A Case Study and Literature Review of the Diagnosis of Danon Disease in Patients Presenting Only with Severe Cardiac Symptoms.

The clinical manifestations of Danon disease, which result from the primary deficiency of the lysosome-associated membrane protein 2 gene, include cardiomyopathy, skeletal myopathy, and different degrees of intellectual disability that vary greatly among patients. The present study reports on two cases of Danon disease in two patients who only presented cardiac symptoms. Cardiac symptoms usually occur in adolescence and during a patient's twenties, and most patients die from heart failure. However, the lab results from these cases suggested that other systems were involved, despite no other clinical symptoms. Significantly, the two patients had elevated serum cardiac troponin I, which often manifests in the acute cardiac phase, especially in severely affected patients with rapidly fatal outcomes. Danon disease is a multi-system involvement disease. Therefore, clinicians must be aware of its complexity when evaluating newly diagnosed patients due to its vastly different clinical course and prognosis and the importance of multidisciplinary management.

Radioiodine adjuvant therapy in differentiated thyroid cancer: An update and reconsideration.

Radioiodine (131I) therapy (RAI) has been utilized for treating differentiated thyroid cancer (DTC) for decades, and its uses can be characterized as remnant ablation, adjuvant therapy (RAT) or treatment for known diseases. Compared with the definite 131I treatment targets for remnant ablation and known disease, 131I adjuvant therapy (RAT) aims to reduce the risk of recurrence by destroying potential subclinical disease. Since it is merely given as a risk with no imaging confirmation of persistence/recurrence/metastases, the evidence is uncertain. With limited knowledge and substance, the indication for RAT remains poorly defined for everyday clinical practice, and the benefits of RAT remain controversial. This ambiguity results in a puzzle for clinicians seeking clarity on whether patients should receive RAT, and whether patients are at risk of recurrence/death from undertreatment or adverse events from overtreatment. Herein, we clarified the RAT indications in terms of clinicopathological features, postoperative disease status and response to therapy evaluation, and retrospectively examined the clinical outcomes of RAT as reported in current studies and guidelines. Furthermore, given the evolution of nuclear medicine imaging techniques, it can be expected that the future of RAT may be advanced by nuclear medicine theranostics (i.e., 131I whole-body scan, PET/CT) by accurately revealing the biological behaviors, as well as the underlying molecular background.

Genome-wide DNA methylation and gene expression patterns of androgenetic haploid tiger pufferfish (Takifugu rubripes) provide insights into haploid syndrome.

Androgenesis is an important chromosome set manipulation technique used in sex control in aquaculture. Haploid embryos exhibit haploid syndrome with body abnormalities and even die during early embryonic development. In this study, we used whole genome bisulfite sequencing (WGBS) to investigate the genome-wide DNA methylation profiles in haploid females (1n-X) and males (1n-Y), and diploid females (2n-XX) and males (2n-XY) of tiger pufferfish (Takifugu rubripes), an economically important fish in China. A total of 96.32 Gb clean data was produced. Differentially methylated regions (DMRs) were found between haploids and diploids, which may be related to abnormal development and early embryonic death in haploids. There were 3,641 hyper-methylated differentially methylated genes (DMGs) and 2,179 hypo-methylated DMGs in haploid vs. diploid comparisons in both females and males. These DMGs were mainly related to genomic stability maintenance and cell cycle regulation. slf1, actr8, gas2, and pbrm1 genes were selected to validate the methylation sequencing. After combining the methylation data with the corresponding transcriptome data, we identified several genes, including guca2a, myoc, fezf2, rprml, telo2, s100a1, and marveld1, which exhibited differential expression levels modulated by DNA methylation. In conclusion, our study revealed different methylation and expression profiles between haploid and diploid T. rubripes for the first time. Several DMGs were identified between different ploidy levels, which may be related to haploid syndrome formation. The results expand the understanding of the effects of ploidy on the early development of teleosts and provide knowledge about target genes and networks to improve the survival rate of haploids.

Longitudinal Spinous-Splitting Laminoplasty with Coral Bone for the Treatment of Cervical Adjacent Segment Degenerative Disease: A 5-Year Follow-up Study.

