A medical condition that is present at or before birth is called Congenital Disorder. These conditions, also referred to as birth defects, can be acquired during the fetal stage of development or from the genetic makeup of the parents. There are numerous variations of congenital malformations of the bone and soft tissue of the head and spine, Congenital anomalies of the spine may be simple and benign, causing no spinal deformity, or they may be complex.
Types of Congenital Malformations of Spine
• Spina Bifida
• Tethered Cord
• Lipoma of the Spinal Cord
• Neurenteric Cyst
Spina bifida is the most common of a group of birth defects known as neural tube defects, which affect the central nervous system. There are 3 types of spina bifida.
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1) Spina bifida occulta
The mildest form of spina bifida, spina bifida occulta describes a group of neural tube birth defects that can affect the spinal column. This is composed of bones, or vertebrae, which support the nerve center of the spinal cord, which is responsible for carrying all nerve signals in between the body and the brain.
2) Meningocele
A meningocele is a form of spina bifida. It is a congenital malformation that arises from an error in the normal development of the central nervous system, particularly the spinal cord and spine. It is an out-pouching of the coverings of the spinal cord that results in a defect in the bone and soft-tissue coverings of the back part of the spine.
3) Myelomeningocele
A myelomeningocele is a defect of the backbone (spine) and spinal cord. Before birth, the baby's backbone, spinal cord and the structure they float in (spinal canal) do not form or close normally.
Tethered Cord
Cord is said to be tethered when it is attached abnormally within the boney spine. There are two ways the spinal cord can become tethered.
1) If your child was born with spina bifida then the cord could become tethered because of the scar tissue that resulted from surgically closing the spine at birth. This scar tissue causes the cord to attach abnormally.
2) The spinal cord can also become tethered with spina bifida occulta. This can occur without visible outward signs although usually half the children have visible symptoms.
Treatment for Tethered Cord
Patients suspected of having tethered cord may go through the following steps for evaluation and treatment:
- Spinal x-rays
- MRI
- Somatosensory evoked potentials – to evaluate nerves and electrical impulse transmissions through the spinal cord
- Bladder function evaluation by a pediatric urologist
- Tethered cord surgery
Lipoma of the Spinal Cord
A spinal cord lipoma is a fatty mass or tumor within the spinal cord that may or may not be associated with Spina Bifida. It often occurs in a normally positioned cord without any skin or bony abnormalities. The tendency to develop a lipoma is not necessarily hereditary although hereditary conditions, such as familial multiple lipomatosis, may include lipoma development. Cases have been reported where minor injuries are alleged to have triggered the growth of a lipoma, called "post-traumatic lipomas." However, the link between trauma and the development of lipomas is controversial. Risks depend on lipoma size and location. If an important motor nerve runs through the lipoma, then removal may injure this nerve. Intraoperative nerve stimulation helps locate the nerve to ensure its preservation. Biopsies also have the risk of nerve injury.
- Spinal x-rays
- MRI
- Somatosensory evoked potentials – to evaluate nerves and electrical impulse transmissions through the spinal cord
- Bladder function evaluation by a pediatric urologist
- Tethered cord surgery
Treatment for Lipomas
Lipomas are normally removed by simple excision. The removal can often be done under local anaesthetic, and take less than 30 minutes. There are new methods being developed that are supposed to remove the lipomas without scarring. One of them is removal by the use of injection of compounds that trigger lipolysis, such as steroids or phosphatidylcholine.
Lipomas are normally removed by simple excision. The removal can often be done under local anaesthetic, and take less than 30 minutes. There are new methods being developed that are supposed to remove the lipomas without scarring. One of them is removal by the use of injection of compounds that trigger lipolysis, such as steroids or phosphatidylcholine.
Neurenteric Cyst
Neurenteric cysts are not confined to the spinal column but may be found within the brain, mediastinum, abdomen, pelvis, or even in a subcutaneous location. Congenital abnormalities believed to be derived from an abnormal connection between the primitive endoderm and ectoderm during the third week of life.
The Treatment of Intraspinal Neurenteric Cysts
The treatment of intraspinal neurenteric cysts is surgical. They are frequently adherent to the spinal cord or cauda equina to the extent that complete excision is not feasible but even so, evacuation and partial resection of these lesions is usually followed by the same good results as complete excision. In either case, neurological function usually improves and recurrence after partial resection may take years to become symptomatic.
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