Spina Bifida

INTRODUCTION:-

Spina bifida is a developmental anomaly involving the neural tube characterized by defective closure of the bony encasement of the spinal cord through which the spinal cord and meninges may or may not protrude. This results in an incompletely formed spinal cord and allows the abnormal portion of the spinal cord to stick out through the opening in the bones. There may or may not be a fluid filled sac surrounding the open spinal cord. The most common location of the malformations is the sacral and lumbar areas of the spinal cord

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 TYPES:-

Spina bifida malformations fall into four categories:

  •  spina bifida occulta
  •  spina bifida cystica (myelomeningocele)
  •  Meningocoele

Spina bifida occulta is a usually harmless form of spina bifida in which there is a small defect or gap in a few of the small bones (vertebrae) that make up the spine. The spinal cord and nerves are usually normal, and most affected individuals have no problems caused by this minor abnormality.

Meningocoele is the rarest form of spina bifida in which a cyst or lump consisting of membranes surrounding the spinal cord pokes through the open part of the spine. The spinal cord and the nerves are usually normal. The cyst, which can be as small as a nut or as large as a grapefruit, can be removed by surgery, allowing the baby to develop normally.

Spina bifida cystica Meningomyelocoele is the most severe form of spina bifida. In this form, the cyst holds nerve roots of the spinal cord and often the cord itself. Or there may be no cyst, but only a fully exposed section of the spinal cord and nerves. Spinal fluid may leak out, and the area often is covered with sores. Affected babies are at high risk of infection until the back is closed surgically. In spite of surgery, varying degrees of leg paralysis and bladder and bowel control problems remain.

A large percentage of children born with spina bifida have hydracephalus, the accumulation of fluid in the brain. Hydrocephalus is controlled by a surgical procedure called shunting which relieves the fluid buildup in the brain by redirecting it to the abdominal area

CAUSES:-

  • Genetic factors like heredity
  • Environmental factors, such as nutrition:- The most important risk factor for spina bifida is poor nutrition, especially a diet deficient in folic acid
  • Exposure to harmful substances, probably contribute to spina bifida.
  • Spina bifida does seem to run in families, although with mixed patterns of inheritance. Having a child with spina bifida increases the chance that another child will also have spina bifida by 8 times. In about 95% of cases of spina bifida, however, there is no family history of neural tube defects.

SYMPTOMS:-

Meningocoele and myelomeningocele are evident at birth. Other than the obvious spinal cord deformity, symptoms are caused by complications of spina bifida. The most common complications include the following:

  • Abnormalities at the lower spine are always accompanied by upper spine abnormalities (Arnold-Chiari malformation), causing subtle coordination problems that usually can be improved by physiotherapy
  • The lumbar nerves control the muscles in the hip, leg, knee and foot, and help to keep the body erect Spine, hip, foot, and leg deformities are often due to imbalances in muscle strength and function resulting mostly from residual paralysis, but with a spasticity component.
  • The sacral nerves control some of the muscles in the feet, urinary bladder and bowel and the ability to have an erection.The most common bladder and bowel problems are inability to voluntarily relax the muscles (sphincters) that hold urine in the bladder and stool in the rectum.
  • Another common residual problem is hydrocephalous (accumulation of fluid in the brain) affecting about 90% of people with spina bifida. Having some fluid around the brain is normal and healthy, but in spina bifida the fluid often cannot drain naturally. Without treatment, this extra fluid can cause neurological problems or mental retardation; however, these individuals are of normal intelligence if their hydrocephalus is treated aggressively. Hydrocephalus often recurs gradually after treatment.
  •  tethered Spinal cord Syndrome:-many children with myelomeningocele have or develop a tethered spinal cord. The cord is attached to surrounding tissues and cannot move up and down freely as it normally does.  Symptoms such as lower body pain, leg weakness, scoliosis, numbness, foot or leg deformities, hip dislocation or incontinence. The problems can worsen as the child grows and the tethered cord is stretched.
  •  Urinary tract disorders (due to poor drainage)and obesity (due to inactivity) are common complications of spina bifida.
  • Pathological bone fractures occur in as many as 25% of people with spina bifida. A pathologic fracture is a break that occurs because of weakness or disease in the bones, not solely because of injury. A very minor injury can worsen a pathologic fracture, causing pain and bringing the fracture to medical attention
  • Growth hormone deficiency resulting in short stature is common in people with spina bifida. On average they are several inches shorter than siblings or peers.
  • Psychological, social, and sexual problems occur more often in people with spina bifida than in the general population.
  •  Allergy to latex (a natural rubber used in medical gloves, some types of elastic, balloons, and many other common items) is very common in people with spina bifida. This is thought to be a result of intense exposure to latex in the early years of life because of frequent surgeries and other medical procedures. Anallergic reaction to latex can be life threatening. Even individuals with no apparent symptoms or disability from spina bifida may have subtle or mild signs or neurological problems. Some, for example, have a dimple, darkening or small hairy patch on the skin overlying the base of the spine. Others have a fatty growth called an epidural lipoma that forms within the spinal canal; this is usually harmless but may result in tethering of the spinal cord.