This study was designed to analyze the causes of cervical adjacent segment degenerative disease (ASDis), evaluate the surgical outcomes of longitudinal spinous-splitting laminoplasty with coral bone (SLAC) during cervical reoperation, and accumulate data on reoperation with SLAC in a primary hospital. Based on the inclusion and exclusion criteria, we conducted a retrospective study involving 52 patients who underwent cervical reoperation for ASDis using SLAC at the spinal surgery department of the Beijing Jishuitan Hospital from 1998 to 2014. Among them, 39 were treated with anterior cervical fusion and internal fixation during the first operation (anterior cervical corpectomy with fusion [ACCF], n = 24; anterior cervical discectomy and fusion [ACDF], n = 11; and cervical disc arthroplasty [CDA], n = 4). Outcomes were the Japanese Orthopaedic Association (JOA) score, neck disability index (NDI) score, upper limb/neck and shoulder evaluated using a visual analogue scale (VAS), and rates of ASDis. In patients who underwent an anterior cervical approach in the first instance, the incidence of ASDis was significantly higher in the C3/4 gap than in the other gaps. In the ACCF group, the lateral radiograph of the cervical spine revealed that the distance between the anterior cervical plate and the adjacent segment disc was <5 mm in 15 (62.5%) cases and five (12.8%) cases, respectively, the internal fixation screws broke into the annulus of the adjacent segment. After the first SLAC, ASDis developed at C2/3 and C3/4 in four (30.8%) and eight (61.5%) cases, respectively. After reoperation, all cases were followed up for >5 (average, 6.2) years. The pre-reoperation and last follow-up values were as follows: mean Japanese Orthopaedic Association score, 10.2 ± 1.5 vs 15.5 ± 0.7 (P = 0.03); neck disability index, 26.2 vs 13.6 points (P = 0.01); upper-limb visual analog scale (VAS) score, 6.1 vs 2.6 points (P = 0.04); and neck and shoulder VAS score, 6.6 vs 2.1 points (P = 0.03). SLAC is a simple technique in which the local anatomy is clearly visible and satisfactory clinical outcomes are obtained.

[Consistency comparison of the parameters of the lumbar spine-pelvic sagittal plane between the whole-spine EOS images system and traditional X-ray].

OBJECTIVE: To explore the consistency of the parameters of the lumbar spine pelvic sagittal plane between the whole spine EOS images (EOS) and traditional X-ray imaging. METHODS: A total of 50 patients (26 males and 24 females) hospitalized in the Spine Surgery Department of Beijing Jishuitan Hospital from May to July 2019 were selected for standard standing EOS full-length spine anterolateral and traditional X-ray lumbar pelvic anterior and lateral X-rays. Two attending physicians used Surgimap software to measure the pelvic incidence (PI), pelvic tilt (PT), sacral slope (SS), lumbar lordosis (LL) of the two types of images and repeated these process after two weeks. The consistency test (reliability analysis) was performed on the results measured by two physicians, and the results measured at the two time points were tested for intra-observer consistency (repeatability analysis).The data were combined to perform consistency and difference tests for the parameters between two types of images finally. RESULTS: The mean values of PI measured by EOS imaging and traditional X-ray imaging were(50.5±12.6)° and (51.4±12.2)°, mean difference 0.9, 95% credible interval (0.2-1.6), P=0.020; the mean values of PT were (16.2±8.9)° and (16.9±8.6)°, mean difference 0.7, 95% credible interval (-0.6-2.0), P=0.283; the mean values of SS were (34.3±9.9)° and (34.5±10.4)°, mean difference 0.2, 95% credible interval (-1.2-1.5), P=0.800;the mean values of LL were (42.7±14.9)° and (43.3±15.3)°, mean difference 0.6, 95% confidence interval (-0.8-2.0), P=0.149. The difference in PI between the two imaging methods was statistically significant (P =0.020, P <0.05), but the average difference was small (0.9°), there was no clinical difference. There were no significant differences in PT, SS and LL between the two imaging methods (P>0.05). Inter-group reliability analysis showed excellent agreement between the two physicians in measuring lateral PI, PT, SS and LL using Surgimap software (correlation coefficients within EOS imaging were 0.984, 0.993, 0.980, 0.989;correlation coefficients within X-ray imaging were 0.975, 0.985, 0.976, 0.988). Repeatability analysis showed that PI, PT, SS and LL measured by the two attending physicians at two time points had excellent consistency(ICC within the group was 0.963-0.996). CONCLUSION: In the local lumbar pelvis segment, the PI, PT SS and LL measured by EOS imaging and traditional X-ray imaging had good agreement, and there was no difference in guiding clinical application.

