Brain Tumors


Few things are more frightening than finding out your child has a brain tumor. Yet it's a fear many parents have had to face. Brain tumors are now the second most common group of childhood cancers, after leukemia, affecting approximately 2,300 children each year.

Some can be cured relatively easily; others have a poorer outlook. All require a very specialized treatment plan involving a team of medical specialists, and all are likely to take a tremendous physical and emotional toll on the kids and their families.

What Is a Brain Tumor?

A tumor is any mass caused by abnormal or uncontrolled growth of cells. Tumors in the brain are categorized according to several factors, including where they're located, the type of cells involved, and how quickly they're growing.

Medical terms doctors may use to describe brain tumors include:

  • Primary vs. secondary: Primary brain tumors originate in the brain. Secondary brain tumors are made up of cells that have spread (metastasized) to the brain from somewhere else in the body. In children, most brain tumors are primary. The opposite is true in adults.
  • Benign vs. malignant: Benign tumors are slow-growing, noncancerous, and do not spread to surrounding tissue. Malignant tumors, on the other hand, are cancerous. Fast-growing and aggressive, they can invade nearby tissue and also are more likely to recur after treatment. Though malignancies are generally associated with a worse outlook, in the brain, benign tumors can be just as serious, especially if they're in a critical location (such as the brain stem, which controls breathing) or grow large enough to press on vital brain structures.
  • Localized vs. invasive: A localized tumor is confined to one area and is generally easier to remove, as long as it's in an accessible part of the brain. An invasive tumor has spread to surrounding areas and is more difficult to remove completely.
  • Grade: The grade of a tumor indicates how aggressive it is. Today, most medical experts use a system designed by the World Health Organization (WHO) to identify brain tumors and help make a prognosis. The lower the grade, the less aggressive the tumor and the greater the chance for a cure. The higher the grade, the more aggressive the tumor and the harder it may be to cure.

What Causes a Brain Tumor?

Most brain tumors in children originate when a normal cell begins to grow abnormally and reproduce too rapidly. Eventually these cells develop into a mass called a tumor. The exact cause of this abnormal growth is unknown, though research continues on possible genetic and environmental causes.

Some kids are more susceptible to developing brain tumors due to certain genetic conditions. Diseases such as neurofibromatosis, von Hippel-Lindau disease, Li-Fraumeni syndrome, and retinoblastoma are all associated with a higher risk of brain tumors.

Signs and Symptoms

A brain tumor can cause symptoms in a number of ways: by directly destroying brain cells, by causing swelling at the tumor site, by causing a buildup of fluid in the brain (hydrocephalus), and by increasing pressure within the skull. A range of symptoms can develop as a result.

Signs or symptoms vary depending on a child's age and the location of the tumor, but may include:

  • seizures
  • weakness of the face, trunk, arms, or legs
  • slurred speech
  • difficulty standing or walking
  • poor coordination
  • headache
  • in babies, a rapidly enlarging head

Because early warning signs can be gradual and may mimic those of other common childhood conditions, brain tumors can be difficult to diagnose. So it's always wise to discuss any symptoms that concern you with your child's doctor.

Diagnosis

A doctor who suspects that a child has a brain tumor will order imaging studies of the brain: a CT scan or MRI, possibly both. These procedures let doctors see inside the brain and pinpoint the area of the tumor. Although both are painless, they do require children to be very still. Kids usually don't require sedation for CT scans, which can be done fairly quickly. Most, however, do need to be sedated for an MRI scan.

If imaging studies reveal a brain tumor, then surgery is likely to be the next step. A pediatric neurosurgeon will try to remove the tumor; if complete removal is not possible, then partial removal — or at least a biopsy (removal of a sample for microscopic examination) — may be done to confirm the diagnosis.

A pediatric pathologist (a doctor who helps diagnose diseases in children by looking at body tissues and cells under a microscope) and a neuropathologist (a pathologist who specializes in diseases of the nervous system) then review the tissue to classify and grade the tumor.

Special tests such as karyotyping, PCR, FISH, and gene expression profiling might be used to analyze the genetic makeup of the tumor cells. Using these tests to get specific information about cancer cells helps doctors develop the best treatment plan for someone with a brain tumor.

Treatment

Most pediatric brain tumor patients require treatment with some combination of surgery, radiation therapy, and chemotherapy. Advancements in all three areas have contributed to better outcomes over the last few decades.

The care of a child with a brain tumor is very complicated and requires close coordination between many members of a medical team. Members of this team typically include:

  • a pediatric neuro-oncologist (a doctor who specializes in treating cancers of the brain or spine)
  • a pediatric neurologist (a doctor who specializes in disorders of the nervous system)
  • a pediatric neurosurgeon (a surgeon who operates on the brain or spine)
  • a radiation therapist (a specialist who administers radiation therapy)
  • rehabilitation medicine specialists, including speech, physical, and occupational therapists
  • psychologists and social workers

These experts must choose a child's therapy very carefully because the potential for long-term effects, particularly from radiation, is high. (Radiation therapy, though often effective, can cause injury to the developing brain, especially in younger patients.) Striking the delicate balance between giving just enough therapy to cure the child, but not so much as to damage healthy cells and cause unnecessary side effects, is probably the most difficult aspect of treating brain tumors.

