Spinal Deformity Study Group
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Tracheal Perforation in a Neonate: a Devastating
Letters to Editor Tracheal perforation in a size of endotracheal tube, cuff overinflation, cough and vigorous movements of head and neck.[2,3] neonate: A devastating Neonatal tracheal injury is ascribed to factors like complication following traumatic traumatic delivery, weak trachea, congenital tracheal endotracheal intubation stenosis, ring agenesis, metal stylets, rigid endotracheal tube, excessive external laryngeal pressure and [1,4,5] Sir, prolonged ventilation. Tracheal injury in neonates following endotracheal In our case, rigorous attempts at intubation along with intubation represents an uncommon complication excessive hyperextension of head and neck due to altered rarely described in literature but carries high anatomy are likely to have contributed to tracheal injury. morbidity and a mortality rate of 70%.[1] We describe Multiple attempts at intubation are known to result in a [4] a case of neonatal tracheal perforation following false tract and are related to anterior tracheal lesions. multiple attempts at endotracheal intubation due to an Incidentally, our case was an undiagnosed Pierre unanticipated difficulty in an emergency situation in Robins Sequence. A small receding mandible, tongue an undiagnosed case of Pierre Robin Sequence. immobility and cleft palate are identified as independent factors for difficult airway with a risk of upper airway A 4-week-old female baby presented to the emergency obstruction, difficult mask holding, difficult intubation, department with respiratory difficulty. In view of severe leak through the cleft resulting in inadequate ventilation respiratory distress and desaturation (SpO2- 60%) a as well as passage of the endotracheal tube into the decision was made to provide ventilatory support to cleft.[6] Successful airway management in such situation the neonate. -
Lordosis, Kyphosis, and Scoliosis
SPINAL CURVATURES: LORDOSIS, KYPHOSIS, AND SCOLIOSIS The human spine normally curves to aid in stability or balance and to assist in absorbing shock during movement. These gentle curves can be seen from the side or lateral view of the spine. When viewed from the back, the spine should run straight down the middle of the back. When there are abnormalities or changes in the natural spinal curvature, these abnormalities are named with the following conditions and include the following symptoms. LORDOSIS Some lordosis is normal in the lower portion or, lumbar section, of the human spine. A decreased or exaggerated amount of lordosis that is causing spinal instability is a condition that may affect some patients. Symptoms of Lordosis include: ● Appearance of sway back where the lower back region has a pronounced curve and looks hollow with a pronounced buttock area ● Difficulty with movement in certain directions ● Low back pain KYPHOSIS This condition is diagnosed when the patient has a rounded upper back and the spine is bent over or curved more than 50 degrees. Symptoms of Kyphosis include: ● Curved or hunched upper back ● Patient’s head that leans forward ● May have upper back pain ● Experiences upper back discomfort after movement or exercise SCOLIOSIS The most common of the three curvatures. This condition is diagnosed when the spine looks like a “s” or “c” from the back. The spine is not straight up and down but has a curve or two running side-to-side. Sagittal Balance Definition • Sagittal= front-to-back direction (sagittal plane) • Imbalance= Lack of harmony or balance Etiology • Excessive lordosis (backwards lean) or kyphosis (forward lean) • Traumatic injury • Previous spinal fusion that disrupted sagittal balance Effects • Low back pain • Difficulty walking • Inability to look straight ahead when upright The most ergonomic and natural posture is to maintain neutral balance, with the head positioned over the shoulders and pelvis. -
The Genetic Heterogeneity of Brachydactyly Type A1: Identifying the Molecular Pathways