Robot-Assisted Minimally Invasive Transforaminal Lumbar Interbody Fusion in the Treatment of Lumbar Spondylolisthesis.

OBJECTIVE: To compare the clinical efficacy between robot-assisted minimally invasive transforaminal lumbar interbody fusion (robot-assisted MIS-TLIF) and traditional open TLIF surgery in the treatment of lumbar spondylolisthesis. METHODS: According to the inclusion and exclusion criteria, 48 cases with lumbar spondylolisthesis who received surgical treatment from June 2016 to December 2017 in the spinal surgery department of Beijing Jishuitan Hospital were analyzed in this study, including 23 patients who received robot-assisted MIS-TLIF and 25 patients who received traditional open TLIF surgery. The two groups were compared in terms of pedicle screw accuracy evaluated by Gertzbein-Robbins classification on postoperative computed tomography (CT), operation time, blood loss, postoperative drainage, hospitalization, time to independent ambulation, low back pain evaluated by visual analog scale (VAS), lumbar function evaluated by Oswestry Disability Index (ODI), paraspinal muscles atrophy on magnetic resonance imaging (MRI), and complications. RESULTS: Postoperative CT showed that the rate of Grade A screws in the robot-assisted MIS-TLIF group was significantly more than that in the open surgery group (χ2 = 4.698, P = 0.025). Compared with the open surgery group, the robot-assisted MIS-TLIF group had significantly less intraoperative blood loss, less postoperative drainage, shorter hospitalization, shorter time to independent ambulation, and lower VAS at 3 days post-operation (P < 0.05). However, the duration of surgery was longer. The VAS of the robot-assisted MIS-TLIF group decreased from 6.9 ± 1.8 at pre-operation to 2.1 ± 0.8 at post-operation, 1.8 ± 0.7 at 6-month follow-up and 1.6 ± 0.5 at 2-year follow-up. The VAS of the open surgery group decreased from 6.5 ± 1.7 at pre-operation to 3.7 ± 2.1 at post-operation, 2.1 ± 0.6 at 6-month follow-up and 1.9 ± 0.5 at 2-year follow-up. The ODI of the robot-assisted MIS-TLIF group decreased from 57.8% ± 8.9% at pre-operation to 18.6% ± 4.7% at post-operation, 15.7% ± 3.9% at 6-month follow-up and 14.6% ± 3.7% at 2-year follow-up. The ODI of the open surgery group decreased from 56.9% ± 8.8% at pre-operation to 20.8% ± 5.1% at post-operation, 17.3% ± 4.2% at 6-month follow-up and 16.5% ± 3.8% at 2-year follow-up. Paraspinal muscle cross-sectional area in 2-year follow-up in patients of the open surgery group decreased significantly compared to patients of robotic-assisted MIS-TLIF group (P = 0.016). CONCLUSION: In the treatment of lumbar spondylolisthesis, robot-assisted MIS-TLIF may lead to more precise pedicle screw placement, less intraoperative blood loss, less postoperative drainage, less postoperative pain, quicker recovery, and less paraspinal muscle atrophy than traditional open surgery.

Changes in DNA methylation during epigenetic-associated sex reversal under low temperature in Takifugu rubripes.

DNA methylation has frequently been implicated in sex determination and differentiation in teleost species. In order to detect the DNA methylation patterns established during sexual differentiation in tiger pufferfish T. rubripes, we performed comprehensive whole genome methylation sequencing and analyses of the gonads of male, female, and pseudo male. We obtained a total of 33.12, 32.44, and 31.60 Gb clean data for male, female, and pseudo male, with a sequencing depth of 66.44×, 60.47× and 54.86×, respectively. The methylation level of cytosine (C) residues in the genomic DNA from gonads was 11.016%, 10.428%, and 11.083% in male, female, and pseudo male, respectively. More than 65% of C methylation was at CpG sites, and less than 1% was at CHG and CHH sites. In each regulatory element, there were low methylation levels on both sides of the transcription start site, and higher methylation levels in exons, introns, and downstream of genes. The highest mCpG was on chromosome 8 and the lowest mCpG was on chromosome 5. Comparisons of whole-genome DNA methylation between pairs of samples revealed that there were 3,173 differentially methylated regions (DMRs) between female and male, and 3,037 DMRs between male and pseudo male, corresponding to 0.232% and 0.223% of the length of the genome, respectively. There were only 1,635 DMRs between female and pseudo male, representing 0.127% of the length of the genome. A number of differentially methylated genes (DMGs) related to sex determination and differentiation were selected, such as amhr2 and pfcyp19a. After Bisulfite Sequencing PCR (BSP) verification, amhr2 was exhibited low methylation level in normal males and pseudo male, and high methylation level in normal females but pfcyp19a showed low methylation level in normal females and high methylation level in normal males and pseudo males. These results provide information about the molecular epigenetic mechanisms of DNA methylation during low-temperature induced masculinization of tiger pufferfish, and increase our understanding of the mechanisms of sex determination and differentiation in this important aquaculture fish species.

