Pediatrics

Embolization

Embolization is a nonsurgical procedure for clogging small blood vessels and blocking the flow of blood.

Embolization is used to manage complex lesions (abnormal body tissues), such as arteriovenous malformations and combined vascular malformations. It is often used as part of an overall treatment strategy that combines a variety of approaches. Patients may also need embolization prior to surgery to prevent bleeding. When lesions are extensive and surgery is not possible, embolization may improve the overall quality of life by controlling the lesion and lessening the symptoms.

Arteriovenous and combined vascular malformations may require a series of two to three treatments to block all of the abnormal vessels. Treatments usually are spaced several months apart. Long-term follow-up and evaluation is required to determine if re-treatment is necessary.

Your child’s procedure will be scheduled by an Interventional RN or technologist. Before the day of the procedure, your child may be seen in the Interventional Radiology clinic where you will meet the radiologist, nurse practitioner and RN who will care for your child. The radiologist will discuss the procedure with you and answer any questions you may have. The nurse will give you instructions for the day of the procedure.

You will be able to stay with your child until just before the procedure is performed and will be escorted to the surgical waiting area for the duration of the procedure.

Your child will receive general anesthesia and will not experience pain during the procedure.

A doctor called an interventional radiologist places a tiny plastic tube called a catheter through the femoral artery or vein, which is located in the upper leg. The doctor then positions the tip of the catheter into an abnormal artery or vein and injects small foam-like particles or titanium coils. This closes the blood vessel and prevents the flow of blood into the malformation.

X-rays are taken during the procedure so the doctor can clearly see which blood vessels need embolization. When the procedure is completed, the catheter is removed and pressure is applied to the catheter site to prevent bleeding. Embolization usually takes three to four hours.

As your child begins to wake up from the anesthesia, he/she will be taken to the Post Anesthesia Care Unit (PACU) where he/she will be closely watched. You may stay with your child in the PACU. Your child will be admitted to the hospital after the procedure for 6-23 hours. Your child must lie very still and cannot bend his/her leg for 4-6 hours after the procedure. The radiologist will speak to you about the results after the procedure is complete.

After discharge from the hospital your child may experience mild discomfort or pain at the catheter site. He/She may also have a fever of 100° F or less for the first few days. This is normal. You may give acetaminophen (Tylenol) for discomfort or fever, following the instructions on the label. If needed, the doctor or nurse practitioner will give you a prescription for pain medication. To prevent bleeding at the catheter site, it is important that you do not give aspirin or medications that contain aspirin – unless prescribed by a member of the interventional radiology team. Aspirin may make your child more susceptible to bleeding.

Your child will not have any restrictions on bathing or routine activities; however, heavy lifting, stair climbing and contact sports should be avoided for several days.

Embolization is a safe and effective procedure that has been shown to be invaluable in treating arteriovenous and combined vascular malformations. Most patients do not have problems or serious side effects; however, bleeding or bruising can occur at the catheter site. Clotting of an artery or damage to normal tissue can also occur.

Stenosis

Spinal stenosis is a medical condition in which the spinal canal narrows and compresses the spinal cord and nerves. This is usually due to the spinal degeneration that commonly occurs with aging. It can also sometimes be caused by osteoporosis, spinal disc herniation, or a tumor.

Spinal stenosis may affect the cervical, thoracic or lumbar spine.

Symptoms include:

  • Pain
  • Weakness
  • Tingling of the legs

Surgery for spinal stenosis is the most common spinal operation in people over the age of 50. However, spinal stenosis caused by developmental narrowing of the spinal canal may occur in people in their 20s and 30s.

