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BMC Musculoskelet Disord 2019 Feb 7;20(1):54. Epub 2019 Feb 7.

Faculty of Rehabilitation, Józef Piłsudski University of Physical Education, Marymoncka 34, 00-968, Warsaw, Poland.

Background: The majority of individuals with spinal muscular atrophy (SMA) experience progressive skeletal deformities which may affect the quality of life and mobility. To date, no studies have evaluated the reliability of tests assessing body posture and joint mobility in SMA patients. The purpose of this study was to assess the reliability of Cervical Rotation test (CR), Supine Angle of Trunk Rotation test (SATR), Hip Extension test (HE) and Pelvic Obliquity test (PO) developed to evaluate the musculoskeletal system in SMA individuals. Read More

Eur Spine J 2019 Feb 1. Epub 2019 Feb 1.

Department of Orthopaedics, Key Laboratory of Orthopaedics of Zhejiang Province, The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, 109# Xueyuan Road, Wenzhou, 325027, Zhejiang, China.

Eur Spine J 2019 Jan 24. Epub 2019 Jan 24.

Department of Spine, Shandong Provincial Hospital Affiliated to Shandong University, No. 9677, Jingshi Road, Jinan, Shandong Province, China.

Spine Deform 2019 Mar;7(2):304-311

Division of Orthopaedics, The Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA 19104, USA. Electronic address:

Study Design: Prospective multicenter comparative study.

Objectives: We aimed 1) to survey surgeons and caregivers to rank the surgical indications for spinal fusion of pediatric patients with neuromuscular scoliosis secondary to cerebral palsy in order of importance and 2) to characterize the agreement of surgeons and caregivers on major (top three) indications.

Summary Of Background Data: Surgery for spinal deformity in children with cerebral palsy is a multifaceted and individualized decision that may lead to miscommunication during informed consent. Read More

Spine Deform 2019 Mar;7(2):286-292

Center for Spinal Disorders, Department of Orthopedic Surgery, Nicklaus Children's Hospital, 3100 S.W. 62nd Ave, Miami, FL 33155, USA.

Study Design: Single-center retrospective study.

Objective: To analyze two-year postoperative outcomes following spinopelvic fixation in pediatric patients using the anatomic trajectory (AT) portal for iliac screws.

Summary: Iliac fixation is crucial in situations requiring fusion to sacrum. Read More

Spine Deform 2019 01;7(1):33-39

Department of Orthopaedic Surgery, Boston Children's Hospital, Boston, MA, USA.

Design: Retrospective comparative study.

Objective: The purpose of this study is to measure SSI outcomes before and after implementation of our center's multidisciplinary clinical pathway protocol for high-risk spinal surgery.

Background: Surgical site infections (SSIs) after spinal fusion harm patients and are associated with significant health care costs. Read More

Spine Deform 2019 01;7(1):158-162

Children's Healthcare of Atlanta, Scottish Rite Campus, 1001 Johnson Ferry Road NE, Atlanta, GA 30342-1605, USA. Electronic address:

Background: Children with congenital heart disease (CHD) have been reported to be at increased risk of developing scoliosis following cardiac surgery. Previous sample studies have reported that these patients may safely undergo posterior spinal fusion (PSF) with low complication rates. The goal of this study is to provide an updated analysis of the perioperative complication profile for posterior spinal fusion in a large cohort of pediatric patients with CHD, using a nationwide database. Read More

Spine Deform 2019 01;7(1):112-117

Rady Children's Hospital, 3020 Children's Way, San Diego, CA 92123, USA.

Study Design: Retrospective review of a prospective cohort.

Objective: To identify patient and surgical factors that alter the length of postoperative intensive care unit (ICU) stays after spinal fusion/instrumentation in patients with neuromuscular scoliosis secondary to cerebral palsy (CP).

Summary Of Background Data: High perioperative complication rates in patients with CP contribute to the practice of utilizing the ICU postoperatively for monitoring. Read More

Spine Deform 2019 01;7(1):107-111

Pediatric Orthopaedic Surgery, Washington University School of Medicine, St. Louis, MO 63110, USA.

Objective: The objective of this study is to identify preoperative laboratory values and patient factors that are associated with postoperative respiratory complications in pediatric neuromuscular scoliosis (NMS) populations undergoing posterior spinal fusion (PSF) with instrumentation.