Surgery

Surgeons are having more success than ever removing brain tumors, partly because of new technologies in the operating room — especially those that allow real-time images of the brain to guide surgeons as they operate. These include stereotactic devices, which help target tumors by providing 3D images of the brain; intraoperative MRI, which lets doctors see beyond what is exposed during surgery and more clearly distinguish the boundary between tumor and healthy tissue; and other image-guidance systems that allow for more precise navigation.

Staged surgeries are also being used more frequently. That means that instead of trying to remove a large tumor all at once, surgeons will take a small part, and then attempt to shrink the tumor with chemotherapy and/or radiation therapy. After several months of treatment, the surgeon may go back a second or even a third time to totally remove the rest of the tumor.

After surgery, some tumors may not require any more treatment beyond observation (periodic checkups and imaging scans to watch for trouble). Many, however, require more aggressive therapies, such as radiation therapy, chemotherapy, or a combination of both.

Radiation Therapy

Radiation therapy — the use of high-energy light to kill rapidly dividing cells — is very effective in the treatment of many pediatric brain tumors. However, because the developing brain in children younger than 10 (and especially younger than 5) is highly sensitive to its effects, radiation therapy can have serious long-term consequences. These may include seizures, stroke, developmental delays, learning problems, growth problems, and hormone problems.

As with surgery, the delivery of radiation therapy has changed significantly over the last decade. New computer-assisted technologies have allowed doctors to construct 3D radiation fields that accurately target tumor tissue, while avoiding critical brain structures, such as the hearing centers. Still, the advantages and disadvantages of using radiation therapy should always be discussed with your doctor.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells. Chemotherapy is often administered through a special long-lasting intravenous (IV) catheter called a central line, and may require frequent hospital stays. Although chemotherapy has many short-term side effects (fatigue, nausea, vomiting, hair loss, etc.), it has fewer long-term side effects than radiation therapy. Many children with brain tumors are treated with chemotherapy in order to delay or avoid radiation treatment.

Unlike brain tumors in adults, many pediatric brain tumors are highly sensitive to the effects of chemotherapy and respond well to high doses of it. However, giving a child high-dose chemotherapy can cause serious damage to the bone marrow (the spongy material inside bones that produces blood cells).

To prevent bone marrow damage from becoming permanent, a procedure called "stem-cell rescue" may be done. The patient's own bone marrow or blood stem cells are collected and stored until after the high-dose chemotherapy is completed. Then the marrow or stem cells are infused back into the patient to help the damaged bone marrow recover.

Common Types of Brain Tumors

There are many different types of pediatric brain tumors, ranging from those that can be cured with minimal therapy to those that cannot be cured even with aggressive therapy.

Some of the most common types are:

Astrocytomas

Astrocytomas come in four major subtypes: juvenile pilocytic astrocytoma (grade 1), fibrillary astrocytoma (grade 2), anaplastic astrocytoma (grade 3), and glioblastoma multiforme (grade 4). The higher the grade, the more aggressive the tumor.

Another important feature of astrocytomas is location, because where the tumor is directly affects the chance for a cure. For example, astrocytomas in the cerebrum and cerebellum tend to be low grade and located closer to the brain's surface, and so often can be treated with surgery alone. Optic pathway gliomas (which occur near the eyes) and astrocytomas (in the central parts of the brain) cannot be easily removed and often require a longer, more complex treatment approach.

Ependymomas

When located in the top part of the brain, these tumors often can be cured by surgery alone. However, when located in the center or back portion of the brain, they usually require much more aggressive therapy and can be difficult to cure.

Brain Stem Gliomas

Diffuse pontine gliomas, the most common subtype of brain stem gliomas, are located in a part of the brain that does not tolerate surgery. Patients with brain stem gliomas typically are treated with radiation therapy alone, although both surgery and chemotherapy have been used, with little success. Long-term survival rates are low for children with these tumors.

Medulloblastomas and Primitive Neuroectodermal Tumors (PNETs)

These related tumors, which are identical under the microscope, differ primarily in their location. Medulloblastomas are located in the back part of the brain, near the brain stem, while PNETs can be located anywhere else in the brain. Both tumors require aggressive therapy, including surgery, chemotherapy, and radiation therapy. Although these are dangerous cancers and the treatments are intense, cure rates are improving every decade. In fact, the majority of children with these types of tumors can now be cured.

Craniopharyngiomas

These rare, benign tumors are slow-growing. Although they do not spread, they can cause serious problems by putting pressure on critical brain structures. Treatment typically involves surgery and radiation, although in some cases surgery alone is used. Long-term survival is excellent.

Germ Cell Tumors

These tumors usually arise from two special areas in the midline of the brain, the area around the pituitary gland and the pineal gland. They can be difficult to treat, but a cure is often possible with aggressive surgery, radiation, and chemotherapy. Germinomas, the most common type of germ cell tumor, can be treated with either radiation therapy, chemotherapy, or both depending on the circumstances.

Late Effects

"Late effects" are problems that patients can develop after cancer treatments have ended. For survivors of pediatric brain tumors, late effects may include cognitive delay, seizures, growth abnormalities, hormone deficiencies, visual and auditory problems, and the possibility of developing a second cancer, including a second brain tumor. Because these problems sometimes don't become apparent until years after treatment, careful observation and regular screenings are needed to catch them as early as possible.

