ObjectiveTo evaluate the effectiveness of modified "eggshell" osteotomy for the treatment of thoracolumbar kyphoscoliosis. MethodBetween April 2009 and June 2014, 19 patients with spinal deformity underwent modified "eggshell" osteotomy consisting of preserving posterior bony structures initially and enlarging surgical field for cancellous bone removal. There were 14 males and 5 females with an average age of 37.8 years (range, 18-76 years) and with a median disease duration of 7 years (range, 1-40 years). The disease causes included ankylosing spondylitis in 13 cases, spinal tuberculosis in 3 cases, and chronic vertebral compression fracture in 3 cases. Eleven patients showed single kyphosis and 8 patients had kyphoscoliosis. Preoperative Cobb angle of kyphosis was (64.2±30.1) °, while Cobb angle of scoliosis was (19.9±12.8) °. Apical vertebraes were T10 in 1 case, L1 in 3 cases, L2 in 7 cases, T10, 11 in 2 cases, T12, L1 in 4 cases, T12-L2 in 1 case, and T10-L1 in 1 case. Preoperative visual analogue scale (VAS) and Japanese Orthopaedic Association (JOA) score were 6.1±1.9 and 15.2±5.6, respectively. According to Frankel criteria for spinal cord function, 16 cases were rated as grade E and 3 cases as grade D before operation. Cobb angle, VAS, and JOA scors were used to assess relief of symptom. ResultsThe operation time was 215-610 minutes (mean, 343 minutes); intraoperative blood loss ranged from 900 to 3000 mL (mean, 1573 mL). All incisions healed primarily. Delayed onset ischemia-reperfusion injury of spinal cord occurred in 1 case at 6 days after operation, and symptoms alleviated after conservative treatments. All 19 cases were followed up 14-76 months (mean, 46 months). No loosening or breakage of internal fixation was observed during follow-up. Cobb angle of kyphosis, Cobb angle of scoliosis, VAS and JOA scores at 1 week after operation and last follow-up were significantly improved when compared with preoperative ones (P<0.05) . VAS and JOA scores at last follow-up were significantly improved when compared with scores at 1 week after operation (P<0.05) , but no significant difference was found in Cobb angle of both kyphosis and scoliosis between at 1 week after operation and at last follow-up (P>0.05) . At 1 week after operation, the correction rate for kyphosis was 34.1%-93.4% (mean, 62.2%), and the correction rate for scoliosis was 42.4%-100% (mean, 68.9%). At 48 months after operation, 3 patients with preoperative impaired spinal cord function achieved full recovery. ConclusionsModified "eggshell" osteotomy owns the advantages of shorter operation time and less intraoperative blood loss, thus it is able to correct thoracolumbar kyphoscoliosis safely and effectively.
ObjectiveTo review the research progress of pathogenesis mechanism of spinal deformity in neurofibromatosis type 1 (NF1). MethodsRecent literature concerning the pathogenesis mechanism of spinal deformity in NF1 was extensively reviewed, and current developments of the correction of spinal deformity and NF1 and the pathogenesis mechanism were summarized. ResultsThe pathogenesis mechanism of spinal deformity in NF1 is not yet clearly known. Current theories include erosion and stress of neurofibromas, melatonin-related decreased contractility of paraspinal muscles, osteopenia and osteoporosis, sexual precocity and mesoderm dysplasia. ConclusionThe clinical manifestations of NF1 may cause the spinal deformities in patients with NF1. The research of pathogenesis mechanism of spinal deformity in NF1 will be conducive to further understanding, diagnosis and treatment of NF1-related spinal deformity.
Objective To develop a multidisciplinary nursing program for Prader-Willi syndrome with spinal deformity and evaluate its effectiveness in clinical practice. Methods In July 2016, a multidisciplinary collaborative team was established before the treatment of children with Prader-Willi syndrome complicated with spinal deformity. For the nursing difficulties in the perioperative period, relevant literature was consulted, and a multidisciplinary collaborative nursing plan was formulated, which included nutrition management and blood glucose control, management of obstructive sleep apnea hypopnea syndrome, behavioral intervention related to mental change, and early identification and management of complications. The developed multidisciplinary collaborative nursing program was applied to three children with Prader-Willi syndrome complicated with spinal deformity in Peking Union Medical College Hospital from July 2016 to October 2018, and their postoperative recovery was evaluated. Results One child had inguinal skin ulceration when admitted to hospital, which was significantly improved after active treatment. The perioperative blood glucose level control of the three children was satisfactory, and there was no postoperative gastrointestinal dysfunction, deep wound infection, respiratory complications, internal fixation failure or other complications, and no asphyxia, fall, loss, or other adverse events. The follow-up compliance after discharge was 100%. Conclusions Multidisciplinary collaboration programs can escort patient’ safety and promote their recovery, improve the professional level of nursing staff, and reflect the nursing value. As a working mode, it can be further popularized and used for reference in the nursing of other difficult diseases.