Summary Of Background Data: PSF in NMS patients are high-risk surgeries. Respiratory complications are the most common postoperative event, with rates up to 28. Read More

Int J Mol Sci 2018 Dec 17;19(12). Epub 2018 Dec 17.

John Walton Muscular Dystrophy Research Centre, Newcastle University, Newcastle upon Tyne NE1 3BZ, UK.

The neuromuscular junction (NMJ) appears to be a site of pathology in a number of peripheral nerve diseases. Charcot-Marie-Tooth (CMT) 4C is an autosomal recessive, early onset, demyelinating neuropathy. Numerous mutations in the gene have been shown to underlie the condition often associated with scoliosis, foot deformities, and reduced nerve conduction velocities. Read More

Orv Hetil 2018 Dec;159(50):2144-2149

Fogászati és Szájsebészeti Klinika, Pécsi Tudományegyetem, Klinikai Központ Pécs, Dischka Győző u. 5., 7621.

From an anatomic and functional point of view, the stomatognathic system and the upper cervical spine are closely connected. Together with the complex neuromuscular relationships, this generates an important field of cooperation between dentists and orthopedics. The aim of this case report was to demonstrate the improvements of temporomandibular joint (TMJ) function after orthopedic surgery in case of a patient with idiopathic scoliosis. Read More

Eur Spine J 2019 Feb 27;28(2):324-344. Epub 2018 Nov 27.

, Stockholm, Sweden.

Introduction And Purpose: Little information exists on surgical characteristics, complications and outcomes with corrective surgery for rigid cervical kyphosis (CK). To collate the experience of international experts, the CSRS-Europe initiated an international multi-centre retrospective study.

Methods: Included were patients at all ages with rigid CK. Read More

World J Orthop 2018 Oct 18;9(10):185-189. Epub 2018 Oct 18.

Department of Orthopaedic Surgery, Children's Hospital of Philadelphia, Philadelphia, PA 19104, United States.

Pelvic instrumentation for neuromuscular scoliosis has been part of neuromuscular scoliosis surgery since the era of the Luque Galveston construct. Unit Rod (Medtronic Sofamor-Danek, Nashville, TN) instrumentation brought with it the concept of cantilever correction by placing the implants in the pelvis and then gradually bringing the rod to the spine by sequentially tightening the sublaminar wires, with the goal of creating a level pelvis over a straight spine. More recently surgeons have utilized pedicle screw constructs in which the corrective strategies have varied. Read More

Spine Deform 2018 Nov - Dec;6(6):781-786

Department of Orthopedic Surgery, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. Electronic address:

Background: Early-onset scoliosis (EOS) is a potentially life-threatening condition that requires early intervention to prevent severe deformity and pulmonary compromise. Observational studies have demonstrated that children receiving multiple procedures requiring general anesthesia are at a higher risk for adverse neurocognitive outcomes. We sought to characterize anesthetic exposure in EOS patients and to determine risk factors for increased exposure. Read More

Spine Deform 2018 Nov - Dec;6(6):736-740

Washington University School of Medicine, 660 S Euclid Ave, St. Louis, MO 63110, USA. Electronic address:

Study Design: Prospective consecutively enrolled cohort.

Objectives: To evaluate paraspinal muscle concentration of intravenously administered vancomycin, at predetermined time points, during posterior spinal fusion (PSF) with instrumentation in neuromuscular scoliosis (NMS).

Summary: Surgical site infection (SSI) after PSF for NMS can be a devastating complication, which may lead to prolonged antibiotic use, multiple additional surgical procedures, pseudarthroses, and sepsis. Read More

Plast Reconstr Surg 2019 Jan;143(1):152e-164e

Seoul, Republic of Korea From the Department of Plastic Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine.

Background: Single-stage latissimus dorsi neuromuscular transfer has been a valuable option for dynamic smile reanimation. However, there is a paucity of studies evaluating the potential donor morbidity in such cases. The present study aimed to comprehensively analyze the donor morbidity following functional latissimus dorsi muscle transfer. Read More

Spine (Phila Pa 1976) 2018 Oct 5. Epub 2018 Oct 5.

Children's Orthopaedic Center, Children's Hospital Los Angeles.