In some cases, short-term effects may improve with the help of physical, occupational, or speech therapy and may continue to improve as the brain heals. In other cases, kids may experience side effects that are longer term, including learning disabilities; medical problems such as diabetes, growth delay, or delayed or early puberty; physical disabilities related to movement, speech, or swallowing; and emotional problems linked to the stresses of diagnosis and treatment.

Be aware of the potential for physical and psychological late effects, especially when the time comes for your child to return to school, activities, and friendships. Talk to teachers about the impact treatment has had on your child and discuss any accommodations that may need to be made, including a limited schedule, additional rest time or bathroom visits, modifications in homework, testing or recess activities, and medication scheduling. Your doctor can offer advice on how to make the transition easier.

Caring for Your Child

Parents often struggle with how much to tell a child who is diagnosed with a brain tumor. Though there's no one-size-fits-all answer for this, experts do agree that it's best to be honest — but to tailor the details to your child's degree of understanding and emotional maturity.

Give as much information as your child requires, but not more. And when explaining treatment, try to break it down into steps. Addressing each part as it comes — visiting various doctors, having a special machine take pictures of the brain, needing an operation — can make the big picture less overwhelming.

Kids should be reassured that the brain tumor is not the result of anything they did, and that it's OK to be angry or sad. Really listen to your child's fears, and when you feel alone, seek support. Your hospital's social workers can put you in touch with other families of children with brain tumors who've been there and may have insights to share.

Also be aware that it's common for siblings to feel neglected, jealous, and angry when a child is seriously ill. Explain as much as they can understand, and enlist family members, teachers, and friends to help keep some sense of normalcy for them. And finally, as hard as it may be, try to take care of yourself. Parents who get the support they need are better able to support their child.

Bell's Palsy


Bell's palsy is a sudden weakness or paralysis on one side of the face that makes it hard for a person to move the mouth, nose, or eyelid and can make that side of the face droop or look stiff. It occurs when one of the facial nerves is not functioning properly, often after a virus.

What Causes Bell's Palsy?

When the facial nerve is working properly, it carries a host of messages from the brain to the face. These messages may tell an eyelid to close, one side of the mouth to smile or frown, or salivary glands to make spit. But if the nerve swells and is compressed, as happens with Bell's palsy, these messages don't get sent correctly. The result is weakness or temporary paralysis of the muscles on one side of the face.

Bell's palsy is most often connected with a viral infection such as HSV-1 (the virus that causes cold sores), Epstein-Barr (the virus that causes mononucleosis), or influenza. It's also associated with ear infections, bad colds, the infectious agent that causes Lyme disease, and trauma to the head or face.

In a few people, the immune system's response to a viral infection leads to inflammation of the facial nerve. Because it's swollen, the nerve gets compressed as it passes through a small hole at the base of the skull and stops sending the correct messages from the brain to the face — messages that, among other things, tell one eyelid to blink, and tell the front of the tongue to taste things.

Signs and Symptoms

Often the virus or infection that causes Bell's palsy is so mild that some kids don't ever feel sick. Usually the virus or infection has passed before the symptoms of Bell's palsy start to appear.

Bell's palsy tends to happen quickly. Some kids may feel pain behind or in front of their ears for a few hours or even days before the facial weakness sets in.

Because Bell's palsy only happens to one facial nerve at a time, it only affects one side of the face. Some kids have only slight weakness; others may not be able to move that side of their face at all. This may make one half of the child's face, especially the mouth, seem to droop or sag.

Other symptoms may include:

  • a feeling as though one side of the face is "twisting" or "tugging" (this is caused by the healthy side making facial expressions — the muscles pull on the weak side)
  • headache
  • trouble tasting at the front of the tongue
  • trouble producing saliva
  • sounds seeming louder than usual in one ear
  • difficulty fully shutting one eye, causing watering
  • twitching in the eye
  • a dry or irritated eye

Bell's palsy affects only the facial muscles, so if other parts of the body are stiff or paralyzed, talk with your doctor right away, as it may be a sign of a different condition.

Diagnosis and Treatment

There isn't a specific test for Bell's palsy, so to eliminate other possible causes, your doctor will evaluate how long it took for the symptoms to develop and where the weakness or paralysis is.

People who have strokes usually have weakness only in the lower half of their face and may have it in their arms and legs as well. The problems caused by tumors usually take longer to develop (the symptoms start more slowly and get more serious over a longer period of time) than Bell's palsy does.

The doctor will ask about whether your child is experiencing weakness or paralysis in other parts of the body, and if he or she is having other problems, like double vision or trouble swallowing. The doctor will also want to know about any head injuries.

If you live in an area of the country where Lyme disease is common, your doctor will do a blood test to check for that infection.

If the facial paralysis lasts more than a few months, the doctor is likely to recommend further tests, such as X-rays, computed tomography (CT) scans, and magnetic resonance imaging (MRI) to rule out other problems. The doctor might also recommend that your child have an electromyography (EMG), which tests how well the muscles are responding to the nerves' signals.

Because the virus or infection that leads to Bell's palsy has usually passed, there is usually no treatment for Bell's palsy. In some cases, medications that help reduce the swelling faster can be used.

Typically, there is nothing that a doctor can do to treat Bell's palsy. It goes away once the swelling of the nerve goes down and the nerve recovers from any damage that the swelling caused. The nerve has to renew itself and that can only happen with time.

