marfan patient with pectus excavatum

Pectus Excavatum and Marfan Syndrome

The inherited anterior chest wall deformities pectus excavatum and pectus carinatum occur in a lot of people worldwide.

Scientific data proves that 70% of all patients with Marfan syndrome (MFS) have pectus excavatum.

The severity of the dent in their chest can worsen with age. This negatively affects patients, especially on a psychological level.

In this article, I will write about scientifically proven ways that are effective to repair the deformity in patients with Marfan syndrome.

What is Marfan Syndrome?

child with mfs and pectus excavatum

Marfan syndrome is a hereditary disease of the connective tissue.

How intensely the patients are affected varies.

It is shown that Marfan syndrome is created with a mutation in the Fibrillin 1 (FBN1), which is a gene that produces fibrillin (glycoprotein that helps the development of elastic fibers located in the connective tissue).

The mutation leads to a connective tissue disorder.

As of February 2020, there is still no known cure for the Marfan syndrome.

However, with proper treatment, the life expectancy of these patients can be normal.

This means that the pectus excavatum deformity must be corrected for the patients to reach optimal health.

It is proven to lower the lifespan for about ten years if left untreated.

The deformity will impair the functioning of the heart and lungs.

Adults and children with MFS should avoid competitive and contact sports.

They also shouldn't perform any heavy lifting exercises.

The extra high blood pressure and rapid heartbeat will hurt their aorta.

Comparatively, light exercises performed in moderation will be suitable for these patients.

They are shown to lower their blood pressure and heart rate.

KEY POINT:

Marfan syndrome is a connective tissue disorder that can shorten lifespan for about 10 years if untreated. There isn't a known cure for this.

Pectus Excavatum is a Major Symptom of MFS

pectus excavatum symptoms marfan syndrome

According to RareDiseases.com, concave chest deformity is one of the few major symptoms of Marfan syndrome. 

The other signs include:

  • Excessive growth of the bones in the legs and arms.
  • Sideways curving of the spine (scoliosis). Usually, the curve pattern is in an "S" or "C" shape.
  • Eye lenses displacement (ectopia lentis).
  • Nearsightedness (myopia). You can see things close to you, but the distant objects are blurry.
  • A weakening of the artery wall which leads to abnormal expansion. This is also known as aneurysm.
  • Tear in the main artery (aorta), that carries blood away from the heart.
  • Mitral valve prolapse, which is a condition where the two valve flaps of the mitral valve don't close regularly. Instead, they protrude up into the left atrium.
  • Backward blood flow through the aortic and mitral valves, every time the left ventricle contracts (aortic and mitral regurgitation).

Is Nuss Procedure Safe for Marfan Patients?

Studies have shown that the Nuss procedure is safe for patients with Marfan syndrome.

Generally, Marfan patients have a more severe deformity case compared to those who don't suffer from Marfan syndrome.

Because of that, they need two or more Nuss bars to be inserted underneath their breastbone for a successful correction.

Investigations revealed that this increases infection rates.

Nevertheless, infections aren't a major concern.

Scientists discovered that Marfan patients with pectus excavatum should postpone the surgery until their skeletal system development is finished.

That is because the chest of the younger Marfan patients isn't stable after the operation.

There is a high chance that the chest will fall back after the Nuss bars are removed.

Research showed the Pectus bars removal surgery should be performed three years after the first surgery.

The bar removal procedure will require no more than 20 minutes in most patients.

KEY POINT:

Nuss procedure is a safe way to surgically correct pectus excavatum in Marfan patients.

Non-Surgical Pex Correction for Marfan

Isaiah Austin is a basketball player that has been diagnosed with Marfan syndrome.

He is a 7'1 NBA Draft Prospect with a 7'5 wingspan.

He is a classic example of a patient with MFS who managed to build a fantastic looking, and functional body, even with a connective tissue disorder.

The non-operative treatment of inverted chest for patients with Marfan syndrome is mostly based upon the usage of the vacuum bell device and wearing posture corsets.

This device is placed over the dent's deepest point and provides suction to lift the sternum to a normal position.