Study Design: Multicenter retrospective OBJECTIVE.: To compare pelvic obliquity correction and reoperation rate in neuromuscular scoliosis patients who had their pelvis included in a posterior spinal fusion (pelvic fusion, PF) at their index procedure vs revision procedures.

Summary Of Background Data: There is limited information on outcomes specific to fusing to the pelvis for neuromuscular scoliosis in a revision operation versus index surgery. Read More

Acta Chir Orthop Traumatol Cech 2018 ;85(2):89-93

Ortopedická klinika Lékařské fakulty Masarykovy univerzity a Fakultní nemocnice Brno.

PURPOSE OF THE STUDY The Growth Guidance System (GGS) represent a relatively new alternative to the traditional growing systems used for the treatment of early onset scoliosis. Ranking among the main aims of this surgical treatment is a three-dimensional correction of the deformity, maintenance of spinal growth and postponement of the necessity of final treatment by spondylodesis. MATERIAL AND METHODS Our study retrospectively evaluates the results of surgical correction in a group of 35 patients treated by GGS technique. Read More

Int J Spine Surg 2018 Jan 30;12(1):15-21. Epub 2018 Mar 30.

Division of Spine Surgery, Department of Orthopaedic Surgery, Warren Alpert Medical School of Brown University, Providence, Rhode Island.

Patients with Parkinson's disease (PD) commonly develop severe spinal deformity, including scoliosis, antecollis, camptocormia, and Pisa syndrome. The etiology of PD-associated spinal deformity is not completely understood and in most cases is likely due to multiple interrelated factors, including central dystonia and focal myopathy. Once spinal deformity has occurred, surgery is often the only modality that can correct the condition, although control of the movement disorder through medication and deep brain stimulation may slow progression. Read More

Acta Orthop Traumatol Turc 2018 Nov 27;52(6):438-441. Epub 2018 Sep 27.

Uludag University College of Medicine, Department of Orthopaedics and Traumatology, Bursa, Turkey.

Objective: The aim of this study was to examine the use of magnetically controlled growing rods as a method of providing spinal improvement while preventing thoracic insufficiency in patients with early-onset scoliosis (EOS).

Methods: Of a total of 13 patients, 4 patients underwent a dual magnetic rod implantation, while 9 patients had a single magnetic rod procedure. The study group comprised 12 (93%) female and 1 (7%) male patients. Read More

Acta Orthop Traumatol Turc 2018 Nov 25;52(6):435-437. Epub 2018 Sep 25.

Uludag University College of Medicine, Orthopedics and Traumatology Department, Bursa, Turkey. Electronic address:

Introduction: Neuromuscular scoliosis leads to a wide range of spinal disorders which disturb the musculoskeletal system. The aim of this study is to compare the clinical and radiological results of posterior spinal fusion with and without extending the instrumentation to iliac bones in treatment of neuromuscular scoliosis.

Methods: Medical records and radiographies of 36 patients with neuromuscular scoliosis who underwent posterior instrumentation between 2011 and 2015 were reviewed. Read More

Acta Chir Orthop Traumatol Cech 2018 ;85(3):194-198

Ortopedická klinika Lékařské fakulty Masarykovy univerzity a Fakultní nemocnice Brno 2 Klinika radiologie a nukleární medicíny Lékařské fakulty Masarykovy univerzity a Fakul.

PURPOSE OF THE STUDY Neuromuscular deformities of the spine represent surgically uneasy to solve problems as well as serious handicaps causing sitting instability, pressure ulcers as well as pain. The aim of our study is to conduct a retrospective clinical analysis of the results of surgical correction of these deformities. This paper presents the use of a recent technique of sacral-alar-iliac (S2AI) screws and its comparison with other techniques of pelvic stabilisation. Read More

J Pediatr Orthop 2018 Sep 20. Epub 2018 Sep 20.

Center for Thoracic Insufficiency Syndrome, Children's Hospital of Philadelphia, Philadelphia, PA.

Background: Over the past 100 years, many procedures have been developed for correcting restrictive thoracic deformities which cause thoracic insufficiency syndrome. However, none of them have been assessed by a robust metric incorporating thoracic dynamics. In this paper, we investigate the relationship between radiographic spinal curve and lung volumes derived from thoracic dynamic magnetic resonance imaging (dMRI). Read More

Muscle Nerve 2019 Jan 26;59(1):129-133. Epub 2018 Oct 26.