Robot-Assisted Versus Fluoroscopy-Guided Pedicle Screw Placement in Transforaminal Lumbar Interbody Fusion for Lumbar Degenerative Disease.

OBJECTIVE: To compare the clinical accuracy and perioperative outcomes for pedicle screw placement in transforaminal lumbar interbody fusion (TLIF) between the robot-assisted (RA) technique and fluoroscopy-guided (FG) technique. METHODS: Seventy-seven patients scheduled to undergo RA (n = 43) and FG (n = 44) TLIF surgery were included. Patient demographics, radiographic accuracy, and perioperative outcomes were recorded and compared. The accuracy of pedicle screw placement was according to the Gertzbein and Robbins scale and facet joint violation. Perioperative outcomes mainly included operative time, radiation exposure, and revisions. RESULTS: Of the 176 screws in the RA group, 164 screws were grade A, and 9, 2, and 1 screws were grades B, C, and D, respectively. Of the 204 screws in the FG group, 175 screws were grade A, with 16 screws scored as grade B, 8 screws scored as grade C, 3 screws scored as grade D, and 2 screws scored as grade E. The rate of perfect screw position (grade A) was higher in the RA group than in the FG group (93.2% vs. 85.8%, respectively; P = 0.020). In the FG group, 191 screws (93.6%) were clinically acceptable (groups A and B), whereas more acceptable screw positions were achieved in the RA group (98.3%; P = 0.024). Fewer screws in the RA group violated the proximal facet joint (5 vs. 24 screws, respectively; P = 0.001). The radiation dose was lower in the RA group (25.9 ± 14.2 vs. 70.5 ± 27.3 µSv, respectively; P < 0.001). Two screws in the FG group required a revision, but no revision was required in the RA group. CONCLUSIONS: RA pedicle screw placement is an accurate and safe procedure in TLIF for lumbar degenerative disease.

Robot-assisted Percutaneous Transfacet Screw Fixation Supplementing Oblique Lateral Interbody Fusion Procedure: Accuracy and Safety Evaluation of This Novel Minimally Invasive Technique.

OBJECTIVES: Percutaneous transfacet screw fixation (pTSF) is a minimally invasive posterior fixation technique supplementing oblique lateral interbody fusion (OLIF) for lumbar spinal disorders. Accurate screw insertion is difficult to achieve and technically demanding under 2-D fluoroscopy. Recently developed robot-assisted spinal surgery demonstrated a high level of accuracy of pedicle screw insertion and a low complication rate. No published study has reported this combination technique. The aim of our study was to evaluate the accuracy and safety properties of the combination of both minimally invasive techniques: robot-assisted pTSF supplementing the OLIF procedure. METHODS: This was an experimental and prospective study. Selected consecutive patients with lumbar degenerative disorders received robot-assisted pTSF supplementing the OLIF procedure using the TianJi Robot system operated by one senior surgeon from March to October 2018. The accuracy of screw insertion and perioperative screw-related complications were evaluated. Assessment of the accuracy of screw insertion included intraoperative robotic guidance accuracy and incidence of screw encroachments. Intraoperative robotic guidance accuracy referred to translational and angular deviations of screws, which were assessed by comparing the planned and actual screw trajectories guided by the robot on reconstructed images using TianJi Robot Planning Software. Screw encroachments were evaluated on postoperative CT images and classified by a grading system (A, excellent; B, good; C, poor). Screw-related complications including intraoperative pin skidding, screw malposition and adjustment, together with postoperative neurological symptoms that correlated with screw malposition were recorded. RESULTS: Ten patients, with an average age of 60.2 years, were selected and recruited in this study. All cases were degenerative lumbar spinal disorders, out of which there were 6 cases of Meyerding Grade I degenerative spondylolisthesis. Twenty-four transfacet screws were inserted by robotic assistance. Instrumented levels included nine segments at L4-5 level and three segments at L3-4 level. Two patients had both L4-5 and L3-4 level fixation. The average surgical time was 3.3 h (SD, 0.8 h). The mean blood loss was 90 mL (SD, 32 mL). Intraoperative guidance accuracy showed 1.09 ± 0.17 mm (ranging from 0.75 to 1.22 mm) translational deviation and 2.17° ± 0.39° (ranging from 1.47° to 2.54°) angular deviation. The gradings of screw encroachment were: 17 screws (71%) with Grade A, 6 screws (25%) with Grade B, and 1 screw (4%) with Grade C. Only one pin skidding occurred intraoperatively and revised subsequently. No postoperative neurological complications were found. CONCLUSION: Our preliminary study of robot-assisted pTSF supplementing the OLIF procedure showed a high level of accuracy for screw insertion and this minimally invasive combination technique was found to be a feasible and safe procedure.

