A Case Report of Gorham-Stout Syndrome with Kyphoscoliosis / 罕见病研究

This article reported the treatment of Gorham Stout syndrome (GSS) with kyphoscoliosis. The patient was an 11-year-old male who was presented with kyphoscoliosis, first developed 4 years ago, accompanied by incomplete paralysis of both lower limbs. The syndrome worsened in the previous year. He had a history of chylothorax, ventilation dysfunction, and osteoporosis. The patient underwent skull traction in another hospital for 7 months, which was ineffective. Finally, he underwent posterior correction, internal fixation, and bone graft fusion (C4-T8) in our hospital. The postoperative clinical outcomes and the posterior correction were satisfactory, with significant improvement in incomplete paralysis of both lower limbs. This article aims to improve the understanding of GSS and provided reference for its diagnosis and treatment through a typical case report and review of previous literature.

The Performance of Graduation from Traditional Growing Rods with Apical Control Technique in Patients with Congenital Early-Onset Scoliosis / 罕见病研究

  Objective  Different from other etiologies of early-onset scoliosis (EOS), congenital early-onset scoliosis (CEOS) is mainly linked to vertebral anomalies, such as formation failures and segmentation failures at the apex segments. So far, there is little research on CEOS patients who have completed traditional growing rods (TGR) treatment, and the initial outcomes of TGR with or without apical control technique (ACT) are different. Therefore, we compared the "final" results of CEOS patients who completed TGR treatment with or without ACT.  Methods  We conducted a retrospective study of CEOS patients who completed TGR treatment from 2007—2020. They either had final fusion or were followed up after reaching skeletal maturity. We split the patients into two groups based on whether they had ACT with TGR or not. The ACT-TGR group had apical vertebrectomy/hemivertebrectomy with short fusion and TGR. The TGR-only group had only TGR. We looked at their demographic features, radiographic measurements, and complications.  Results  This study enrolled 46 CEOS patients, of which 13 patients were in the ACT-TGR group and 33 patients in the TGR group. The ACT-TGR group had a longer distraction interval (1.17 years vs. 0.75 years). The ACT-TGR group had a larger preoperative main curve [87.00(63.50, 98.00)], but the residual curve degrees were comparable between the two groups at the last follow-up (P=0.354). At the last follow-up, the T1-12 and T1-S1 heights were similar between the two groups. The ACT-TGR group had a lower number of implant-related complications per patient (0.77 vs. 1.48). Three patients in the ACT-TGR group underwent final fusion, while 17 patients in the TGR group underwent final fusion (P=0.060).  Conclusions  Both ACT-TGR and traditional TGR coud effectively correct deformity and preserve spinal growth in CEOS patients. ACT-TGR had a better corrective effect on patients with severe deformity and did not have a significant impact on spinal height. For patients with acceptable correction, spontaneous fusion and without implant failure, retaining the implant and continuing observation could be a strategy for graduating from growing rod treatment.

Progress in the Research of the Spinal Deformity Related to Marfan Syndrome / 罕见病研究

Marfan syndrome(MFS) is an autosomal dominant systemic connective tissue disease. The incidence rate of MFS is about 2-3 per 10 000. Main cause of MFS is FBN1 gene mutation. About 2/3 of MFS patients have spinal deformities, showing symptoms of scoliosis, thoracic lordosis and lumbar kyphosis, severe spondylolisthesis, dural dilatation and pedicle dystrophy. MFS scoliosis develops with age and may continue even after bone maturation. Conservative treatments such as brace are usually ineffective. Surgical treatment of main curve > 40°-45 °is recommended, but due to the special anatomical structure of MFS patients, such surgical complications as dural leakage, failure of internal fixation and revision surgery are not uncommon.