Department of Medical Genetics, Institute of Mother and Child, Kasprzaka 17a St, 01-211, Warsaw, Poland.

Introduction: Mutations in the TRPV4 gene are associated with neuromuscular disorders and skeletal dysplasias, which present a phenotypic overlap.

Methods: Next-generation sequencing and Sanger sequencing were used to analyze the TRPV4 gene.

Results: We present 2 Polish families with TRPV4-related disorder harboring the same p. Read More

JB JS Open Access 2018 Mar 12;3(1):e0045. Epub 2018 Mar 12.

Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Japan.

Background: The surgical treatment of congenital heart disease is reported to be associated with a high prevalence of scoliosis, although the detailed etiology is unknown. Surgical interventions involving the rib cage are considered to increase the risk of scoliosis. However, whether the cardiac condition or the procedure performed makes patients more susceptible to the development of spinal deformity is controversial. Read More

J Dent Anesth Pain Med 2018 Aug 28;18(4):261-265. Epub 2018 Aug 28.

Department of Anesthesiology and Reanimation, Erzincan Binali Yıldırım University, Faculty Of Medicine, Erzincan, Turkey.

Rett syndrome (RS) is a neurodevelopmental disorder characterized by loss of cognitive, motor, and social skills, epilepsy, autistic behavior, abnormal airway patterns, gastroesophageal reflux, nutritional problems, and severe scoliosis. Although girls with RS show normal or near-normal growth until 6-8 months, they lose their skills after that. The anesthetic management of these patients requires care because of all these clinical features. Read More

J Orthop Surg Res 2018 Sep 5;13(1):227. Epub 2018 Sep 5.

Department of Orthopedic Surgery, Okayama University Hospital, 2-5-1 Shikata-cho, Okayama City, Okayama, 700-8558, Japan.

Background: Intraoperative neuromonitoring using motor evoked potentials (MEP) satisfactorily detects motor tract integrity changes during spinal surgery. However, monitoring is affected by "anesthetic fade," in which the stimulation threshold increases because the waveform amplitude decreases with the accumulation of propofol. Therefore, the purpose of this study was to clarify the effect of anesthetic fade on transcranial MEPs by investigating the time-dependent changes of amplitude during spinal deformity surgeries. Read More

Spine (Phila Pa 1976) 2018 Aug 31. Epub 2018 Aug 31.

Department of Orthopedic Surgery, Toronto Western Hospital, University of Toronto, Toronto, ON.

Study Design: A case report.

Objective: The aim of this study was to report the use of erector spinae plane (ESP) blocks as part of an opioid-free multimodal anesthetic regimen and its impact on postoperative pain and opioid requirements following spine surgery.

Summary Of Background Data: Posterior spinal fusion surgery is highly painful and usually requires significant amounts of opioids for adequate perioperative analgesia; this is commonly associated with adverse effects, including opioid-induced hyperalgesia, nausea, and ileus. Read More

J Child Orthop 2018 Aug;12(4):406-412

Growing Spine Foundation, Milwaukee, Wisconsin, USA.

Purpose: To investigate paediatric orthopaedists' cast practices for early onset scoliosis regarding patient selection, cast application, radiographic evaluation, treatment cessation and adjunctive bracing.

Methods: A casting survey was distributed to all paediatric orthopaedists in Children's Spine and Growing Spine Study Groups (n = 92). Questions included physician and patient characteristics, technique, treatment, outcomes, radiographic measurements and comparison to other treatments. Read More

Eur Spine J 2018 Aug 24. Epub 2018 Aug 24.

Division of Orthopedics, The Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA, 19104, USA.

Background: Neuromuscular scoliosis is often treated with posterior spinal fusion, with or without anterior release, and either a same-day or staged, 2-day procedure.

Methods: We retrospectively reviewed 222 patients from a prospectively collected, multi-center database of patients with cerebral palsy scoliosis with 2-year follow-up. Baseline characteristics, perioperative, radiographic, and HRQoL measures were compared in six sub-analyses: (1) staged versus same-day surgeries, (2) posterior-only fusion (PSF) versus anterior-posterior spinal fusion (APSF), (3) same-day versus staged PSF, (4) staged versus same-day APSF, (5) same-day PSF versus same-day APSF, (6) staged PSF versus staged APSF. Read More

Spine Deform 2018 Sep - Oct;6(5):607-613

Riverside County Regional Medical Center, 26520 Cactus Ave., Moreno Valley, CA 92555, USA.