In some cases doctors can offer medications to help reduce the swelling or prescribe an eye patch or eye drops if the child's eye is dry.

Most people recover fully within 1 to 3 months, whether or not the condition is treated, although some will have permanent weakness in their face afterwards. It is very unusual for anyone to get Bell's palsy twice.

Making sure your child eats well and gets plenty of sleep are important to healing. Activities and sports participation don't have to be limited as long as your child can close the eye (to protect it). If the eye can't be closed, talk with the doctor about using protective glasses or a patch to prevent eye damage.

Helping Kids Cope

Bell's palsy can be tough for kids because it affects their appearance so dramatically. Reassure your child that his or her face will soon return to normal.

If your child is being teased, consider talking with teachers, school counselors, and coaches — you might even want to speak to the class, if your child is comfortable with that. You also can practice responses with your child in case people ask what's wrong, stare, or say unkind things — explaining that it's a temporary medical problem that isn't contagious will probably satisfy most questioners.

With a little patience, and some extra doses of love and support from you, your child will be feeling better very soon.

Abusive Head Trauma (Shaken Baby Syndrome)


Abusive head trauma/inflicted traumatic brain injury or AHT (also called shaken baby/shaken impact syndrome or SBS) is a form of inflicted head trauma.

AHT can be caused by direct blows to the head, dropping or throwing a child, or shaking a child. Head trauma is the leading cause of death in child abuse cases in the United States.

How These Injuries Happen

Unlike other forms of inflicted head trauma, abusive head trauma results from injuries caused by someone vigorously shaking a child. Because the anatomy of infants puts them at particular risk for injury from this kind of action, the vast majority of victims are infants younger than 1 year old. The average age of victims is between 3 and 8 months, although these injuries are occasionally seen in children up to 4 years old.

The perpetrators in these cases are most often parents or caregivers. Common triggers are frustration or stress when the child is crying. Unfortunately, the shaking may have the desired effect: although at first the baby cries more, he or she may stop crying as the brain is damaged.

Approximately 60% of identified victims of shaking injury are male, and children of families who live at or below the poverty level are at an increased risk for these injuries as well as any type of child abuse. It is estimated that the perpetrators in 65% to 90% of cases are males — usually either the baby's father or the mother's boyfriend, often someone in his early twenties.

When someone forcefully shakes a baby, the child's head rotates about the neck uncontrollably because infants' neck muscles aren't well developed and provide little support for their heads. This violent movement pitches the infant's brain back and forth within the skull, sometimes rupturing blood vessels and nerves throughout the brain and tearing the brain tissue. The brain may strike the inside of the skull, causing bruising and bleeding to the brain.

The damage can be even greater when a shaking episode ends with an impact (hitting a wall or a crib mattress, for example), because the forces of acceleration and deceleration associated with an impact are so strong. After the shaking, swelling in the brain can cause enormous pressure within the skull, compressing blood vessels and increasing overall injury to its delicate structure.

Normal interaction with a child, like bouncing the baby on a knee, will not cause these injuries, although it's important to never shake a baby under any circumstances because gentle shaking can rapidly escalate.

What Are the Effects?

AHT often causes irreversible damage. In the worst cases, children die due to their injuries.

Children who survive may have:

  • partial or total blindness
  • hearing loss
  • seizures
  • developmental delays
  • impaired intellect
  • speech and learning difficulties
  • problems with memory and attention
  • severe mental retardation
  • cerebral palsy

Even in milder cases, in which babies looks normal immediately after the shaking, they may eventually develop one or more of these problems. Sometimes the first sign of a problem isn't noticed until the child enters the school system and exhibits behavioral problems or learning difficulties. But by that time, it's more difficult to link these problems to a shaking incident from several years before.

Signs and Symptoms

In any abusive head trauma case, the duration and force of the shaking, the number of episodes, and whether impact is involved all affect the severity of the infant's injuries. In the most violent cases, children may arrive at the emergency room unconscious, suffering seizures, or in shock. But in many cases, infants may never be brought to medical attention if they don't exhibit such severe symptoms.

In less severe cases, a child who has been shaken may experience:

  • lethargy
  • irritability
  • vomiting
  • poor sucking or swallowing
  • decreased appetite
  • lack of smiling or vocalizing
  • rigidity
  • seizures
  • difficulty breathing
  • altered consciousness
  • unequal pupil size
  • an inability to lift the head
  • an inability to focus the eyes or track movement

Diagnosis

Many cases of AHT are brought in for medical care as "silent injuries." In other words, parents or caregivers don't often provide a history that the child has had abusive head trauma or a shaking injury, so doctors don't know to look for subtle or physical signs. This can sometimes result in children having injuries that aren't identified in the medical system.

And again, in many cases, babies who don't have severe symptoms may never be brought to a doctor. Many of the less severe symptoms such as vomiting or irritability may resolve and can have many non-abusive causes.

Unfortunately, unless a doctor has reason to suspect child abuse, mild cases (in which the infant seems lethargic, fussy, or perhaps isn't feeding well) are often misdiagnosed as a viral illness or colic. Without a diagnosis of child abuse and any resulting intervention with the parents or caregivers, these children may be shaken again, worsening any brain injury or damage.

