The natural history of spinal deformity in patients with Coffin-Lowry syndrome.

PURPOSE: Little is known about the natural history of spinal deformities in Coffin-Lowry syndrome (CLS). Our goal was to evaluate the spinal deformity progression and clinical impact. METHODS: In this institutional review board-approved study, we performed a multinational retrospective review of six male CLS patients, aged 13 to 22 years at final follow-up, for a mean of 7.25 years (3 to 13). RESULTS: All showed delayed skeletal maturity. Three had calcifications of their lower cervical ligamentum flavum, all experienced neural axis abnormalities, including lower extremity weakness, numbness and tingling and in one, quadriparesis. Only two were ambulatory at final follow-up.All had significant spinal abnormalities, including severe progressive thoracic lordosis, thoracolumbar kyphosis and scoliosis. All had undergone spinal fusion or were being evaluated for surgery. CONCLUSION: CLS is a rare X-linked mutation in the RSK2 gene, affecting between 1/50 000 to 100 000 people. There are two reports in the literature of patients with calcifications of their ligamentum flavum. Both had neural axis abnormalities and one had acute onset quadriplegia. Analysis of their ligamentum flavum found abundant central calcifications. Despite our small cohort we found 50% had calcifications and 100% had neurologic consequences associated with those calcifications. There was a 100% rate of deformity progression.They all exhibited delay in skeletal maturity, which mandates longer follow-up and has implications for surgical planning.From our cohort and literature review, the natural history of CLS supports frequent patient evaluation and a lower threshold for correction of spinal deformities. Aiming to avoid spinal cord compression and improve or avoid neurological deterioration. LEVEL OF EVIDENCE: IV - retrospective study.

Pedicle morphology in thoracic adolescent idiopathic scoliosis: is pedicle fixation an anatomically viable technique?

STUDY DESIGN: A radiographic study of thoracic pedicle anatomy in a group of adolescent idiopathic scoliosis (AIS) patients. OBJECTIVE: To investigate the anatomic constraints of the thoracic pedicles and determine whether the local anatomy would routinely allow pedicle screw insertion at every level. SUMMARY OF BACKGROUND DATA: In spite of the clinical successes reported with limited thoracic pedicle screw-rod constructs for thoracic AIS, controversy exists as to the safety of this technique. MATERIAL AND METHODS: Twenty-nine patients with right thoracic AIS underwent preoperative thoracic CT scans and plain radiographs. Anatomic parameters were measured from T1 to T12. RESULTS: Information on 512 pedicles was obtained. The transverse width of the pedicles from T1 through T12 ranged from 4.6-8.25 mm. The medial pedicle to lateral rib wall transverse width from T1 through T2 ranged from 12.6 to 17.9 mm. Measured dimensions from the CT scans showed the actual pedicle width to be 1-2 mm larger than would have been predicted from the plain radiographs. Age, Risser grade, curve magnitude, and the amount of segmental axial rotation did not correlate with the morphology or size of the thoracic pedicles investigated. In no case would pedicle morphology have precluded the passage of a pedicle screw. CONCLUSION: Based on the data identified in this group of adolescent patients, it is reasonable to consider pedicle screw insertion at most levels and pedicle-rib fixation at all levels of the thoracic spine during the treatment of thoracic AIS.

Aneurysmal bone cysts of the spine: excision and stabilization.

The treatment of aneurysmal bone cysts (ABCs) of the spine remains controversial in the literature. Treatment options have included radiation, curettage and bone graft, extirpation, and various combinations of these. Conspicuously missing in previously published articles and texts are guidelines for dealing with the instability and deformity that often accompany ABCs of the spine. The index case in this report highlights the potentially devastating effects of treating the tumor in isolation without addressing the concomitant deformity and instability. The status of the structural integrity of the spine must be assessed before initiating treatment. If instability or deformity or both are already present or if the amount of osseous tissue to be resected may render the spine unstable, then instrumentation and fusion should be performed at the time of surgical resection or before other forms of therapy. We present three cases of ABCs of the spine in which the tumor itself was treated with surgical extirpation and the associated deformity and instability were treated with spinal instrumentation and long fusions.

Technical aspects and nursing considerations of limb lengthening.

Limb lengthening in children is a complex undertaking which involves a technically proficient surgeon, experienced nurses and a multidisciplinary health care team. The decisions surrounding a limb lengthening process in children require a thorough and individual approach. This article provides an overview of the detailed planning and implementation of the limb lengthening process. Included will be discussion of patient selection, surgical planning, nursing interventions and overall involvement of the health care team in the multidisciplinary approach.

The Luque trolley revisited. Review of nine cases requiring revision.

This study is a retrospective review of nine patients who underwent Luque instrumentation without fusion from 1982-1984. Average age at surgery was 9 years. Average preoperative curve was 51 degrees (30 degrees-70 degrees). All nine patients have had at least one revision. All of the revisions were technically difficult secondary to extensive fibrosis and weakened laminar bone. Spontaneous fusion was documented in all nine patients, limiting further correction. Final follow-up curves averaged 51 degrees (25 degrees-90 degrees). Average gain in spinal height was 5.8 cm (2.3 in) but only a small portion was derived from the instrumented levels. Segmental spinal instrumentation without fusion in immature patients was not effective in control of spinal deformity, nor did it allow anticipated growth under the instrumented regions.

Pseudoaneurysm after foot surgery.

Pseudoaneurysm (PA) is recognized as a rare complication after pediatric foot surgery. We identified the incidence, pertinent clinical features, and response to surgical intervention in PA as a complication of foot surgery. Four PAs were identified after 2,756 foot operations, an overall incidence of 0.14%. These patients typically had symptoms between 2 and 3 months after index operation with an enlarging, pulsatile, compressible mass in the plantar medial aspect of the foot. Arteriography was helpful in planning surgical intervention. Operative treatment consisted of ligation and excision of PA in all patients. Final outcome of foot deformity surgery was not compromised.

An evaluation of spinal deformity in metastatic breast cancer.

Between October 1984 and January 1988 31 magnetic resonance (MR) imaging studies were performed on 27 patients with metastatic vertebral breast cancer (MVBC). The MR images were reviewed to determine the extent and type of sagittal spinal deformity, and whether spinal canal compromise was present. Adjunct studies were compared to determine the pathogenesis of spinal deformity and the etiology of spinal canal compromise. An analysis of the data revealed that a consistent pattern of sagittal spinal deformity exists with MVBC, and a classification system was developed to describe the stages of vertebral deformity. Criteria are suggested for identifying metastatic spinal instability. A protocol is presented for treating patients with metastatic spinal involvement. By understanding the natural history of metastatic spinal deformity, instability and spinal canal compromise can be recognized and treated early, before the onset of progressive deformity and neurologic sequelae.