Archive for June, 2010

Legg-Calvé-Perthes Disease

Posted by on Sunday, 27 June, 2010

Legg-Calvé-Perthes disease is a temporary condition in children in which the ball-shaped head of the thigh bone, referred to as the femoral head, loses its blood supply. As a result, the femoral head collapses. The body will absorb the dead bone cells and replace them with new bone cells. The new bone cells will eventually reshape the femoral head of the thigh bone. Legg-Calvé-Perthes disease causes the hip joint to become painful and stiff for a period of time.

Legg-Calvé-Perthes disease goes through four phases of changes that affect the head of the femur. The phases include:

LCPD

Phase 1 – Blood supply is absent to the femoral head and the hip joint becomes inflamed, stiff, and painful. Portions of the bone turn into dead tissue. The ball of the thigh bone becomes less round in appearance on x-rays. This phase can last from several months up to one year.

Phase 2 – The body cleans up the dead bone cells and replaces them with new, healthier bone cells. The femoral head begins to remodel into a round shape again. The joint is still irritated and painful. This phase can last from one to three years.

Phase 3 – The femoral head continues to model itself back into a round shape with new bone. This phase lasts for one to three years.

Phase 4 – Normal bone cells replace the new bone cells. This last phase can last a few years to complete the healing process.

The cause of Legg-Calvé-Perthes disease is unknown. It is four times more likely to occur in boys than girls.

Legg-Calvé-Perthes disease commonly affects first-born children and is typically seen in children 4 to 8 years of age. The majority of cases affect only one hip.

The child typically complains of pain in his/her hip that is aggravated by activity. Sometimes, they will also experience pain in their thigh or knee area. The child usually walks with a limp and reports that rest will alleviate the pain.

The symptoms of Legg-Calvé-Perthes disease may resemble other conditions or medical problems of the hip. Always consult your child’s physician for a diagnosis.

In addition to a complete medical history and physical examination, diagnostic procedures for Legg-Calvé-Perthes disease may include:

  • x-rays – a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
  • bone scans – a nuclear imaging method to evaluate any degenerative and/or arthritic changes in the joints; to detect bone diseases and tumors; to determine the cause of bone pain or inflammation.
  • magnetic resonance imaging (MRI) – a diagnostic procedure that uses a combination of large magnets, radio frequencies, and a computer to produce detailed images of organs and structures within the body.
  • arthrograms – a diagnostic imaging test to study the non-bony structures of joints.
    blood tests

Specific treatment for Legg-Calvé-Perthes disease will be determined by your child’s physician based on:

  • your child’s age, overall health, and medical history
  • the extent of the condition
  • your child’s tolerance for specific medications, procedures, or therapies
  • expectations for the course of the condition
  • your opinion or preference

The goal of treatment is to preserve the roundness of the femoral head and to prevent deformity while the condition runs its course. Treatment options are dependent upon the amount of hip pain, stiffness, and x-ray changes over time, as well as how much of the femoral head has collapsed.

Typically, the first step of treatment is to regain hip motion and eliminate pain that results from the tight muscles around the hip and the inflammation inside the joint.

Treatment may include:

  • rest
  • activity restrictions
  • medications
  • bed rest and traction
  • casting or bracing (to hold the femoral head in the hip socket, permit limited joint movement, and allow the femur to remold itself into a round shape again)
  • surgery (to hold the femoral head in the hip socket)
  • physical therapy (to keep the hip muscles strong and to promote hip movement)
    crutches or wheelchair (in some cases)

The ultimate goal in Legg-Calvé-Perthes disease is to diagnose the condition early in order to allow as much time as possible to let the femoral head remodel back into a round shape. Other treatment goals include controlling pain, maintaining hip motion, and preventing continued hip deformity.

The two most critical factors that determine the outcome are the child’s age and how much of the femoral head is affected by this condition.

The more severe the case, the greater the likelihood that the child may experience limited hip motion, differences in leg lengths, and further hip problems in adulthood.


Anorectal Malformation

Posted by on Tuesday, 1 June, 2010

Anorectal malformations are birth defects (problems that happen as a fetus is developing during pregnancy). With this defect, the anus and rectum (the lower end of the digestive tract) do not develop properly. “Ano” refers to the anus (the opening at the end of the large intestine through which stool passes when a baby has a bowel movement). “Rectal” refers to the rectum (the area of the large intestine just above the anus)

anorectal malformation

During a bowel movement, stool passes from the large intestine to the rectum and then to the anus. Muscles in the anal area help to control when a bowel movement occurs. Nerves in the area help the muscles sense the need for a bowel movement and also stimulate muscle activity.