If shaken baby syndrome is suspected, doctors may look for:

  • hemorrhages in the retinas of the eyes
  • skull fractures
  • swelling of the brain
  • subdural hematomas (blood collections pressing on the surface of the brain)
  • rib and long bone (bones in the arms and legs) fractures
  • bruises around the head, neck, or chest

The Child's Development and Education

What makes AHT so devastating is that it often involves a total brain injury. For example, a child whose vision is severely impaired won't be able to learn through observation, which decreases the child's overall ability to learn.

The development of language, vision, balance, and motor coordination, all of which occur to varying degrees after birth, are particularly likely to be affected in any child who has AHT.

Such impairment can require rigorous physical and occupational therapy to help the child acquire skills that would have developed on their own had the brain injury not occurred.

As they get older, kids who were shaken as babies may require special education and continued therapy to help with language development and daily living skills, such as dressing themselves.

Before age 3, a child can receive speech or physical therapy through the Department of Public Health/ Early Intervention. Federal law requires that each state provide these services for children who have developmental disabilities as a result of being abused.

Some schools are also increasingly providing information and developmental assessments for kids under the age of 3. Parents can turn to a variety of rehabilitation and other therapists for early intervention services for children after abusive head trauma. Developmental assessments can assist in improving education outcomes as well as the overall well-being of the child.

After a child who's been diagnosed with abusive head trauma turns 3, it's the school district's responsibility to provide additional special educational services.

Preventing AHT

Abusive head trauma is 100% preventable. A key aspect of prevention is increasing awareness of the potential dangers of shaking.

Finding ways to alleviate the parent or caregiver's stress at the critical moments when a baby is crying can significantly reduce the risk to the child. Some hospital-based programs have helped new parents identify and prevent shaking injuries and understand how to respond when infants cry.

The National Center on Shaken Baby Syndrome offers a prevention program, the Period of Purple Crying, which seeks to help parents and other caregivers understand crying in normal infants. By defining and describing the sometimes inconsolable infant crying that can sometimes cause stress, anger, and frustration in parents and caregivers, the program hopes to educate and empower people to prevent AHT.

One method that may help is author Dr. Harvey Karp's "five S's":

  1. Shushing (using "white noise" or rhythmic sounds that mimic the constant whir of noise in the womb, with things like vacuum cleaners, hair dryers, clothes dryers, a running tub, or a white noise CD)
  2. Side/stomach positioning (placing the baby on the left side — to help digestion — or on the belly while holding him or her, then putting the sleeping baby in the crib or bassinet on his or her back)
  3. Sucking (letting the baby breastfeed or bottle-feed, or giving the baby a pacifier or finger to suck on)
  4. Swaddling (wrapping the baby up snugly in a blanket to help him or her feel more secure)
  5. Swinging gently (rocking in a chair, using an infant swing, or taking a car ride to help duplicate the constant motion the baby felt in the womb)

If a baby in your care won't stop crying, you can also try the following:

  • Make sure the baby's basic needs are met (for example, he or she isn't hungry and doesn't need to be changed).
  • Check for signs of illness, like fever or swollen gums.
  • Rock or walk with the baby.
  • Sing or talk to the baby.
  • Offer the baby a pacifier or a noisy toy.
  • Take the baby for a ride in a stroller or strapped into a child safety seat in the car.
  • Hold the baby close against your body and breathe calmly and slowly.
  • Call a friend or relative for support or to take care of the baby while you take a break.
  • If nothing else works, put the baby on his or her back in the crib, close the door, and check on the baby in 10 minutes.
  • Call your doctor if nothing seems to be helping your infant, in case there is a medical reason for the fussiness.

To prevent potential AHT, parents and caregivers of infants need to learn how to respond to their own stress. It's important to talk to anyone caring for your baby about the dangers of shaking and how it can be prevented.

Treating Scoliosis

Every person's spine can curve. A certain amount of curvature is necessary for us to balance, move, and walk properly. But 3 people out of 100 have scoliosis, a condition that causes the spine to curve too much in the wrong direction.

No one knows what causes the most common form of scoliosis, called idiopathic scoliosis. The condition can be hereditary, so a child who has scoliosis might have family members who have it. Girls are much more prone to developing severe cases of spinal curvature than boys are.

Scoliosis can develop very gradually, in most cases it isn't diagnosed until a child is between the ages of 10 and 14. Most cases of scoliosis don't require treatment, but even when they do, kids can usually resume an active life after treatment.

About Scoliosis

In scoliosis, the spine becomes abnormally curved from side to side into an "S" shape. No one knows what the underlying cause is in most cases of scoliosis. If the curve gets severe, it can be visible and cause discomfort. If the curve gets really severe, it can lead to other health problems, possibly affecting the lungs, heart, and joints.

Most of the time, scoliosis is mild enough that it doesn't affect a child's life and requires no medical treatment. In some cases, doctors will have a child with scoliosis wear a back brace or perform surgery to correct the problem.

Diagnosing Scoliosis

Some kids show visible signs of scoliosis. Sometimes a curvature of the spine is obvious or one shoulder blade is noticeably higher than the other one. Other times, though, it's not so obvious. And because scoliosis doesn't hurt or happen suddenly, it's not always easy to diagnose.

Some schools in the United States test for scoliosis. But the most accurate way to diagnose it is to see a doctor. Doctors routinely check kids for scoliosis during regular physical exams.

Treating Scoliosis

In most cases, scoliosis does not require medical treatment. The doctor just regularly checks the child's spine to make sure the curve doesn't become any larger.

