In-Depth

How does a child get a pectus excavatum?

A pectus excavatum is caused by an overgrowth of cartilage during chest wall development before birth. These cartilages are extra long and push the sternum backward.

Pectus excavatum is not always noticed at birth. It is usually apparent by the time a child is 2 to 3.

o It can become more severe during later childhood and progress further as your child goes through puberty.

It is four times more common in boys than girls and occurs more often in families where one member has the abnormality.

Pectus excavatum is associated with other muscle and bone abnormalities, particularly scoliosis (15 percent of cases).

What causes a pectus excavatum?

We don't know. Some studies investigating a genetic component are underway. Although the majority of cases don't involve a family history, there are many that do&emdash;enough to warrant the suspicion that genes play a significant role. The genetic story is likely to be complex, though.

What are the symptoms of pectus excavatum?In infancy, symptoms of funnel chest can include:

frequent lingering colds that often develop into pneumonia a hollow depression in the chest that may be broad and shallow, deep and narrow, or

assymetric

difficulty breathing

In older children, symptoms of funnel chest can include:

breathing difficulty upon exertion or exercise chest pain

frequent respiratory infections

a lateral curvature of the spine, absence of the curve of the upper back, hooked shoulders and a broad thin chest

The symptoms of pectus excavatum may resemble other conditions or medical problems. Always talk to your child's physician for a diagnosis.

Is a pectus excavatum dangerous?

Not typically, though it can be troublesome.

If the pectus excavatum is causing exercise limitations, heart problems or if your child is concerned about how it looks, it can be repaired by surgery.

Treatment & Care

How is a pectus excavatum treated?

Funnel chest can be surgically repaired. There are two options, both of which require your child to be put under general anesthesia:

The Welsh - The surgeon makes an incision in your child's chest wall, removes the cartilage wedged between the ribs and breastbone and then repositions the freed-up breastbone.

o A bar is left in the chest wall to maintain the right shape for six months, during which time the child has to refrain from activities that might involve a collision, like football.

o The technique leaves a scar visible on the chest, but fixes the problem.

The Nuss -The incisions are made on the side, a bar is inserted laterally through the chest and the breastbone is lifted forward.

o No cartilage is removed.

o Scarring is less extensive.

On the other hand, any asymmetry that's there before the operation will remain to some degree. And there's just as much pain throughout recovery. Also, the bar has to stay in for at least a year, and sometimes two. Surgeons at Children's specialize in both techniques.

What happens after surgery?

Your child will go to the recovery room, and then be transferred to the surgical floor after approximately one to two hours.

Your child may have a small rubber tube (drain) underneath the incision. This will be removed one to two days after surgery.

Your child will be helped to walk the day after surgery.

Your child can eat/drink if feeling well the day after surgery.

It is not necessary to remove the stitches. They are under the skin and will dissolve.

Your child will need to see the surgeon two weeks after surgery to check how the area is healing.

Your child should not return to gym or participate in contact sports until after this postoperative visit with the surgeon. The doctor or nurse practitioner will give you exact instructions.

What if there are complications after surgery?

Call your doctor if your child has:

an increase in redness or swelling around the wound drainage or bleeding from the incision

a fever of 100.5 degree or higher

severe pain that does not get better with pain medicine

shortness of breath

Q&A with Jay Wilson, MD: Pectus excavatum and carinatum

The chest wall deformities pectus excavatum and carinatum have incidences of approximately one in 500 and 1,500 respectively. Here, Jay M. Wilson, MD, senior associate in Children’s Hospital Boston’s Department of Surgery, discusses the range of severity, available treatments and psycho-social impact of the condition.

How do these chest wall deformities present?

Both are apparent during physical exams. Excavatum is an indentation of the chest wall. Carinatum is a protuberance of the chest wall. Either may run the gamut from barely perceptible to severe. I’ve seen indentations of a chest wall that could hold a quart of water, while others are characterized by only a few millimeters’ depression. They may taper gradually or draw to a sudden peak or depression. Asymmetry is fairly common and a combination of excavatum and carinatum is possible.

When do these deformities become apparent?

We can often see both deformities in newborns and during early childhood. Other times, though, it may not be apparent until the child is 9 or 10. It’s rare for either excavatum or carinatum to

show up after that. The typical course is from mild to more severe, with growth spurts contributing most to severity.

