A Unique Case of Cervical Myelopathy in an Adult Patient with Scheie Syndrome.

INTRODUCTION: Scheie syndrome is an extremely rare systematic disease that represents the most attenuated form of mucopolysaccharidosis Type I disorder. Although associated with a variety of manifestations, Scheie syndrome leading to the development of cervical myelopathyis yet to be reported. Our purpose was to present a unique case of a Scheie syndrome patient, who underwent surgery due to cervical myelopathy, and to discuss the clinical and imaging findings, as well as the challenges and outcomes of surgical treatment. CASE REPORT: A 33-year-old man with Scheie syndrome presented with neck and radicular pain, upper extremity weakness, and insecure gait. The workup studies revealedcervical spine stenosis at multiple levels, caused by accumulation of soft tissue, within the cervical spinal canal. D espite the high risks of anesthesia, and the patient's inherent poor bone quality that could lead to failure of spinal fusion, we decided to proceed with surgery; indeed, decompressive laminectomies combined with C1-7 posterior stabilization led to immediate pain relief. Despite counter advised, the patient returned to sports rather early, and 6months after index procedure neck pain relapsed, while screw breakage and cutout occurred at the level of C7. Consequently, the initial instrumentation was revised and extended at T2 level. At 2years follow-up, the patient remained continuously pain-free and ambulatory. CONCLUSION: Although cervical myelopathy in Scheie syndrome represents an extremely rare entity, it can make a severe impact on patients' quality of life. If timely managed though, these patients can be offered a significant relief from symptoms. Surgery is rather challenging and treating physicians should be aware of the high risks of anesthesia. Especially spine surgeons should be aware of the nature of the disease, since it may not allow for fusion, causing instrumentation to fail

Sonication Improves the Diagnosis of Megaprosthetic Infections

Limited data are available for the diagnosis of patients with tumors with infected endoprosthetic reconstructions. The purpose of this study was to evaluate whether sonication is effective for the diagnosis of infection and to compare it with tissue cultures. The files of 58 patients who underwent revision surgery for suspected infected endoprosthetic reconstructions were reviewed. Cultures were performed on 5 tissue samples obtained from each patient and on fluid obtained by sonication of the megaprosthesis. The sensitivity, specificity, and negative and positive predictive values of tissue and sonication fluid cultures were evaluated. Overall, tissue and sonication fluid cultures confirmed an infection in 42 of the 58 patients. In 36 of the 42 infected endoprosthetic reconstructions, tissue and sonication fluid cultures identified the same bacterial isolate. In 5 cases, a bacterial isolate was identified only in sonication fluid cultures, and in 1 case, a bacterial isolate was identified only in tissue cultures. The sensitivity and negative predictive value of sonication fluid cultures were statistically significantly better than those of tissue cultures, while the specificity and positive predictive value were not different between the 2 culture types. Compared with tissue cultures for the diagnosis of infected megaprostheses in patients with tumors, sonication fluid cultures are associated with a better sensitivity and negative predictive value and a similar specificity and positive predictive value. Therefore, sonication should be considered a useful adjunct for the optimal diagnosis and management of these patients. [Orthopedics. 201x; xx(x):xx-xx.]

Rib Hump Deformity Correction in Patients with Adolescent Idiopathic Scoliosis: A Comparison of Three Spinal Fusion Systems.

The aim of the present study is to control the hypothesis that the rib hump deformity can be adequately corrected when applying vertebral derotation. We retrospectively studied patients treated with full pedicle screw systems (group A), hybrid constructs (group B), and Harrington rod instrumentation (group C). No costoplasties were performed in the patients included in our study. Derotation was applied in groups A and B. The rib hump deformity was assessed on lateral radiographic studies by rib index (RI). Of the 72 patients that were finally included in our study, 30 patients (24 females and 6 males; mean age, 14.5 ± 2.2 years) were treated with a full pedicle screw system, 23 patients (19 females and 4 males; mean age, 13.8 ± 1.9 years) were treated with a hybrid construct, and 19 patients (16 females and 3 males; mean age, 14.3 ± 2 years) received the Harrington rod instrumentation. In all groups RI was significantly corrected after surgery. Before surgery no difference in RI was found among groups; however, after surgery RI was found significantly higher in group C as compared to groups A and B. The between-group analysis revealed that the correction of RI, and thereby the rib hump deformity correction, did not significantly differ among the three patient groups. In conclusion, it cannot be suggested based on the present study that vertebral derotation alone can offer an adequate correction of the rib hump deformity. Further, the development of rib cage deformity and its degree of interdependence with the scoliotic spinal deformity has to be further investigated and assessed, as it seems that it may not necessarily result directly from the primary vertebral deformity

Complications of spine surgery for metastasis

The spinal column represents the third most common site for metastases after the lungs and the liver, and the most common site for metastatic bone disease. With life-extending advances in the systemic treatment of cancer patients, the surgical proce-dures performed for spinal metastases will increase, and their related complications will increase unavoidably. Furthermore, considering the high complication rates reported in the spinal literature regarding spine surgery overall, it becomes clear that a better understanding of complications that the cancer patients with spinal metastases may experience is necessary. This article aims to summarize and critically examine the current evidence for complications after spine surgery for metastatic spinal disease, in both the perioperative and postoperative period. This paper would be useful for the treating physicians of these patients in their clinical practic

Aggressive Chordomas: Clinical Outcome of 13 Patients

none11siThe authors reviewed the files of all patients with chordomas who were admitted and treated at their institutions from 1975 to 2012. Patients were categorized by early local recurrence and metastasis. Aggressive clinical behavior was defined as local recurrence and metastasis within 24 months of diagnosis and adequate treatment (wide en bloc resection with microscopically negative tumor margins). According to these criteria, 13 patients (14.3%) had aggressive chordomas, including 7 men and 6 women, with mean age of 54 years (range, 37-65 years) at diagnosis and treatment. All patients had preoperative tumor biopsy, followed by resection with partial (7 patients) or total sacrectomy (6 patients). In all cases, biopsy and histologic analysis of resected tumor specimens showed conventional chordomas. Resection margins were wide (grossly negative) in 6 patients and wide contaminated in 7 patients. Mean maximum tumor diameter was 11.8 cm (range, 5-21 cm). Mean follow-up was 43 months (range, 8-131 months). Rates of local recurrence, metastasis, and death were evaluated. At the last follow-up, all patients had local recurrence at a mean of 13 months (range, 5-22 months). Histologic examination of recurrent tumors showed a dedifferentiated chordoma with a fibrosarcoma component in 2 patients and no histologic change in the remaining patients. In addition, 8 patients had metastases at a mean of 13 months (range, 4-24 months) and died of their disease. All histologic findings of metastatic lesions were similar to those of primary tumors. Early diagnosis of aggressive tumors requires close follow-up of patients with chordomas. Metastasis is common, with resultant poor survival. [Orthopedics. 201x; xx(x):xxx-xxx.].noneMavrogenis, Andreas F; Angelini, Andrea; Panagopoulos, Georgios N; Pala, Elisa; Calabrò, Teresa; Igoumenou, Vasilios G; Katzouraki, Galatia; Megaloikonomos, Panayiotis D; Pneumaticos, Spyros G; Papagelopoulos, Panayiotis J; Ruggieri, PietroMavrogenis, Andreas F; Angelini, Andrea; Panagopoulos, Georgios N; Pala, Elisa; Calabrò, Teresa; Igoumenou, Vasilios G; Katzouraki, Galatia; Megaloikonomos, Panayiotis D; Pneumaticos, Spyros G; Papagelopoulos, Panayiotis J; Ruggieri, Pietr