The effects of early spinal fusion on pulmonary function and thoracic development in patients with early-onset scoliosis / 中华骨科杂志

Early-onset scoliosis (EOS) is defined as the scoliosis occurs before 10 years old. Such patients with severe scoliosis often require early surgical intervention, but spinal fusion may also affect their thoracic development and lung function. Based on etiology, EOS can be classified as congenital, neuromuscular, syndrome-related and idiopathic scoliosis. The clinical goal is to control the progression of the curve while allowing the spine and chest to grow as much as possible to promote the development of alveolar. Clinical treatments include physiotherapy, plaster and brace correction as well as surgery. Patients of EOS were usually at the critical stage of thoracic and lung development due to their young age. In addition, the combination of severe thoracic deformity may also lead to life-threatening cardiopulmonary disorder and related complications considering the clinical inconsistency and complexity of EOS. Thoracic anatomical changes brought by scoliosis itself can limit chest wall movement and reduce lung compliance, resulting in changes in thorax diameter and compression of thoracic volume, leading to restrictive ventilation dysfunction. And spinal fusion can effectively correct curve and control progression, which still remains as the primary surgical option for severe EOS patients nowadays. However, early spinal fusion can also lead to deformation of lung tissue, collapse and malformation of alveolar while limiting the height and growth rate of thoracic cavity, hindering the circulatory system and leading to respiratory dysfunctionof children. Spinal growth restriction, crankshaft phenomenon and restricted alveolar proliferation may play a role in thisprocess. The present review retrospectively summarized the effects and possible mechanisms of early spinal fusion on lung function and thoracic development in patients with EOS, aiming to further provide guidance for clinical decisions.

Preliminary results of osteotomy with short fusion and dual growing rod technique for congenital scoliosis / 中华骨科杂志

Objective To evaluate the clinical outcomes of the hybrid technique of posterior osteotomy with short segmental fusion and dual growing rod technique for severe rigid congenital scoliosis.Methods Seven patients (2males,5 females) undergoing this hybrid technique for severe rigid congenital scoliosis in our hospital from 2006 to 2011 were retrospectively studied.The average age was 5.9 years (range,2-10).The Risser sign of all the patients was 0.The follow-up time was 59.4 months (range,36-83 months).The patients' charts were reviewed.The analysis included age at initial surgery and the latest follow-up,number and frequency of lengthening,and complications.Radiographic evaluation included measured changes in scoliosis Cobb angle,thoracic kyphosis,lumbar lordosis,trunk shift,length of T1-S1 and instrumentation.Results All patients were treated with posterior osteotomy with short segmental fusion and dual growing rod technique.There were 48 total surgeries,41 of which were lengthening procedures,for 7 patients.The average lengthening was 5.9 per patient.The mean scoliosis improved from 81.4° to 40.1 ° after initial surgery and was 41.1 ° at the final follow-up.The average T1-S1 length was of 1.12 cm per year.The Campbell's space available for lung ratio increased from 0.87 to 0.97.Conclusion Osteotomy with short fusion could help to improve the correction of the growing rod and eliminate the large asymmetric growth potential around the apex,with little influence to the length of the spine.Dual growing rod technique could maintain correction achieved at initial surgery while allowing spinal growth to continue.However,this technique is relatively more aggressive and technique demanding.And the patients need numbers of surgeries.

Complications and prophylaxis strategies of posterior hemivertebra resection for congenital scoliosis / 中华外科杂志