With an anorectal malformation, several abnormalities can occur, including the following:

  • The anal passage may be narrow.
  • A membrane may be present over the anal opening
  • The rectum may not connect to the anus
  • The rectum may connect to part of the urinary tract or the reproductive system through a passage called a fistula.

The treatment for the malformation depends on which type of abnormality is present.
As a fetus is growing in its mother’s uterus before birth, different organ systems are developing and maturing. The lower end of the intestinal tract forms fairly early in pregnancy.

In a fetus, the lower portion of the large intestine and the urinary tract start off as one large mass of cells. Certain steps have to take place in the 7th to 10th weeks of gestation for the rectum and anus to separate from the urinary tract and form properly. Sometimes, these steps do not occur as they should, and the rectum and/or anus may not develop normally. Nothing that the mother did during pregnancy can be shown to have caused the malformation.

Most of the time, the cause for an anorectal malformation is unknown. Rarely, autosomal recessive inheritance has been observed in some studies. This means that each parent unknowingly carries a gene for the condition, and the baby receives both copies of the gene. Couples who are gene carriers have a one in four or 25 percent chance for the malformation to reoccur in a future pregnancy. Up to one third of babies who have genetic syndromes, chromosomal abnormalities, or other congenital defects also have anorectal malformations.

Anorectal malformations occur in about 1 in 5,000 live births. Boys are at a slightly higher risk for this abnormality than girls.

Anorectal malformations cause abnormalities in the way a baby has a bowel movement. These problems will vary depending on the type of malformation.

When the anal passage is narrow, a baby may have a difficult time passing a bowel movement, causing constipation and possibly discomfort. If there is a membrane over the anal opening, the baby may be unable to have a bowel movement.

“Ano” refers to the anus (the opening at the end of the large intestine through which stool passes when a baby has a bowel movement)

“Rectal” refers to the rectum (the area of the large intestine just above the anus)

When the rectum is not connected to the anus but there is a fistula present, stool will leave the baby’s body through the fistula instead of the anus. This can cause infections. If the rectum is not connected to the anus and there is not a fistula present, there is no way for stool to leave the intestine. The baby will be unable to have a bowel movement.

Your baby’s physician will perform a physical examination when your baby is born, and will look at the anus to see if it is open. Diagnostic imaging tests may be performed to further evaluate the problem, including:

  1. Abdominal x-rays – diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
  2. Abdominal ultrasound (Also called sonography.) – a diagnostic imaging technique which uses high-frequency sound waves and a computer to create images of blood vessels, tissues, and organs. (Ultrasounds are used to view internal organs as they function, and to assess blood flow through various vessels.)
  3. Computed tomography scan (Also called a CT or CAT scan.) – a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.

 

The treatment of an anorectal malformation may depend on the following:

  • The extent of the problem
  • The overall health of the baby and medical history.
  • The opinion of the physicians involved in the baby’s care
  • Expectations for the course of the disease
  • Your opinion and preference

The majority of babies with an anorectal malformation will need to have surgery to correct the problem. The type and number of operations necessary depends on the type of abnormality the baby has, including the following:

anus repair

  1. Narrow anal passage
Babies who have the type of malformation that causes the anal passage to be narrow may not need an operation. A procedure known as anal dilatation may need to be performed periodically to help stretch the anal muscles so stool can pass through.
  2. Anal membrane
Babies with this type of malformation will have the membrane removed in surgery. Anal dilatations may need to be performed to help with any narrowing of the anal passage that is present.
  3. Lack of rectal/anal connection, with or without a fistula
These babies will need a series of operations in order to have the malformation repaired.

Toilet training should be started at the usual age, which is generally when the child is between two and three years old. However, children who have had anorectal malformations repaired may be slower than others to gain bowel control. Some children may not be able to gain good control over their bowel movements, while others may be chronically constipated, depending on the type of malformation and its repair. Your child’s physician can explain the outlook for your child.

Children who had the type of malformation that involves an anal membrane or a narrow anal passage are usually able to gain good control over their bowel movements after repair of an anorectal malformation. Children with more complex variations of an anorectal malformation may have need to participate in a bowel management program in order to help them have control over their bowel movements and prevent constipation. The nurses and other healthcare professionals that work with your child’s physicians can help tailor a program to your child’s needs.