Operations used to treat stenosis include:

  • Anterior Cervical Discectomy and Fusion — A small incision in the front of the neck is used to access the upper spine. The ruptured or herniated disc is removed and replaced with a small bone plug, which eventually grows to connect the two adjacent vertebrae.
  • Cervical Corpectomy — Part of the vertebra and discs are removed and replaced with a bone graft or a metal plate and screws to support the spine.
  • Decompressive Laminectory — The roof of the vertebrae, called the lamina, is surgically removed. The procedure also may include removing part of the disc or fusing the vertebrae (spinal fusion).
  • Foramenotomy — The area where nerve roots leave the spinal canal, called the foramen, is removed. This procedure can be performed using a minimally invasive approach with an endoscope, an instrument that allows the surgeon to see inside the body through a tiny incision. The surgeon can then use other tiny incisions to perform the surgery, avoiding the discomfort and muscle atrophy associated with the traditional open technique that uses a large incision.
  • Laminoplasty — The compressive bone in the back of the neck is gently lifted off of the spinal cord creating a new “roof” over the spinal cord and nerve roots. This procedure effectively decompresses the spinal cord over multiple segments without the need for fusion or hardware. It also minimizes the chance of spinal instability or deformity that may result from the traditional laminectomy procedure.
  • Laminotomy — Only a small portion of the lamina is removed.
  • Medial Facetectomy — Part of the bone structure in the spinal canal, called the facet, is removed.
  • Cervical Disc Replacement — Instead of fusing the affected area, the natural disc material is replaced with a metal and plastic prosthesis that maintains or restores the motion segment. This will hopefully prevent degeneration of the next disc level.

Cervical Stenosis

Stenosis in the neck, also called the cervical spine, affects the upper part of the body including the arms and hands. Stenosis is the narrowing of the bony canal that protects the spinal cord and its branching nerves to the point where it injures the spinal cord or nerves.

This may be caused by a number of conditions including:

  • Bone spurs
  • Rupture of the spinal discs

Cervical stenosis may cause pain, numbness, or weakness in the legs. The pain may move from one part of the body to another but is often most noticeable in the neck.

If the stenosis is severe and is not responding to other treatment methods, surgery to widen the spinal canal may be necessary. Because bone continues to deteriorate, additional treatment may be needed several years after even successful surgery.

Lumbar Stenosis

Stenosis in the lower back is called lumbar stenosis. It is often characterized by radiating pain in the buttocks and legs.

Frequently people afflicted with lumbar stenosis have varying degrees of low back discomfort. The pain typically occurs most often during activities and is relieved by resting, sitting or bending forward. In some cases, the pain is centralized in the lower legs and feet. In severe cases, it also can impact continence (bowel and bladder control) and sexual function.

The initial treatment for stenosis is to treat the symptoms rather than the condition itself. These treatments include:

  • Rest
  • Posture changes, such as lying with the knees drawn up to the chest or leaning forward while walking, may relieve the pressure on the nerves
  • Medication such as aspirin or ibuprofen to relieve inflammation and pain
  • Physical therapy
  • Losing weight
  • Corticosteroid injections to reduce inflammation and relieve pain
  • A cervical collar

If several months of treatment have not improved the symptoms, and if the stenosis is severe, surgery to widen the spinal canal may be necessary. Because bone continues to deteriorate, additional treatment may be needed several years after even successful surgery.

Scoliosis

Everyone’s spine has natural curves. These curves round our shoulders and make the lower back curve slightly inward. But some people have spines that also curve from side to side, a common condition called scoliosis. Scoliosis is defined as curvature of the spine greater than 10 degrees, as measured on an X-ray. Anything less is simply due to normal variation. On an X-ray, a spine with scoliosis looks more like an “S” or a “C” than a straight line. Some of the bones in a scoliotic spine also may have rotated slightly, making the person’s waist or shoulders appear uneven.

Scoliosis affects approximately 20 million people in North America, about 2 percent of the population, including children and adults. The condition tends to run in families. If someone in a family has scoliosis, the likelihood of another family member having it is much higher — about 20 percent.

As the population ages, adult degenerative scoliosis is becoming more common, with the condition typically developing at between age 50 to 70. The condition can have devastating effects on a person’s life in later years.

Treatment in this older population presents particular challenges due to other back conditions, such as osteoporosis. But significant advances — including minimally invasive surgery, new neuro-monitoring techniques and 3-dimensional imaging — allows older patients to receive limited doses of anesthesia and benefit from quicker recovery.

