Indications for surgical treatment of thoracolumbar kyphosis in patients with Mucopolysaccharidosis

Introduction. Circumferential fusion of kyphosis in patients with MPS is currently accepted as the most effective surgical approach. However, long-term results remain debatable. This study assesses the effectiveness of posterior-only compared to circumferential fusion. Methods. Eleven patients (7 male, 4 female) with MPS and thoracolumbar kyphosis underwent surgical treatment. Hurler Syndrome (type I) was diagnosed in 5 patients, Morquio Syndrome (type IV) in 2, and Maroto-Lamy (type VI) in 4 patients. Indications for surgical treatment included more than 40° kyphosis, sagittal spinal imbalance, progressive neurological symptoms and severe pain. In 3 cases, patients underwent circumferential arthrodesis combining anterior and posterior approaches. In 8 cases, instrumentation included hooks and/or pedicular screws, placed two levels above and two levels below the deformity apex. The follow-up period ranged from 2 to 5 years. Results. In 8 cases solid spinal fusion was achieved. Complications after surgical treatment were observed in 4 patients (36%). PJK developed in one case 2 years after surgery, pseudarthrosis was observed in one case, wound suppuration was observed in one case, and a broken metal rod in one case. Conclusions. Surgical treatment of MPS patients with thoracolumbar kyphosis is accompanied by a high risk of complications when circumferential stabilization is performed. Most authors and our data show that the most optimal method of surgical treatment of thoracolumbar deformation is dorsal correction and fixation in combination with a wide laminectomy at the level of stenosis. The second stage includes the anterior decompression and interbody fusion. However, if the patient’s lung function is dramatically compromised, and a high risk of respiratory complications exists, surgery may be limited to only posterior correction and fixation in conjunction with a wide laminectomy, which allows to achieve a comparable level of fixation with a lower risk of complications

Risk factors and surgical treatment of craniovertebral stenosis in patients with Maroteaux-Lamy syndrome (Mucopolysaccharidosis type VI)

Introduction. Atlantoaxial instability with the outcome of myelopathy and spastic tetraparesis are commonly described in patients with MPS VI type. The accumulation of glycosaminoglycans behind the odontoid process leads to a gradual development of the spinal canal stenosis and compression of the spinal cord in the cervical spine. These lesions lead to neurological disorders and loss of quality of life. Methods. Nine patients with MPS type VI. Of them 3 males and 6 females aged 14 to 35 years (mean age 20.8 years). All patients presented with craniovertebral stenosis of some degree and underwent posterior spinal canal decompression with cervical fusion. Neurological symptoms were observed in 7 of all cases preoperatively. Functional assessment and evaluation of neurological status was conducted in all cases. CT and MRI evaluation was performed at the atlantoaxial level before surgery and at follow-up. Results. The average follow-up period was 2.9 years. Seven of the nine patients demonstrated regression of neurological symptoms. In two patients the neurological status was unchanged. Solid fusion was achieved in 6 cases. Complications from surgery we observed in 3 patients. One patient died one year after surgery due to unrelated causes, there was one case of pseudarthrosis one case of implant instability and one case of early postoperative wound suppuration. Conclusion. The majority of patients with type VI MPS present with some degree of spinal stenosis at the atlantoaxial level. Based on our experience, these patients require close neurological and radiographic monitoring as early as possible. In our view, surgical treatment of patients with type VI MPS should be considered before the onset and progression of neurological symptoms

Smith-Petersen osteotomy effectiveness compared to anterior release procedures in surgical treatment of Lenke type I idiopathic scoliotic deformities

Introduction: rigid idiopathic scoliosis deformities are traditionally treated using a two-stage approach. However, multilevel Smith-Petersen osteotomies allow to mobilize the main curve and to omit the anterior release stage. Materials and methods: the results of 72 patients aged from 14 to 21 years with an idiopathic scoliosis of Lenke type I and angle of deformity from 70° to 90° (average angle 81.3°) were analyzed. In 35 patients, one-stage treatment was performed in combination with multilevel Smith-Petersen osteotomies. In 38 patients – two-stage operative treatment (anterior release + posterior fusion) was performed. In all patients, the deformities were rigid (correction of less than 25% with the traction test). All patients were examined radiographically. Radiographs were performed right after surgery and 3, 6 and 12 months after surgery. Results. In-group I the average degree of deformity was 72.67 °. The mobility of the main curve in all cases was below 25%. All patients underwent SPO (from 5 to 8 levels). Correction and fixation were carried out using hybrid and screw instrumentation. The average correction angle was 49.94 ° or 68.7%. In-group II the average degree of deformity was 73.92°. The mobility of the main curve was below 25%. All patients underwent anterior release (4 to 6 levels of discectomy). Over the next 7-14 days, halo-gravity traction was carried out. The second stage was performed using posterior correction and fusion using hybrid or screw instrumentation. The average correction angle was 48.73°, or 65.9%. Conclusion: the use of Smith-Petersen osteotomy in patients with rigid idiopathic Lenke type I scoliosis with a degree of deformity between 70° and 90° allows for one-step correction that yields comparable results with two-stage surgical treatment. The number of SPO levels should be at least 5

СРАВНИТЕЛЬНЫЙ АНАЛИЗ ЛЕЧЕНИЯ ЭОЗИНОФИЛЬНОЙ ГРАНУЛЕМЫ С ПОРАЖЕНИЕМ ПОЗВОНОЧНИКА КОРСЕТОМ И ХИРУРГИЧЕСКИМ ВМЕШАТЕЛЬСТВОМ

The results of treatment of 72 patients with pathological fracture of vertebra bodies against eosinophilic granuloma a spine are analysed. Orthopedic corset technologies are used in treatment of 42 patients, surgical treatment was applied. Orthopedic corset may be used in patients with eosinophilic granuloma of backbone. This method of treatment was used in a case of the absence of spine secondary deformations and neurologic semiology. Orthopedic corset treatment is associated with long immobilization on the average within 1,5-2 years and never leads to a complete recovery of the damaged spine. Surgical treatment consists in use only at loss of height of a body to 30-40%, when destruction of spine more severe it is necessary to use operative treatment in two stages. Absolute indications to surgical treatment are the neurologic deficit and secondary deformations of a spine. Surgical treatment allows to reduce terms of treatment till 3-4 months and quickly to return the patient to an active life.Проанализированы результаты лечения 72 пациентов с патологическими переломами тел позвонков на фоне эозинофильной гранулемы позвоночника. У 42 больных использованы корсетные технологии, у 30 применялось хирургическое лечение. Использование корсета возможно при отсутствии вторичных деформаций позвоночника и неврологической симптоматики. Оно связано с длительной иммобилизацией в среднем в течение 1,5-2 лет и никогда не приводит к полному восстановлению пораженного позвонка. Хирургическое лечение заключается в использовании только эндофиксаторов при потере высоты тела до 30-40%. При большем разрушении позвонка необходимо использовать двухэтапное оперативное лечение. Абсолютными показаниями к хирургическому лечению являются неврологическая симптоматика и вторичные деформации позвоночника. Хирургическое лечение позволяет сократить сроки лечения до 3-4 месяцев и быстро вернуть пациента к активной жизни