Scoliosis and BMI in patients with Prader-Willi syndrome.

Although scoliosis is commonly seen in patients with Prader-Willi syndrome, the patterns and extent of the deformity may change along their growth. Increased body weight is another issue in these patients, and its relationship with scoliosis is still controversial. The aim of this study was to evaluate scoliosis in patients with PWS, and its relationship with BMI. This was a retrospective cohort study in which a series of radiographic images and BMI from each patient were collected, and the data were rearranged following the age at which they were recorded. These patients were subsequently labeled as non-Scoliotic (<10°), Moderate (10° - 39°), and Severe (≥40°) according to their final Cobb angle, also as Normal (≤85%), Overweight (86%-95%), and Obese (≥95%) according to final BMI percentage. Thirty-four patients with age from 1 to 20 years old were recruited for this study, and the mean length of follow-up was 6.6 years. The prevalence of scoliosis was 71% (24 patients in Moderate, and 9 patients in Severe), and 65.6% were either overweight (11 patients) or obese (10 patients). The mean BMI percentage in non-scoliotic patients was 93.10 ± 13.84, which was significantly higher than that of the scoliotic groups ( P = 0.0180). When looking at the longitudinal change, the non-Scoliotic group had high BMI since childhood, and obese patients had less spine deformity also from early childhood. In this study, we found that the prevalence of scoliosis in Taiwanese population with PWS was 71% without gender preference. Not every patient had a high BMI, and obese patients seemed to have significantly less chance to develop scoliosis. Level III.

Expert consensus for PVCR in severe, rigid and angular spinal deformity treatment: The Kunming consensus.

In the past decades, an increasing number of surgeons started using posterior vertebral column resection (PVCR) to treat severe, rigid and angular spinal deformities. Little high-level evidence is available to guide surgical treatment. The aims of our study were to identify important surgical strategies and key technical points of Chinese experts who have extensive experience in the management of severe, rigid and angular spinal deformities using PVCR, and to standardize and unify the current core concepts. Workgroups of consensus were formed by selecting nationwide representing experts and comprehensive consultations. Eight task forces for major issues were established, then retrieval of literature, collection of expert opinions and writing of review articles were carried out. A modified Delphi process was chosen in round-table forum with three face-to-face meetings. Consensus was reached with items graded more than seven points including: indications and contraindications of PVCR; review PVCR in the evolution of spinal osteotomies; The corrective mechanism and safety of spinal cord; monitoring and responses of spinal cord crisis; characteristics and therapeutic outcome of pulmonary function; management of bleeding during PVCR; relationship of pedicle screw insertion and spinal cord safety; and analysis of non-neurologic complications and prevention strategies. In conclusion, The essential properties regarding PVCR procedure are tightly linked with various factors such as medical and surgical indication, range and level of vertebral column resection, strategies of correction, corrective efficiency and control of neurological risk. PVCR is used mainly for severe, rigid spinal deformity that is not manageable by other osteotomy techniques.

Gorham syndrome with postoperative respiratory failure and requiring prolonged mechanical ventilation.

Gorham syndrome is a rare disease that presents as progressive osteolysis, and may affect any part of the skeleton. The pathologic process involves the replacement of normal bone by aggressively expanding but non-neoplastic vascular tissue, resulting in massive osteolysis of the adjacent bone. If the spine and ribs are affected, the subsequent kyphosis and chest wall deformity may cause severe restrictive ventilatory impairment. We report a 34-year-old male with Gorham syndrome presenting as progressive kyphosis, severe back pain, unstable gait, and exertional dyspnea. Pulmonary function testing revealed severe restrictive ventilatory impairment. He underwent spinal surgery but could not be extubated after surgery. Postoperative left lower lung pneumonia and respiratory failure required prolonged mechanical ventilation. After a weaning program of pressure support ventilation and T-piece spontaneous breathing trials, he was successfully weaned from mechanical ventilation.

Enhancement of recombinant human BMP-7 bone formation with bmp binding peptide in a rodent femoral defect model.

Bone morphogenetic binding peptide (BBP) is an 18.5 kDa fragment of a bone matrix protein peptide. A rat femoral defect model was used to test the effect of BBP combined with recombinant human bone morphogenetic protein-7 (rhBMP-7) to induced bone healing. Two doses of BBP (500 and 1000 µg) were tested with two doses of rhBMP-7 (2 and 5 µg), and the results were compared with a positive control (10 µg rhBMP-7). Bone healing was evaluated by radiology, manual palpation, microcomputed tomography, and histology. The high dose of 10 µg of rhBMP-7 resulted in a consistent 100% bone union rate and a mature histological appearance on histology, and was used as a positive control. When 1000 µg of BBP was combined with lower doses of BMP-7 (2 µg rhBMP-7 or 5 µg rhBMP-7) significant differences were seen in radiographic scores, manual palpation, and bone volume, when compared to 2 µg rhBMP-7 or 5 µg rhBMP-7 alone. The combination of 1000 µg of BBP and 5 µg rhBMP-7 also achieved 100% fusion rate, induced a larger amount of bone formation, and yielded similar maturity of bone marrow when compared with the high dosage 10 µg rhBMP-7 group. This study demonstrated that when combined together, BBP can enhance the bone healing of rhBMP-7. Improved healing imparted by the addition of BBP may result in lesser amounts of rhBMP-7 needed to achieve union in the clinical setting.