Scoliosis may be divided into five types:

  • Congenital Scoliosis — Congenital means that a person is “born with” scoliosis. Congenital scoliosis is caused by an abnormality of one or more vertebrae where they fail to form properly. This can be seen on X-ray and directly in the operating room.
  • Idiopathic Scoliosis — This is the most common form of scoliosis. The name idiopathic means “the cause is unknown.” Those with this type of scoliosis are otherwise healthy and normal. The spine shows no abnormality of the bones themselves on X-rays or by looking at it directly in the operating room. Idiopathic scoliosis may involve pain, which is more common in adults with scoliosis.While the overall incidence is equal in females and males, progressive or severe idiopathic scoliosis is about six to seven times more frequent in females.
  • Neuromuscular Scoliosis — This type of scoliosis occurs in people who have a disease of the nervous system, such as cerebral palsy.
  • Postural Scoliosis — Also known as “hysterical scoliosis,” postural scoliosis may be a result of pain, as a patient tilts to relieve the pain. It can be reversed by relieving the pain or by having the patient lie flat. X-rays don’t show any abnormality of the vertebrae.
  • Syndromic Scoliosis — This type of scoliosis occurs in people with a syndrome, such as Marfan syndrome or one of the skeletal dysplasias such as achondroplasia.

Progressive, severe scoliosis can produce three major problems:

  • If the part of the spine in the chest, called the thoracic spine, curves more than 60 degrees, the volume of the chest can be reduced, potentially compromising the function of the heart and lungs. For example, the heart may have to work harder to pump the normal volume of blood or the affected individual may have shortness of breath.
  • Severe curvature of the lower half of the spine that connects the chest with the pelvis, known as the lumbar spine, may push the contents of the abdomen against the chest and interfere indirectly with heart and lung function. Curvature of the lumbar spine also may alter sitting balance and posture.
  • Severe curvature of either the thoracic (upper) or the lumbar (lower) spine, or both, eventually becomes visible to others. The resulting tilting and twisting of the back, shoulders and pelvis may produce an appearance that the individual finds unacceptable. If idiopathic scoliosis affects a girl around the time of puberty, when body image is developing hand-in-hand with self-esteem, the condition can pose significant psychological and emotional challenges.

Traditional medical management of scoliosis is determined by the severity of the curvature and skeletal maturity, which together help predict the likelihood of progression.

Conventional treatment options include:

  1. Observation
  2. Physical therapy
  3. Occupational Therapy
  4. Chiropractic
  5. Bracing
  6. Surgery

Sclerotherapy

Sclerotherapy is a nonsurgical way to treat certain types of vascular malformations, which are abnormal clusters of blood vessels that occur during fetal development.

Sclerotherapy is used to reduce the size of venous and lymphatic malformations or to eliminate pain that is associated with them. Depending on the depth, location and extent of these malformations, sclerotherapy is sometimes used with other therapies, including surgical removal of the malformation.

When your child’s procedure is scheduled, you will be given specific information from the interventional nurse for arrival time and eating instructions. Your child may be seen in the HVMC clinic and/or the interventional clinic prior to scheduling the procedure. The radiologist will discuss the treatment plan with you at the clinic visit and you will meet the nurse practitioner, nurse and interventional radiology technologist that will assist during your child’s procedure.

Your child will receive general anesthesia and will not experience pain during the procedure. You will be able to stay with your child until anesthesia is administered for the procedure. You will be escorted to the surgical waiting area where the doctor will talk to you after the procedure.

Using X-ray and ultrasound equipment, a doctor called an interventional radiologist, places a small needle into the vascular malformation and injects it with a chemical substance called a sclerosant. The sclerosant causes clotting, inflammation and eventual shrinkage of the malformation. There are several different agents the doctor may use; it depends on the type of malformation being treated and the intended result.

