Treatments for Marfan syndrome
Treatments for Marfan syndrome
The list of treatments mentioned in various sources
for Marfan syndrome
includes the following list.
Always seek professional medical advice about any treatment
or change in treatment plans.
Marfan syndrome: Is the Diagnosis Correct?
The first step in getting correct treatment is
to get a correct diagnosis.
Differential diagnosis list for Marfan syndrome may include:
Marfan syndrome: Research Doctors & Specialists
Research all specialists including ratings, affiliations, and sanctions.
Latest treatments for Marfan syndrome:
The following are some of the latest treatments for Marfan syndrome:
Discussion of treatments for Marfan syndrome:
Genes and Disease by the National Center for Biotechnology (Excerpt)
Beta blockers have been used to control some of the cardiovascular symptoms of Marfan syndrome; however, they are not effective against the skeletal and ocular problems, which can also be serious.
(Source: Genes and Disease by the National Center for Biotechnology)
Questions and Answers about Marfan Syndrome: NIAMS (Excerpt)
There is no cure for Marfan syndrome. To develop one,
scientists may have to identify and change the specific gene responsible
for the disorder before birth. However, a range of treatment options can
minimize and sometimes prevent complications. The appropriate
specialists will develop an individualized treatment program; the
approach the doctor uses depends on which systems have been
affected.
- Skeletal--Annual evaluations are important to detect any
changes in the spine or sternum. This is particularly important in
times of rapid growth, such as adolescence. A serious deformity can
not only be disfiguring but can also prevent the heart and lungs from
functioning properly. In some cases, an orthopedic brace or surgery
may be recommended to limit damage and disfigurement.
- Eyes--Early, regular eye examinations are key to catching
and correcting any vision problems associated with Marfan syndrome. In
most cases, eyeglasses or contact lenses can correct the problem,
although surgery may be necessary in some cases.
- Heart and blood vessels--Regular checkups and
echocardiograms help the doctor evaluate the size of the aorta and the
way the heart is working. The earlier a potential problem is
identified and treated, the lower the risk of life-threatening
complications. Those with heart problems are encouraged to wear a
medical alert bracelet and to go to the emergency room if they
experience chest, back, or abdominal pain. Some heart valve problems
can be managed with drugs such as beta-blockers, which may help
decrease stress on the aorta. In other cases, surgery to replace a
valve or repair the aorta may be necessary. Surgery should be
performed before the aorta reaches a size that puts it at high risk
for tear or rupture. Following heart surgery, extreme care must be
followed to prevent endocarditis (inflammation of the lining of the
heart cavity and valves). Dentists should be alerted to this risk;
they are likely to recommend that the patient be prescribed protective
medicines before they perform dental work.
- Nervous system--If dural ectasia (swelling of the covering
of the spinal cord) develops, medication may help minimize any
associated pain.
- Lungs--It is especially important that people with Marfan
syndrome not smoke, as they are already at increased risk for lung
damage. Any problems with breathing during sleep should be assessed by
a doctor.
Pregnancy poses a particular concern due to the stress on
the body, particularly the heart. A pregnancy should be undertaken only
under conditions specified by obstetricians and other specialists
familiar with Marfan syndrome and be followed as a high-risk
condition.
While eating a balanced diet is important for maintaining
a healthy lifestyle, no vitamin or dietary supplement has been shown to
help slow, cure, or prevent Marfan syndrome.
(Source: excerpt from Questions and Answers about Marfan Syndrome: NIAMS)
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Book Excerpts: Treatment of Marfan syndrome
Treatments of Marfan syndrome: Online Medical Books
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for more information about the treatments of Marfan syndrome.