MRI classification of interspinous ligament degeneration of the lumbar spine: intraobserver and interobserver reliability and the frequency of disagreement.

Posterior spinal ligament pathology is becoming increasingly recognized as a significant cause of low back pain. Despite the growing clinical importance of interspinous ligament degeneration in low back pain patients, formal reliability studies for the magnetic resonance imaging (MRI) evaluation of interspinous ligaments have not been performed. We proposed an MRI classification system for interspinous ligament degeneration and conducted a comprehensive reliability and reproducibility assessment. Fifty patients who had low back pain with or without leg discomfort (26 males and 24 females) with a mean age of 48.8 years (range 23-85 years) were studied. The classification for lumbar interspinous ligament degeneration was developed on the basis of the literature using mid-sagittal T1- and T2-weighted images. Three spine surgeons independently graded a total of 200 interspinous ligament levels. Intraobserver and interobserver reliability were assessed by kappa statistics. The frequency of disagreement was also identified. The intraobserver agreement was excellent in all readers (kappa range 0.840-0.901). The interobserver agreement was lower as expected, and was substantial to excellent (kappa range 0.726-0.818). Overall complete agreement was obtained in 87.8% of all interspinous ligament levels. A difference of 1, 2, and 3 grades occurred in 8.1, 3.0, and 1.1% of readings, respectively. This proposed MRI classification of interspinous ligament degeneration was simple, reliable, and reproducible. Its use as a standardized nomenclature in clinical and radiographic research may be recommended.

Magnetic resonance imaging grading of interspinous ligament degeneration of the lumbar spine and its relation to aging, spinal degeneration, and segmental motion.

OBJECT: Degenerative changes of the interspinous ligaments (ISLs) have generally been ignored in previous studies. Factor-related causes, the effects that these changes have on other structures within the spinal functional unit, and their relation to kinematic changes in the spine are lacking. In this study, the authors evaluated the reliability of a proposed MR imaging grading system of ISL degeneration (ISLD). They also investigated the relationship between ISLD and aging, disc/facet joint degeneration, and lumbar segmental motion. METHODS: The authors studied 256 lumbar motion segments from L-2 to S-1 in 64 patients (35 men and 29 women) with a mean age of 46.08 years (range 23­85 years). An MR imaging­based grading system for ISLD was developed and ranged from Grade A (mild) to Grade D (severe). The reliability was tested, and the correlation of the grade with the severity of the disc and facet joint degeneration was examined. The segmental motion of each functional unit was measured using flexion/extension MR imaging, and their relationships with ISL grades were identified. RESULTS: Grade A was observed in 115 levels (44.9%), Grade B in 105 (41.0%), Grade C in 15 (5.9%), and Grade D in 21 levels (8.2%). The kappa coefficients for intraobserver and interobserver agreements were substantial to excellent (intraobserver [0.871] and interobserver [0.721­0.807]). Grade D was observed primarily in elderly patients. Segmental motion tended to decrease in the most severe grade, with a significant difference in angular mobility. As the severity of ISLD increased, the severity of disc/facet joint degeneration increased (p < 0.001 and p < 0.05, respectively). CONCLUSIONS: The authors proposed a reliable and reproducible grading system that may be used to investigate spinal kinematics in association with ISLD. The authors' findings illustrated the distribution of ISLD grades. The most severe grade occurred primarily in elderly patients. Mobility decreased in the most severe grade; therefore, the stage of ISLD should be taken into consideration when evaluating spinal stability.

Absence of bone morphogenetic protein-2 in human breast milk after spinal surgery.

