Special article: Update on the magnetically controlled growing rod: tips and pitfalls

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Next generation of growth-sparing techniques: preliminary clinical results of a magnetically controlled growing rod in 14 patients

Session 3A - Early Onset Scoliosis: Paper no. 33SUMMARY: Growth-sparing techniques are commonly used for the treatment of progressive EOS. The standard growing rod (GR) technique requires multiple surgeries for lengthening. The preliminary results of MCGR has shown the comparable outcomes to standard GR without the need for repeated surgery which can be expected to reduce the overall complication rate in GR surgery. INTRODUCTION: The growing rod (GR) technique for management of progressive Early-Onset Scoliosis (EOS) is a viable alternative but with a high complication rate attributed to frequent surgical lengthenings. The safety and efficacy of a non-invasive Magnetically Controlled Growing Rod (MCGR) has been previously reported in a porcine model. We are reporting the preliminary results of this technique in EOS. METHODS: Retrospective review of prospectively collected multi-center data. Only patients who underwent MCGR surgery and at least 3 subsequent spinal distractions were included in this preliminary review. Distractions were performed in clinic without anesthesia or analgesics. T1-T12 and T1-S1 height and the distraction distance inside the actuator were analyzed in addition to conventional clinical and radiographic data. RESULTS: Patients (N=14; 7 F and 7 M) had a mean age of 8y+10m (3y+6m to 12y+7m) and underwent a total of 14 index surgeries (SR: index single rod in 5 and DR: dual rod in 9) and 91 distractions. There were 5 idiopathic, 4 neuromuscular, 2 congenital, 2 syndromic and one NF. Mean follow-up (FU) was 10 months (5.8-18.2). Mean Cobb changed from 57° pre-op to 35° post-op and correction was maintained (35°) at latest FU. T1-T12 increased by 4 mm for SR and 10 mm for DR with mean monthly gain of 0.5 and 1.39, respectively. T1-S1 gain was 4 mm for SR and 17 mm for DR with mean monthly gain of 0.5 mm for SR and 2.35 mm for DR. The mean interval between index surgery and the first distraction was 66 days and thereafter was 43 days. Complications included one superficial infection in (SR), one prominent implant (DR) and minimal loss of initial distraction in three after index MCGR (all SR). Overall, partial loss of distraction was observed following 14 of the 91 distractions (one DR and 13 SR). This loss was regained in subsequent distractions. There was no neurologic deficit or implant failure. CONCLUSION: MCGR appears to be safe and provided adequate distraction similar to the standard GR technique without the need for repeated surgeries. DR patients had better initial curve correction and greater spinal height. No major complications were observed during the short follow-up period. The FDA has not cleared the drug and/or medical device for the use described in this presentation (i.e., the drug or medical device is being discussed for an ‘off label’ use).postprin

Painful rib hump: a new clinical sign for detecting intraspinal rib displacement in scoliosis due to neurofibromatosis

BACKGROUND: Spinal cord compression and associate neurological impairment is rare in patients with scoliosis and neurofibromatosis. Common reasons are vertebral subluxation, dislocation, angulation and tumorous lesions around the spinal canal. Only twelve cases of intraspinal rib dislocation have been reported in the literature. The aim of this report is to present a case of rib penetration through neural foramen at the apex of a scoliotic curve in neurofibromatosis and to introduce a new clinical sign for its detection. METHODS: A 13-year-old girl was evaluated for progressive left thoracic kyphoscoliotic curve due to a type I neurofibromatosis. Clinical examination revealed multiple large thoracic and abdominal "cafe-au-lait" spots, neurological impairment of the lower limbs and the presence of a thoracic gibbous that was painful to pressure at the level of the left eighth rib (Painful Rib Hump). CT-scan showed detachment and translocation of the cephalic end of the left eighth rib into the adjacent enlarged neural foramen. The M.R.I. examination of the spine showed neither cord abnormality nor neurogenic tumor. RESULTS: The patient underwent resection of the intraspinal mobile eighth rib head and posterior spinal instrumentation and was neurologically fully recovered six months postoperatively. CONCLUSION: Spine surgeons should be aware of intraspinal rib displacement in scoliotic curves in neurofibromatosis. Painful rib hump is a valuable diagnostic tool for this rare clinical entity

Rib head protrusion into the central canal in type 1 neurofibromatosis

Intraspinal rib head dislocation is an important but under-recognized consequence of dystrophic scoliosis in patients with neurofibromatosis 1 (NF1). To present clinical and imaging findings of intraspinal rib head dislocation in NF1. We retrospectively reviewed clinical presentation, imaging, operative reports and post-operative courses in four NF1 patients with intraspinal rib head dislocation and dystrophic scoliosis. We also reviewed 17 cases from the English literature. In each of our four cases of intraspinal rib head dislocation, a single rib head was dislocated on the convex apex of the curve, most often in the mid- to lower thoracic region. Cord compression occurred in half of these patients. Analysis of the literature yielded similar findings. Only three cases in the literature demonstrates the MRI appearance of this entity; most employ CT. All of our cases include both MRI and CT; we review the subtle findings on MRI. Although intraspinal rib head dislocation is readily apparent on CT, sometimes MRI is the only cross-sectional imaging performed. It is essential that radiologists become familiar with this entity, as subtle findings have significant implications for surgical management

Atlanto-axial rotatory fixation in a girl with Spondylocarpotarsal synostosis syndrome

We report a 15-year-old girl who presented with spinal malsegmentation, associated with other skeletal anomalies. The spinal malsegmentation was subsequently discovered to be part of the spondylocarpotarsal synostosis syndrome. In addition, a distinctive craniocervical malformation was identified, which included atlanto-axial rotatory fixation. The clinical and the radiographic findings are described, and we emphasise the importance of computerised tomography to characterize the craniocervical malformation complex. To the best of our knowledge, this is the first clinical report of a child with spondylocarpotarsal synostosis associated with atlanto-axial rotatory fixation

Surgical treatment of scoliosis: a review of techniques currently applied

In this review, basic knowledge and recent innovation of surgical treatment for scoliosis will be described. Surgical treatment for scoliosis is indicated, in general, for the curve exceeding 45 or 50 degrees by the Cobb's method on the ground that