Objectives/summary Of Background Data: Segmental sublaminar spinal instrumentation without fusion results in guided growth and correction of deformity in early-onset scoliosis (EOS). The purpose of this study is to report the outcomes of a series of children with low-tone EOS that were treated surgically with a modification of a Luque trolley technique.

Methods: This study is a retrospective chart and radiographic review of a single-center series of 13 consecutive children who met inclusion criteria with documented progression of scoliosis greater than 25°. Read More

Iowa Orthop J 2018 ;38:147-152

Background: It is unknown what role specific tasks associated with personal care, positioning, communication and social interaction, and comfort and emotions play in predicting the overall health and quality of life of individuals with non-ambulatory cerebral palsy (CP). In this study, we prospectively evaluated which of these factors were significant predictors of overall health and quality of life.

Methods: Parents and guardians of non-ambulatory children, adolescents and young adults with CP were prospectively recruited from the Cerebral Palsy Clinic of a large pediatric academic hospital. Read More

Neurology 2018 Sep 10;91(10):e917-e930. Epub 2018 Aug 10.

From the Department of Neurology (K.R., I.D., C.H., C.D., J.B.S.), RWTH Aachen University; JARA-BRAIN Institute Molecular Neuroscience and Neuroimaging (K.R., I.D., C.H., C.D., J.B.S.), Forschungszentrum Jülich GmbH and RWTH Aachen University, Germany; Department of Molecular Neuroscience (P.G.), Ataxia Center, UCL Institute of Neurology, London, UK; Unit of Genetics of Neurodegenerative and Metabolic Diseases (C.M.), Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy; ICM (Brain and Spine Institute) Sorbonne Universités (A.D.), UPMC Univ Paris 06 UMR S 1127, and INSERM U 1127, CNRS UMR 7225 and APHP, Pitié-Salpêtrière University Hospital, Genetic Department, Paris, France; Department of Neurology (S.B.), Medical University Innsbruck, Austria; Department of Neurology (T.K.), Friedrich Baur Institute, University Hospital of the Ludwig-Maximilians-Universität München; German Center for Neurodegenerative Diseases (DZNE) (T.K.), Munich; Munich Cluster for Systems Neurology (SyNergy) (T.K.), Munich, Germany; Reference Unit of Hereditary Ataxias and Paraplegias (F.J.R.d.R.G.), Department of Neurology, IdiPAZ, Hospital Universitario La Paz, Madrid, Spain; Department of Neurodegenerative Diseases (L.S.), Hertie-Institute for Clinical Brain Research, University of Tübingen; Department of Neurology (I.G.), University Hospital of Bonn; German Center for Neurodegenerative Diseases (DZNE) (I.G.), Bonn; Department of Neurology (K.B.), Philipps University of Marburg, Germany; and Laboratory of Experimental Neurology (M.P.), Université Libre de Bruxelles, Brussels, Belgium.

Objective: To provide a systematic evaluation of the broad clinical variability in Friedreich ataxia (FRDA), a multisystem disorder presenting mainly with afferent ataxia but also a complex phenotype of nonataxia symptoms.

Methods: From the large database of the European Friedreich's Ataxia Consortium for Translational Studies, 650 patients with genetically confirmed FRDA were included. Detailed data of medical history documentation, questionnaires, and reports on clinical features were analyzed to provide in-depth description of the clinical profile and frequency rates of phenotypical features with a focus on differences between typical-onset and late-onset FRDA. Read More

J Neurosurg Pediatr 2018 Nov;22(5):591-594

Departments of1Neurosurgery and.

OBJECTIVESpine surgery is less common in children than adults. These surgeries, like all others, are subject to complications such as bleeding, infection, and CSF leak. The rate of incidental durotomy in the pediatric population, and its associated complications, has scarcely been reported in the literature. Read More

J Spine Surg 2018 Jun;4(2):342-348

Department of Orthopaedic Surgery, Cleveland Clinic, Cleveland, OH, USA.