BACKGROUND CONTEXT: Bone morphogenetic protein-2 (BMP-2) has been used extensively in recent years to enhance the process of spinal fusion. However, numerous side effects have been reported. This raises concerns regarding the safety of using this material in human beings. There are no published reports, that we are aware of, that demonstrate detectable levels of BMP-2 in human milk. PURPOSE: The purpose of this study was to determine the presence/absence of recombinant human bone morphogenetic protein-2 (rhBMP-2) in milk samples taken from a patient who underwent spinal surgery using rhBMP-2 shortly after delivering a child. STUDY DESIGN/SETTING: The study design comprises a case report dealing with analyzing milk from a patient who underwent spinal surgery using rhBMP-2 shortly after delivering a child. METHODS: We report the analysis of milk samples from a patient who underwent spinal surgery using rhBMP-2 shortly after delivering a child to determine the presence/absence of rhBMP-2. The milk samples were taken before and after the spinal surgery. Enzyme-linked immunosorbent assays were performed repeatedly to the samples. RESULTS: The standard curve for the assay had an r(2) value of 0.9853 indicating an acceptable degree of statistical reliability. The dose range for the standard curve was 62.5 to 2,000 pg/mL. None of the samples had an optical density greater than that of the lowest standard. CONCLUSIONS: No rhBMP-2 was detected in human milk in this single case report. Although this result seemed promising, it did not eliminate the concern regarding the potential risk of rhBMP-2 on child development. We still need further studies including more cases to verify this conclusion.

Bone morphogenetic protein-2 and bone marrow aspirate with allograft as alternatives to autograft in instrumented revision posterolateral lumbar spinal fusion: a minimum two-year follow-up study.

STUDY DESIGN: Retrospective cohort study. OBJECTIVE: Compare the efficacy of rhBMP-2 and bone marrow aspirate with allograft (BMAA) as alternatives to autograft in instrumented revision posterolateral lumbar fusion (PLF). SUMMARY OF BACKGROUND DATA: The use of autogenous bone graft is the current gold standard in revision PLF; however, this practice is associated with significant donor-site morbidity. Revision PLF pose the additional challenges of a less than ideal fusion environment and a limited quantity of autogenous bone graft. rhBMP-2 and BMAA have been shown to be acceptable bone graft substitutes in several primary orthopedic procedures. The role of these bone graft substitutes in instrumented revision PLF has yet to be determined. METHODS: Sixty-two patients (125 levels) who underwent instrumented revision PLF with a minimum 2-year follow-up were included. Group 1 contained 24 patients (13 single- [group 1A] and 11 multilevel [group 1B]) who underwent instrumented revision PLF using rhBMP-2 on an absorbable collagen sponge. Group 2 included 18 patients (7 single- [group 2A] and 11 multilevel [group 2B]) with procedures using BMAA. Group 3 consisted of 20 patients (10 single- [group 3A] and 10 multilevel [group 3B]) with procedures using autograft. Demographic, surgical, and clinical data were collected from medical records. Time to solid fusion mass formation, fusion rate, complications, and clinical outcomes were evaluated. The progression of the fusion mass was evaluated by reviewing radiographs. A diagnosis of nonunion was based on exploration during an additional revision surgery or evidence of nonunion on dynamic radiographs or computerized tomography. Clinical outcomes were assessed using a visual analog scale (VAS) before surgery and at 6-week, 6-, 12-, and 24-month follow-ups. RESULTS: Overall fusion rate was 93.5% (58/62). All single-level revision PLF achieved solid fusion. Groups 1B and 3B achieved 100% fusion, (11/11) and (10/10), respectively;whereas group 2B had a fusion rate of 63.6% (7/11). Group 1 also developed a solid fusion mass earlier than the other groups. There was a significant decrease between preoperative and 2-year postoperative VAS scores in all groups, but no significant difference among groups. Three patients in group 2 required an additional revision surgery. CONCLUSION: rhBMP-2 may be an appropriate alternative to autogenous bone graft in both single- and multilevel revision PLF, whereas BMAA may be appropriate as a substitute in single-level revision PLF. The use of BMAA in single-level revisions may be a more cost-effective option than rhBMP-2.

Superior mesenteric artery syndrome complicating staged corrective surgery for scoliosis.

Superior mesenteric artery syndrome (SMAS) is a rare complication following correction of scoliosis with either nonoperative or operative methods. If the patient diagnosed with this syndrome is not managed timely and adequately, mortality may result. We report two cases of SMAS complicating staged corrective surgery for scoliosis using modern segmental derotation instrumentation system. The aim of this report is to highlight the clinical presentations, laboratory findings, radiologic features, and management of the syndrome. The first patient had the syndrome after two-staged scoliosis surgery with halo traction between two stages, and the second patient after three-staged scoliosis surgery with halo traction between the first and second surgeries. The first patient responded well to conservative treatment. However, the second patient failed to respond to conservative treatment and needed a gastrojejunostomy operation to bypass the duodenal obstruction. Clinicians treating post scoliosis surgery patients should always have a high index of suspicion for this potential life-threatening condition. Early diagnosis will enable a multidisciplinary team approach to be initiated early to provide optimal care for the patient. Nutritional and fluid supplementation is mandatory during conservative treatment. The duration for trial of conservative treatment should not exceed 1 week.