<p><b>OBJECTIVE</b>To evaluate the surgical outcomes and related complications of posterior hemivertebra resection with transpedicular instrumentation in the treatment of congenital scoliosis caused by fully-segmented non-incarcerated hemivertebra.</p><p><b>METHODS</b>From January 2003 to January 2012, one hundred and forty consecutive cases of congenital scoliosis treated by posterior hemivertebra resection with transpedicular instrumentation were investigated retrospectively. Radiographs were reviewed to determine the type and location of the hemivertebra, the coronal curve magnitude, sagittal alignment, compensatory cranial curve and compensatory caudal curve preoperatively, postoperatively and at the latest follow-up. Operative reports and patient charts were reviewed to record operation time, fusion level and complications.</p><p><b>RESULTS</b>One hundred and fifty-one posterior hemivertebra resections in 140 patients aged 2 to 45 years (average 10.8 years) with non-incarcerated hemivertebra were evaluated. All the patients were followed up from 3 to 119 months (average 25 months). The average fusion level was 5.0 segments (2-11 segments). There was a mean improvement of 71.3% in the segmental scoliosis from 42.5° before surgery to 10.6° at the time of the latest follow-up, and a mean improvement of 66.8% in segmental kyphosis from 29.5° to 7.2° at the same periods. There were 14 complications (13 patients), 3 pedicle fractures, 2 rod breakages, 2 pedicle elongation, 2 removed implants for prominent implants, 2 delayed wound healing, 2 additional surgeries for curve progression, 1 prolonged respiratory support. There was no neurological complication.</p><p><b>CONCLUSIONS</b>Posterior hemivertebra resection with transpedicular instrumentation is a safe and effective procedure for congenital scoliosis patients.Neurological complication is rare, but implant-related complication still remains a challenge.</p>

Perioperative complications and risk factors of one-stage posterior vertebral column resection for severe spinal deformity / 中华骨科杂志

Objective To investigate the perioperative complications and risk factors of one-stage posterior vertebral column resection (VCR) for severe spinal deformity.Methods From September 2004 to July 2012,39 patients with severe and fixed spinal deformity underwent one-stage posterior VCR,including 15 males and 24 females,aged from 3 to 53 years (average,16.9 years).There were 24 cases of kyphoscoliosis (mean coronal Cobb angle:85.1°,mean sagittal Cobb angle:92.9°),7 cases of scoliosis (mean coronal Cobb angle:81.1°),and 8 cases of kyphosis (mean sagittal Cobb angle:94.4°).Eleven patients had neurological compromise.The perioperative complications and related risk factors of 39 patients were retrospectively analyzed.Results All patients were followed up for 3 to 72 months (average,29.4 months).There were 15cases (13 patients) of perioperative complications.Neurological complications occurred in 6 patients (15.4％),among whom one patient presented complete paraplegia after surgery and 5 patients presented transient paresthesia or muscle weakness.Adults had a significantly higher incidence of neurological complications than teenagers.The incidence rate of neurological complications was 36.4％ in patients with preoperative neurological compromise,while 7.1％ in patients without preoperative neurological compromise.All patients with postoperative neurological complications had kyphosis before operation,and the incidence of neurological complications increased significantly in patients with severe kyphosis (Cobb angle ≥90°).Prolonged respiratory support was conducted in 4 cases.Rupture of the parietal pleura occurred in 3 cases (7.7％),cerebrospinal fluid leak in 1 case (2.6％),and pulmonary infection in 1 case (2.6％).Conclusion One-stage posterior VCR is effective in the treatment of severe and rigid spinal deformity.However,the perioperative complications,especially the neurological complications are common.The risk factors of neurological complications include preoperative neurological compromise,degree of kyphosis and the age of patients.

Protective effect of glucocorticoid preconditioning on the myocardial ischemic and reperfused rabbit heart / 西安交通大学学报(医学版)

Objective To study the protective effect of glucocorticoid preconditioning on the myocardial ischemic and reperfused hearts.Methods Totally 18 rabbits were randomly divided into three groups: myocardial ischemia-reperfusion model(model),high-dose glucocorticoid given by one time group(high-dose group) and low-dose glucocorticoid given by several times group(low-dose group),with six rabbits in each group.Myocardial ischemia was induced by left anterior descending coronary artery ligation.ST segments were recorded by the BL-420 biological signal acquisition system.Plasma malondial dehyde(MDA) was examined before ischemia,at 15 min after ischemia and 30 min after reperfusion;ischemic heart muscles were prepared with cryotomy and stained histochemically.Succinic dehydrogenase activity was observed in the ischemic region.Results There was shorter time of ST-segment recovery in the high-dose group and the low-dose group than that in the model group.Serum level of MDA in the high-dose group was lower than in the low-dose group(P