Patients need to wear it for about two hours a day, for about a year.

The use of the device yields amazing improvements in repairing the chest wall abnormality in Marfan patients. Scientific facts support this.

Making pectus excavatum less visible by performing bodybuilding workouts with heavy weights is not recommended for MFS patients.

I'd recommend a program where the sufferer will perform the compound exercises in the 20-30 rep range for two sets, with 2 minutes rest between sets.

After a couple of months, the patient will see significant bodily improvements, particularly in the chest area, which will greatly improve the patient's confidence.

KEY POINT:

Vacuum bell device therapy and improving posture with bracing can help Marfan patients repair their deformity.

Quality of Life of Marfan Patient with Pectus Excavatum

If you have Marfan connective tissue syndrome and suffer from the concave chest deformity, you must improve the quality of most common life issues.

The biggest problem that I regularly noticed in MFS Facebook groups was thinness and being underweighted.

Being underweight as a patient with genetic connective tissue disorder and pectus excavatum will further exaggerate your lack of physical strength and endurance.

This will be more evident while you're working out in the gym. You won't have any fat storage that is needed to release energy.

There is no way you can build muscle this way.

You need extra calories, fat, and protein if you want to bulk up and make the dent in your anterior chest wall less noticeable.

Also, you will do more harm than good if you're trying to work out malnourished. You'll feel lightheaded, dizzy, and can even faint.

A drastic decrease in calorie intake and doing intense workouts will lead to starvation and illness.

In many cases, this can get to the point of being malnourished.

This occurs when the Marfan patient doesn't get the required nutrients.

These patients consume insufficient amounts of food because of a lack of appetite.

According to MedicalNewsToday, this can lead to health issues, sicknesses, slow injury recovery times, increased risks of infections, and lack of focus.

Calculate BMI

I highly recommend you to calculate your Body Mass Index (BMI). This is a value received from the height and weight of the individual.

It tells whether you are malnourished, underweight, overweight, or whether you're in a healthy weight range.

I stumbled upon a concerned mother that entered her daughter's BMI into an online calculator. She was suffering from Marfan syndrome.

The message she received back was shocking.

It said that she needed to immediately bring her daughter to the emergency room because she was severely malnourished.

KEY POINT:

The combination of Marfan syndrome and pectus excavatum can negatively affect the patients and lower their life quality.

Gaining Weight with Marfan Syndrome and Pectus Excavatum

Most Marfan syndrome patients with pectus excavatum have trouble obtaining weight, particularly when they're younger.

Usually, they are underweight, tall, thin, with long slender arms, legs, fingers, and toes.

If you add scoliosis and flexible joints to this mix, you'll see an awkward-looking body that causes psychological discomfort in these patients.

Because of this, gaining weight would be an amazing way to make their bodies look more natural.

I stumbled upon a few Marfan patients with a concave chest that complained about not being able to gain weight. 

Regardless of how many protein shakes and weight gainer supplements they consumed, they still couldn't get their weight up.

They convinced me that they ate a lot, but still had trouble bulking.

I advised them to track their daily calorie intake.

After they did that, they noticed that they weren't eating as much quality food as they thought.

That was their biggest problem.

Stay Away from Junk Food

The nutritionists and the Gastroenterologists usually advise Marfan patients with an inverted chest to eat anything they want.

They say the extra calories will be very helpful in the weight gain process.

However, this makes patients fall in love with junk food.

Salt and sugar becomes their best friend, and greens and veggies their biggest enemy.

I would advise the Marfan syndrome patients with funnel chest to not eat any junk food just to gain weight.

That's a faster way to bulk up, but it will harm your health.

Marfan Patients Gain Weight in Their Stomach

Also, the majority of Marfan patients accumulate a lot of weight in their stomach.

Eating junk food will only help you obtain mass in this area.

However, some Marfan patients still can't gain weight.

Doctors still don't know the exact cause of this.

The sunken chest is a lot more noticeable when you're underweight.

Some speculate that the undeveloped muscles of Marfan patients can be related to that.

