What is Thoracic Dystrophy?

There is not a lot of information out there about platythorax, so I am quoting two different people from a support group with their permission of course. I will update this section as I find more documented sources.


"Platythorax is a form of pectus excavatum that has a wide, flattened appearance. Whereas standard pectus excavatum is recognized by an often deep indentation at the sternal area, platythorax has a more shallow, wide indentation, or hardly any visible indentation at all. In platythorax, the indentation is not limited to the sternal area, but includes wider portions of the chest, sometimes both the upper and lower portions, and can affect either one side of the ribcage or both.


Because of the visual differences between standard pectus excavatum and the flattened appearance of platythorax, it is common for doctors and others to visually diagnose platythorax as "mild" or even "normal." However, pectus excavatum can present itself in many forms, and the depth or lack of a visual dent may not determine the severity of the pectus or its symptoms. 


Platythorax may lead to a greater degree of heart and lung compression than that in a patient with standard pectus excavatum having a similar Haller Index. A larger portion of the chest may be pressing inward, giving the organs less room to shift to either side of the spine. Platythorax pectus excavatum may be visible at birth, or may develop later. Some individuals have been born with standard pectus excavatum that flattened into platythorax later in life either naturally or following failed surgeries.


How do you know if you have platythorax pectus excavatum? Here are some common considerations:

  1. Either a small sternal dent or none at all, while still experiencing other signs or symptoms of pectus excavatum.
  2. The ribs bend inwardly at a sharper angle at the sides than what it normal.
  3. Excessive flaring of the ribs with flatness above them.
  4. Often, a general sloping appearance of the front of the chest. The chest may slope inward just below the collarbone, and then slope back out at the lower ribs, often creating a "potbellied" appearance.
  5. Poor posture that includes a forward rolling of the shoulders.
  6. In females, poorly fitting bras and clothing that droops in the front. "

"Pectus excavatum (a Latin term meaning hollowed chest) is the most common congenital deformity of the anterior wall of the chest, in which several ribs and the sternum grow abnormally. This produces a caved-in or sunken appearance of the chest. It can either be present at birth or not develop until puberty.

Pectus excavatum is sometimes considered to be cosmetic; however, depending on the severity, it can impair cardiac and respiratory function and cause pain in the chest and back. People with the condition may experience negative psychosocial effects, and avoid activities that expose the chest.Pectus excavatum is sometimes referred to as cobbler's chest, sunken chest, the crevasse, funnel chest or "as having a dent in one's chest"

before surgery

The following images are from Melissa Leslie. Here is an example where Pectus Excavatum turned into platythorax as her chest collapsed after a botched surgery. Her chest continued to collapse into very severe PE. Melissa received two bars from Dr. Jaroszewski in January 2017 and is currently recovering and doing well.

Here I will try to present just the facts and cite them directly from their source. If you have any addition information or sources please email me at PEWilliamsJourney@gmail.com.


after the Nuss Procedure

Melissa Leslie was also kind enough to share her thoughts about platythorax - "I've had both kinds of PE and I found platythorax to be extremely painful, especially at the sharp edges of what I called the B shape. My bones felt like a sharp angle to me and then it went flat across my chest. It was especially painful in those two sharp sections before it flattened across my chest. The rounded part of the B would obviously be my back. Some people could have platythorax, but then also regular PE in a different section. It's way less common, and doctors often dismiss it because they don't even fully understand it, unless they are well trained in the area."

Information

Haller Index vs. Correction Index

"The Haller index has been classically used as a measure of the severity of the pectus deformity, where a HI >3.25 is considered an indication for consideration for surgical correction.


An innate fault of this measurement, as St Peter et al pointed out in their paper, is the fact that this number relies heavily on the cross sectional diameter of the chest (measurement a), which varies between patients. They noted that when they measured the HI in patients with a pectus deformity, there was a 45% cross-over with normal patients. Essentially, the authors suggest that the HI is not a reliable measure of severity of a pectus deformity."

"Pectus carinatum is an uncommon birth defect in which a child's breastbone protrudes outward abnormally. Sometimes the deformity isn't noticeable until after the adolescent growth spurt.

For most children and teens, the main issue with pectus carinatum is the way it looks. However, some will also have problems with shortness of breath, especially during exercise.

While surgical repair is an option for people with severe pectus carinatum, the use of a brace to help flatten the chest is the preferred treatment for children whose bones are still growing. The brace is worn up to 23 hours a day, and results can begin to be seen in just a few months."

after the Nuss Procedure

before surgery

"Pectus excavatum is a condition in which a person's breastbone is sunken into his or her chest. In severe cases, pectus excavatum can look as if the center of the chest has been scooped out, leaving a deep dent.

While the sunken breastbone is often noticeable shortly after birth, the severity of pectus excavatum typically worsens during the adolescent growth spurt.

Also called funnel chest, pectus excavatum is more common in boys than in girls. Severe cases of pectus excavatum can eventually interfere with the function of the heart and lungs. But even mild cases of pectus excavatum can make children feel self-conscious about their appearance. Surgery can correct the deformity."

"As an alternative, the authors recommend using a measurement they referred to as the correction index. Simply put, CI is the ratio of the anticipated rise in the sternal defect after bar placement (measurement a-b), to the maximal anterior to posterior dimension of the inner chest, multiplied by 100.

The question then becomes, what CI should be used as an indication for surgery?  The authors suggested that a 10% or higher CI reflects a pectus deformity severe enough to warrant correction."

What is Pectus Carinatum?

A "normal"  CT scan

platythorax after botched surgery

What is Pectus Excavatum?

very severe pectus excavatum

My CT scan with a 3.7 Haller Index

It is also known as "Jeune syndrome" and is a rare genetic disorder that affects the way a child’s cartilage and bones develop. It begins before the child is born. Thoracic Dystrophy affects the child's rib cage, pelvis, arms and legs. Usually, problems with the rib cage cause the most serious health problems for children with Jeune syndrome. Their rib cages (thorax) are smaller and narrower than usual. This can keep the child's lungs from developing fully or expanding when the child inhales. The child may breathe rapidly and shallowly. They may have trouble breathing when they have an upper or lower respiratory infection, like pneumonia.

Breathing trouble can range from mild to severe. In some children, it is not noticeable, aside from fast breathing. In most children, breathing problems are serious. About 60% to 70% of children with this condition die from respiratory failure as babies or young children.

Children with Thoracic Dystrophy who survive often develop problems with their kidneys, another serious feature of Jeune syndrome. Over time they may experience renal failure. As a result, few children with Jeune syndrome live into their teen years. Children with Jeune syndrome have a form of dwarfism. They are short in stature, and their arms and legs are shorter than most people’s.

Yet, another name for Jeune syndrome is Asphyxiating Thoracic Dystrophy. This diagnosis is grouped with other chest problems called thoracic insufficiency syndrome (TIS).

"Its a type of pectus where the entire thorax is generally flattened - make a heart shape with your hands - that is like a cross-section of a regular PE. A platythorax is more like a flattened oval. there is usually a depression too, but it is sometimes called 'mild' pectus, even though the Haller can be pretty bad, because it doesn't look dramatic.


I also thought that a regular chest would look like you were holding a really big burger, and a platythorax would be more like holding a panini."

What is Platythorax?