What are the causes?

They’re unknown. Some studies investigating a genetic component are underway. Although the majority of cases don’t involve a family history, there are many that do—enough to warrant the suspicion that genes play a significant role. The genetic story is likely to be complex, though.

What are the options for treatment?

Excavatum is amenable to surgical repair. The first option is called the Welsh, during which the surgeon makes an incision in the chest wall, removes the cartilage wedged between the ribs and breastbone, and then repositions the freed up breastbone as appropriate. A bar is left in the chest wall to maintain the right shape for six months, during which time the child has to refrain from activities that might involve a collision, like football. The technique leaves a scar visible on the chest, but allows for a precise resolution of asymmetries.

With a technique called the Nuss, the incisions are made on the side, a bar is inserted laterally through the chest and the breastbone is lifted forward. No cartilage is removed. Scarring is less extensive. On the other hand, any asymmetry that’s there pre-operatively will remain post-operatively to some degree. And although we’re not breaking and resetting bones like we are in the Welsh, there’s as much pain throughout recovery. Also, the bar has to stay in for at least a year, and sometimes two. Surgeons at Children’s specialize in both techniques.

What is the best timing of an excavatum repair and how can a carinatum be resolved?

Twenty-five years ago, surgeons operated when the child was as young as 4. The cartilage content of the bones made repositioning easier and healing was faster. But too often, the condition would recur, especially during growth spurts. So we prefer to intervene, if we intervene at all, during the child’s teen years. By then, they have most of their vertical height. One more growth spurt won’t contribute much to a recurrence. As for resolving carinatum, we recommend bracing, as pressure over time reforms the chest wall.

What are the indications for treatment?

For mild cases of either condition, there’s really no reason for intervention. A shallow excavatum is unlikely to affect the operation of the heart or lungs at all. A mild carinatum, if anything, gives the thoracic organs more room to function. Appearance, too, is normal in these cases.

In more severe cases, the approach is different. A severe excavatum can impinge on the heart and lungs, but it’s important not to overstate the threat. At extremes of exercise, there is a slight deficit in the heart’s stroke volume, and the volume of blood the heart can pump out with each beat. That’s because the heart has less room to fill when it relaxes. Pulmonary function tests also show a slight deficit in severe cases of excavatum. But again, this occurs only at extremes of exercise. These kids are not at any elevated risk for sudden death. With severe cases of

carinatum, the problem is one of form rather than physiology. In both cases, the emotional impact can be devastating. Some kids with severe pectus are perfectly content with the shape of their chests. But those who suffer psychologically can suffer deeply.

What are the benefits of repair?

It’s not yet clear whether surgical repair of excavatum really improves physiology. When we intervene at around age 12 or 13, when the lungs are already fully formed and the heart is almost fully formed. But the change in appearance can be drastic. For basic moral reasons and demonstrable psycho-social ones, we believe every child has a right to a normal appearance.

Pectus Excavatum Surgery Abroad

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Pectus excavatum is the most common form of congenital chest wall deformity. It is sometimes also referred to as 'funnel chest'. Pectus excavatum is the abnormal growth found in the costal cartilages between the sternum (breastbone) and ribs. In such an abnormal growth in the cartilages, the sternum appears sunken or, on in somecases, rotates to one side or the other.

The overall incidence of pectus excavatum is reported to be 1 in 1000. As of now, the exact cause of pectus excavatum is unclear. Definitive research says that 46 percent of patients have a family history of pectus excavatum. Pectus excavatum tends to affect males more often than females. This has led many experts and surgeons to believe that some genetic factors are involved with pectus excavatum, including male gender.

Pectus excavatum causes numerous problems; moderate to severe PE can result in major complications. In moderate and severe pectus excavatum, the patient's heart compresses and displaces into the left chest. This limits the amount of blood, the heart is capable of pumping per beat. This may become evident when the patient is working out and his heart has to beat faster to compensate for this compression.

In moderate to very severe defects, the heart displaces to the sternum's left or the midline. This causes a murmur, which is actually due to the pressure on the system causing rough flow instead of a smooth flow.

In human anatomy, one of the main functions of the rib cage is to act as a shield for the lungs and heart. In pectus excavatum, the rib cage is shaped differently. The heart and lungs are still protected by the ribs but the concave shape of the rib cage may cause these vital organs to be compressed. This restriction may prevent normal contractions leading to cardiac limitations.