If your child needs medical treatment, the doctor might order X-rays to get a clearer view of the spine and refer your child to an orthopedist, a doctor who specializes in treating medical conditions involving the bones.

The orthopedist may mention the Cobb angle, a measure of the curvature of the spine in degrees. The number of degrees helps the doctor decide what type of treatment is necessary. A scoliosis curve of 10 to 15 degrees usually means that nothing needs to be done except for regular checkups until pubertal maturation and growth are complete (the curvature of the spine usually doesn't get worse after that point). If the curve is 20 to 40 degrees, the orthopedist will generally suggest a back brace. A Cobb angle of 40 or 50 degrees or more may mean that surgery is necessary. Keep in mind that when the orthopedist draws the lines for the Cobb angle, there is some "measurement error." This means that the number will change a little from one X-ray to another (up to about 5 degrees), even though the curve hasn't changed.

About 20% of kids with scoliosis need to wear a back brace, which acts as a holding device that keeps the spine from developing more of a curve. A brace won't ever make the spine straight, but if it does its job well, the curve won't increase more than 5 or 10 degrees and surgery can be avoided.

The many different types of back braces are typically made of lightweight materials. Some braces are made to be worn 18 to 20 hours a day; others are worn only at night.

Several different types of braces are used, and the one that the orthopedist chooses depends on the child, the location of the curve, and the severity of the curve. The most common type is a TLSO (thoracic-lumbar-sacral-orthosis) brace. This is a low-profile brace, which means it comes up under the arms and fits beneath the clothes. Here are some examples of the TLSO brace:

  • Wilmington jacket. This brace was developed at the Alfred I. duPont Hospital for Children in Wilmington, Delaware. It's constructed of lightweight plastic and can be worn under clothes, so it isn't visible. It's designed to be worn as much as possible, at least 18–20 hours per day.
  • Charleston brace. This is also a low-profile brace, and it bends the spine in an effort to straighten the curve as much as possible to prevent the curve from worsening. This brace puts the child's body in an awkward position, though, so it can only be worn while sleeping.

Sometimes, even with a brace, surgery becomes necessary to correct the curvature of the spine. Overall, braces prevent surgery about 70% of the time.

If an operation is necessary, the orthopedic surgeon performs a procedure that will cause the separate bones of the spine where the curve is to grow into one solid piece of bone. This is called a spine fusion, and helps prevent further side-to-side curving. The surgeon also uses metal rods, hooks, and wire to keep the spine straight until the bits of bone join together with the vertebrae.

The surgery usually takes between 3 and 4 hours. The child is usually home within 1 week, and should be able to do normal activities right away (walking, dressing, climbing stairs, etc.). The child may need about a month off from school to get over the soreness from surgery and recover strength, and after 3 or 4 months can take part in most regular activities. After 6 to 12 months, the child can return to almost all activities.

After 3 to 6 months, the bone fusion is typically complete. The area where the rods are is "stiffer" than before, but the rods don't limit movement much — it's still possible to bend down and move all different ways. The rods are left in the patient's back, but only because taking them out would involve another operation, which isn't necessary.

With the right kind of treatment — whether it involves a brace or surgery — almost every child with scoliosis can have an active, normal life.

Nursemaid's Elbow


Whenever you reach out to a young child, be mindful not to pull or jerk his or her hands or arms. A quick tug of a toddler's arm can easily result in a slip or subluxation (partial dislocation) of an elbow bone. This is called "nursemaid's elbow" or pulled elbow.

Nursemaid's elbow most commonly occurs in kids 1 to 3 years old, though infants and older kids can experience it, too.

In younger children, the ligaments (bands of muscle that hold bones together) are loose and bones are not yet fully developed, making it easy for them to slip in and out of place.

As kids get older, however, their ligaments tighten, bones enlarge and harden, and the risk of nursemaid's elbow significantly decreases.

Causes of Nursemaid's Elbow

The elbow joint connects the humerus (upper arm bone) to the radius and ulna (lower arm bones). The rounded tip of the radius (the radial head) is surrounded by a ligament that is sometimes loose in kids.

If not tight enough, the ligament may slip over the radial head if a small amount of pressure is applied. This causes the bones to move out of place, or subluxate.

Any of the following can cause a subluxation:

  • Jerking a child's arm. Pulling a toddler along while walking or quickly grabbing his or her hand can jerk the arm, resulting in slipping of the radial head. Use caution when taking a child by the hand.
  • Pulling a child up by the hands. Pulling on hands or forearms can put stress on the elbows. Never pick up a toddler or infant by the hands or wrists. Lifting under the armpits is the safest way to lift a child.
  • Swinging a toddler by the arms. Any type of swinging by holding the hands or wrists can strain the elbow joint and should be avoided.
  • Breaking a fall with the arm. The natural response to falling is outstretching an arm for protection. The elbow can overextend during this type of injury, resulting in a slip of the radial head.
  • Rolling over in an awkward way. Sometimes rolling over in a crib, bed, or on the floor can cause nursemaid's elbow in infants and very young children.

When Your Child Is Injured

A child with nursemaid's elbow will probably not look outwardly injured because the subluxation does not cause the arm to twist or bend awkwardly.

