The role of melatonin in the pathogenesis of adolescent idiopathic scoliosis (AIS).

The cause of adolescent idiopathic scoliosis (AIS) in humans remains obscure and probably multifactorial. At present, there is no proven method or test available to identify children or adolescent at risk of developing AIS or identify which of the affected individuals are at risk of progression. Reported associations are linked in pathogenesis rather than etiologic factors. Melatonin may play a role in the pathogenesis of scoliosis (neuroendocrine hypothesis), but at present, the data available cannot clearly show the role of melatonin in producing scoliosis in humans. The data regarding human melatonin levels are mixed at best, and the melatonin deficiency as a causative factor in the etiology of scoliosis cannot be supported. It will be an important issue of future research to investigate the role of melatonin in human biology, the clinical efficacy, and safety of melatonin under different pathological situations. Research is needed to better define the role of all factors in AIS development.

Uninstrumented posterolateral spinal arthrodesis: is it the gold standard technique for I degrees and II degrees grade spondylolisthesis in adolescence?

We retrospectively reviewed the outcome of uninstrumented posterolateral spinal arthrodesis in 49 patients with lumbar isthmic spondylolisthesis grades I degrees and II degrees in adolescent patients in the time of surgery, who participate at follow-up, between 1980 and 1995. The goal of our study is to analyse the clinical and radiographic imaging at long follow-up in uninstrumented posterolateral arthrodesis and to evaluate the efficiency and the validity of surgical technique in young patients (<18 years). All patients had failed previous conservative treatment. The average age at follow-up was 33.5 years (range 25-42 years) and the average follow-up time was 19.7 years (range 12-27 years). The clinical outcome measures were the Oswestry Disability Index, the SF-36, and the visual analogic score. All measures assessed the endpoint outcomes at 20 years after surgery. The outcome of spinal fusion was good with 43 (87.7%) patients attaining solid fusion, pseudoarthrosis in 6 patients (12.3%). None of our patients complained of excessive postoperative wound pain. Additionally, no complications, such as wound infection, were encountered. Satisfactory results were obtained in 94% of patients and this was closely associated with the rate of successful fusion. The results suggest that clinical outcome is closely related to the attainment of solid fusion.

Evaluation of conservative treatment of non specific spondylodiscitis.

The objective of this study was to analyse the presentation, aetiology, conservative management, and outcome of non-tuberculous pyogenic spinal infection in adults. We performed a retrospective review of 56 patients (35 women and 21 men) of pyogenic spinal infection presenting over a 7-year period (1999-2006) to the Department of Spinal Surgery of Hesperia Hospital. The medical records, radiologic imaging, bacteriology results, treatment, and complications of all patients were reviewed. The mean age at presentation was 47.8 years (age range 35-72 years), the mean follow-up duration was 12.5 months. The most common site of infection was lumbar spine (n: 48), followed by the thoracic spine (n: 8). Most patients were symptomatic for between 4 and 10 weeks before presenting to hospital. The frequently isolated pathogen was Staphylococcus aureus in 31 of 56 cases (57.6%). Percutaneous biopsies were diagnostic in 57% of patients; the open biopsy was indicated if closed biopsy failed and when the infection was not accessible by percutaneous technique. The patients were managed by conservative measures alone, including antibiotic therapy and spinal bracing. The mean period of antibiotic therapy was 8.5 weeks (range 6-9 weeks), followed by oral antibiotics for 6 weeks. All patients had a supportive spinal brace for mean 8 weeks (range 6-10 weeks). The duration of the administration of oral antibiotics was dependent on clinical and laboratory evidence (white cell count, erythrocyte sedimentation rate, C-reactive protein) that the infection was resolved. The follow-up MR gadolinium scans were essential to monitor the response to medical treatment. The diagnosis of pyogenic spinal infection should be considered in any patient presenting with severe localised unremitting back and neck pain, especially when accompanied with systemic features, such as fever and weight loss and in the presence of elevated inflammatory markers. The conservative management of infection with antibiotic therapy and spinal bracing was very successful.

Late post-operative paraparesis after rib penetration of the spinal canal in a patient with neurofibromatous scoliosis.