[Underlying mechanisms of the heavy metal tolerance of mycorrhizal fungi].

Mycorrhizal fungi are ubiquitous in natural ecosystems and can form symbiotic associations with the majority of terrestrial plants. They can be detected even in heavy metal-contaminated soils, while some fungal strains show strong heavy metal tolerance and could potentially be used in bioremediation of contaminated soils. We reviewed current research progresses in the underlying mechanisms of heavy metal tolerance of mycorrhizal fungi, with focuses on habitat selection, physiological adaptation and functional genes. Future research perspectives were proposed to promote the basic research and development of mycorrhizal technology for remediation of heavy metal-contaminated soils.

Arbuscular mycorrhizal symbiosis influences arsenic accumulation and speciation in Medicago truncatula L. in arsenic-contaminated soil.

In two pot experiments, wild type and a non-mycorrhizal mutant (TR25:3-1) of Medicago truncatula were grown in arsenic (As)-contaminated soil to investigate the influences of arbuscular mycorrhizal fungi (AMF) on As accumulation and speciation in host plants. The results indicated that the plant biomass of M. truncatula was dramatically increased by AM symbiosis. Mycorrhizal colonization significantly increased phosphorus concentrations and decreased As concentrations in plants. Moreover, mycorrhizal colonization generally increased the percentage of arsenite in total As both in shoots and roots, while dimethylarsenic acid (DMA) was only detected in shoots of mycorrhizal plants. The results suggested that AMF are most likely to get involved in the methylating of inorganic As into less toxic organic DMA and also in the reduction of arsenate to arsenite. The study allowed a deeper insight into the As detoxification mechanisms in AM associations. By using the mutant M. truncatula, we demonstrated the importance of AMF in plant As tolerance under natural conditions.

Chromium resistance of dandelion (Taraxacum platypecidum Diels.) and bermudagrass (Cynodon dactylon [Linn.] Pers.) is enhanced by arbuscular mycorrhiza in Cr(VI)-contaminated soils.

In a greenhouse pot experiment, dandelion (Taraxacum platypecidum Diels.) and bermudagrass (Cynodon dactylon[Linn.] Pers.), inoculated with and without arbuscular mycorrhizal fungus (AMF) Rhizophagus irregularis, were grown in chromium (Cr)-amended soils (0 mg/kg, 5 mg/kg, 10 mg/kg, and 20 mg/kg Cr[VI]) to test whether arbuscular mycorrhizal (AM) symbiosis can improve Cr tolerance in different plant species. The experimental results indicated that the dry weights of both plant species were dramatically increased by AM symbiosis. Mycorrhizal colonization increased plant P concentrations and decreased Cr concentrations and Cr translocation from roots to shoots for dandelion; in contrast, mycorrhizal colonization decreased plant Cr concentrations without improvement of P nutrition in bermudagrass. Chromium speciation analysis revealed that AM symbiosis potentially altered Cr species and bioavailability in the rhizosphere. The study confirmed the protective effects of AMF on host plants under Cr contaminations.

Effect of total disc replacement on atypical symptoms associated with cervical spondylosis.