Scoliosis & Kyphosis:
Treatment
(In a Page: Signs and Symptoms)
- Scoliosis
–Treat underlying cause if applicable (e.g., tumor)
–<20–25° of deformity: Observation
–20–40° of deformity: Bracing (preferably to be worn 23 hours/day); bracing stops progression only; Milwaukee brace (includes neck ring) gives best results but poor compliance; lumbosacral orthosis (Boston brace) has poorer results but better compliance
–>40° of deformity: Surgery (posterior spinal fusion with rods) is usually indicated; progression is very likely
–More aggressive treatment is usually indicated if progression >5°, female, younger, or if secondary, treatment generally more aggressive - Kyphosis: Bracing or surgery, similar to scoliosis
>
>
» READ BOOK EXCERPT ONLINE »
Source: In a Page: Signs and Symptoms, 2004
Scoliosis:
Treatment
(In A Page: Pediatric Signs and Symptoms)
-
Treatment options include observation while child is growing, bracing, and surgery
–Many curves do not progress enough to require treatment
–Spinal curve progression may occur despite bracing; however, for idiopathic scoliosis, response to brace wearing is dose-related and many patients do not like to wear the brace
-
Exercise and electrical stimulation have not been shown to alter natural progression of curve
-
Bracing for curves in 20–40° degree range may slow curve progression but does not reduce the magnitude of curve despite a well-made brace and compliance
-
Surgery is reserved for progressive curves >40° in skeletally immature (Risser scale 0–1) and >50° in skeletally mature patients
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Source: In A Page: Pediatric Signs and Symptoms, 2007
Asymmetric Limbs:
Treatment
(In A Page: Pediatric Signs and Symptoms)
-
Since underlying cause of an asymmetric limb is very broad, treatment varies depending on the etiology
-
In certain cases, especially in those in which specific treatment would make a difference (DDH, SCFE, infection, inflammation malignancy), diagnosis must be made as soon as possible to prevent permanent damage
-
Consider surgical (shortening or lengthening procedures, prostheses) and nonsurgical (orthotics, pressure stockings)
-
Angular deformity should be looked at carefully and corrected before or at the same time of length equalization to avoid joint dislocation
» READ BOOK EXCERPT ONLINE »
Source: In A Page: Pediatric Signs and Symptoms, 2007
Marfan syndrome:
Treatment
(Professional Guide to Diseases (Eighth Edition))
Attempts to stop the degenerative process have met with little success. Therefore, treatment of Marfan syndrome is basically aimed at relieving symptoms — for example, surgical repair of aneurysms and ocular deformities. In young patients with early dilation of the aorta, prompt treatment with beta-adrenergic blockers may decrease ventricular ejection and protect the aorta; extreme dilation requires surgical replacement of the aorta and the aortic valve. Steroids and sex hormones have been successful (especially in girls) in inducing precocious puberty and early epiphyseal closure to prevent abnormal adult height. Genetic counseling is important, particularly because pregnancy and resultant increased cardiovascular workload can produce aortic rupture.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
Scoliosis:
Treatment
(Professional Guide to Diseases (Eighth Edition))
Only two treatments effectively treat scoliosis: spinal bracing and surgery. If monitored closely, a properly constructed and fitted brace can successfully halt progression of a curve in approximately 70% of cooperative patients. Most braces should be worn over a long T-shirt or similar article of clothing for 23 hours a day. However, mild curvatures may require less. Exercises must be done daily both in and out of the brace to maintain muscle strength. Patients should be seen for follow-up and brace adjustment every 3 months. Radiographs should be repeated at 6-month intervals. As the skeleton matures, as seen radiographically, brace wear should be gradually decreased until it’s worn only at night.
The primary indications for surgery are relentless curve progression (usually curves over 40°) or significant curve progression despite bracing. Surgery corrects lateral curvature by posterior spinal fusion and internal stabilization with metal rods. A distraction rod on the concave side of the curve “jacks” the spine into a straight position and provides an internal splint. An alternative procedure, anterior spinal fusion, corrects curvature with vertebral staples and an anterior stabilizing cable. Some spinal fusions may require postoperative immobilization in a brace. Postoperatively, periodic checkups are required for several months to monitor stability of the correction.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
Scoliosis:
Treatment
(Handbook of Diseases)
The severity of the deformity and potential spine growth determine appropriate treatment, which may include such noninvasive measures as close observation, exercise, or a brace. For more serious deformity, surgery or a combination of methods may be needed. To be most effective, treatment should begin early, when spinal deformity is still subtle.
Noninvasive measures
A curve of less than 25 degrees is mild and can be monitored by X-rays and an examination every 3 months. An exercise program that includes sit-ups, pelvic tilts, spine hyperextension, push-ups, and breathing exercises may strengthen torso muscles and prevent curve progression. A heel lift also may help.
