High-grade spondyloretrolisthesis in a 12-year-old girl with neurofibromatosis type 1: a case report and literature review

Neurofibromatosis (NF) type 1 is characterized by several skin, endocrine, central nervous system and musculoskeletal manifestations, spine deformities being the most common, affecting up to 64% of patients. Thoracic kyphoscoliosis is the most common deformity observed; however, high-grade spondylolisthesis and dural defects such as dural ectasia can also be found. The aim of this study is to describe a case of high-grade spondyloretrolisthesis in an NF-1 patient, associated with dural ectasia and extensive lumbar laminectomies, and to discuss our management and review the current literature on this controversial topic. A 12-year-old girl with NF-1 who had undergone extensive lumbar laminectomies in an outside facility presented to our emergency department complaining of back pain and lower limbs upper motor neuron symptoms. Image studies showed a high-grade lumbar spondyloretrolisthesis associated with dural ectasia. The first step of treatment was spine immobilization using a Boston brace. An anterior approach was used, and an L2 corporectomy was performed, using a Moss type cage between L1 and L3 with an instrumented arthrodesis and autologous bone graft for stabilization purposes. The second step planned was a posterior approach for arthrodesis and instrumentation, but after an extensive discussion with the parents and the patient, the parents did not agree to the procedure planned for the patient. A brace was used for 1 year while rehabilitation was performed. At the 1-year follow-up, there was a 70° kyphosis at the thoracolumbar junction but it was clinically stable, with an acceptable sagittal balance. Dural ectasia is not a common finding in children with NF-1; however, it should be identified as its presence may predispose to spine instability and as a consequence the development of a high-grade spondyloretrolisthesis. Even though a few cases have been reported, we believe that it is important to consider the option of a double approach to achieve a better correction in both planes and a good outcome. If an extensive laminectomy is involved, it is mandatory to perform a posterior fusion and instrumentation

Ewing sarcoma of clavicle in children: report of 5 cases

Ewing sarcoma accounts for about 2% to 3% of childhood tumors and can occur in any bone, but it is most often found in extremities and central axis. Ewing sarcoma affecting clavicle is uncommon. We report 5 cases of Ewing sarcoma of clavicle treated in our institution. The mean age at the time of diagnosis was 11.6 years (2 to 15 y). There were 3 males and 2 females. All patients received chemotherapy before and after the surgery. Likewise, radiotherapy was administrated after tumor excision in one patient. Different methods of surgical treatment were used and included: claviculectomy, claviculectomy and vascularized fibular autograft, and claviculectomy and clavicle allograft. One patient died during the follow-up because of the recurrence of the tumor and presence of metastatic disease. The remaining 4 patients had good functional outcomes with no pain of the shoulder at final follow-up. In conclusion, Ewing sarcoma of clavicle is a very rare tumor and different methods of surgical treatment can be used. Clavicle often can be resected with no need for reconstruction, especially in young children. Autografts or allografts can restore the normal anatomy of the shoulder

Simultaneous juxta-epiphyseal proximal phalanx fracture with flexor tendon entrapment in a child: a case report and review of literature

Juxta-epiphyseal/Salter-Harris fractures are the most common hand fractures in children and the proximal phalanx is involved in most cases. In the absence of soft-tissue interposition, these growth plate injuries are simple to reduce and are stable. However, in some cases, flexor tendon entrapment could be present. We report on an 11-year-old girl who sustained a fall onto her outstretched hand with subsequent injuries in her long, ring, and small fingers. Plain radiographs showed a severely displaced juxta-epiphyseal proximal phalanx fracture in her ring finger associated with mildly displaced juxta-epiphyseal proximal phalanx fractures of the long and small fingers. Fracture reduction could not be achieved after a closed reduction attempt. An open reduction and stabilization using Kirschner wires was performed in the fourth and fifth fingers, because of entrapment of the flexor digitorum profundus tendon. Excellent functional as well as radiological outcomes were achieved. These types of injuries are very uncommon and a high index of suspicion on the basis of clinical as well as radiological findings is needed to make an early diagnosis and for adequate treatment. Multiple proximal phalangeal fractures could be associated with the simultaneous entrapment of flexor tendons in different fingers as in our case; this is important to keep in mind as it is useful when planning the definitive surgical treatment and doing so will have a positive impact on the final functional as well as radiological outcomes

Complications and associated risk factors at screw removal in slipped capital femoral epiphysis treated by cannulated stainless steel screws

PURPOSE: The aim of our study is to report our complication rate and analyze the associated risk factors when removing cannulated stainless steel screws for SCFE fixation. METHODS: This was a multicenter retrospective study of patients who underwent removal of cannulated stainless steel screws after a mean time of 2.03 years of implantation. Thirty-two patients were included (38 hips) with a mean of 13.7 years of age during screw removal surgery. The mean post-removal follow up time was 1.6 years. In all cases the removal of screws was done systematically. Demographic data, possible risk factors related to removal failure, as well as post-removal complications such as post-removal fractures, infections and scar issues were recorded. RESULTS: A removal failure rate of 15.79 % (6/38) was found. The removal surgical time was longer than the initial fixation time but without statistical significance (70.78 vs 61.84 m, p = 0.196). However, the duration of screw implantation (r(2): 7.09; IC: 1.12–13.06) and screw head bony coverage (r(2): 21.32; IC: 5.58–37.06) were both related to this prolonged time. Multivariant analysis revealed that a fully threaded cannulated screw had the lowest removal failure risk (OR: 0.3; IC: 0.14–0.61). There were no postremoval complications recorded. CONCLUSIONS: We recommend to use full threaded cannulated stainless steel screws and to perform the procedure as soon as the physis are closed to decrease the surgical time. It is a safe procedure based on a low rate of complications such as post-removal fractures, infection and scar issues

Distal radius definitive epiphysiodesis for management of Kienböck´s disease in skeletally immature patients

PURPOSE: Kienböck’s disease is an unusual pathology with unknown aetiology within the pediatric population. Several treatment options have been described in the literature; however, there are neither large series nor homogeneous studies that have clarified which treatment is best. The aim of this study was to describe five cases in which definitive distal radius epiphysiodesis was performed as an alternative method to the classic distal radius shortening osteotomy in skeletally immature patients with Kienböck’s disease. METHODS: The clinical charts and radiographs of four patients (five wrists) diagnosed as having Kienböck’s disease and treated by definitive distal radius epiphysiodesis were reviewed. RESULTS: All patients were symptomatically free with full range of motion at 4.25 months (range, three to six months) after surgery. In all cases, shortening of the distal radius was achieved (p = 0.032). CONCLUSIONS: This new technique is a simple, effective, and minimally invasive procedure with low morbidity and good clinical and radiological outcomes