Rib displacement into the spinal canal is a rare cause of paraplegia or paraparesis in patients affected by neurofibromatous scoliosis. We describe a case of paraparesis in a 14-year-old child affected by neurofibromatous dystrophic kyphoscoliosis, treated with combined posterior and anterior spinal arthrodesis. Seventeen days after the surgical treatment the patient developed clinical signs and symptoms of paraparesis. A CT scan showed the head of the fifth rib protruding into the spinal canal with cord compression. Rib resection and posterior cord decompression were carried out following complete neurological recovery.

Surgical treatment of spinal deformities in Duchenne muscular dystrophy: a long term follow-up study.

BACKGROUND: Surgical treatment of spinal deformities in Duchenne muscular dystrophy (DMD) is influenced by a number of factors which have proven to be a difficult challenge. Each case should be carefully evaluated, considering not only the natural history of the spinal deformity, but also the patient's general condition. These should be thoroughly assessed through clinical and radiographic investigations together with other medical specialists. Life expectancy should be determined according to the cardio-respiratory function, and both preoperative and postoperative quality of life should be taken into consideration, trying to imagine the functional status of each patient after surgery. METHODS: From February 1985 to February 2000, 58 patients with spinal deformity in DMD were surgically treated. Of 25 patients that were operated on between 1985 and 1995, only 20 were followed-up after 5 years because 5 of them had died during this time. Therefore, the present study focuses on the results obtained in 20 cases. The 20 cases reviewed presented with a mean angular value of scoliosis equal to 48 degrees (range 10-92 degrees). Spinal fusion with our modified Luque technique [6] was performed in 19 cases, whereas CD instrumentation was applied in only one case. RESULTS: At the 5 year follow-up (range 5.6-10 years), the age ranged from 18 to 24 years and averaged 20.4 years. The postoperative angular value of scoliosis averaged 22 degrees (58%, range 0-43 degrees), the mean correction at follow-up was 28 degrees (range 0-60 degrees), and the mean loss of correction was equal to 6 degrees (range, 0-11 degrees). Vital capacity showed a slow progression, slightly inferior to its natural evolution in untreated patients. The severest complication was the death that occurred in one of the patients. CONCLUSIONS: According to the present study, an early surgery (angular value lower than 35-40 degrees) dramatically reduces the rate of risk factors associated with spinal deformities in DMD, and its advantages far exceed the disadvantages, above all in terms of quality of life.

Surgical correction of dystrophic spinal curves in neurofibromatosis. A review of 56 patients.

STUDY DESIGN: A presentation of the results from 56 patients with dystrophic spinal deformities caused by neurofibromatosis surgically managed from 1971 to 1992. OBJECTIVES: To focus on the need for combined anterior and posterior fusion in the presence of severe spinal dystrophic changes. SUMMARY OF BACKGROUND DATA: It has been stated that the most effective management for dystrophic curves is early and aggressive surgery. METHODS: The patients were divided into two groups: Type I scoliosis (kyphosis < 50 degrees) and Type II kyphoscoliosis (kyphosis > 50 degrees). Results were evaluated in relation to the type of surgery performed: single posterior instrumented fusion or preplanned combined anterior and posterior fusion. RESULTS: At a mean follow-up period of 15 years (range, 5-22 years), all patients appeared to be stabilized, after a total of 120 surgical interventions. In Group I, the posterior instrumented fusion failed in nine patients (47%), and in Group II it failed in seven patients (63%). The preplanned combined anterior and posterior fusion failed in two patients (33%) in Group I and in four patients (20%) in Group II. The failure incidence of the posterior instrumented fusion alone and of the planned anterior and posterior fusion was 53% (16 patients) and 23% (6 patients), respectively. CONCLUSIONS: The severe dystrophic curve with anterior vertebral scalloping always requires combined anterior and posterior stabilization, particularly in younger patients, even if the sagittal curves have not become pathologic by the time of presentation.

Surgical treatment of scoliosis associated with myelomeningocele.

Twenty-nine patients (mean age 12 years) with severe thoracolumbar and lumbar scoliosis due to myelomeningocele were treated by spinal fusion (7 by posterior arthrodesis with instrumentation, 3 by anterior arthrodesis with instrumentation, 19 by combined anterior and posterior fusion with instrumentation). Fusion was extended to the sacrum in 15 patients. Mean period of follow-up was 6.2 years. The average Cobb angle changes were as follows: thoracic and thoracolumbar curves preoperatively 86 degrees to 45 degrees at follow-up (the final average curve correction was 47%); lumbar curves preoperatively 97 degrees to 48 degrees at follow-up (the final average curve correction was 50%). Average pelvis obliquity changed from 26 degrees to 13 degrees at follow-up with an average correction of 49%. The combined anterior and posterior instrumentation and fusion gave the best correction of deformity (the final average thoracic and thoracolumbar curve correction was 55%; the final average lumbar curve correction was 61%). Independent of the method of stabilization, post-operative wound infection was a serious problem (24%). The combined fusion-instrumentation method reduced the rate of pseudoarthrosis to 14%.