In addition, the defect tends to restrict the amount of air entering the lungs. This, again, becomes evident during physical activity, when the patient feels that he cannot keep up with his peers. Other problems include psychosocial and emotional disturbances of having a chest deformity.

Some of the pectus excavatum symptoms are:

Chest pain: research has suggested that almost two thirds of surgical patients with pectus excavatum have a history of chest pain

Shortness of breath during exercise: patients experience a shortness of breath or have lack of stamina when they are exercising.

Psychosocial effects: patients with pectus excavatum are conscious and insecure about the deformity in their chest wall.

Diagnosis for Pectus excavatum

Before surgical treatment is decided, a pectus deformity is diagnosed. Doctors observe the patient having a chest deformity or having difficulty in breathing. The next step is an x-ray or CT scan or even MRI scan, to better understand the pectus deformity's scope and structure.

Then the patient's Haller index is measured. The Haller is calculated by obtaining the measurement of inside left to inside right of the ribcage and dividing that by the distance from the sternum to the spine. A normal chest has an index of 2.5 and a Haller Index greater than 3.25 is generally considered severe.

Non-Surgical Treatments for Sunken Chest

Pectus excavatum is damaging only in the severest of the cases. For most cases, doctors don't advice surgery and ask the patient to consider non-surgical treatments. Since attitude often plays a significant role in adjusting to a pectus deformity and patient's well-being, many patients go for psychological counseling. Depending on the severity of the condition, counseling therapies help the child develop coping strategies.

Physiotherapies, such as exercises and improvement of posture, sometimes provide benefit in terms of appearance, and lung and heart performance. Body-developing exercises can worsen appearance because of larger pectoral muscles that amplify the chest deformity. But certain aerobic exercises have proved beneficial for patients suffering from pectus excavatum. In addition, any patient who tends to slouch or has rounded shoulders stands to gain from certain techniques.

Surgery Treatment for Pectus excavatum

Doctors usually recommend surgery only after the patient has reached mid-teens, this is due to the fact that, in several past instances, younger patients had their chest buckle-in again after the surgery. Although improvement in cosmetic appearance is a bonus benefit, the surgeries are carried out mainly for physiologic reasons, not for cosmetic.

Surgical procedures include:

The Nuss Procedure is a less invasive procedure, developed in the 1980s, to correct the more common excavatum in younger patients (age 5-15). Nuss surgery involves, the making of small incisions to insert one or more metal rods behind the sternum. The bars force the sternum into its proper position. These bars are then left in place for about two years before they're removed. Doctors do not recommend this procedure for older patients whose bones are thicker and more brittle, or for patients with carinatum.

Ravitch Procedure. In this, the sternum is detached from the ribs and turned around to lie flat. It is then reconnected and reinforced with metal struts or rods to hold its proper position during recovery. Ravitch is a major invasive procedure, which creates a significant scar and calls for painkillers. The patient is made to exercise causing shortness of breath and forcing the lungs to expand to their actual capacity. Only non-contact activities are prescribed for 4 months, while the bones are healing. Following this, a second surgery is programmed to remove the metal rods. The procedure is the most common and highly successful one, but is complex and the recovery can be slow.

A newer, invasive reconstructive procedure is the Leonard Procedure. In the Leonard procedure, the cartilage is removed from the lower ribs and the sternum undergoes modifying. Then chest cavity is rebuilt and reshaped using wires to reconnect and support the bones. The wires are fixed to an external plastic "Jewitt" brace, which is worn for six weeks as a traction device.

A fourth type of surgical procedure is Silicone Implants, that can provide some amount of cosmetic improvement for patients, especially in the case of older patients, with minor to moderate cases of excavatum.

Pectus carinatum V/s Pectus excavatum

Pectus carinatum, or protrusion of the breast, also called pigeon breast, is a completely different malformation. Pectus carinatum (PC, or "pigeon chest") in which the sternum is raised and so the chest pushed out. It is a complete reverse condition of pectus excavatum.

Another significant difference is, in pectus carinatum the heart is in its normal position and murmurs are very rare, whereas, in pectus excavatum the heart is displaced to the left of the sternum or the midline which may cause a murmur.

