Percutaneous Intervention in Kyphoscoliosis—Tackling a Tortuosity

Spine deformities have several associations with compromised cardiorespiratory function. Percutaneous coronary interventions rely on fluoroscopic anatomical landmarks to guide procedures, and distortion of the mediastinal soft tissue and skeletal anatomy in conditions like kyphoscoliosis can pose unique challenges for the interventional cardiologist. Here, we report a case of elderly kyphoscoliotic male patient with worsening angina, who underwent successful percutaneous coronary intervention via the radial route.

Free Hand One Stage Correction of Kyphoscoliosis

Background: Scoliosis is a complex musculoskeletal torsional deformity of spine that includes: Lateral curvature in the anterior-posterior plane with a Cobb angle greater than 10 degrees, Angulation in the sagittal plane, or Rotation in the transverse plane. Scoliosis classified into neuromuscular, idiopathic, or congenital. Radiological evaluation is done by plain radiography, computed tomography (CT), and magnetic resonance (MR). We aimed to evaluate patients with idiopathic kyphoscoliosis who underwent a freehand posterior approach for scoliosis correction through transpedicular screws fixation. Setting: Al-Azhar University Hospitals. Subjects and Methods: Study was performed on 12 patients with idiopathic kyphoscoliosis who underwent a freehand posterior approach for scoliosis correction through transpedicular screws fixation in Al-Azhar University Hospitals between 2015 to 2018 & follow up for one year. Results: Early outcome showed improved Cobb’s angle and coronal balance significantly after surgery. Correction is 95% in 2 cases (17%), 80% correction in 8 cases (66%), 60% correction in 2 cases (17%). 2 cases have CSF leakage which improved with conservative treatment. Late follow-up shows improvement of patient deformity and cosmetic appearance to the degree of patient satisfaction occur in 7 cases (58%) associated with high SRS-30 total scores. However, assessment of the pain in comparison to preoperative assessment show improvement of pain in 4 cases (33%), persistent in 4 cases (33%), appear in 4 cases (33%). Conclusion: Freehand one-stage correction management of idiopathic kyphoscoliosis through the posterior approach is a safe and effective for near-total correction of deformity with a good outcome with minimal complications.

Surgical treatment of severe scoliosis and kyphoscoliosis by stages

Background Although previous reports had reported the use of temporary internal distraction as an aid to correct severe scoliosis,two-stage surgery strategy （less invasive internal distraction followed by posterior correction and instrumentation） has never been reported in the treatment of patients with severe spinal deformity.This study aimed to report the results of the surgical treatment of severe scoliosis and kyphoscoliosis by two-stage and analyse the safety and efficacy of this surgical strategy in the treatment of severe spinal deformities.Methods A total of 15 patients with severe scoliosis,kyphoscoliosis or kyphosis who underwent two-stage surgeries （less invasive internal distraction followed by posterior correction and instrumentation） were studied based on hospital records.Pretreatment radiographs and radiographs taken after first surgery （internal distraction by two small incisions）,before second surgery （posterior correction,instrumentation and fusion）,one week after second surgery and final follow-up were measured.Subjects were analyzed by age,gender,major coronal curve magnitude,flexibility of major curve,major sagittal curve magnitude before first surgery,after first surgery,before second surgery,after second surgery and at final follow-up.Complications related to two-stage surgeries were noted in each case.Results The average major curve magnitude was 129.4° （range,95° to 175°）,reduced 58.9° or 45.4% after first stage surgery and reduced 30.6° or 24.6% after second stage surgery.The loss of correction during the interval between two surgeries was 7.1%.The total major coronal curve correction was 81.4°or 62.9%.At the final follow up,the average loss of correction of major coronal curve was 3.9° and the final average correction rate was 59.7%.The average major sagittal curve magnitude was 80.3° （range,30° to 170°）,and the total major sagittal curve correction was 48.2°.Loss of correction averaged 4.0° for major sagittal curve and the final correction averaged 42.2°.Clinical complications were noted in the peri-operative and long-term periods.Conclusions Two-stage surgery was a safe and effective surgical strategy in this difficult population.Using two-small-incision technique,the first stage surgery was less invasive.No permanent neurologic deficit was noted in this series.

Autogenous tibial strut grafts used in severe kyphoscoliosis: surgical policies and preliminary results

Background Surgery for severe kyphoscoliosis is frequently unsatisfying because of loss of correction, high rate of pseudarthrosis and neurological complications. Several authors reported that the anterior fusion with strut grafts might improve the surgical outcome. This article describes and assesses the surgical strategies and the application of autogenous tibial strut in the treatment of severe kyphoscoliosis. Methods Severe kyphoscoliosis patients （n = 39 ） admitted from April 1998 to September 2003 formed the study group. Different surgical strategies had been used according to the flexibility, neurological function and curve level. All patients received staged anterior and posterior surgery with a tibial strut used in the anterior fusion. The patients were followed up for 9 months to 6 years （ mean 37 months）. Results The mean preoperative and postoperative kyphosis was 82° and 52° respectively, and the mean seoliosis was 84° and 44° respectively. Complications included pseudarthrosis （2 eases, one with graft fracture and the other with hook displacement） , posterior elements fractures （4） , pleura penetrations （3, in the plastic surgery of the thoracic cage） , dura tear （2） , exudative pleuritis （2, in the anterior surgery） , and tibia fracture of the harvesting site （ 1 ）. The mean loss of correction in coronal and sagittal plane was 6° and 7° respectively. Except for 1 ease, the patients with incomplete paraplegia showed improvements to varying extents. Conclusion Autogenous tibial strut can provide excellent support to the kyphoscoliotic spine： it reduces pseudarthrodic rate, loss of correction and complications of graft harvest, it also prevents the occurrence of neurological impairment.

Identification of a novel nonsense mutation in kyphoscoliosis peptidase gene in an Iranian patient with myofibrillar myopathy

Myofibrillar myopathies(MFMs)are rare genetic and slowly progressive neuromuscular disorders.Several pathogenic mutations have been reported in MFM-related genes including DES,CRYAB,MYOT,LDB3 or ZASP,FLNC,BAG3,FHL1 and DNAJB6.Although MFMs is commonly inherited in an autosomal dominant manner,the inheritance pattern and novel mutated genes are not thoroughly elucidated in some cases.Here,we report discovery of a novel nonsense mutation in a 29-year-old Iranian male patient with motor disorders and deformity in his lower limbs.His parents are second cousins.Hereditary Motor Sensory Neuropathy as initial genetic diagnosis was ruled out.Whole exome sequencing using NGS on Illumina Hi-Seq4000 platform was performed to identify the disease and possible mutated gene(s).Our data analysis identified a homozygous nonsense unreported c.C415T(p.R139X)variant on kyphoscoliosis peptidase(KY)gene(NM_178554:exon4).Sanger sequencing of this mutation has been performed for his other related family members.Sequencing and segregation analysis was confirmed the NGS results and autosomal recessive inheritance pattern of the disease.