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The tethered spinal cord is a condition in which the bottom of the spinal cord is fixed to an immovable structure such as the sac surrounding the spinal cord, the tissues surrounding the sac such as the fat under the skin, or even the skin itself. This prevents normal movement of the spinal cord with growth of the child and everyday movement such as bending forward. Many but not all children who are born with this condition will have a birthmark on their back such as a dimple, unusual patch of hair, lump of fatty tissue under the skin, or a small area of abnormally thin skin in the midline of the back. They may also have other signs such as back pain, leg pain, tingling or numbness in their legs, urinary incontinence, abnormal posture, stiffness in the legs or deformity of the feet. If your child has one of these symptoms or signs, an imaging test such as an MRI or ultrasound of the spine may be ordered. If this does show findings of a tethered cord, then a neurosurgical evaluation may be requested by your child’s pediatrician. The different types of tethered spinal cord include myelomeningocele (often referred to as spina bifida), lipomyelomeningocele (the bottom of the spinal cord blends with fatty tissue that tethers or anchors it to the skin), tight or fatty filum terminale (the spinal cord is anchored by a thin band of fatty tissue to the bottom of the sac surrounding it), meningocele (the spinal cord is covered by a sac containing spinal fluid that bulges outward on the back) and myelocystocele ( the bottom of the spinal cord flares into a cyst that contains spinal fluid and is covered by fat).
Hydrocephalus is a condition where there is an imbalance in, or obstruction to, the flow and reabsorption of cerebrospinal fluid (CSF) in the brain. It may occur as an isolated congenital anomaly, along with other complex congenital anomalies such as spina bifida, as a consequence of prematurity, following a head injury, or in association with a lesion in the brain such as a brain tumor or cyst. Normally the CSF circulates within the ventricles, or fluid chambers, deep within the brain and then is resorbed into the bloodstream via a large vein called the superior sagittal sinus that courses along the midline surface of the brain. A brain tumor or cyst adjacent to or within a ventricle can interfere with CSF circulation and allow CSF to accumulate within the ventricles. Prematurity and trauma, on the other hand, can lead to bleeding within the ventricles that can cause scarring that will interfere with absorption of the fluid. Children who develop hydrocephalus in infancy, or even before they are born, may have a bulging soft spot (anterior fontanel), irritability, vomiting, excessive sleepiness, eyes that tend to look downwards towards the feet (sunsetting eyes) and rapid head growth. Older children will often have headaches, especially at night or early in the morning, abnormal eye movements, vomiting or change in school performance. Ultrasound, CT or MRI imaging will show the enlarged ventricles in a child suspected of having hydrocephalus. Sometimes evidence of the elevated pressure in the head caused by the excess fluid can be seen by examining the back of the eye and looking for swelling of the nerve that supplies vision, a situation called papilledema.
Spina bifida is a complex birth defect affecting approximately 0.8 per 1,000 live births, although the incidence has declined over the years and there is much variation in incidence from region to region across the world. A combination of genetic and environmental factors may lead to a baby being born with an open sac on the back exposing a portion of the spinal cord (this part of the spina bifida complex is called the myelomeningocele and can be anywhere along the midline of the back) as well as hydrocephalus (buildup of excess cerebrospinal fluid in the brain) in most cases. As a result of the abnormal development of the brain and spinal cord very early on, well before the child is born, children with spina bifida face a number of unique neurosurgical, orthopedic, urologic and developmental challenges. Here at Cincinnati Children’s we are uniquely positioned to help these patients and their families in our multidisciplinary spina bifida clinic, allowing patients to see all their surgical specialists (neurosurgery, orthopedics and urology) as well as developmental medicine, orthotics, physical medicine and rehabilitation, and transition specialists when appropriate. It is recognized as an approved center for spina bifida by the Spina Bifida Association and won their 2008 Outstanding Service Provider Award. Expectant mothers carrying a fetus diagnosed as having spina bifida, based on medical criteria for eligibility, may be offered the opportunity to participate in ongoing national trials such as the MOMS trial, which is studying fetal surgery for this condition in hopes of improving outcomes at certain select centers in the United States. We maintain a positive outlook for our patients with spina bifida and seek to provide our families with a supportive environment with 21st- century resources and facilities. We are able to offer the latest in neurosurgical care as provided by our BrainSUITE operating room with intraoperative MRI , a pediatric ICU with portable CT scanner, and a new outpatient facility built specifically to meet the needs of patients with developmental or physical challenges. There, specially designed waiting and examining rooms provide a safe, accessible, bright, friendly and calming environment for our patients and families.
More and more children are coming to neurosurgical attention because a CT or MRI, initially done for other reasons, has found an arachnoid cyst of the brain. This is a collection of CSF (cerebrospinal fluid) contained within a pocket of delicate membranes. They can occur almost anywhere in the brain but are most common in a part of the brain in front of the ear called the middle fossa. Other common sites include the region behind the eyes (suprasellar), the area behind the ears (cerebellopontine angle), the back part of the brain (retrocerebellar) and the surface of the brain (convexity). Boys are almost twice as likely to have an arachnoid cyst as girls, and in general, they are not thought to be hereditary. A decision as to whether an arachnoid cyst requires treatment is one that will be discussed with your child’s neurosurgeon. Factors that may influence a decision to treat an arachnoid cyst would include cysts that are enlarging over time, those that are causing headaches directly over the area of the cyst, those thought to be causing seizures, motor weakness, hormonal problems, problems with eyesight, or those that rupture and cause bleeding around the brain. Many cysts cause no symptoms and your child’s neurosurgeon may recommend that a course of observation is most appropriate, with counseling about any activity restrictions for your child. Some cysts may be susceptible to rupture with trauma and your neurosurgeon may recommend an avoidance of contact sports for that reason. Sometimes an MRI or CT will show that a child has hydrocephalus (buildup of spinal fluid) in the ventricles (normal fluid chambers of the brain) in addition to the arachnoid cyst. In those cases, your neurosurgeon may recommend some type of shunt placement in addition to, or in place of, surgery on the cyst itself.
Spasticity is defined as an abnormal increase in muscle tone that makes muscles resist the normal stretching that occurs during use. It may occur in patients with cerebral palsy (CP) as well as following severe head injuries, stroke and spinal cord injuries. Even when present from birth, it may not be initially identified during infancy, especially in children with CP. Although it may improve or stay the same as a child gets older, it typically does not worsen once a diagnosis has been established. Muscle tone is regulated by nerve cells in the spinal cord that receive input from stretch receptors in the muscles and by cells in the brain that release chemical substances that bathe these special nerve cells in the spinal cord. When there is an imbalance in this pathway, spasticity can result. Symptoms of spasticity can include muscle stiffness, muscle tightness, pain and cramping in the muscles of the arms or legs, and easy fatigability. Hip dislocations, as well as severe contractures of the tendons in the arms and legs, can result from this excess tightness of the muscles. If the spasticity is severe enough to require treatment, a team of specialists from the departments of physical medicine and rehabilitation, neurosurgery, orthopaedics and physical and occupational therapy will evaluate your child and make appropriate recommendations. The goals of any treatment are to reduce abnormal muscle tone, improve function, reduce or prevent pain, help with care and prevent contractures. There are two operations offered by our neurosurgical department. Your neurosurgeon and the other members of the team will include you in a discussion about which operation may be best for your child: implantation of a baclofen pump or selective dorsal rhizotomy. Typically, these options are considered once medical therapy has been tried extensively.
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