DIAGNOSIS

For some children, evaluation for spina bifida begins before they are born.

  • A prenatal test measures the level of maternal serum alpha fetoprotein(MSAFP, or AFP), which is unusually high in women carrying a foetus with spina bifida or other neural tube defect
  • This test usually is done as one of 3 tests known together as the “triple screen.” The triple screen includes AFP, ultrasound, and testing of amniotic fluid.
  • Any pregnant woman who has a high level of AFP should undergo 2 additional tests that are very accurate in detecting severe spina bifida: ultrasound of the fetal spine and testing of the amniotic fluid for AFP.
  • Amniotic fluid is the fluid that surrounds the fetus in the womb. A small amount of the fluid is removed through a large needle and tested in various ways that might indicate abnormalities in the fetus. Removal of amniotic fluid is a safe, routine procedure called aminocentesis

The evaluation of a child with spina bifida includes the following:

  • Thorough analysis of individual medical history (if not newborn), family medical history, and details of the pregnancy and birth.
  • Physical examination focusing on neurological deficits, musculoskeletal deformities, and psychological evaluation; in an older child, assessment of developmental, social, and learning disorders.
  • Evaluation of critical body systems such as vascular system,heart, lungs, and kidneys to ensure child’s ability to undergo surgery.
  • Imaging studies are done as needed to detect new or worsening complications.
  • X-rays are used to detect vertebral abnormalities, scoliosis, hip deformities, pathologic fractures, and other bony abnormalities that are common and may or may not cause pain and other symptoms in spina bifida.
  • Ultrasound may be used to assess an area of pain that suggests pathologic fracture.
  • CT scan of the head is used to evaluate for hydrocephalus.
  • MRI provides the best detailed information on spinal cord abnormalities and the extent of cord and nerve root involvement.
  •  Gait analysis is an assessment of the child’s walking skills. Walking involves many different body systems, including the brain, the sensory organs (such as the eyes), the spinal cord and nervous system, the bones and joints of the neck, back, hips, legs, and feet, and the muscles, tendons, and ligaments.
  • he findings of gait analysis can be used to help people with spina bifida improve their ability to walk.

TREATMENT

  • Treatment for spina bifida depends on the severity of the condition.
  • Most people with spina bifida occulta require no treatment at all.
  • Children with meningocele typically require surgical removal of the cyst and survive with little, if any, disability.
  • Children with myelomeningocele, however, require complex and often lifelong treatment and assistance. Almost all of them survive with appropriate treatment starting soon after birth. Their quality of life depends at least partially on the speed, efficiency, and comprehensiveness with which that treatment is provided.

A child born with myelomeningocele requires specialty care.

  • The child should be transferred immediately to a center where newborn surgery can be performed.
  • Treatment with antibiotics is started as soon as the myelomeningocele is recognized; this prevents infection of the spinal cord, which can be fatal.
  • The operation involves closing the opening in the spinal cord and covering the cord with muscles and skin taken from either side of the back. The most common complications are tethered spinal cord and hydrocephalus, which can have very severe consequences

Other Therapy

A child’s emotional and social development can be deeply affected by physical disabilities such as spina bifida. For their all round development:-

Physical therapy is imperative so as to ensure maximum functional independence.

  • Most of these children can be educated in mainstream schools. They should be evaluated for learning disorders
  • Children should be in the least restrictive environment possible. They should learn skills that help them be as independent as possible.
  • They should be encouraged to participate with their peers in age-appropriate activities such as clubs and teams to the best of their abilities.
  • Children should take on responsibility for their own care as much as possible.

Consult PHYSIOLINE for further consultation and rehabilitation