The genetic heterogeneity of brachydactyly type A1: Identifying the molecular pathways Lemuel Jean Racacho Thesis submitted to the Faculty of Graduate Studies and Postdoctoral Studies in partial fulfillment of the requirements for the Doctorate in Philosophy degree in Biochemistry Specialization in Human and Molecular Genetics Department of Biochemistry, Microbiology and Immunology Faculty of Medicine University of Ottawa © Lemuel Jean Racacho, Ottawa, Canada, 2015 Abstract Brachydactyly type A1 (BDA1) is a rare autosomal dominant trait characterized by the shortening of the middle phalanges of digits 2-5 and of the proximal phalange of digit 1 in both hands and feet. Many of the brachymesophalangies including BDA1 have been associated with genetic perturbations along the BMP-SMAD signaling pathway. The goal of this thesis is to identify the molecular pathways that are associated with the BDA1 phenotype through the genetic assessment of BDA1-affected families. We identified four missense mutations that are clustered with other reported BDA1 mutations in the central region of the N-terminal signaling peptide of IHH. We also identified a missense mutation in GDF5 cosegregating with a semi-dominant form of BDA1. In two families we reported two novel BDA1-associated sequence variants in BMPR1B, the gene which codes for the receptor of GDF5. In 2002, we reported a BDA1 trait linked to chromosome 5p13.3 in a Canadian kindred (BDA1B; MIM %607004) but we did not discover a BDA1-causal variant in any of the protein coding genes within the 2.8 Mb critical region. To provide a higher sensitivity of detection, we performed a targeted enrichment of the BDA1B locus followed by high-throughput sequencing. -
Osteodystrophy-Like Syndrome Localized to 2Q37
Am. J. Hum. Genet. 56:400-407, 1995 Brachydactyly and Mental Retardation: An Albright Hereditary Osteodystrophy-like Syndrome Localized to 2q37 L. C. Wilson,"7 K. Leverton,3 M. E. M. Oude Luttikhuis,' C. A. Oley,4 J. Flint,5 J. Wolstenholme,4 D. P. Duckett,2 M. A. Barrow,2 J. V. Leonard,6 A. P. Read,3 and R. C. Trembath' 'Departments of Genetics and Medicine, University of Leicester, and 2Leicestershire Genetics Centre, Leicester Royal Infirmary, Leicester, 3Department of Medical Genetics, St Mary's Hospital, Manchester. 4Department of Human Genetics, University of Newcastle upon Tyne, Newcastle upon Tyne; 5MRC Molecular Haematology Unit, Institute of Molecular Medicine, Oxford; and 6Medical Unit and 7Mothercare Unit for Clinical Genetics and Fetal Medicine, Institute of Child Health, London Summary The physical feature brachymetaphalangia refers to shortening of either the metacarpals and phalanges of the We report five patients with a combination ofbrachymeta- hands or of the equivalent bones in the feet. The combina- phalangia and mental retardation, similar to that observed tion of brachymetaphalangia and mental retardation occurs in Albright hereditary osteodystrophy (AHO). Four pa- in Albright hereditary osteodystrophy (AHO) (Albright et tients had cytogenetically visible de novo deletions of chro- al. 1942), a dysmorphic syndrome that is also associated mosome 2q37. The fifth patient was cytogenetically nor- with cutaneous ossification (in si60% of cases [reviewed by mal and had normal bioactivity of the a subunit of Gs Fitch 1982]); round face; and short, stocky build. Affected (Gsa), the protein that is defective in AHO. In this patient, individuals with AHO may have either pseudohypopara- we have used a combination of highly polymorphic molec- thyroidism (PHP), with end organ resistance to parathyroid ular markers and FISH to demonstrate a microdeletion at hormone (PTH) and certain other cAMP-dependent hor- 2q37. -
Kyphectomy in Neonates with Meningomyelocele
Child's Nervous System (2019) 35:673–681 https://doi.org/10.1007/s00381-018-4006-4 ORIGINAL PAPER Kyphectomy in neonates with meningomyelocele Nail Özdemir1 & Senem Alkan Özdemir2 & Esra Arun Özer3 Received: 18 August 2018 /Accepted: 18 November 2018 /Published online: 11 December 2018 # Springer-Verlag GmbH Germany, part of Springer Nature 2018 Abstract Purpose Kyphosis is the most severe spinal deformity associated with meningomyelocele (MMC) and is seen in approximately 15% of neonates. Our purpose is to present our clinical experience, to discuss the technique and deformity correction in kyphectomy in neonates with MMC, and to assess its long-term outcomes. Method In this prospective study, the authors reviewed eight cases submitted to surgery between 2013 and 2015. We evaluated clinical characteristics that were analyzed, as were the operative technique employed, and angle range of the kyphosis deformity postcorrection follow-up. Results Neonatal kyphectomy was performed of six females and two males. The mean birth weight was 2780 g, and the mean age at the time of surgery was 5.6 days. There were S-shaped type deformity in lumbar region in all neonates. In the correction of the kyphotic deformity, a total vertebrae were removed from four patient, whereas a partial vertebrectomy was done in four. The mean operative time was 116 min. No patients did not require the blood transfusion. There were no serious complications, and wound closure was successful in all patients. The mean follow-up period was 4 years and 3 months (range 36–61 months), except one patient who died 1 week after discharge. -
