Spina bifida

Read more about this disease, some with Classification – Types – Signs and symptoms – Genetics – Pathophysiology – Diagnosis – Screening – Prevention – Treatment and management – Cures and much more, some including pictures and video when available.

Spina bifida (Latin: “split spine”) is a developmental birth defect involving the neural tube: incomplete closure of the embryonic neural tube results in an incompletely formed spinal cord. In addition, the vertebrae overlying the open portion of the spinal cord do not fully form and remain unfused and open. This 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. Other neural tube defects include anencephaly, a condition in which the portion of the neural tube which will become the cerebrum does not close, and encephalocele, which results when other parts of the brain remain unfused.

Spina bifida malformations fall into four categories: spina bifida occulta, spina bifida cystica (myelomeningocele), meningocele and lipomeningocele. The most common location of the malformations is the lumbar and sacral areas of the spinal cord. Myelomeningocele is the most significant form and it is this that leads to disability in most affected individuals. The terms spina bifida and myelomeningocele are usually used interchangeably.

Spina bifida can be surgically closed after birth, but nerve damage is permanent and this does not restore normal function to the affected part of the spinal cord. An individual with this condition will have dysfunction of the spinal cord and associated nerves from the point of the open defect and below. Intrauterine surgery for spina bifida has also been performed and the safety and efficacy of this procedure is currently being investigated. The incidence of spina bifida can be decreased up to 70 percent when daily folic acid supplements are taken prior to conception.[1]

These vary with the extent of the spinal defect and differ between the subtypes described below.

The most common location of the malformations is the lumbar and sacral areas of the spinal cord. The lumbar nerves control the muscles in the hip, leg, knee and foot, and help to keep the body erect. The sacral nerves control some of the muscles in the feet, bowel and urinary bladder, and the ability to have an erection. Some degree of impairment can be expected in these areas, resulting in varying degrees of paralysis, absence of skin sensation, and poor or absent bowel and/or bladder control as well as curvature of the spine (scoliosis) (depending on the severity and location of the lesion damage on the spine). These individuals are rarely intellectually disabled. Children with spina bifida often have hydrocephalus, which consists of excessive accumulation of cerebrospinal fluid in the ventricles of the brain.[2]

Tethered Spinal Cord syndrome, (with symptoms such as lower body pain, leg weakness, incontinence, scoliosis, numbness) is a common problem associated with spina bifida. Indeed, all spina bifida myelomeningocele patients have Tethered Cord on imaging studies such as Magnetic resonance imaging, but not all will develop symptoms.[1] A tethered cord is thought to result from scar tissue which forms following the initial surgery to close the open defect. Symptoms caused by a tethered cord are rare in infancy and early childhood. Once symptoms develop it is important to make the diagnosis early, before permanent damage is done to the spinal cord and nerves.[3]

According to the Spina Bifida Association of America (SBAA), over 73 percent of people with spina bifida develop an allergy to latex, ranging from mild to life-threatening. The common use of latex in medical facilities makes this a particularly serious concern. The most common approach is to try to avoid development of the allergy by avoiding contact with latex-containing products such as examination gloves, catheters, and many of the products used by dentists.[3]

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