Spinal dysraphisms of the cervicothoracic region in childhood (original) (raw)
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Spinal Dysraphism of a Baby – A Case Report
BIRDEM Medical Journal, 2016
A 8 months old male child reported to paediatric OPD of Shaheed Suhrawardy Medical College Hospital with the complaints of small swelling in the back and tilting of body on one side during sitting and standing posture. His antenatal period was unremarkable, post natal period revealed delayed crying and immunization was not completed. Local examination showed a small hairy patch in lower dorsal region. General and systemic examination revealed no significant abnormality. Consulting physician advised to do X-ray of dorso-lumbar spine (both views), Ultrasonography (USG) of the swelling and FNAC from the swelling. X-ray report concluded as spinal dysraphism with widening of spinal canal. Ultrasonography showed dural ectasia. No malignant or granulomatous cells were found in FNAC. For confirmation of plain x-ray and USG findings, the patient's parent was advised to do MRI of dorsolumbar spine in Radiology and Imaging Department of BIRDEM hospital. MRI findings were compatible with Diastematomyelia with tethered cord as evidenced by two unequal hemicord separated by signal void cleft and low insertion of spinal cord at L4-L5 level. As MRI is less sensitive regarding bone , CT scan was done and CT revealed grossly abnormal vertebral bodies with an osseous spur traversing the entire canal dividing the canal into two halves. Spinal cord was also divided into two unequal cord. Considering X-ray, USG, CT and MRI, final Diagnosis was made as Type I Diastematomyelia with Tethered cord.
Spinal Dysraphisms: A New Anatomical–Clinicoradiological Classification
Indian Journal of Radiology and Imaging
Background Spinal dysraphisms refer to the congenital abnormalities of the spine and spinal cord due to aberrations in the processes of gastrulation, primary neurulation, and secondary neurulation. Embryology of many complex spinal dysraphisms are yet poorly understood and there is no agreeable anatomical–clinicoradiological classification with inclusion of recently documented and complex spinal dysraphisms. Aims and Objectives The main objective of this study was to review the imaging features of spinal dysraphisms with a better understanding of embryological abnormalities and propose a new classification inclusive of all complex and unusual dysraphisms based on anatomical and clinicoradiological correlation. Materials and Methods This was a retrospective single institutional observational study of 391 cases of spinal dysraphism for 10 years in our institution. Of 391 cases included in the study, 204 were males and 187 were females. Also, 123 cases belonged to the 0–6 months age gr...
Spinal Dysraphism: Common Entity in Pediatric Neurosurgery
Nepal Journal of Neuroscience
Introduction: Neural tube defects are among the most common congenital malformations and a major cause of health problems in surviving children, especially in developing countries. Although the incidence of spinal dysraphism has significantly decreased over the last few decades, all over the world; however, the incidence is much higher in developing countries with poor socioeconomic status. The social and economic impact of this disease is not well documented; however, up to 75% of adult survivors may be dependent on parents or other care providers.Aims and Objectives: The aim of this study is to review the demographic profile, clinical pretentions, surgical management and short term outcome of patients presenting with spinal dysraphism Methodology: This is a prospective observational study of cases of spinal dysraphism managed surgically over the period of 2 years from March 2014 to February 2016 in Department of Neurosurgery at Tribhuvan University Teaching Hospital (TUTH), Kathma...
Spectrum of Spinal Dysraphism in Pediatric Patients in a Tertiary Care Hospital
Pakistan Journal Of Neurological Surgery, 2021
Objectives: To report the spectrum of spinal dysraphism presenting in pediatric patients admitted to the Department of Neurosurgery Lady reading hospital Peshawar. Material & Methods: A descriptive case series was conducted and total of 89 patients (age between 2 months to 12 years) were included who underwent the surgical treatment. All patients were examined for clinical and radiological diagnosis of spinal dysraphism. Results: The average age at treatment was 23 ± 39.77 months. The most recurring (52.8%) presenting symptom was the swelling on the back followed by lower limb weakness. Meningocele was reported in 11.2%, myelomeningocele in 47.2%, myelomeningocele & hydrocephalus in 12.4%, tethered cord syndrome in 25.8%, and diastematomyelia 3.4% of patients. Excision combined with the repair was done in 58.4%, release & repair done in 29.2% and endoscopic third ventriculostomy/ventriculoperitoneal shunts with the repair were done in 12.4% patients. Cerebrospinal fluid leak was ...