Spondylolysis and Spondylolisthesis in Children and Adolescents: I
Spondylolysis and Spondylolisthesis in Children and Adolescents: I. Diagnosis, Natural History, and Nonsurgical Management Ralph Cavalier, MD Abstract Martin J. Herman, MD Spondylolysis and spondylolisthesis are often diagnosed in children Emilie V. Cheung, MD presenting with low back pain. Spondylolysis refers to a defect of Peter D. Pizzutillo, MD the vertebral pars interarticularis. Spondylolisthesis is the forward translation of one vertebral segment over the one beneath it. Isth- Dr. Cavalier is Attending Orthopaedic mic spondylolysis, isthmic spondylolisthesis, and stress reactions Surgeon, Summit Sports Medicine and involving the pars interarticularis are the most common forms seen Orthopaedic Surgery, Brunswick, GA. Dr. Herman is Associate Professor, in children. Typical presentation is characterized by a history of Department of Orthopaedic Surgery, activity-related low back pain and the presence of painful spinal Drexel University College of Medicine, mobility and hamstring tightness without radiculopathy. Plain ra- St. Christopher’s Hospital for Children, Philadelphia, PA. Dr. Cheung is Fellow, diography, computed tomography, and single-photon emission Department of Orthopaedic Surgery, computed tomography are useful for establishing the diagnosis. Mayo Clinic, Rochester, MN. Dr. Symptomatic stress reactions of the pars interarticularis or adjacent Pizzutillo is Professor, Department of vertebral structures are best treated with immobilization of the Orthopaedic Surgery, Drexel University College of Medicine, St. Christopher’s spine and activity restriction. Spondylolysis often responds to brief Hospital for Children. periods of activity restriction, immobilization, and physiotherapy. None of the following authors or the Low-grade spondylolisthesis (≤50% translation) is treated similarly. departments with which they are The less common dysplastic spondylolisthesis with intact posterior affiliated has received anything of value elements requires greater caution. -
Failure to Maintain Segmental Lordosis During TLIF for One-Level
European Spine Journal (2019) 28:745–750 https://doi.org/10.1007/s00586-019-05890-w ORIGINAL ARTICLE Failure to maintain segmental lordosis during TLIF for one‑level degenerative spondylolisthesis negatively afects clinical outcome 5 years postoperatively: a prospective cohort of 57 patients Matevž Kuhta1 · Klemen Bošnjak2 · Rok Vengust2 Received: 14 June 2018 / Revised: 28 November 2018 / Accepted: 13 January 2019 / Published online: 24 January 2019 © Springer-Verlag GmbH Germany, part of Springer Nature 2019 Abstract Purpose The present study aimed to determine whether obtaining adequate lumbar (LL) or segmental (SL) lordosis during instrumented TLIF for one-level degenerative spondylolisthesis afects midterm clinical outcome. Methods The study was designed as a prospective one, including 57 patients who underwent single-level TLIF surgery for degenerative spondylolisthesis. Patients were analyzed globally with additional subgroup analysis according to pelvic incidence (PI). Radiographic analysis of spinopelvic sagittal parameters was conducted pre- and postoperatively. Clinical examination including ODI score was performed preoperatively, 1 and 5 years postoperatively. Results Signifcant improvement in ODI scores at 1 and 5 years postoperatively (p < 0.001) was demonstrated. There was a signifcant correlation between anterior shift of SVA and failure to improve SL (p = 0.046). Moreover, anterior SVA shift correlated with increased values of ODI score both 1 and 5 years postoperatively. In low-PI group, failure to correct LL correlated with high ODI scores 5 years postoperatively (r = − 0.499, p = 0.005). Conclusions Failure to correct segmental lordosis during surgery for one-level degenerative spondylolisthesis resulted in anterior displacement of the center of gravity, which in turn correlated with unfavorable clinical outcome 1 and 5 years postoperatively. -