PURPOSE: To determine the efficacy of total disc replacement (TDR) for the treatment of cervical spondylosis associated with atypical symptoms. METHODS: In this retrospective study, patients with myelopathy and/or radiculopathy related to cervical spondylosis that were treated with TDR were contacted by phone. Atypical symptoms involved in the questionnaire included dizziness, headache, nausea, vomiting, blurred vision, tinnitus, palpitations, hypomnesia, and abdominal discomfort. The severity of each atypical symptom was evaluated separately based on an numerical rating scale. The radiographs and charts before and after the surgery were reviewed. Paired samples t tests were used to compare the severity of the symptoms before and after surgery. RESULTS: Between 2003 and 2010, 73 of 133 patients diagnosed with cervical spondylotic myelopathy and/or radiculopathy treated by TDR experienced atypical symptoms before surgery. The mean follow-up was 34.6 months. There were 47 males and 26 females (mean age 48.9 years). Of the 73 patients, 41 were diagnosed with myelopathy; 13 with radiculopathy; and 19 with mixed-type spondylosis. The incidence of each symptom before surgery was dizziness (46.6%), tinnitus (41.1 %), facial flushing and sweating (41.1 %), palpitations (39.7%), headache (35.6%), hypomnesia (30.1%), nausea and vomiting (20.5%), blurred vision (20.5%), and gastroenterologic discomfort (5.5%). The severity of the following symptoms improved after surgery: dizziness (p = 0.000, α = 0.05), headache (p = 0.000, α = 0.05), nausea and vomiting (p = 0.000, α = 0.05), blurred vision (p = 0.004, α = 0.05), tinnitus (p = 0.000, α = 0.05), palpitations (p = 0.000, α = 0.05), hypomnesia (p = 0.010, α = 0.05), and other symptoms (p = 0.030, α = 0.05). The gastroenterologic discomfort did not improve (p = 0.731, α = 0.05). CONCLUSIONS: TDR may have a positive effect on atypical symptoms associated with cervical spondylotic myelopathy and/or radiculopathy.

Intraoperative 3-dimensional navigation and ultrasonography during posterior decompression with instrumented fusion for ossification of the posterior longitudinal ligament in the thoracic spine.

STUDY DESIGN: A retrospective clinical study was conducted. OBJECTIVE: The purpose of this study was to describe the clinical outcomes of intraoperative 3D navigation (ITN) and ultrasonography during posterior decompression and instrumented fusion for thoracic myelopathy due to ossification of the posterior longitudinal ligament (OPLL). SUMMARY OF BACKGROUND DATA: The symptoms caused by thoracic-ossification of the posterior longitudinal ligament (T-OPLL) are usually progressive and do not respond to conservative treatment-surgical intervention is the only effective treatment option. Various methods have been described for the treatment of symptomatic T-OPLL, all of which have limitations. METHODS: The study included 18 patients with T-OPLL who underwent posterior decompression with instrumented fusion from 2006 to 2011. A staged operative procedure was used. First, pedicle screws were placed with ITN and a wide laminectomy was performed with resection of ossification of the ligamentum flavum (if present). With insufficient decompression on intraoperative ultrasonography, additional circumferential decompression was performed through a transpedicular approach. ITN-guided OPLL resection was performed using a burr attached to a navigational tracker. In all cases, posterior instrumented fusion was performed in situ. The outcomes were evaluated with the modified Japanese Orthopaedic Association scores and recovery rates. RESULTS: Intraoperative ultrasonography showed that posterior laminectomy was sufficient in 6 patients; the remaining 12 were treated with additional circumferential decompression. The follow-up period ranged from 1 to 6 years (mean period, 2.8 y). Postoperative transient neurological deterioration occurred in 1 patient, and cerebrospinal fluid leakage occurred in 4 patients. All patients showed neurological recovery with a mean Japanese Orthopaedic Association score that improved from 5.5 points preoperatively to 8.5 points at the final follow-up and a mean recovery rate of 54.5%. CONCLUSIONS: Intraoperative ultrasonography and ITN are safe and reliable imaging methods for posterior decompression and instrumented fusion for thoracic myelopathy caused by OPLL.

Occipital-C2 transarticular fixation for occipitocervical instability associated with occipitalization of the atlas in patients with klippel-feil syndrome, using intraoperative 3-dimensional navigation system.