Neurological complications in segmental spinal instrumentation: analysis of 750 patients.

The authors report on neurological damage caused by the use of sublaminar segmental fixation in the correction of vertebral deformities. Three groups were reviewed: 600 patients instrumented with Harrington rods and segmental wiring, 50 patients treated with the Hartshill system and 100 patients instrumented with Luque bars. All of the patients were operated on using sublaminar wiring fixation. We report two transitory neurological complications among the 600 patients with Harrington rod instrumentation and segmental wiring, two permanent neurological deficits among the 50 cases treated with the Hartshill system and none among the 100 patients instrumented using Luque bars. The purpose of this study is to analyse the causes of these neurological complications, which occurred late in all four of the cases described.

Long-term results of spine surgery in Duchenne muscular dystrophy.

We report the long-term orthopaedic and functional results of segmental spinal instrumentation and fusion in 30 Duchenne patients. Twenty-nine had a mean 59% correction of scoliosis with post-operative immobilization in a brace of only three months on average and with a very limited loss of correction over time. One died after cardiac arrest. The mean vital capacity preoperatively was 57 +/- 17% with a decrease to 34 +/- 13% at 3.9 +/- 2 yr after surgery. The sitting position, aesthetic improvement and the quality of life after spinal fusion have been positively evaluated by the large majority of the patients and their parents. Head control was lost in the 14 patients who developed a more severe extension contracture of the neck measured as a significantly longer chin-sternum distance. More than 90% would have the operation or would give their consent again for their son having the operation.

Spine surgery in spinal muscular atrophy: long-term results.

Fifteen patients with spinal muscular atrophy operated on for scoliosis were reviewed and interviewed. Age at time of surgery averaged 16 yr. Follow-up averaged more than 5 yr. Eleven patients underwent posterior spinal fusion with Luque instrumentation. Four had Harrington instrumentation, with segmental wiring in three. Three out of the 15 also had anterior fusion with Dwyer instrumentation. Patients were immobilized in cast or brace for 6 months, on average, after surgery. The average curve correction was 50%. There was a loss of vital capacity after the operation, which in most cases was regained with time. In functional terms, there were improvements and deteriorations both in the short and long term. Esthetic appearance improved in all patients, as did the perceived well-being. Retrospectively all but one would have the operation again.

Achondroplasia and lumbar spinal stenosis.

Achondroplasia is the most common type of rhizomelic dwarfism. The abnormal spinal development of the achondroplastic dwarf can result in neurologic damage due primarily to the following two syndromes: lumbar spinal canal stenosis and thoracolumbar kyphosis. The authors report the cases observed at their institution, discussing the diagnosis and operative treatment.

Surgical treatment of vertebral deformity due to myelomeningocele.

Surgical treatment of vertebral deformity due to myelomeningocele has always presented important technical problems. Many complications, the foremost of which pseudarthrosis and infection, are reported in the literature. The authors discuss 14 patients with vertebral deformity from myelomeningocele who were treated with different surgical methods and reviewed after an average of eight years. The only conclusion that can be drawn on the basis of both the literature and the present study is that posterior surgery alone is incapable of guaranteeing stable correction over time for severe deformity. Combined anterior and posterior surgery with spinal fusion up to the sacrum yields better results, and the patient is not required to wear a corset afterward.

The nonsurgical treatment of fractures of the dens epistrophei.

The authors examined 25 fractures of the dens epistrophei treated conservatively by several methods in order to evaluate the most suitable treatment for this lesion. A Minerva plaster cast proved to be effective in the treatment of type III fractures, while the halo plaster system obtained the best results in type II fractures.

Hip dislocation in spinal muscular atrophy.