A curve of 25 to 39 degrees requires management with spinal exercises and a brace. (Transcutaneous electrical nerve stimulation may be used as an alternative.)
A brace halts progression in most patients but doesn’t reverse the established curvature. Such devices passively strengthen the patient’s spine by applying asymmetric pressure to skin, muscles, and ribs. Braces can be adjusted as the patient grows and can be worn until bone growth is complete.
Surgery
A curve of 40 degrees or more requires surgery (spinal fusion with instrumentation) because a lateral curve continues to progress at the rate of 1 degree a year even after skeletal maturity.
CLINICAL TIP: Some surgeons may prescribe Cotrel dynamic traction for 7 to 10 days for preoperative preparation. This traction consists of a belt-pulley-weight system. While in traction, the patient should exercise for 10 minutes every hour, increasing muscle strength while keeping the vertebral column immobile.
Surgery corrects lateral curvature by posterior spinal fusion and internal stabilization with a Harrington rod or other fixation devices. A distraction rod on the concave side of the curve “jacks” the spine into a straight position and provides an internal splint.
An alternative procedure, anterior spinal fusion with instrumentation, corrects curvature with vertebral staples and an anterior stabilizing cable. Some spinal fusions may require postoperative immobilization in a brace.
Postoperatively, periodic checkups are required for several months to monitor stability of the correction.
» READ BOOK EXCERPT ONLINE »
Source: Handbook of Diseases, 2003
Salivation, increased:
Patient counseling
(Signs & Symptoms: A 2-in-1 Reference for Nurses)
Teach the patient the importance of proper oral hygiene to prevent odor and dental problems. Remind him to seek regular dental care.
» READ BOOK EXCERPT ONLINE »
Source: Signs & Symptoms: A 2-in-1 Reference for Nurses, 2007
Know how to recognize and manage scoliosis because an early diagnosis and treatment are especiallyimportant to prevent serious consequences:
Treatment
(Avoiding Common Pediatric Errors)
The American Academy of Pediatrics recommends that infants, children,
and adolescents with severe scoliosis be referred to a pediatric orthopedic surgeon. The orthopedist will conduct a standing anteroposterior
roentgenogram of the spine to confirm the diagnosis. Treatment recommendations will depend on the degree, flexibility, and location of the curve.
Current guidelines are to follow closely every 3 to 6 months with spinal
radiographs and photographs for <15-to 20-degree curvature; to use a
Milwaukee brace for between 20 and 40 degrees; bracing or surgery between
40 and 50 degrees; and spinal fusion for >50 degrees.
>
» READ BOOK EXCERPT ONLINE »
Source: Avoiding Common Pediatric Errors, 2008
Teething:
Teething - TREATMENT
(The 5-Minute Pediatric Consult)
- Application of cold/frozen objects locally onto the gums: Many find that cold objects work well, but care must be taken because direct contact with a frozen object may result in local irritation.
- Objects for chewing: Choking hazards, such as raw carrots, must be avoided.
- Teething rings should not be placed around the child’s neck, as they represent a strangulation hazard. Teething rings made prior to 1998 should be discarded as they might contain diisonoyl phthalate, a softening agent now thought to be toxic.
- Acetaminophen (15 mg/kg PO q4h) or ibuprofen (10 mg/kg PO q6h) may be used for pain relief as needed, but should not be given round-the-clock to mask fever.
- Home remedies or treatments given by parents:
- Most over-the-counter preparations marketed for the relief of teething symptoms contain 7.5–10% benzocaine as the active ingredient. Excessive use of benzocaine preparations has been associated with methemoglobinemia.
- Homeopathic remedies may contain a variety of ingredients including belladonna alkaloids, chamomile, and ground coffee. Depending on the size of the child and the amount of medication or herb ingested, toxicity is possible.
- Remedies that have been used in the past and are no longer recommended include: Alcoholic liquors, paregoric, 2% lidocaine solution (excess may result in seizures), lancing the gums, and rubbing the gums with a thimble until the tooth breaks through the gum.
» READ BOOK EXCERPT ONLINE »
Source: The 5-Minute Pediatric Consult, 2008
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