Scoliosis in Paraplegia
Paraplegia (1974), II, 290-292 SCOLIOSIS IN PARAPLEGIA By JOHN A. ODOM, JR., M.D. and COURTN EY W. BROWN, M.D. Children's Hospital, Denver, Colorado in conjunction with ROBERT R. JACKSON, M.D., HARRY R. HA HN, M.D. and TERRY V. CARLE, M.D. Craig Rehabilitation Hospital, Englewood, Colorado WITH the increasing instance of excellent medical care, more children with traumatic paraplegia and myelomeningocele with paraplegia live to adulthood. In these two groups of patients there is a high instance of scoliosis, kyphosis and lordosis. Much attention in the past years has been placed on hips and feet but only in the last decade has there been much attention concentrated on the treatment of the spines of these patients. Most of this attention has been toward the patient with a traumatic paraplegia with development of scoliosis. Some attempts have been made at fusing the scoliotic spine of myelomeningo celes by Harrington Instrumentation but with a rather high instance of complica tions and failures. With the event of anterior instrumentation by Dr. Alan Dwyer of Sydney, Australia, the anterior approach to the spine is becoming widely accepted and very successfully used. This is especially valuable in the correction and fusion of the spine with no posterior elements from birth. MATERIAL FOR STUDY Between March of 1971 and June of 1973, there have been 26 paraplegics with scoliosis who have been cared for by the authors. All of these patients have either been myelomeningoceles with paraplegia or spinal cord injuries, all of whom have had scoliosis, kyphosis, or severe lordosis. -
CERVICAL SPONDYLOSIS Pathogenesis the Canal Diameter Is
CERVICAL SPONDYLOSIS Pathogenesis The canal diameter is reduced by 1. Osteophytes, thickened ligamentum flavum, protruded disc 2. Hyperextension of the spine reduces the canal diameter by shingling effect of lamina and buckling of Ligamentum flavum 3. Retrolisthesis with extension 4. Hypermobility in the level above degenerated disc can cause myelopathy 5. Vascular compromise in spondylosis may cause myelopathy Disc is innervated by sinu‐vertebral nerve formed from ventral nerve root and sympathetic plexus. This nerve turns back at intervertebral foramen and supplies: annulus fibrosus, posterior longitudinal ligament, and periosteum of the vertebra Clinical A B C 1. Axial Pain patterns proved during discography at each level A Level between C2‐3 B “ C3‐4 C “ C4‐5 D “ C5‐6 E “ C6‐7 D E Look for trigger points. Pain is more on extending the neck. 2. Red flags: Night pain Persistent pain > 3months Any associated primary tumour Weight loss and sweat 3. Referred or radicular pain C6 to the thumb, C7 to the middle finger C8 to the little finger C6 nerve root exist between C5‐C6 vertebra. At cervical spine, both disc herneation and stenosis affect the exit root [In the lumbar region, transit root is involved in disc herniation] If more than one nerve root involvement: rule out myelopathy Sometimes, the pain can be referred to heart lungs and TMJ joint from Cervical spondylosis 4. Spurling’s manoeuvre: Extension and lateral rotation to the side of pain [refer clinical examination] Differential diagnosis for radiculopathy Peripheral entrapment syndrome Rotator cuff syndrome Brachial plexitis and herpes Spinal tumours Cardiac ischemia Investigations X rays AP, Lateral, Flexion‐extension lateral MRI is gold standard Myelopathy Types 1. -
Cervical Sagittal Alignment in Adolescent High Dysplastic
Guo et al. Journal of Orthopaedic Surgery and Research (2020) 15:243 https://doi.org/10.1186/s13018-020-01762-y RESEARCH ARTICLE Open Access Cervical sagittal alignment in adolescent high dysplastic developmental spondylolisthesis: how does the cervical spine respond to the reduction of spondylolisthesis? Xinhu Guo, Weishi Li* , Zhongqiang Chen, Zhaoqing Guo, Qiang Qi, Yan Zeng, Chuiguo Sun and Woquan Zhong Abstract Background: Although pelvic and related parameters have been well stated in lumbar developmental spondylolisthesis, cervical sagittal alignment in these patients is poorly studied, especially in high dysplastic developmental spondylolisthesis (HDDS). The purpose of this study is to investigate the sagittal alignment of the cervical spine in HDDS and how the cervical spine responds to reduction of spondylolisthesis. Methods: Thirty-three adolescent