If your doctor clears you to go to the gym and lift weights, this diet will also promote muscle growth, which will make your bodily appearance more attractive.

Nevertheless, in most cases, it is not recommended for Marfan patients to participate in bodybuilding and lifting heavyweights.

In some cases, it can lead to aortic enlargement, a life-threatening problem that can shorten lifespan.

See what Alan C. Braverman, an M.D., Professor of Medicine, and has a clinical interest in MFS, has to say about weightlifting.

Talk With a Nutritionist or Dietician

Just to be safe, I would recommend you talk to a nutritionist or dietician.

Then, consult with a specialist to make sure any foods or supplements don't conflict with some medications you're getting.

Your health and well-being are much more important than a few pounds that you can gain with a diet that may harm your health.

If you want to learn more about proper nutrition and weight gain as a Marfan syndrome patient, I recommend you check this article written by Hien Nguyen-Le, a nutrition therapist specializing in treating eating disorders for over 20 years.

KEY POINT:

Gaining weight as a Marfan patient is very hard. Stay away from junk food and eat quality nutrient foods. Speak with a nutrition for a custom diet to follow.

Nuss Procedure Will Lower Weight

Weight gain will become more problematic when the Marfan patient undergoes a pectoralis excavatum operation.

Even though the Nuss and Ravitch procedures are categorized as minimally invasive, the recovery process in Marfan patients is slower than those without the syndrome.

As mentioned earlier, they need two or more Pectus bars to be inserted beneath their concave breastbone to correct the deformity successfully.

This makes the Nuss procedure very challenging to heal.

Marfan patients with funnel breast should expect to lose five pounds after the surgery.

Years after that, they will have trouble gaining even a few pounds.

Also, an additional surgical procedure is required to remove the Nuss bar out of the patient's chest.

Expect to lose a few pounds, a couple of months after this secondary operation.

If the Marfan patient must undergo a pectus excavatum surgery, please visit the nutritionist or Gastroenterologist a few times after the surgery.

The doctor will calculate your BMI and tell you whether you need more nutrition or not.

KEY POINT:

The Nuss surgery is proven to additionally lower the weight of the sufferer.

Heart Surgery & Nuss Procedure Done Concurrently in MFS Patients

There was a debate on whether the Nuss procedure and an open cardiac surgery should be performed concurrently in pectus excavatum patients with congenital MFS.

However, a recent 2020 research published in January, proves that the single-stage procedure is very safe and effective.

There are seven major advantages of the single-stage procedure in Marfan patients.

  • It doesn't require extra surgery, resulting in extra blood loss.
  • Both surgeries are done with one single general anesthesia, which lowers the dangers related to an additional anesthetic procedure.
  • Lowered number of surgical injuries and postoperative aches.
  • Reduced costs because there will be only one hospitalization, instead of two.
  • Instant improvement in blood flow and respiratory system functioning, as a result of internal organ decompression after lifting the sternum.
  • Lowered amount of opioid medication intake.
  • Instant cosmetic improvement, which will improve the Marfan patient's mental and emotional well-being.

Conclusion

If you suffer from Marfan syndrome and pectus excavatum, or you are a parent of a kid that experiences this, it is very important to be knowledgeable about the subject. 

In this article, I covered everything you need to know about the effectiveness of the Nuss procedure on Marfan patients, how to gain weight, improve the quality of life and much more.

Even though everything in the article is backed up by science, it is critically important to visit the doctor. 

You can discuss with the doctor about the syndrome, and whether the anterior chest wall dent is severe enough to require surgery. 

The information I've written in this article is not a substitute for professional medical advice, examination, or therapy.

The MFS causes cardiovascular complications, involving the heart and blood vessels.  

If you add a severe pectus excavatum in the mix, you'll also get respiratory and cardiac problems, which will drastically decrease the patient's stamina and exercising capacities.

Please seek medical treatment if you're having a few of the Marfan symptoms I mentioned above in the article.

If you have some questions or concerns, please post them in the comment segment underneath, and I'll do my best to answer them.

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