Spinal dysraphic anomalies; classification, presentation and management
Paediatrics and Child Health, 2014
Spinal dysraphism comprises an array of congenital anomalies of spinal cord development. Each of the dysraphic disorders can result in neurological, orthopaedic and urological dysfunction. This potentially confusing array of conditions is best understood from the embryological perspective. Whilst not all of these conditions are managed in the same way similar principles govern the initial investigation, multidisciplinary evaluation and long term follow up of these cases.
Report of two adult with occult cervical spinal dysraphism and scoliosis
Abant Medical Journal, 2014
Spinal disrafizm (SD), embriyogenez sırasında nöral tüpün kapanmasındaki bozukluğa bağlı omurganın kemik ve nöral yapılarının kısmi birleşmesi veya malformasyonudur. Omurganın arka kısmının orta hat boyunca tam olarak kapanmaması söz konusudur. SD sıklıkla torakolomber ve ve lumbosakral seviyelerde oluşur ve servikal SD tüm spinal anomalilerin %1-5'ini oluşturan nadir bir durumdur. SD açık ve kapalı(gizli) olmak üzere iki şekilde ortaya çıkmaktadır. Gizli SD, genellikle düz grafi veya manyetik rezonans görüntüleme sırasında tesadüfen saptanmaktadır. Klinik olarak önemli gizli SD, kas kuvvetsizliği, cilt anormalliği, Klippel-Feil Sendromu, torasik hemivertebra, ayak deformitesi, duysal anormallik, üriner inkontinans, yürüyüş bozukluğu ve skolyoza neden olabilir. Boyun ve sırt ağrısıyla başvuran ve gizli SD ve torakolomber skolyoz saptanan iki erişkin kadın olguyu sunuyoruz. Servikal SD nadiren bildirilmesine rağmen, dermatolojik, nörolojik ve ürogenital hastalıklarla birlikte skolyoz gibi ortopedik bozukluklarla da birlikte ortaya çıkabilir. Spinal dysraphism (SD) is the partial fusion or malformation of bone and neural structures of the spine by errors in the closure of the neural tube during embryogenesis. SD commonly occurs at the thoracolumbar and lumbosacral levels, and cervical SD is an uncommon condition that comprises 1-5% of all spinal anomalies. Occult SD is generally diagnosed incidentally on plain radiography or magnetic resonance imaging. Clinically important occult SD may cause muscle weakness, cutaneous abnormality, Klippel-Feil syndrome, thoracic hemivertebra, foot deformity, sensory abnormality, urinary incontinence, gait abnormality and scoliosis. We report two adult females with occult cervical spinal dysraphism and thoracolumbar scoliosis who presented with neck and shoulder pain. Although cervical SD has rarely been reported, this report highlights that may be associated with orthopedic abnormalities such as scoliosis as well as cutaneous, neurologic and urogenital disorders.
