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Spina Bifida – A Congenital Disease Of The Spine

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Spina Bifida

Spina bifida is defined as a deformity, a defect of the spine that includes several injuries with different severity and prognosis that require a adapted therapeutic approach which is always surgical.
According to the importance of this defect and its associated lesions, spina bifida distinguish several degrees:
  • Spina bifida occulta;
  • Dermal or epidermal fistulas;
  • Meningocele;
  • Myelomeningocele.
Spina Bifida

Spina Bifida

Spina bifida occulta is an elementary lesion, which can be only seen on X-ray. It is  a partial defect of the posterior vertebral arch. If the neurological signs and skin lesions which can be associated are absent, then any course of treatment is not imposed.

Dermal or epidermal fistulas realize a communication between the dural membrane and skin, crossing a bone defect which is more or less discreet. Fistulas can be reduced to a fine communication between the dural membrane and the skin  in which can be found residual elements from the epidermis or dermis, creating a epidermal or dermal fistula, also know as pilonidal cyst.
These fistulas require surgical treatment, but the problems are linked to the existence or not of a complete communications with the dural foil. The most important risk of this treatment is relapse and this complication often occurs if the lesion which were not completely excised.
Meningocele is defined as  a hernia of the dura mater and the arachnoid (membrane which are covering the spinal cord) over a bone defect. This bone defect can be spread over a larger portion of the spine, depending on how many vertebrae are damaged. Meningocele can appear at all levels of the spin but particularly occurs in the  lumbo-sacral region.
This mass can reach a critical level and it is covered by the skin which can have a variable thickness. Meningocele is often associated with lipoma  Often we find a lipoma which can amplify the volume of the protrusion and can mask the underlying  herniated meninges.
Hydrocephalus is associated in at least 80% of cases of myelomeningocele. This malformation consists in engaging of the cerebellum tonsil in the medullary canal, compressing foramen magnum. The result is that the compression produced over the foramen magnum prevent the leakage of the cerebrospinal fluid. This prevent of leakage produces an excessive accumulation of cerebrospinal fluid whcih will compress the brain, resulting in different degrees of retardation.
If the child is born with this disorders, the chances of recovery are virtually nil, but if the hydrocephalus occurs after surgery by cutting the nerve threads (a procedure which is inevitable in the surgical technique applied) there is a degree of recovery by medical gymnastics.
Effects of Spina Bifida
Myelomeningocele is a more complex malformation,in which are interest as  one or more vertebrae, spinal meningeal layers and always are interest the roots of the spinal nerves. It shows different aspects:
  • Myelomeningocele with open medullary area – it has a round, red, bleeding central area, with a upper central hole through which cerebrospinal fluid leaks. Peripheral it is followed by a transition zone, which is covered with a thin skin and make the transition to the normal dermal area. Spine slides are missing and spinal canal has a posterior opening;
  • Myelomeningocele cyst – is the most commonly encountered and is often situated in the lumbar median area. It is covered with thin skin and contain fluid. The coating membrane can crack easily and can lead to loss of cerebrospinal fluid and meningitis;
  • Covered myelomeningocele – it is a rare and less severe variant, which is covered entirely  with normal skin.
Spina Bifida
Neurological manifestations of myelomeningocele
  • Partial or complete motor disorders of the lower limbs, represent a common manifestation;
  • Dislocation of the hip and crooked legs;
  • Flaccid paralysis of the lower limb is more often present than spastic paralysis of the lower limb;
  • Insensitive areas of the limbs and gluteal region and perineum;
  • Partial or complete paralysis for anal and bladder sphincter;
  • Hydrocephalus which can lead to different degrees of retardation.