The surgeries are also different, given the formation of the conditions. The surgical correction for pectus carinatum involves bilaterally removing the cartilages that are affected and the excess cartilage over the sternum. A reverse wedge is carried out on the sternum. This is followed by a bracing that is in a compression system rather than outward rigging as required by pectus excavatum.

Low Cost Nuss Procedure Surgery in India

For some patients suffering from pectus excavatum, in U.S. and U.K., the surgery bills can run into anywhere between $75,000 and $150,000. In such cases, patients can opt to go in for sunken chest repair surgery, in countries like India.

Your medical treatment and surgery can be easily handled in India, as the quality of healthcare available in India is simply one of the best in the world. The surgeons are USA/UK trained and the hospital facilities and equipments are 5-star.

The cost for Nuss procedure surgery in India comes at a fraction of the costs, charged in U.S. and U.K. Medical Tourism Corporation's network of hospitals can provide you pectus excavatum repair surgery from 3,400 -15,000 US dollars.

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Medical Tourism Corporation facilitates affordable pectus excavatum surgery abroad in India. Fill out the free estimate request form for a free quote and more information.

Signs and symptoms

The hallmark of the condition is a sunken appearance of the sternum. The heart can be displaced and/or rotated. Mitral valve prolapse may also be present. Base lung capacity is decreased.[7]

Causes

Researchers are currently unsure as to the actual cause of pectus excavatum but hypothesize genetic defect.[3] Approximately 37% of individuals with pectus excavatum have a first degree family member with the condition.[2] Physiologically, increased pressure in utero, rickets and increased traction on the sternum due to abnormalities of the diaphragm have been postulated as specific mechanisms.[2] Pectus excavatum is also a relatively common symptom of Marfan syndrome.[8] Many children with spinal muscular atrophy develop pectus excavatum due to the diaphragmatic breathing that is common with the disease. Pectus excavatum also occurs in about 1% of persons diagnosed with Celiac disease for unknown reasons.

Pathophysiology

Because the heart is located behind the sternum, and because individuals with pectus excavatum have been shown to have visible deformities of the heart (seen both on radiological imaging and after autopsies), it has been hypothesized that there is impairment of function of the cardiovascular system in individuals with pectus excavatum. While some studies have demonstrated decreased cardiovascular function in pectus excavatum, there has been no consensus reached based on newer physiological tests (such as echocardiography) of the presence or degree of impairment in cardiovascular function in people with pectus excavatum. Similarly, there is no consensus on the degree of functional improvement after corrective surgery.[2]

Diagnosis

Cross sectional scan of a chest with pectus excavatum

Pectus excavatum is initially suspected from visual examination of the anterior chest. Auscultation of the chest can reveal displaced heart beat and valve prolapse. There can be a heart murmur occurring during systole caused by proximity between the sternum and the pulmonary artery.[9] Lung sounds are usually clear yet diminished due to decreased base lung capacity.[7]

Many scales have been developed to determine the degree of deformity in the chest wall. Most of these are variants on the distance between the sternum and the spine. One such index is the Backer ratio which grades severity of deformity based on the ratio between the diameter of the vertebral body nearest to xiphosternal junction and the distance between the xiphosternal junction and the nearest vertebral body.[10] More recently the Haller index has been used based on CT scan measurements. An index over 3.25 is often defined as severe.[11] The Haller index is the ratio between the horizontal distance of the inside of the ribcage and the shortest distance between the vertebrae and sternum.[12]

Chest x-rays are also useful in the diagnosis. The chest x-ray in pectus excavatum can show an opacity in the right lung area that can be mistaken for an infiltrate (such as that seen with pneumonia).[13] Some studies also suggest that the Haller index can be calculated based on chest x-ray as opposed to CT scanning in individuals who have no limitation in their function.[14]

Pectus excavatum is differentiated from other disorders by a series of elimination of signs and symptoms. Pectus carinatum is excluded by the simple observation of a collapsing of the sternum rather than a protrusion. Kyphoscoliosis is excluded by diagnostic imaging of the spine, where in pectus excavatum the spine usually appears normal in structure.