Background: Clinical decision making, preoperative planning, and surgical correction for adolescent idiopathic scoliosis (AIS) has traditionally focused on obtaining the maximum coronal plane correction to improve cosmesis and function. More recently, restoring sagittal alignment has also received increasing attention in AIS patients, correlating with positive health-related quality of life (HRQOL) outcomes in multiple studies. In this realm, cervical sagittal alignment (CSA) has also emerged as one of the variables that may correlate with clinical and functional outcomes in AIS patients undergoing surgical correction. Read More

BMC Musculoskelet Disord 2018 Aug 2;19(1):277. Epub 2018 Aug 2.

Department of Spine Surgery, the Affiliated Drum Tower Hospital of Nanjing University Medical School, 321 Zhongshan Road, Nanjing, China.

Background: It is a great challenge for spine surgeons to correct severe rigid scoliosis. We developed a three- staged correction (one stage posterior release and screw placement, two stage skull-femoral traction and three stage posterior instrumentation) for adult severe scoliosis. The objective of this study is to investigate safety and efficacy of a three- staged correction for adult severe scoliosis. Read More

Adv Pediatr 2018 Aug 12;65(1):249-266. Epub 2018 Jun 12.

Department of Orthopaedic Surgery, Harbor UCLA Medical Center, 1000 West Carson Street, Torrance, CA 90509, USA.

Eur Spine J 2018 Sep 23;27(9):2213-2222. Epub 2018 Jul 23.

Department of Orthopaedics, Key Laboratory of Orthopaedics of Zhejiang Province, The Second School of Medicine of Wenzhou Medical University, The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, 109# Xueyuan Road, Wenzhou, 325027, Zhejiang, China.

Purpose: Neuromuscular scoliosis (NS) is a complicated spinal disorder, and it could be treated through posterior-only approach (POA) or combined anterior-posterior approach (APA), which one is better and how to choose the surgical tactic is still in controversy. So comparing POA with APA parameters in the treatment of NS is meaningful.

Methods: Database of PubMed, Embase and Cochrane Library was systematically searched, and the studies, which focus on the comparisons of POA and APA in the treatment of NS, were included. Read More

J Clin Monit Comput 2018 Jul 20. Epub 2018 Jul 20.

Department of Anesthesiology, Affiliated Drum Tower Hospital of Medical Department of Nanjing University, 321 Zhong Shan Road, Nanjing, Jiangsu, 210008, People's Republic of China.

Transcranial electric motor evoked potentials (TCeMEPs) play an important role in reducing the risk of iatrogenic paraplegia. TCeMEPs could be obviously suppressed by neuromuscular blockade (NMB). The aims of this study were to examine the effects of NMB on TCeMEPs and to determine an appropriate level of partial neuromuscular blockade (pNMB) for TCeMEPs during surgical correction of idiopathic scoliosis under total intravenous anesthesia (TIVA). Read More

Pediatr Radiol 2018 11 18;48(12):1797-1805. Epub 2018 Jul 18.

Department of Radiology, Phoenix Children's Hospital, 1919 E. Thomas Road, Phoenix, AZ, 85016, USA.

Background: Spinal muscular atrophy (SMA) is an autosomal-recessive neuromuscular disorder resulting in progressive muscle weakness. In December 2016, the U.S. Read More

Neurology 2018 Aug 13;91(7):e620-e624. Epub 2018 Jul 13.

From the Department of Neurology, Division of Neuromuscular Medicine (A.V., R.P., K.E., E. Collins, E. Ciafaloni), Department of Imaging Sciences, Division of Diagnostic and Interventional Neuroradiology (S.D., I.Y., P.-L.W.), and Department of Neurology, Division of Child Neurology (J.K.), University of Rochester Medical Center, NY.

Objective: To report our experience delivering intrathecal nusinersen through cervical puncture in patients with spinal muscular atrophy (SMA) with no lumbar access.

Background: SMA is a neuromuscular disorder characterized by profound muscle weakness, atrophy, and paralysis due to degeneration of the anterior horn cells. Nusinersen, the first Food and Drug Administration-approved treatment for SMA, is administered intrathecally via lumbar puncture; however, many patients with SMA have scoliosis or solid spinal fusion with hardware that makes lumbar access impossible. Read More

Neuromuscul Disord 2018 Aug 15;28(8):639-645. Epub 2018 Jun 15.

National Neuromuscular Centre, University Hospital of North Norway, Norway; Department of Medical Genetics, University Hospital of North Norway, Norway.