STUDY DESIGN: Retrospective case series. OBJECTIVE: The aim of this study was to describe the clinical outcomes of cervical reduction and occipital-C2 transarticular (OCTA) fixation with an assistance of intraoperative 3-dimensional navigation system (ITNS) during the treatment of reducible occipitocervical instability (OCI) in patients with Klippel-Feil syndrome (KFS) with occipitalization of the atlas and fusion of C2-C3. SUMMARY OF BACKGROUND DATA: Patients with KFS have congenital fusions of at least 2 cervical segments and may gradually develop symptoms at the hypermobile articulations adjacent to the cervical synostosis. This is particularly common in patients with KFS with occipitalization of the atlas and C2-C3 fusion. These patients may be at risk for instability and neurological complications of the occipitocervical junction that require occipitocervical reconstruction and fusion. Numerous treatment techniques are available for this pathological condition. However, there has yet to be a study of reducible OCI, showing successful treatment with intraoperative reduction and posterior OCTA fixation using ITNS. METHODS: From 2006 to 2011, 9 patients with KFS with reducible OCI attributed to occipitalization of the atlas and C2-C3 fusion were surgically treated. After a limited foramen magnum decompression, reduction of the OCI was conducted by intraoperative cervical traction and extension, followed by OCTA fixation using a direct posterior approach and with the assistance of ITNS. The follow-up period ranged from 6 to 60 months (mean, 31 mo). RESULTS: Good decompression and bone fusion were achieved in all the patients. The clinical symptoms had improved for all patients. There were no intraoperative or postoperative complications. CONCLUSION: In patients with KFS with occipitalization of the atlas and C2-C3 fusion, manual cervical traction in tandem with cervical extension, followed by posterior OCTA fixation and fusion provides a safe, effective treatment of OCI and ventral brainstem impingement. IFTN is a feasible tool for monitoring cervical reduction and OCTA screw insertion in patients with KFS with this pathological condition.

Reversal of anterior cervical discectomy and fusion with cervical artificial disc replacement: regain motion after 9 years fusion.

STUDY DESIGN: Case report and review of the literature. OBJECTIVE: To present a conversion of an anterior cervical discectomy and fusion (ACDF) with a cervical artificial disc replacement in a 39-year-old woman and to review the relevant literature. SUMMARY OF BACKGROUND DATA: Return of functional spinal unit motion 9 years after attempted fusion is extremely rare. METHODS: The patient underwent an attempted anterior cervical discectomy and fusion 9 years earlier for bilateral hand numbness and leg weakness. Most of her neurological deficits had resolved after the index operation, but returned 2 months before the second operation and were unresponsive to nonoperative treatment. Computed tomography (CT) myelography showed recurrence of cervical disc herniation at the cephalad adjacent segment, which compressed the spinal cord. There was still some osteophyte at the C5/6 level that was also causing compression to the spinal cord. A solid fusion was suspected at this level. Surgery was performed to take down the grafted region and replace both levels with artificial disks. RESULTS: The range of motion (ROM) of the revised level at the 6-month follow-up was well preserved, there was no sign of instability at either operated level. The 6-month follow-up CT scan shows that, there was no obvious compression in the spinal canal. The remobilized facet joints of C5/6 segment have not demonstrated further degeneration. The patient's neck symptom and neurological function were significantly recovered. CONCLUSIONS: This case demonstrates application of a cervical artificial disc replacement to restore motion at a level that was previously grafted and fused. In select cases, cervical artificial disc replacement may represent a reasonable alternative to a repeated attempt at fusion. It is imperative that preoperatively lack of fusion of the facet joints be demonstrated on reconstructed CT scanning.

The "X-Factor" index: a new parameter for the assessment of adolescent idiopathic scoliosis correction.

