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  • Spina Bifida

    Dr Hasan Nugud

    Consultant

    Paediatric Surgeon DR/HN/1

  • Spina Bifida

    Spina Bifida (SB), is a neural tube defect (NTD), that occurs when the vertebrae (arches) and /or spinal cord of the fetus fail to fuse in the mid line with or without protrusion (herniation) and dysplasia of the spinal cord ,its associated membranes and distal nerves , resulting in varying degrees of CNS damage .

    Dr/HN/2

  • Spina Bifida

    Spina bifida (SB) itself means split spine.

    When the brain itself is not completely developed the condition is called anencephally.

    When a portion of it the spine, is abnormally formed , spina bifida results,

    The damage is permanent.

    Dr/HN/3

  • Spina Bifida

    Calssification :

    1-Spina bifida occulta,

    2-Spina bifida cystica;

    a-Meningocele,

    b-Myelomeningocele,

    3-Rare types :

    -Amelia,

    -Rachischisis

    Dr/HN/4

  • Spina Bifida

    Spina bifida is actually one of a

    number of conditions called NTD

    that occur when the CNS of the

    fetus fails to fold and fuse at some

    point along it length.

    This can occur anywhere from the

    brain to the end of the spinal

    cord.The bones (ver- tebral

    arches) of the spinal column which

    surround the developing spinal

    cord do not close . Dr/HN/5

  • Spina Bifida

    Spina Bifida Occulta :

    The vertebrae are bifid without a

    menigocele or myelomeningocele

    below the intact skin, commonly

    affects the 5th lumbar and first

    sacral vertebrae

    Dr/HN/6

  • Spina Bifida

    Spina Bifida Occulta :

    The term suggests that the lesion

    is hidden below the intact skin, but

    it is not always so, sometimes the

    presence a signature mark as a

    superficial clue may over lie the

    anomaly such as; a patch of a dark

    hair,pigmented naevus,

    angiomatus area, lipoma, simple

    dimple or sinus. Dr/HN/7

  • Spina Bifida

    Dr/HN/8

  • Spina Bifida

    Dr/HN/9

  • Spina Bifida

    Amelia :

    The spinal cord may be absent, split or

    disorganized,

    Amelia occurs with gross spina bifida

    associated with anencephaly

    Rachischisis:

    The most severe form. Incomplete

    failure of fusion of the neural plates

    Neural tube lies open. No sac is

    present, falttend spinal cord red down

    the back.

    Dr/HN/10

  • Spina Bifida

    During the first 28 days of

    pregnancy the brain and spinal

    cord of an embryo form,

    During this period (28 days), spina

    bifida occurs, before most women

    even know that they are pregnant,

    Occurs in 1/750-1000 pregnancies.

    Dr/HN/11

  • Spina Bifida

    Etiology :

    There is no single known cause of

    SB. Research continues into the

    effects of factors such as heredity ,

    nutrition, environment, pollution,

    and physical damage to the

    embryo.

    The risk of SB occuring in

    subsequent children is certainly

    increased. Dr/HN/12

  • Spina Bifida

    Alfa Feto Protein (AFP):

    AFP is produced by all fetuses but higher in cases of open CNS(NTD)

    Pregnant womans blood can be tested for AFP as early as the 16th week of gestation,

    Amniocentesis at 14-17th weeks of gestation is more accurate ,(disad- vantage of miscarriage).

    Dr/HN/13

  • Spina Bifida

    AFP : (cont)

    AFP testing can detect up to 85%

    of these cases, but this test is not

    specific for SB and can be

    elevated in many other similar

    abnormalities , therefore, further

    testing is recommended.

    Dr/HN/14

  • Spina Bifida

    AFP : (cont..)

    Amniocentesis is offered to

    mothers with increased risk of

    having babies with a birth defect,

    (previous spina bifida, or taking

    drugs for siezures, or over 35

    years of age or positive family

    history) .

    Dr/HN/15

  • Spina Bifida

    USS Scan :

    Uss at 18-20 weeks of gestation will show the defect in the spinal bones and certain changes in the brain in 90% on NTD cases.

    Dr/HN/16

  • Spina Bifida

    Spina Bifida Occulta :

    Neural abnormalities are uncommon

    The bony diffect is of no clinical

    significance,

    Few patients may develop progressive

    neurological signs during spurt growth

    which occurs most often between 8 and

    14 years of age.