Autosomal recessive Charcot-Marie-Tooth disease (CMT) is considered rare and phenotypic descriptions are scarce for the different subgroups. Mutations in the SH3TC2 gene, causing recessive demyelinating CMT type 4C have been found in several Norwegian CMT patients over the last years. We aimed to estimate a minimum prevalence and to study the genotypic and phenotypic variability of CMT4C in Norway. Read More

J Pediatr Orthop 2018 Jul 2. Epub 2018 Jul 2.

Children's of Alabama, Department of Orthopaedic Surgery, University of Alabama at Birmingham, Birmingham, AL.

Background: Large national databases have been increasingly used in recent years to determine the rate of adverse events and identify factors associated with altered surgical outcomes. This can be especially useful to evaluate rare events such as 30-day mortality. Despite differences between national pediatric databases, there have been no comparison studies in the pediatric orthopaedic population. Read More

Neurosurg Focus 2018 Jul;45(VideoSuppl1):V2

2Pediatric Orthopedic Surgery, Amiens University Medical Center, Amiens, France.

Fusionless surgery coupled with growing rod techniques is increasingly advocated for the treatment of early-onset scoliosis in general and neuromuscular scoliosis in particular. Iliosacral screws have excellent biomechanical characteristics but are hard to place safely. Here, the authors report on robot-assisted iliosacral screw positioning as part of growing rod surgery for the fusionless correction of early-onset scoliosis. Read More

Chin Med J (Engl) 2018 Jul;131(13):1569-1574

Department of Neurology, Chinese People's Liberation Army General Hospital, Beijing 100853, China.

Background: Myoclonic epilepsy with ragged red fibers (MERRF) syndrome is characterized by myoclonus, generalized epilepsy, cerebellar ataxia, and ragged red fibers (RRFs) in the muscle. T-to-C transition at nucleotide position 14709 in the mitochondrial tRNA glutamic acid (tRNA) gene has previously been associated with maternally inherited diabetes and deafness. However, the association between MERRF and mitochondrial T14709C mutation (m. Read More

J Orthop Surg (Hong Kong) 2018 May-Aug;26(2):2309499018782556

1 Division of Orthopaedics, Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Purpose: Feeding difficulties are common among patients with cerebral palsy (CP) and neuromuscular (NM) scoliosis. We theorize that posterior spinal fusion (PSF) reduces intra-abdominal pressure, resulting in improved feeding and subsequent weight gain. We hypothesized that, among nonambulatory patients with CP and NM scoliosis, we would observe significant gain in weight following PSF. Read More

Eur J Orthop Surg Traumatol 2018 Jun 18. Epub 2018 Jun 18.

University of Tennessee-Campbell Clinic, Memphis, TN, USA.

Objective: The purpose of this study was to assess the impact of patient position on the magnitude of the coronal Cobb angle measurements in relation to the change of position using plain radiograph on non-ambulatory children with myelodysplasia. Whole-spine radiographs with the patient sitting generally are preferred for the diagnosis and monitoring of progression of scoliosis in neuromuscular patients. Supine, supine traction, and sitting push-up positions have been used as substitutes, although there is no general consensus validating if these positions correlate with the sitting position. Read More

Spine (Phila Pa 1976) 2019 Jan;44(1):60-67

Texas Childrens Hospital, Houston, TX - USA.

Study Design: Prospective case series of nine children with early-onset scoliosis (EOS) treated by a single surgeon with a novel implant, the magnet-driven growing rod (MdGR) in a publicly funded health care service accounting for "payer costs" (PC) incurred.

Objective: The aim of this study was to compare the cost-effectiveness of MdGR versus conventional growing rods (CGRs) with respect to the PC incurred for treating EOS at 5 years.

Summary Of Background Data: Cost estimate and mathematical modeling study projections of MdGR have shown despite high insertional costs, it breaks even with CGR by 3 to 4 years. Read More

Spine Deform 2018 Jul - Aug;6(4):460-466

Department of Orthopaedic Surgery, Children's Hospital Colorado, Aurora, CO, USA. Electronic address:

Study Design: Retrospective prognostic study.

Objective: The purpose of this study assess was to assess the results of spinal fusion and identify factors associated with the development of post-spinal fusion infections in patients with myelomeningocele.

Background: Surgical correction of neuromuscular scoliosis secondary to myelomeningocele is known to be associated with a high complication rate. Read More