Treatment

Treatment for pectus excavatum can involve either invasive or non-invasive techniques or a combination of both. Before an operation proceeds several tests are usually to be performed. These include, but are not limited to, a CT scan, pulmonary function tests, and cardiology exams (such as auscultation and ECGs).[3] After a CT scan is taken the Haller index is measured. The patient's Haller is calculated by obtaining the ratio of the transverse diameter (the horizontal distance of the inside of the ribcage) and the anteroposterior diameter (the shortest distance between the vertebrae and sternum).[15] A Haller Index of greater than 3.25 is generally considered severe, while normal chest has an index of 2.5.[12][16][17] The cardiopulmonary tests are used to determine the lung capacity and to check for heart murmurs.

Surgery

Surgical correction has been shown to repair any functional symptoms that may occur in the condition, such as respiratory problems or heart murmurs, provided that permanent damage has not already arisen from an extremely severe case.[3]

Original Ravitch technique

The Older Ravitch technique is an invasive surgery that was introduced in 1949,[18] and developed in the 1950s to treat the condition. This procedure involves creating an incision along

the chest through which the cartilage is removed and the sternum detached. A small bar is then inserted underneath the sternum to hold it up in the desired position. The bar is left implanted until the cartilage grows back, typically about 6 months. The bar is subsequently removed in a simple out-patient procedure. The Ravitch technique is not widely practiced because it is so invasive. It is often used in older patients, where the sternum has calcified, when the deformity is asymmetrical, or when the less invasive Nuss procedure has proven unsuccessful.[19]

Modern Ravitch technique

The newer Modified Ravitch technique addresses many of the concerns posed by the traditional method and allows for the complete repair in one surgery with no need for a second surgery. Using a new "dissolving plate" to hold the sternum in place while healing, the newer method allows for a shorter healing time as well as proper muscle placement.

Nuss procedureMain article: Nuss procedure

Lung exercisers, used after corrective surgery to avoid pneumonia and increase base lung capacity

X-Ray of a 15 year old male after undergoing the Nuss procedure

Since then, Dr. Donald Nuss, based at Children's Hospital of The King's Daughters (CHKD) in Norfolk, Virginia, has developed a technique that is minimally invasive.[20][21] The Nuss procedure involves slipping in one or more concave steel bars into the chest, underneath the sternum. The bar is flipped to a convex position so as to push outward on the sternum, correcting the deformity. The bar usually stays in the body for about two years, although many surgeons are now moving toward leaving them in for up to five years. When the bones have solidified into place, the bar is removed through outpatient surgery.

Vacuum bellMain article: Vacuum bell (medicine)

A relatively new alternative to surgery is the vacuum bell. It consists of a bowl shaped device which fits over the caved-in area; the air is then removed by the use of a hand pump. The vacuum created by this lifts the sternum upwards, lessening the severity of the deformity. As it is such a recent device there is currently no information as to whether it is effective in the long term.[22]

Cosmetic and light treatments

The cosmetic appearance of pectus excavatum can be treated with a dermal filler called Bio-Alcamid.[23] However, as this does nothing to alleviate the actual deformity it will not prevent any physiological symptoms caused by the condition.

Mild cases have also reportedly been treated with corset-like orthopedic support vests and exercise.[24][25]

There are also prosthetic implants available to fill the depressed area. Solid silicone implants have been successfully used for many years with acceptable results in some cases.[26][27] More recently a porex implant has been used which is a similar material used to replace skull in brain surgery and severe head injuries.[28]

Magnetic mini-mover procedure

The magnetic mini-mover procedure (3MP) is a technique used to correct pectus excavatum by using two magnets to realign the sternum with the rest of the chest and ribcage.[29] One magnet is inserted 1 cm into the patients body on the lower end of the sternum, the other is placed externally onto a custom fitted brace. These two magnets generate around 0.04 tesla (T) in order to slowly move the sternum outwards over a number of years. The maximum magnetic field that can be applied to the body safely is around 4 T, making this technique safe from a magnetic viewpoint.[29] The 3MP technique's main advantages are that it is more cost-effective than major surgical approaches such as the Nuss procedure and it is considerably less painful postoperatively. One potential adverse effect of 3MP is inactivation of implanted devices such as an artificial pacemaker. Since the 3MP is still in a trial period it is unknown whether long term wearing of the magnet will affect the skin or other vital organs.

Epidemiology

Pectus excavatum occurs in an estimated 1 in 150-1000 births, with male predominance (male-to-female ratio of 3:1). Occurrences of the condition in family members have been reported in 35% to 45% of cases.[7][30]