However, a child may exhibit warning signs that should signal a red flag to parents. Here's what to look for:

  • The child refuses to use an arm. A child will not be able to use the injured arm without pain. Therefore, the arm is usually kept in a fixed, straight position or with a slight bend in the elbow.
  • Use of the arm is painful. Toddlers may tell you the forearm hurts, or they may cry or shout out in pain if you touch it. This doesn't necessarily indicate a case of nursemaid's elbow. Fractures and bruises will hurt, too. You'll just know something isn't right and a doctor needs to check it out.

Contact your doctor if you suspect a case of nursemaid's elbow. Do not attempt to put the bone back into place yourself. Timely treatment by a medical professional can ease pain and reduce the risk of further complications.

At the Doctor's Office

If your family doctor is not able to treat nursemaid's elbow, an orthopedic specialist may provide care. A doctor at a local emergency department can also see your child.

The doctor will first determine whether your child has nursemaid's elbow. The arm and shoulder will be felt to make sure there's no swelling or other abnormality, which could point to a fracture instead. If no swelling is present, the doctor will attempt to manually push the radial head back into place. The medical term for this is reduction.

Your child may be given some medicine for anxiety relief before the reduction, but anesthesia is not needed. The procedure is very quick and takes only a few seconds. A child is usually asked to sit on a parent's lap while the doctor attempts to reduce the elbow. During this procedure, the arm is taken from a straight position and quickly bent upwards. The doctor will listen and feel for a "pop" or "click" sound, indicating the bones are back in place. A child may experience a moment of pain in the elbow at the time of the reduction. After treatment, most kids have full use of the arm within 5 to 10 minutes.

Some cases may require more than one try to successfully reduce the elbow. Occasionally, a child may not use the arm after the reduction for a brief time, fearing it will be painful. If discomfort continues, the doctor may put the arm in a sling and recommend acetaminophen or ibuprofen for pain relief.

Kids who've had nursemaid's elbow are at risk of a recurrence, so it's important to be careful when picking up your child or holding hands. Never jerk, swing, or tug on a young child's hands or arms.

Muscular Dystrophy


Muscular dystrophy (MD) is a genetic disorder that gradually weakens the body's muscles. It's caused by incorrect or missing genetic information that prevents the body from making the proteins it needs to build and maintain healthy muscles.

A child who is diagnosed with MD gradually loses the ability to do things like walk, sit upright, breathe easily, and move the arms and hands. This increasing weakness can lead to other health problems.

There are several major forms of muscular dystrophy, which can affect a child's muscles in different levels of severity. In some cases, MD starts causing muscle problems in infancy, while in others, symptoms don't appear until adulthood.

There is no cure for MD, but researchers are quickly learning more about how to prevent and treat the condition. Doctors are also working on improving muscle and joint function, and slowing muscle deterioration so that kids, teens, and adults with MD can live as actively and independently as possible.

What Are the First Symptoms of Muscular Dystrophy?

Many kids with muscular dystrophy follow a normal pattern of development during their first few years of life.

But in time common symptoms begin to appear. A child who has MD may start to stumble, waddle, have difficulty going up stairs, and toe walk (walk on the toes without the heels hitting the floor). A child may start to struggle to get up from a sitting position or have a hard time pushing things, like a wagon or a tricycle. It is also common for a young child with MD to develop enlarged calf muscles, a condition called calf pseudohypertrophy, as muscle tissue is destroyed and replaced by fat.

How Is Muscular Dystrophy Diagnosed?

When a doctor first suspects that a child has muscular dystrophy, he or she probably will do a physical exam, take a family history, and ask about any problems - particularly those affecting the muscles - that the child might be experiencing.

In addition, the doctor may perform a series of tests to determine what type of MD a child may have and to rule out any other diseases that may be causing a problem. This might include a blood test to measure levels of serum creatine kinase, an enzyme that's released into the bloodstream when muscle fibers are deteriorating. Elevated levels of this enzyme indicate that something is causing muscle damage.

The doctor also may do a blood test to check a child's DNA for gene abnormalities, or a muscle biopsy to examine a muscle tissue sample for patterns of deterioration and abnormal levels of dystrophin, a protein that helps muscle cells keep their shape and length. Without dystrophin, the muscles break down.

Types of Muscular Dystrophy

The different types of muscular dystrophy affect different sets of muscles and result in different degrees of muscle weakness.

Duchenne muscular dystrophy is the most common and the most severe form of the disease. It affects about 1 out of every 3,500 boys. (Girls can carry the gene that causes the disease, but they usually have no symptoms.) This form of MD occurs because of a problem with the gene that makes dystrophin. Without this protein, the muscles break down and a child becomes weaker.

In cases of Duchenne muscular dystrophy, symptoms usually begin to appear around age 5, as the pelvic muscles begin to weaken. Most kids with this form of MD need to use a wheelchair by age 12. Over time, their muscles weaken in the shoulders, back, arms, and legs. Eventually, the respiratory muscles are affected, and a ventilator is required to assist breathing. Kids who have Duchenne muscular dystrophy typically have a life span of about 20 years.

Although most kids with Duchenne muscular dystrophy have average intelligence, about one-third of them experience learning disabilities and a small number of them have mental retardation.

While the incidence of Duchenne is known, it's unclear how common other forms of MD are because the symptoms can vary so widely between individuals. In fact, in some people the symptoms are so mild that the disease goes undiagnosed.