    Lipoma

    Dr/HN/17

  • Spina Bifida

    Spina Bifida Occulta : (cont...)

    Progressive deformity of the feet or

    changes in micturation are very

    suggestive and myelography is indicated

    when these occur,

    In case of objective neurological signs or

    myelographic evidence of cord or root

    displacement ,patient should be

    explored.

    Local lesion removal for cosmetic reason Dr/HN/18

  • Spina Bifida

    Spina Bifida Occulta : (cont..)

    Dermal sinus ;

    Should be completely excised before infection supervenes to remove the risk of meningitis when the track happens to communicate with or is in close proximity to the subarachnoid space. If already inf-ected, the sinus should be excised after the antibiotic infection control

    Dr/HN/19

  • Spina Bifida

    Split Notochord Syndrome :

    Occasionally , embryos develop

    with partial duplication of

    notochord, yolk sac herniates

    between them,

    Diastematomyelia is due to fusion

    of the medial pedicles of a pair of

    hemi-vertevrae to form a bony spur

    which lies between the split halves

    of spinal cord. Dr/HN/20

  • Spina Bifida

    Vesicointestinal fissure, or extrophy

    of the cloaca is usually associated

    with a myelomeningocele and is a

    severe variation of the split

    notochord syndrome.

    Split notochord with yolk sac hernia,

    Split notochord with yolk sac fistula,

    Split notochord with gut fistula,

    Split notochord with

    diastematomyelia Dr/HN/21

  • Spina Bifida

    Different

    variants of

    split

    notochord

    Dr/HN/22

  • Spina Bifida

    Spina Bifida Cystica :

    Meningocele;

    About 5-6% of SB cystica,

    A failure of the vertebral arch to form and a protrusion of the meninges through the gap to form a simple meningeal sac lined by arachnoid membrane and dura containing CSF and only occasionally nerve tissue.

    Dr/HN/23

  • Spina Bifida

    Meningocele

    Spina Bifida Cystica

    Dr/HN/24

  • Spina Bifida

    Menigocele : (cont..)

    A cystic swelling with a cover which

    may vary from a thin translucent

    membrane to normal skin,

    In the great majority the cord is

    normally formed and there is

    neurological defect, but each case

    must be watched with care because

    of associated spinal cord dysplasia.

    Dr/HN/25

  • Spina Bifida

    Meningocele : (cont..)

    The cyst may be large to several

    inches in diameter while the neck

    is often narrow,

    The cyst may be tense, specially if

    the baby is crying or when

    pressure is applied to the

    fontanelle, otherwise soft and

    fluctuant,

    Dr/HN/26

  • Spina Bifida

    Meningocele : (cont..)

    The skin over the sac is generally

    intact, but it is occasionally

    ulcerated, which is, however , more

    typical of myelomeningocele,

    A lipoma round the base of the sac

    may give the false impresion of a

    wide base,

    Radiology will show that the inter-

    peduncular gap is not so extensive. Dr/HN/27

  • Spina Bifida

    Meningocele : (cont..)

    Hdrocephalus is rare,

    Normal mental state,

    Usually, no neurol. abnormalities.

    If the skin is intact no urgency ,

    The repair can be done at any

    convinient time during infancy.

    Dr/HN/28

  • Spina Bifida

    Meningocele : (cont..)

    If the sac is ulcerated it should be repaired immediately after birth, be- fore significant infection supervenes,

    But if this opportunity is missed epithelium should be allowed to cover the sac, which is then excised at a convenient time later in infancy.

    Dr/HN/29

  • Spina Bifida

    SB Cystica :

    Myelomeningocele;

    94% of spina bifida cystica ,

    The most serious variety,

    Consists of a bifid spine with prot- rusion and dysplasia of the menin- ges and spinal cord and is always acoompanied by neurological signs

    Dr/HN/30

  • Spina Bifda

    Myelomeningocele : (cont..)

    The spinal cord remains exposed and the surface (neural plague), looks like granulation tissue,

    At the upper end the central canal may be recognized as a fistula discharging cerebro- spinal- fluid (CSF).

    Dr/HN/31

  • Spina Bifida

    Myelomeningocele : (cont..)

    This tissue is surrounded by a thin

    translucent membrane

    representing the meninges

    (arachnoid membrane with nervous

    tissue visible on the surface known

    as , neural plague), and

    circumferentially an area of ill-

    formed skin is almost always

    present. Dr/HN/32

  • Spina Bifida

    My

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