Spinal Dysraphism; Frequency of Rare Cases of Spinal Dysmorphism in View of Mri Findings
THE PROFESSIONAL MEDICAL JOURNAL, 2017
ORIGINAL PROF-3787 ABSTRACT… Introduction: MRI plays an important role in characterising spinal dysraphisms. The reason behind this is quality of MRI showing very high contrast resolution of soft tissues and the ability to detect fat in the lesion.T2 weighted images provide excellent contrast between the sub-arachnoid spaces and neural tissue while evaluating spinal dysraphisms. Objectives: To determine the frequency of rare disorders of spinal dysraphism in view of MRI findings of spinal dyspharism patients. Study Design: retrospective. Duration: the study was conducted from 1 st September 2015 to 30 th November 2015. Settings: Radiology department, Allied hospital Faisalabad. Methods: A total of 20 consecutive pediatric age group patients presented with clinical suspicion of spinal dysraphism were included. MRI L/S spine was carried out on 1.5Tesla Philips and diagnosis of various disorders of spinal dysraphism was made. Conclusion: MRI is the first choice examination for the detection of occult spinal dysraphism. Its timely diagnosis improving surgical & medical outcome as well as better & long term care of patients with spinal dysraphism. Majority of patients in the current study had tethered cord (75%), myelomeningocele (20%), myelocele (10%), lipomyelocele (10%) Dermal sinus tract (10%) & diastematomyelia (10%) followed by associated spinal lipomas (5%), caudal agenesis (5%) & anterior meningocele (5%).
Congenital Spine and Spinal Cord Malformations— Self-Assessment Module
American Journal of Roentgenology, 2010
Congenital abnormalities of the spine and spinal cord are referred to as spinal dysraphisms. This article reviews nor mal embryological development of the spine and spinal cord and the imaging findings of congenital abnormalities of the spine and spinal cord with particular focus on MRI. Conclusion Knowledge of the normal development of the spine and spinal cord provides a framework for understanding these complex entities.
Indian Journal of Pediatrics, 1999
Spinal dysraphism (SO) is characterized by maldevelopment of neural tube, notochord, mesoderm and cutaneous ectoderm. Incidence of SD is 2–4/1000 live births. One hundred and nineteen patients operated from January 1991-June 1996 at Department of Neurosurgery, All India Institute of Medical Sciences, were studied. Only 21 patients (17.6%) presented when they were less than one year old and 17 patients came in adult age group (> 16 years). Lumbar and lumbosacral region was the commonly involved site in 81 patients (74.7%). Weakness of lower limbs (74%), difficulty in walking (54%),muscle atrophy (41.2%) were the commonest indicators of motor system involvement. Loss of sensation, trophic ulcer, backache were seen in 45, 14, 10 patients respectively. Cutaneous lipoma (26%), hypertrichiosis (20%), dermal sinus (13.4%), midline dimples (7%) were the important cutaneous markers. Foot and limb deformity was seen in 25% cases. Tethering of cord, syringomyelia & split cord malformation were the most common radiological findings. Only 10% of our patients had hydrocephalus that requiredshunt. Out on 119 cases operated, 43 improved. Vwenty had sensory improvement and 18 showed motor improvement. Fifteen patients regained continence. Twelve patients were lost to follow-up. Sixtyseven patients had no change in neurological status, post-operatively. Six cases deteriorated in terms of motor or sensory deficit and one patient lost continence. CSF leak (8%) and wound infection (6%) were the common complications. Six patients required second surgery as T.R Shunt (4), rotation flap (1), reexploration and duraplasty(1).
Postnatal Intracranial Findings Following Fetal Repair of Spinal Dysraphisms
Journal of Computer Assisted Tomography, 2020
Objective: Our objective is to document the imaging appearance in the intracranial compartment at the time of the infants' first postnatal brain MR imaging after fetal repair for spinal dysraphisms. Methods: Twenty-nine patients were evaluated on fetal and postnatal magnetic resonance imaging for a series of features of Chiari II malformation. Results: Of the 29 infants, 55% had resolution of tonsillar ectopia, and 62% showed a dorsal outpouching of the near the foramen magnum on postnatal magnetic resonance imaging. The majority had persistence of Chiari II features including: prominent massa intermedia (93%), tectal beaking (93%), towering cerebellum (55%), flattening of the fourth ventricle (90%), hypoplastic tentorium (97%), and tonsillar hypoplasia (59%). Conclusions: Normally positioned or minimally descended, oftentimes hypoplastic tonsils in the presence of a posterior fossa configuration typical of Chiari II, was the most common presentation. An additional documented feature was an outpouching of the dorsal thecal sac between the opisthion and the posterior arch of C1.