepiphyseal Dysplasia congenita are particularly challenging to treat. Addressing these deformities requires a holistic surgical strategy. The aim of this case report is to highlight an original preoperative protocol combining atlantoaxial instability stabilization by halo jacket with progressive correction of a thoracic kyphoscoliosis using continuous elongation by tension plaster cast as described by Stagnara. METHOD: A 16-year-old patient with spondyloepiphyseal dysplasia congenita presenting a severe thoracic kyphoscoliosis associated with atlantoaxial instability causing cervical myelopathy was managed through a preoperative protocol combining a halo jacket with a Stagnara elongation cast allowing progressive correction of the kyphoscoliosis while stabilizing the cervical instability. RESULTS: The preoperative protocol allowed a safer and a more effective surgical spine fusion, reducing neurological risks, improving the final correction, and offering better postoperative recovery. Clinical and radiological solid fusion was observed at 3-year-follow-up with significant improvement of the patient's quality of life. CONCLUSION: This case report highlights the effectiveness of the preoperative preparation combining a halo jacket with a Stagnara elongation cast to safely stabilize the cervical spine while correcting the thoracic kyphoscoliosis. This strategy can serve as a valuable tool for spine teams when addressing complex spinal deformities particularly in this patient with SEDc. LEVEL OF EVIDENCE: Level IV.

Reconstruction of isolated hypoplasia of the patella by a modified Galeazzi procedure: a case report.

BACKGROUND: Isolated Patellar Aplasia Hypoplasia is a very rare autosomal dominant disorder. Its treatment depends on the clinical manifestations that can vary widely. The lack of active extension, which can be responsible for frequent falls due to a knee instability, is the most frequent and disabling manifestation. We report an original technique that is a modification of the Galeazzi technique for recurrent dislocation of the patella to gain active extension in case of PTLAH. CASE REPORT: A 7-year-old Caucasian boy with isolated Patellar Aplasia Hypoplasia and an extension lag of the right knee has been treated by a modified Galeazzi technique. The tendons of the semi-tendinous and gracilis muscles have been harvested and their distal insertion was kept intact. Both tendons were fixed over the top of the patella to restore knee active extension. After 6 years of follow up the patient is symptom free with a strong active extension of the operated knee. CONCLUSION: Reconstruction of isolated hypoplasia of the patella by a modified Galeazzi procedure is a safe and reliable technique for skeletally immature patients offering satisfying long-term outcomes.

Growing rods for early-onset scoliosis in Ehlers-Danlos disease.

PURPOSE: To study the results and complications of Traditional Growing Rods (TGR) for the treatment of Early-Onset Scoliosis (EOS) in patients with Ehlers-Danlos syndrome (EDS). METHODS: This is a retrospective study of patients with EDS treated for EOS between 2012 and 2018 by TGR. For each patient, we evaluated the evolution of the Cobb angle of the main coronal curve before the first surgery, postoperatively and at the last follow-up. We also noted every complication. RESULTS: Four patients have been evaluated. Age at the index surgery ranged from 3- to 6-year-old. All the patients had a Kyphoscoliosis. The preoperative Cobb angle of the main coronal curve ranged from 70° to 104°. Prior to surgery, a progressive correction of the spinal deformity by Halo-gravity traction was performed for all patients. One patient had several complications with a poor result after final fusion. Three patients did not sustain any complication and presented good results. Follow-up ranged from 3 to 8 years. For two of our patients, further lengthening procedures are still needed. CONCLUSIONS: TGR is a good surgical option for the treatment of EOS in EDS. A progressive preoperative correction is recommended. Anchor-related complications must be minimized in these patients with an important kyphosis. Level of evidence Level IV.

Congenital pseudarthrosis of the forearm treated with induced membrane technique: A case report.

Congenital pseudarthrosis of the forearm (CPF) is an uncommon entity frequently described in association with neurofibromatosis type 1. Surgical treatment Is required. This is a challenging pathology in pediatric orthopedic surgery due to the difficulty of achieving a satisfying result in terms of bone defect healing. Many techniques are described such us free vascularized fibula, illizarov and induced membrane. We describe a first case of the forearm non union associated with neurofibromatosis type 1 successfully treated with 2 steps of induced membrane. Whatever the technique selected it's remain challenging problem in pediatric orthopaedic.

Kienböck's disease in an 11-year-old girl: a case report.

Kienböck disease is a pathology that remains uncommon in children, therefore the number of published cases of Kienböck's disease before skeletal maturity is limited. The etiology of Kienböck's disease is still controversial. Although many therapeutic methods are described in the literature. There is no consensus treatment for this pathology. We describe a case of Kienböck's disease of an 11-year-old girl who presented with avascular necrosis of the lunate bone confirmed by the radiologic pattern. And who was treated with 10 weeks of splinting with satisfying clinical outcome. Wrist pain and other symptoms resolved after 2 months. A magnetic resonance imaging (MRI) confirmed partial revascularization of the lunate. After 18 months of follow-up the patient remains asymptomatic.