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    Spinal Deformities (Congenital)

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    deathmetal017
    This document discusses spinal deformities known as occult spinal dysraphism (OSD). OSD is a failure of closure of the spine without exposure of neural tissue. It can cause neurological symptoms and orthopedic deformities. Diagnosis involves imaging like MRI and treatment is surgical detethering of the spinal cord. Detethering improves symptoms in many patients, though re-tethering remains a risk. The document covers classification, embryology, pathophysiology, symptoms, diagnosis, treatment complications and outcomes of OSD and other spinal deformities like meningocele and meningomyelocele.

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    Attribution Non-Commercial (BY-NC)
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    Spinal Deformities (Congenital)

    Uploaded by

    deathmetal017
    23 views27 pages
    This document discusses spinal deformities known as occult spinal dysraphism (OSD). OSD is a failure of closure of the spine without exposure of neural tissue. It can cause neurological symptoms and orthopedic deformities. Diagnosis involves imaging like MRI and treatment is surgical detethering of the spinal cord. Detethering improves symptoms in many patients, though re-tethering remains a risk. The document covers classification, embryology, pathophysiology, symptoms, diagnosis, treatment complications and outcomes of OSD and other spinal deformities like meningocele and meningomyelocele.

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    Spinal

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    This document discusses spinal deformities known as occult spinal dysraphism (OSD). OSD is a failure of closure of the spine without exposure of neural tissue. It can cause neurological symptoms and orthopedic deformities. Diagnosis involves imaging like MRI and treatment is surgical detethering of the spinal cord. Detethering improves symptoms in many patients, though re-tethering remains a risk. The document covers classification, embryology, pathophysiology, symptoms, diagnosis, treatment complications and outcomes of OSD and other spinal deformities like meningocele and meningomyelocele.

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    23 views27 pages

    Spinal Deformities (Congenital)

    Uploaded by

    deathmetal017
    This document discusses spinal deformities known as occult spinal dysraphism (OSD). OSD is a failure of closure of the spine without exposure of neural tissue. It can cause neurological symptoms and orthopedic deformities. Diagnosis involves imaging like MRI and treatment is surgical detethering of the spinal cord. Detethering improves symptoms in many patients, though re-tethering remains a risk. The document covers classification, embryology, pathophysiology, symptoms, diagnosis, treatment complications and outcomes of OSD and other spinal deformities like meningocele and meningomyelocele.

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    of 27

    Spinal deformities

    (Congenital)
    Dep’t of Neurosurgery
    CMC,Ludhiana
    Introduction

    • Failure of closure of the spine referred to as


    spinal dysraphism or spina bifida.
    • May be associated defect in the soft tissues,
    creating open spina bifida
    (myelomeningocele etc.).
    • If no or minimal soft tissue defect so that
    neural tissue is not exposed-referred to as
    occult spinal dysraphism (OSD).
    Classification

    • Occult or hidden
    • Aperta : Visible

    According to site
    Cervical/Dorsal/Occipital/ Lumbosacral
    Anterior or posterior
    Embryology
    • lower lumbar and sacral region : secondary neurulation at
    about 28-30 days of age and may be more common in males.
    • upper lumbar region or higher :primary neurulation-formation
    of the neural tube at 21days gestational age, and may be more
    common in females.
    • OSD due to defects in separation of embryonic tissues after 30
    days of age.
    • May be due to combination of genetic and environmental
    factors. Due to use of folic acid in cereal and as peri-
    conceptual supplement (0.4 mg/day),
    • 2.5% Chance if sibling has the disease.
    Pathophysiology
    • In the embryo, distal spinal cord undergoes
    retrogressive differentiation and filum terminale (glio-
    ependymal strand) forms.
    • Spinal column (bone) elongates and grows more quickly
    than spinal cord, so conus moves up-at term lies at L2-3
    vertebral level and by 3 months at L1-2 level.
    • If spinal cord tethered at site of dysraphism, can’t move
    up and becomes stretched.
    • Stretching can increase due to growth until the teens;
    later deterioration probably due to excessive stretching/
    superimposed degenerative change in spine.
    Symptoms and Signs
    • Cutaneous stigmata: hypertrichosis, capillary
    hemangioma, dermal sinus, subcutaneous lipoma,
    caudal appendage.
    • Neurological symptoms/signs: back and leg pain,
    weakness (UMN or LMN), sensory and/or reflex
    change.
    • Orthopedic deformities: club foot, small leg,
    asymmetric feet/legs, scoliosis.
    • Urological problems: frequency/urgency,
    neurogenic bladder, incontinence.
    cutaneous signs
    OSD-Leg changes
    OSD-Scoliosis
    Diagnosis

    • History and Physical.


    • Imaging: plain x-rays, CT, MRI.
    • Urodynamic testing.
    • EMG/nerve conduction studies.
    • In Utero testing :USG, Amniocentesis
    MRI-tethered spinal cord
    MRI-tethered spinal cord cont.
    MRI-spinal cyst/spina bifida
    MRI-lipomyelomeningocele
    MRI-lipomyelomeningocele cont.
    Treatment

    • Natural history: majority show neurological


    deterioration with non-surgical treatment.
    (Keating-urinary incontinence in 26% of
    infants and 92% of older children with
    lipomyelomeningocele; Hoffman-85% of
    untreated kids with lipomyelomeningocele
    showed decreased neurological function by
    age 5 yrs.)
    Treatment (cont)
    • Treatment of choice=surgical de-tethering.
    • In most studies, back and leg pain improved
    post-op.(86% improved-Huttmann et al).
    • Spasticity better in 71%.
    • Bladder dysfunction improved in 44%.
    • Sensorimotor deficits better in only 35%.
    • Worse outcome if deficits present >5 yrs.
    Pre-op.
    Complications

    • Reported complication rates vary and are


    generally about 5 -10%.
    • Post-op problems include: loss of
    sphincter control/incontinence, increased
    weakness or sensory deficits, persistent
    pain, infection, spinal instability.
    Long Term Outcome

    • Literature rates of re-tethering vary from 10-


    50%.
    • The longer patients are followed, the higher the
    rate of re-tethering is likely to be.
    • Almost all post-op MRI scans still show radiologic
    evidence of tethering!
    • Therefore only treat patients if symptomatic.
    Meningocele

    • 1-2/1000
    • 1/3 have neurological deficits
    • Surgical repair with water-tight dural
    closure
    Meningomyelocele

    • 1-2/1000 live birth


    • Failure of complete
    closure of caudal
    neural tube
    • 85% occur in lumbar
    region
    Split cord Malformation

    • SCM I : Two hemi cords each with dural


    sac , bony spur
    • SCM II Single sac surrounding two parts
    Conclusion

    • Developmental anomalies of the spinal


    cord and spine are important in practice
    • Greater awareness is important as newer
    techniques of detethering and closure can
    help prevent and reduce neurological
    deficits

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