Depending on the size and shape of the malformation, more than one injection may be needed. The procedure usually takes two to three hours; however, its beneficial effects may not be seen for up to two months.

As your child begins to wake up from the anesthesia, he/she will be taken to the Post Anesthesia Care Unit (PACU), where he/she will be closely observed. You will be brought to the PACU once your child is settled in and assessed. The injection areas are usually quite swollen and bruised. This is normal and is not cause for concern. If your child has severe swelling, he/she may be given a steroid medication, such as prednisone, to help reduce this. The radiologist will speak to you about the results after the procedure. When your child is ready, he/she will be moved from the PACU to a patient room, where he/she will remain overnight for careful observation or may be discharged from the PACU.

After the procedure, your child may experience some discomfort related to swelling and bruising. Swelling and bruising gradually decrease over the first week, but do not go away completely until the blood clots are absorbed, which may take up to two months. Skin inflammation is sometimes caused if the sclerosant leaks onto adjacent skin. If this occurs your physician or nurse practitioner will prescribe medication to reduce the discomfort. It is important that you do not give aspirin or medications that contain aspirin – unless prescribed by a member of the interventional radiology team. Aspirin may make your child more susceptible to bleeding.

After sclerotherapy your child will not have any restrictions on bathing or other routine activities; however, heavy lifting and contact sports should be avoided for two weeks. Your child may also have an ACE wrap to the site of the sclerosing. Children who have sclerotherapy to a leg or hip may need to use crutches for 3-4 days.

About 25 percent of patients require more than one treatment. These treatments generally are spaced at three-month intervals.

Although sclerotherapy has been proven to be safe and effective, side effects occasionally occur. These may include blistering, scarring, infection, numbness due to nerve damage and localized tissue loss.

Polycystic Ovarian Syndrome

Polycystic ovarian syndrome (PCOS) is a hormonal disorder in which a woman’s body produces abnormally high levels of the male hormone, called androgens. These high levels of androgens prevent the ovaries from producing enough progesterone, which is necessary for a normal menstrual cycle. This results in undeveloped egg follicles, which turn into small cysts in the ovaries that prevent ovulation.

PCOS affects approximately five to ten percent of women of childbearing age and is a leading cause of infertility. The condition is also the most common hormonal disorder among pre-menopausal women. Women of all ethnicities may be affected.

The exact cause of PCOS is unknown. However, research suggests that genetics may play a part, since women who have female relatives with PCOS are at a higher risk of developing the syndrome. Obesity, diabetes, being insulin resistant and having problems with the adrenal, thyroid or pituitary glands may also contribute to PCOS.

Symptoms develop within a few years of puberty, though sometimes they appear later in life. Symptoms may worsen during a woman’s prime reproductive years — between the ages of 20 and 40 — particularly in women who gain a significant amount of weight.

Symptoms of PCOS vary for each woman. However, common symptoms may include:

  • Irregular periods
  • Excess body or facial hair
  • Acne
  • Difficulty maintaining a healthy weight

PCOS may also be associated with depression, difficulty getting pregnant, predisposition to type II diabetes and other long-term health problems.

Currently, there is no cure for PCOS. Medical treatment of PCOS varies by the patient and their goals. The condition can be managed by treating symptoms with medications and by adapting a healthy, well-balanced lifestyle.

Treatments include:

  • Lowering of insulin levels
  • Weight loss
  • Treatment of hirsutism or acne
  • Restoration of fertility, which may include a surgical procedure called ovarian drilling may be recommended to induce ovulation
  • Restoration of regular menstruation, and prevention of endometrial hyperplasia and endometrial cancer

Kleine-Levin Syndrome

Kleine-Levin syndrome is a rare disorder is characterized by recurring but reversible periods of excessive sleep (up to 20 hours per day).

Kleine-Levin syndrome primarily affects adolescent males (approximately 70 percent of those with Kleine-Levin syndrome are male).

Symptoms may be related to malfunction of the hypothalamus and thalamus, parts of the brain that govern appetite and sleep.