Becker muscular dystrophy is similar to Duchenne, but it is less common and progresses more slowly. This form of MD affects approximately 1 in 30,000 boys. It too is caused by insufficient production of dystrophin.

With this form of MD, symptoms typically begin during the teen years, then follow a pattern similar to Duchenne muscular dystrophy. Muscle weakness first begins in the pelvic muscles, then moves into the shoulders and back. Many children with Becker have a normal life span and can lead long, active lives without the use of a wheelchair.

Myotonic dystrophy, also known as Steinert's disease, is the most common adult form of muscular dystrophy, although half of all cases are diagnosed in people who are younger than 20 years old. It is caused by a portion of a particular gene that is larger than it should be. The symptoms can appear at any time during a child's life.

The main symptoms include muscle weakness, myotonia (in which the muscles have trouble relaxing once they contract), and muscle wasting, where the muscles shrink over time. Kids with myotonic dystrophy also can experience cataracts and heart problems.

Limb-girdle muscular dystrophy affects boys and girls equally. Typically, symptoms begin when kids are between 8 and 15 years old. This form of MD progresses slowly, affecting the pelvic, shoulder, and back muscles. The severity of muscle weakness varies from person to person. Some kids develop only mild weakness while others develop severe disabilities and as adults need a wheelchair to get around.

Facioscapulohumeral muscular dystrophy can affect both boys and girls, and the symptoms usually first appear during the teen years. This form of muscular dystrophy tends to progress slowly.

Muscle weakness first develops in the face, making it difficult for a child to close the eyes, whistle, or puff out the cheeks. The shoulder and back muscles gradually become weak, and kids who are affected have difficulty lifting objects or raising their hands overhead. Over time, the legs and pelvic muscles also may lose strength.

Other types of muscular dystrophy, which are rare, include distal, ocular, oculopharyngeal, and Emery-Dreifuss.

Caring for a Child With Muscular Dystrophy

Though there's no cure for MD yet, doctors are working to improve muscle and joint function, and slow muscle deterioration in kids who are living with the condition.

If your child is diagnosed with muscular dystrophy, a team of medical specialists will work with you and your family. That team will likely include: a neurologist, orthopedist, pulmonologist, physical and occupational therapist, nurse practitioner, cardiologist, registered dietician, and a social worker.

Muscular dystrophy is often degenerative, so kids may pass through different stages as the disease progresses and require different kinds of treatment. During the early stages, physical therapy, joint bracing, and the medication prednisone are often used. During the later stages, doctors may use assistive devices such as:

  • physical therapy and bracing to improve your child's flexibility
  • power wheelchairs and scooters to improve your child's mobility
  • a ventilator to support your child's breathing
  • robotics to help your child perform routine daily tasks

Physical Therapy and Bracing

Physical therapy can help a child to maintain muscle tone and reduce the severity of joint contractures with exercises that keep the muscles strong and the joints flexible.

A physical therapist also uses bracing to help prevent joint contractures, a stiffening of the muscles near the joints that can make it harder to move and can lock the joints in painful positions. By providing extra support in just the right places, bracing can extend the time that a child with MD can walk independently.

Prednisone

If a child has Duchenne muscular dystrophy, the doctor may prescribe the steroid prednisone to help slow the rate of muscle deterioration. By doing so, a child with muscular dystrophy may be able to walk longer and live a more active life.

There is some debate over the best time to begin treating a child with prednisone, but most doctors prescribe it when a child with MD is 5 or 6 years old, or when the child's strength begins to show a significant decline. Prednisone does have side effects, though. It can cause weight gain, which can put even greater strain on a child's already-weak muscles. It also can cause a loss of bone density and, possibly, lead to fractures. If your doctor prescribes prednisone, he or she will closely monitor your child.

Spinal Fusion

Many children who have the Duchenne and Becker forms of muscular dystrophy develop severe scoliosis - an S- or C-shaped curvature of the spine that develops when the back muscles are too weak to hold the spine erect. Some kids who have severe cases of scoliosis undergo spinal fusion, a surgery that can reduce pain, lessen the severity of the spine curvature so that a child can sit upright and comfortably in a chair, and ensure that the spine curvature doesn't have an effect on the child's breathing. Typically, spinal fusion surgery only requires a short hospital stay.

Respiratory Care

Many kids with muscular dystrophy also have weakened heart and respiratory muscles. As a result, they can't cough out phlegm and sometimes develop respiratory infections that can quickly become serious. Good general health care and regular vaccinations are especially important for children with muscular dystrophy to help prevent these infections.

Assistive Devices

A variety of new technologies are available to create independence and mobility for kids with muscular dystrophy.

Some kids with Duchenne muscular dystrophy may use a manual wheelchair once it becomes difficult to walk. Others go directly to a motorized wheelchair, which can be equipped to meet their needs as muscle deterioration advances.

Robotic technologies also are under development to help kids move their arms and perform activities of daily living.

If your child would benefit from an assistive technological device, it's a good idea to contact your local chapter of the Muscular Dystrophy Association (see the Additional Resources tab for a link to their website) to ask about financial assistance that might be available. In some cases, health insurers cover the cost of these devices.

The Search for a Cure

Researchers are quickly learning more about what causes the genetic disorder that leads to muscular dystrophy, and about possible treatments for the disease. If you'd like to know more about the most current research on muscular dystrophy, contact the local chapter of the Muscular Dystrophy Association, or talk to your child's doctor.