patients with lumbar developmental spondylolisthesis who received preoperative and postoperative whole-spine x-rays were reviewed. They were divided into the HDDS group (n = 24, 13.0 ± 2.2 years old) and the low dysplastic developmental spondylolisthesis (LDDS) group (n = 9, 15.6 ± 1.9 years old). Spinal and pelvic sagittal parameters, including cervical lordosis (CL), were measured and compared between groups. In the HDDS group, the postoperative parameters were measured and compared with those before surgery. Results: HDDS group had a higher proportion of cervical kyphosis (70.8% vs. 22.2%, P = 0.019), and there was a significant difference in CL between the two groups (− 8.5° ± 16.1° vs. 10.5° ± 11.8°, P = 0.003). CL was correlated with the Dubousset’s lumbosacral angle (Dub-LSA), pelvic tilt (PT), and thoracic kyphosis (TK). -
Acquired Degenerative Changes of the Intervertebral Segments at And
European Journal of Radiology 50 (2004) 134–158 Acquired degenerative changes of the intervertebral segments at and suprajacent to the lumbosacral junction A radioanatomic analysis of the nondiscal structures of the spinal column and perispinal soft tissues J. Randy Jinkins a,b,∗ a Department of Radiologic Sciences, Downstate Medical Center, State University of New York, Brooklyn, NY 11203, USA b Fonar Corporation, 110 Marcus Drive, Melville, NY 11747, USA Received 3 October 2003; received in revised form 9 October 2003; accepted 13 October 2003 Abstract A review of the imaging features of normal and degenerative anatomy of the spine on medical imaging studies shows features that have been largely overlooked or poorly understood by the imaging community in recent years. The imaging methods reviewed included computed tomography (CT) with multiplanar reconstructions and magnetic resonance imaging (MRI). A routine part of the MRI examination included fat-suppressed T2 weighted fast-spin- or turbo-spin-echo acquisitions. As compared to the normal features in asymptomatic volunteers, alterations in the observed CT/MRI morphology and MR signal characteristics were sought in symptomatic individuals. Findings in symptomatic subjects which departed from the normal anatomic features of the posterior spinal elements in asymptomatic volunteers included: rupture of the interspinous ligament(s), neoarthrosis of the interspinous space with perispinous cyst formation, posterior spinal facet (zygapophyseal joint) arthrosis, related central spinal canal, lateral recess (subarticular zone) and neural foramen stenosis, posterior element alterations associated with various forms of spondylolisthesis, and perispinal muscle rupture/degeneration. These findings indicate that the posterior elements are major locations of degenerative spinal and perispinal disease that may accompany or even precede degenerative disc disease. -
Promising: Process Improvement in Psychosocial Health
PROMISing: Process Improvement in Psychosocial Health Carly Woodmark MS │ Dereesa Reid MBA │ Daniel Bouton MD SHC-Portland │ Department of Performance Improvement Abstract no. 20 Shriners Team And Patients PROMISing Changes Shriners Hospitals for Children is a network of 22 non-profit medical facilities across North America. Benefits of PROMIS Intervention Pre-op Post-op Since 1924, SHC-Portland has treated a wide range of pediatric orthopedic conditions, from fractures to rare diseases and syndromes. Our Integrated Practice Unit of multi-disciplinary Minor burden of taking PROMIS is offset by quality professionals provide a comprehensive approach through specialized evaluation and treatment communication of meaningful progress between along with rehabilitative services to restore each child physically, emotionally, and socially. Below is patient/family & physician during clinic visit. a list of common conditions treated at SHC-Portland. Medical providers can demonstrate improvements Skeletal abnormalities – Osteogenesis imperfecta (OI), osteochondritis dissecans (OCD lesions), from interventions & adjust care management if Blount disease, skeletal dysplasias, etc. needed. Outcome Performance Improvement Neuromuscular conditions – Cerebral palsy, myelomeningocele (spina bifida), Muscular dystrophy, spinal muscular atrophy After one year of data collection, rates of Minimal Clinical Important Difference (MCID) were assessed for all patient-reported domains in both surgical and non-surgical populations. Multivariate Hand/Upper extremity