Symptoms occur as “episodes”, typically lasting a few days to a few weeks. Episode onset is often abrupt, and may be associated with flu-like symptoms. During episodes, the following may be observed:

  • Excessive food intake
  • Irritability
  • Childishness
  • Disorientation
  • Hallucinations
  • An abnormally uninhibited sex drive

Mood can be depressed as a consequence, but not a cause, of the disorder. Affected individuals are completely normal between episodes, although they may not be able to remember afterwards everything that happened during the episode. It may be weeks or more before symptoms reappear. Episodes eventually decrease in frequency and intensity over the course of eight to 12 years.

There is no definitive treatment for Kleine-Levin syndrome and watchful waiting at home, rather than pharmacotherapy, is most often advised. Stimulant pills, including amphetamines, methylphenidate, and modafinil, are used to treat sleepiness but may increase irritability and will not improve cognitive abnormalities. Because of similarities between Kleine-Levin syndrome and certain mood disorders, lithium and carbamazepine may be prescribed and, in some cases, have been shown to prevent further episodes. This disorder should be differentiated from cyclic re-occurrence of sleepiness during the premenstrual period in teen-aged girls, which may be controlled with birth control pills. It also should be differentiated from encephalopathy, recurrent depression, or psychosis.

Interstitial Lung Disease

Interstitial lung disease (ILD) refers to a group of lung diseases affecting the tissue and space around the air sacs of the lungs (the interstitium). It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivascular and perilymphatic tissues.

Interstitial lung diseases include:

  • Hypersensitivity Pneumonitis
  • Idiopathic Pulmonary Fibrosis
  • Sarcoidosis

Hypersensitivity Pneumonitis

Hypersensitivity pneumonitis (HP) is an interstitial lung disease caused by repeated inhalation of certain fungal, bacterial, animal protein or reactive chemical particles, called antigens. While most people who breathe in these antigens don’t develop problems, in some people, the body’s immune reaction to these particles causes inflammation of the lung. In some cases, parts of the lungs may become scarred.

HP should not be confused with the more common types of allergies, which are caused by small amounts of proteins in the environment such as dust mites, cat dander, pollen and grass. Having seasonal or environmental allergies has nothing to do with having or developing HP.

It’s not known why a minority of people exposed to these antigens develop HP. Their genetics and environment may interact to make them more susceptible to the disease.

Hypersensitivity pneumonitis (HP) is subdivided into two forms: acute and chronic. Symptoms differ for each form.

  • The acute form of HP occurs after heavy, often short-term exposure to the antigen. Symptoms appear relatively suddenly and include fever, chills, fatigue, breathlessness, chest tightness and cough. If the person is removed from the antigen exposure, the symptoms usually resolve over 24 to 48 hours. Recovery is often complete.
  • The chronic form of HP is thought to occur due to longer term, low-level exposure to the antigen, and it often causes more subtle symptoms. Patients with chronic HP often describe chronic symptoms, such as shortness of breath or cough, that have gotten worse. Symptoms may worsen at work, at home or wherever the patient is being exposed to the antigen, but most often, patients with chronic HP haven’t had acute episodes.

The best treatment is to avoid the provoking allergen, as chronic exposure can cause permanent damage. Corticosteroids such as Prednisolone may help to control symptoms but may produce side-effects.

In addition to medications and removal of the antigen, pulmonary rehabilitation — a structured exercise and educational program designed for patients with chronic lung disease — is an important and effective treatment for patients with chronic HP.

Lung transplant may be an effective treatment option for some patients.

Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis, or IPF, is a condition that causes progressive scarring of the lungs. Fibrous scar tissue builds up in the lungs over time, affecting their ability to provide the body with enough oxygen.

IPF affects more than 100,000 people in North America, with 30,000 to 40,000 new cases diagnosed each year. Typically the disease is found in people between the ages of 50 and 70 and affects men more frequently than women. Most patients are former smokers. There are no proven risk factors for IPF, but a minority of patients does have a family history of lung scarring.

The cause of the condition is unknown.