The correction rate (CR) and fulcrum bending correction index (FBCI) based on the fulcrum bending radiograph (FBR) were parameters introduced to measure the curve correcting ability; however, such parameters do not account for contributions by various, potential extraneous "X-Factors" (e.g. surgical technique, type and power of the instrumentation, anesthetic technique, etc.) involved in curve correction. As such, the purpose of the following study was to propose the concept of the "X-Factor Index" (XFI) as a new parameter for the assessment of the correcting ability of adolescent idiopathic scoliosis (AIS). A historical cohort radiographic analysis of the FBR in the setting of hook systems in AIS patients (Luk et al. in Spine 23:2303-2307, 1998) was performed to illustrate the concept of XFI. Thirty-five patients with AIS of the thoracic spine undergoing surgical correction were involved in the analysis. Plain posteroanterior (PA) plain radiographs were utilized and Cobb angles were obtained for each patient. Pre- and postoperative PA angles on standing radiograph and preoperative fulcrum bending angles were obtained for each patient. The fulcrum flexibility, curve CR, and FBCI were determined for all patients. The difference between the preoperative fulcrum bending angle and postoperative PA angle was defined as Angle(XF), which accounted for the correction contributed by "X-Factors". The XFI, designed to measure the curve correcting ability, was calculated by dividing Angle(XF) by the fulcrum flexibility. The XFI was compared with the curve CR and FBCI by re-evaluating the original data in the original paper (Luk et al. in Spine 23:2303-2307, 1998). The mean standing PA and FBR alignments of the main thoracic curve were 58.3° and 24.5°, respectively. The mean fulcrum flexibility was 58.8%. The mean postoperative standing PA alignment was 24.7°. The mean curve CR was 58.0% and the mean FBCI was 101.1%. The mean XFI was noted as 1.03%. The CR was significantly positively correlated to curve flexibility (r = 0.66; p < 0.01).The FBCI (r = -0.47; p = 0.005) and the XFI (r = -0.45; p = 0.007) were significantly negatively correlated to curve flexibility. The CR was not correlated to Angle(XF) (r = 0.29; p = 0.089).The FBCI (r = 0.97; p < 0.01) and the XFI (r = 0.961; p < 0.01) were significantly positively correlated to Angle(XF). Variation in XFI was noted in some cases originally presenting with same FBCI values. The XFI attempts to quantify the curve correcting ability as contributed by "X-Factors" in the treatment of thoracic AIS. This index may be a valued added parameter to accompany the FBCI for comparing curve correction ability among different series of patients, instrumentation, and surgeons. It is recommended that the XFI should be used to document curve correction, compare between different techniques, and used to improve curve correction for the patient.

[Transforaminal lumbar interbody fusion by minimally invasive approach in the treatment of simple foraminal stenosis].

OBJECTIVE: To study the short-term clinical outcome of transforaminal lumbar interbody fusion (TLIF) by minimally invasive approach in the treatment of simple foraminal stenosis (SFS). METHODS: Thirty four cases (M: F = 19: 15) diagnosed with SFS from January. 2007 to March. 2009 were included. Age ranged from 47 to 62 (mean: 57) years old. Patients generally had degenerative changes and decreased disc height on X-ray films at the indexed level. No stenosis within the spinal canal was found on myelograph and CTM. Sagittal images of MRI usually demonstrated narrowed foramina. Selective radiculograph and radicular blocking was used to make the definite diagnosis and to locate the involved nerve root. Double paramedian intermuscular approaches were adopted in all cases. Minimally invasive retractor systems, such as X-Tube and Pipeline etc., were used to facilitate the exposure, pedicle screw instrumentation and TLIF. RESULTS: The average operation time was 2.76 hours. The average blood loss was 387.16 ml. All patients were encouraged to ambulate 2-3 days post-operation. The incisions healed uneventfully. At the final follow-up, instrumentations were maintained in an excellent position in all cases and JOA score improved by 31.14%. CONCLUSIONS: TLIF by minimally invasive approach is an effective method in treating patients with lumbar foraminal stenosis. The short-term clinical outcomes are satisfactory. This approach may avoid the injuries to paraspinal muscles, posterior ligaments, dura and nerve root of the uninvolved side.

[Comparison between the qualitative similarity and the quantitative similarity of chromatographic fingerprints of traditional Chinese medicines].

To explore the differences between the qualitative similarity and the quantitative similarity of chromatographic fingerprints of traditional Chinese medicines, the quantitative similarity calculated by vector shadow C%, apparent quantitative similarity R%, quantitative similarity P%, etc. were firstly proposed to disclose the quantitative information characteristics of traditional Chinese medicines fingerprints. The HPLC fingerprints of both the standard Fructus gardeniae and the ten batches of Fructus gardeniae produced in different places were evaluated by the new parameters to obtain good results. The contrasted fingerprint contained 35 peaks while geniposide was selected as the reference peak. The HPLC fingerprint had good precision and reproducibility with the RSD of the relative retention time less than 1.5% and the RSD of the relative peak area within 5%. The qualitative similarity and quantitative similarity between each crude drug and the contrasted fingerprint were quantitatively calculated, the values of C%, P%, etc., were applied in the quality control practice, which had less errors. What is more, this method could be used for the overall quality control of Fructus gardeniae and especially suits for qualitative and quantitative evaluations of the chromatographic fingerprints both in chemical constituent distribution and in contents. The quantitative parameters such as C% and P% can be used to objectively, authentically and thoroughly display the content information characteristics. When they combined with the qualitative similarity, it will be the good method to evaluate the chromatographic fingerprints of traditional Chinese medicines.