Hip conditions in 49 patients affected with spinal muscular atrophy were studied: 35 of these were affected with the intermediate form of the disease (patients were able to sit but not walk), and 14 with the mild form (patients were able to walk). The Perkins method was used to measure the migration percentage of the femoral head. Thirty-one percent of the patients affected by the intermediate form of the disease had normal hips, 38% had unilateral or bilateral subluxation, 11% had hip dislocation. In the mild form of the disease, 50% of the patients had normal hips, 28% had unilateral or bilateral subluxation, and 22% had a hip dislocation. In the intermediate form of the disease there was a linear correlation between migration percentage and age, and between migration percentage and scoliosis. In the patients affected with the mild form of the disease who were able to walk, and in the patients affected with the intermediate form and fitted with orthoses who were able to stand, or to walk, there was no hip dislocation. Hence, walking with or without orthoses seems to be an important factor in preventing hip dislocation.

Scoliosis in spinal muscular atrophy: natural history and management.

This is a report on the natural history of 109 patients with infantile spinal muscular atrophy (SMA). All 18 children with the severe form died, as did five of the 52 with the intermediate form. Functional ability was more or less stable in the 47 living patients with the intermediate form, but 16 of the 39 with mild SMA lost the ability to walk. Ventilatory function was severely impaired in those with intermediate SMA: orthopaedic treatment for scoliosis in this group did not prevent the curves from worsening with age. Scoliosis was also severe in patients with mild SMA who had stopped walking: surgical treatment in six cases resulted in stable correction of the spine, with functional and cosmetic improvement and without impairment of respiratory function.

The surgical treatment of late instability of flexion-rotation injuries in the lower cervical spine.

The authors reviewed 12 cases of anterior dislocation of the lower cervical spine due to flexion-rotation trauma, with late instability after closed treatment. The 12 patients were treated at the Instituto Ortopedico Rizzoli between 1977 and 1982. Epidemiology, causes, type of lesion, and associated complications were studied. After an average period of 3 years (range: 2-6), correction appeared stable in all of the patients. In 4 patients, posterior stabilization with plates was performed after presurgical correction in a halo-thoracic cast. In 4 patients, reduction with combined, treatment consisting of anterior release and posterior stabilization with plates was performed after failure of presurgical correction. Finally, in 4 patients, stabilization was obtained with anterior fusion alone without reduction of the dislocation.

Surgical treatment of vertebral deformities in neurofibromatosis.

Vertebral deformity in neurofibromatosis can present in two basic forms which determine the prognosis and treatment. In the first form, which is rare, there are no gross dystrophic changes, while in the second, which is the more common, such changes are widespread. Neurofibromatosic scoliosis where there are no signs of bone dystrophy poses no particular therapeutic problems. However, the surgical treatment of the dystrophic form is much more complex and the results far more uncertain. There is a high incidence of pseudarthrosis following posterior arthrodesis and fixation with Harrington rods, and this has shown the need for combined posterior and anterior arthrodesis in all cases of neurofibromatosic kyphoscoliosis. Furthermore, posterior arthrodesis alone does not provide protection against future relapse even when kyphosis is initially absent. Delayed kyphosis, due to subsidence of the vertebral bodies, frequently appears after six months and necessitates a second operation to reinforce the posterior arthrodesis. However, a greater guarantee of stability can be achieved simply by performing combined anterior and posterior arthrodesis at the outset.

Spinal deformities in Marfan's syndrome.

Marfan's syndrome is an hereditary disorder of the connective tissue which in its most classic form includes cardiovascular, ocular and skeletal manifestaions. Scoliosis is frequently present (40-75 per cent); the type of curve is similar to that seen in idiopathic scoliosis, but progression is often much more severe. One of our patients, a woman aged thirty-two who had never been treated, had two 180 degree curves. There is a high incidence (50 per cent) of double curves (right thoracic and left lumbar) and a 23 per cent incidence of triple curves. Vertebral inversion (thoracic lordoscoliosis) is very common and often accompanied by severe respiratory insufficiency, particularly if it is associated with funnel chest. Conservative treatment is almost invariably ineffective and should be reserved only for very slight curves. All curves of 50 degrees or more should be surgically corrected. A 40-50 per cent correction can be achieved without risk. In view of the high incidence of pseudarthrosis, systematic review and, if necessary, revision of the arthrodesis is to be recommended. In the more severe cases of kyphosis it is advisable also to perform an anterior arthrodesis. Surgery is contraindicated in the presence of severe mitral or aortic insufficiency or aortic aneurysm.