Symptoms and signs of IPF often appear gradually and include:

  • Shortness of breath, particularly during or after physical activity
  • Chronic, dry hacking cough
  • Crackles in the lungs heard through a stethoscope
  • Rounding of the fingernails, a condition called clubbing

Symptoms of IPF may mimic those of other diseases that cause lung scarring, so diagnosing IPF often involves ruling out other conditions. Several visits with your doctor may be needed to finalize your diagnosis and treatment approach.

There is no cure for IPF and currently no satisfactory treatment exists. Medical studies suggest a potential role for several therapies, but further study is needed.

Pulmonary rehabilitation — a structured exercise and educational program designed for patients with chronic lung disease — is important for patients with IPF. Lung transplant may be an effective treatment option for some patients.

Sarcoidosis

Sarcoidosis is a disorder that causes inflamed tissue called nodules or granulomas to develop in the body’s organs, most often the lungs. It can also affect the skin, eyes, nose, muscles, heart, liver, spleen, bowel, kidney, testes, nerves, lymph nodes and brain. Nodules in the lungs can lead to narrowing of the airways and inflammation, also called fibrosis, of lung tissue.

Sarcoidosis affects people of all ages, races and gender, though it most commonly occurs in people between 20 to 40 years old. Children are rarely diagnosed with the disease. In very few cases, more than one family member is affected. African Americans are three to four times more likely to have sarcoidosis and may have a more severe form of the disease than people of European descent.

The exact cause of sarcoidosis is not known.

Symptoms of sarcoidosis may differ in each person and depends on the organs affected. Frequently, the condition causes mild symptoms and resolves on its own without treatment. In approximately half of all patients, sarcoidosis is detected on a routine chest X-ray before any symptoms develop.

The most common symptoms of sarcoidosis involving the lungs include:

  • Cough
  • Shortness of breath
  • Chest pain, which is usually a vague tightness of the chest, but can occasionally be severe and similar to the pain of a heart attack
  • Fatigue
  • Weakness
  • Fever
  • Weight loss

There is no specific treatment to cure the condition at this time. Fortunately, between 30 and 70% of patients do not require therapy because the nodules gradually resolve on their own and leave behind few, if any, signs of inflammation or other complications.

However, treatment is necessary in some cases. Medications are available that effectively suppress symptoms and help reduce lung inflammation, the impact of nodules and prevent the development of lung fibrosis. These include:

  • Corticosteroids
  • Methotrexate
  • Cyclophosphamide and Azathioprine
  • Antimalarial Medications (Hydroxychloroquine)
  • Colchicine

A number of other medications are currently being investigated for the treatment of sarcoidosis

Deformity

Spine deformities can cause debilitating pain and seriously impact function, self-image and overall quality of life.

Spine deformities include:

  • Ankylosing Spondylitis
  • Kyphosis
  • Scoliosis
  • Spondylolisthesis

Patients with complex spinal deformities may require:

  • Surgery
  • Rehabilitation
  • Pain control
  • Physical medicine
  • Bracing
  • Orthotics

Ankylosing Spondylitis

Ankylosing spondylitis is an inflammatory condition that involves the spine and skeleton of the head and trunk. The disorder causes inflammation and pain in joints in the spine, pelvis and other parts of the skeleton. In addition, parts of the spine, the sacroiliac joints where the hips join the lower back, or the hips may fuse, or grow, together.

The typical patient is a young male, aged 20–40. Men are affected more than women by a ratio about of 3:1. The disease usually takes a more painful course in men than women.

Symptoms include:

  • Pain
  • Stiffness, especially in the morning
  • Functional limitation

When the disorder affects the spine, it also may result in progressive deformity including curvature of the back, called kyphosis, and the inability to stand up straight.

No cure is known for ankylosing spondylitis, although treatments and medications are available to reduce symptoms and pain. Physical therapy and exercise, along with medication, are the most common forms of therapy.

Kyphosis

Kyphosis describes the exaggerated curve of the spine that results in a rounded or hunched back. Kyphosis may develop for several reasons. Postural kyphosis in children and adolescents may be related to habit and posture rather than underlying spinal deformity. In contrast, structural kyphosis refers to a round-back posture that is not reversible by paying attention to your posture and making an effort to sit and stand up straight. In adolescents, structural kyphosis may be caused by initial spine development with a rounded shape that is made worse by further growth. In the elderly, compression fractures characteristically result in loss of height and kyphotic deformity.

Symptoms of kyphosis include:

  • Back pain
  • Difficulty standing with an upright posture
  • Early fatigue to the back and legs

Kyphosis causes a bowing of the back, seen as a slouching back, as well as breathing difficulties. Severe cases can cause great discomfort and even lead to death.

Treatments include:

  • Body braces
  • Physical therapy
  • Surgery (in severe cases)

Spondylolisthesis

Spondylolisthesis is a condition in which one vertebra slips forward on the one below it.

In children, spondylolisthesis may occur as the result of a birth defect that affects the back of the spine or be caused by stress fractures within the back part of the spine. Spondylolisthesis is the most common cause of low back pain in adolescent athletes. In older people, the most common cause is degeneration of the discs between the vertebrae. With aging, the discs lose moisture, dry out and flatten, bringing the bones on either side closer together to the point where one slips forward on the other.

Typical symptoms of spondylolisthesis include:

  • Pain across the lower back
  • Hamstring tightness
  • Cosmetic deformity
  • Radiculopathy
  • Abnormal gait

However, a person can have the condition and not have pain.

Most often, treatment for spondylolisthesis includes:

  • Physical therapy to strengthen the back muscles
  • Pain medication
  • Bed rest
  • Wearing a back brace or corset

Children and adolescents whose spines have slippage greater than 30 percent to 50 percent may be candidates for spinal fusion surgery. Children and adults who have persistent pain despite non-operative care also may be considered for surgery.

Surgery for spondylolisthesis may involve decompression of the nerve roots by removing bone and/or intervertebral disc material, followed by fusion of the vertebrae with or without bracing.

Cystic Fibrosis

Cystic fibrosis (CF) is a chronic, progressive and frequently fatal genetic disease of the body’s mucus glands that often causes severe respiratory and digestive disorder. The disease causes the body to produce an abnormally thick, sticky mucus that obstructs the pancreas, preventing enzymes from reaching the intestines to digest food. The sweat glands and the reproductive system also are usually involved.

CF is caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR). In North America, cystic fibrosis affects about 30,000 children and adults. Caucasians of Northern European ancestry have the greatest chance of being carriers of the cystic fibrosis gene.

Cystic fibrosis has a variety of symptoms including:

  • Very salty-tasting skin
  • Persistent coughing, wheezing or pneumonia
  • Excessive appetite but poor weight gain
  • Bulky stools

The sweat test is the standard diagnostic test for cystic fibrosis. This simple and painless test measures the amount of salt in the sweat. A high salt level can indicate that a person has CF.

People who carry traits for these disorders may not have symptoms. However, if both parents are carriers of the same abnormal gene, the chance of having an affected child is 1 in 4, or 25 percent, for each pregnancy. A special blood test, called a carrier test, may be able to tell if you carry such a trait.

While there are no cures for cystic fibrosis there are several treatment methods. Disease management is aimed at maximizing organ function and consists of proactive treatment of airway infection, and encouragement of good nutrition and an active lifestyle. Current treatments only delay the decline in organ function at best. Targets for therapy are the lungs, gastrointestinal tract, the reproductive organs, and psychological support.

With improved treatments, the lifespan of individuals with cystic fibrosis has increased so that on average it now is about 30 years.

Cyclic Vomiting Syndrome

Cyclic Vomiting Syndrome (CVS) is characterized by episodes or cycles of severe nausea and vomiting that last for hours, or even days, that alternate with intervals with no symptoms.

Although originally thought to be a pediatric disease, CVS occurs in all age groups. In children CVS starts most often between the ages of 3 and 7.

Each episode of CVS is similar to previous ones, meaning the episodes tend to start at the same time of day, last the same length of time, and occur with the same symptoms and level of intensity. Episodes can be so severe that a person has to stay in bed for days, unable to go to school or work.

CVS has four phases:

Symptom-free interval phase. This phase is the period between episodes when no symptoms are present.
Prodrome phase. This phase signals that an episode of nausea and vomiting is about to begin. Often marked by nausea—with or without abdominal pain—this phase can last from just a few minutes to several hours. Sometimes, taking medicine early in the phase can stop an episode in progress. However, sometimes there is no warning; a person may simply wake up in the morning and begin vomiting.
Vomiting phase. This phase consists of nausea and vomiting; an inability to eat, drink, or take medicines without vomiting; paleness; drowsiness; and exhaustion.
Recovery phase. This phase begins when the nausea and vomiting stop. Healthy color, appetite, and energy return.
Many people can identify a specific condition or event that triggered an episode, such as an infection. Common triggers include:

Emotional stress and excitement (in children)
Anxiety and panic attacks
Colds
Allergies
Sinus problems
The flu
Eating certain foods such as chocolate or cheese
Eating too much
Eating just before going to bed
Hot weather
Physical exhaustion
Menstruation
Motion sickness
A person who experiences the following symptoms for at least 3 months—with first onset at least 6 months prior—may have CVS:

Vomiting episodes that start with severe vomiting—several times per hour—and last less than 1 week
Three or more separate episodes of vomiting in the past year
Absence of nausea or vomiting between episodes
A person with CVS may experience:

Abdominal pain
Diarrhea
Fever
Dizziness
Sensitivity to light during vomiting episodes
Continued vomiting may cause severe dehydration that can be life threatening. Symptoms of dehydration include thirst, decreased urination, paleness, exhaustion, and listlessness. A person with any symptoms of dehydration should see a health care provider immediately.

The relationship between migraine and CVS is still unclear, but medical researchers believe the two are related.

The severe vomiting that defines CVS is a risk factor for several complications:

Dehydration. Vomiting causes the body to lose water quickly. Dehydration can be severe and should be treated immediately.
Electrolyte imbalance. Vomiting causes the body to lose important salts it needs to keep working properly.
Peptic esophagitis. The esophagus—the tube that connects the mouth to the stomach—becomes injured from stomach acid moving through it while vomiting.
Hematemesis. The esophagus becomes irritated and bleeds, so blood mixes with vomit.
Mallory-Weiss tear. The lower end of the esophagus may tear open or the stomach may bruise from vomiting or retching.
Tooth decay. The acid in vomit can hurt teeth by corroding tooth enamel.
CVS is hard to diagnose because no tests—such as a blood test or x ray—can establish a diagnosis of CVS.

Treatment varies, but people with CVS generally improve after learning to control their symptoms. People with CVS are advised to get plenty of rest and sleep and to take medications that prevent a vomiting episode, stop one in progress, speed up recovery, or relieve associated symptoms.

Once a vomiting episode begins, treatment usually requires the person to stay in bed and sleep in a dark, quiet room. Severe nausea and vomiting may require hospitalization and intravenous fluids to prevent dehydration. Sedatives may help if the nausea continues.

Sometimes, during the prodrome phase, it is possible to stop an episode from happening. For example, people with nausea or abdominal pain before an episode can ask their doctor about taking ondansetron (Zofran) or lorazepam (Ativan) for nausea or ibuprofen (Advil, Motrin) for pain. Other medications that may be helpful are ranitidine (Zantac) or omeprazole (Prilosec), which help calm the stomach by lowering the amount of acid it makes.

People whose episodes are frequent and long-lasting may be treated during the symptom-free intervals in an effort to prevent or ease future episodes. Medications that help people with migraine headaches are sometimes used during this phase, but they do not work for everyone. Taking the medicine daily for 1 to 2 months may be necessary before one can tell if it helps.