Funnel chest deformity

Overview

Pectus deformities are responsible for around 95 percent of congenital chest wall defects, with Funnel chest deformity being the most frequent. When the breastbone pulls inwards and there is a dip between the ribs, this is known as a funnel chest deformity.

Some persons who have Funnel chest have no additional symptoms. Others may suffer from impaired heart or lung function. Some persons with pectus excavatum may feel timid or self-conscious because it changes their physical form. The location of the breastbone can be corrected with treatment.

What are chest wall deformities?

A chest wall deformity is a modest to severe structural malformation of the chest wall. Deformities of the chest wall arise when the cartilage that joins the ribs grows irregularly. It is unknown why this occurs, although the ailment appears to run in families.

The two most common types of chest wall deformity are:

• When the breastbone presses inward, it causes pectus excavatum, also known as funnel chest or concave chest. Sunken chests are seen in children with this sort of malformation. Pectus excavatum is the most prevalent kind of chest wall malformation, affecting one in every 300 to 400 children and affecting boys three times more than girls.
• Pectus carinatum is a disorder in which the breastbone and ribs protrude. It is also known as pigeon chest or elevated chest. For some youngsters, both sides of the chest protrude, while for others, one side protrudes more than the other. The disorder affects around one out of every 1,500 children and is more frequent in boys than in girls.

The most frequent congenital chest wall defect is pectus excavatum (90%), followed by pectus carinatum (5-7%), cleft sternum, pentalogy of Cantrell, asphyxiating thoracic dystrophy, and spondylothoracic dysplasia. When a youngster is 1 or 2 years old, chest wall abnormalities are commonly seen for the first time. The deformity may be minor at initially and worsen throughout adolescence, when a child's bones and cartilage are quickly expanding.

Children with a chest wall abnormality may exhibit no symptoms other than self-consciousness about their looks. Some youngsters, however, have difficulties breathing during activity and are more susceptible to respiratory infections and asthma.

What is Funnel Chest?

Pectus excavatum, also known as sunken or funnel chest, is a congenital chest wall deformity characterized by aberrant growth of numerous ribs and the sternum, resulting in a concave, or caved-in, look in the anterior chest wall.

While the abnormality affects the third to seventh costocartilages or ribs, the xiphisternum is the most severely affected location. Although the malformation can be symmetrical, it is more often asymmetrical and can affect other parts of the thorax. A pectus deformity might be detected in a newborn or develop later in infancy.

Funnel chest malformation affects around 1 in every 300-400 births, with males predominating (male-to-female ratio of 3:1). More than 90% of cases are diagnosed within the first year of life. The look of the chest worsens and symptoms arise with fast bone growth in the early adolescent years.

Many patients are not brought to a pediatric surgeon's attention until they and their families detect changes. Young teens may be disturbed by the sight of the chest. Teenage patients typically express issues with self-esteem and body image perception. Psychological problems are common in elderly individuals.

Funnel Chest Causes

It is uncertain what causes pectus excavatum. It is most likely the outcome of a genetic abnormality that causes aberrant musculoskeletal growth. This aberrant development pattern is most likely caused by the cartilaginous part of the rib. Pectus excavatum and pectus carinatum are most likely caused by abnormalities in rib formation and development. The sternum is assumed to be pushed in by aberrant development at the rib and cartilage articulation in pectus excavatum.

Again, the precise mechanism causing this aberrant development pattern is unknown. Increased labor of breathing, as seen in young children during exercise or play activity, may contribute to the evolution of the pectus deformity, especially during the early adolescent years. However, there is no scientific evidence to back up such a claim.

Pectus excavatum is characterized by aberrant bone and cartilage formation in the anterior chest wall, often affecting 4-5 ribs on either side of the sternum. The severity of the abnormality ranges from minor to severe, and some people have substantial asymmetry between the right and left sides. The precise process underlying this aberrant bone and cartilage outgrowth is unknown, and no known hereditary flaw has been linked to the development of pectus excavatum. Despite the lack of an obvious genetic marker, 35% of instances with pectus deformity are believed to be familial. Furthermore, the disorder is linked to Marfan syndrome and Poland syndrome.

What are the symptoms of Funnel chest deformity?

The symptoms of pectus excavatum generally fall into three categories:

• Pain. It is unclear what is causing the pain. It might be related to the way the pectoral muscles traverse the chest to connect to the ribs and breastbone. When this happens, the discomfort is typically not severe or long-lasting.
• Reduced exercise tolerance. This is most likely connected to the previously indicated effect of the breastbone malformation on the heart.
• Appearance. Many people with pectus excavatum are self-conscious about the appearance of their chests. While the chest is uncovered (like when swimming), other youngsters are likely to notice and comment or make fun of the appearance. This may be enough to make a youngster change his or her conduct. The youngster may no longer want to swim, will not shower with other children during gym class, will change clothes away from other children, and will avoid any activities that may draw attention to the abnormality. This has a substantial impact on certain youngsters, who may retreat socially to some extent. These psychological issues might last until puberty and adulthood.

Funnel Chest Diagnosis

The sternal defect is plainly visible in the lateral image of the chest radiograph. Further imaging investigations may detect misplaced vertebral bodies as well as varied degrees of scoliosis. Patients with moderate pectus excavatum may have few or no symptoms; nonetheless, every one to two years, a cardiopulmonary examination is recommended to establish a baseline and monitor for progression. To assess for secondary or related abnormalities of a clinical condition, a complete evaluation comprises a chest radiograph, pulmonary function tests (spirometry, plethysmography, and respiratory muscle strength assessment), EKG, and echocardiography.

In elderly individuals, pulmonary function tests may indicate obstructive or restrictive lung disease, as well as air trapping with an increase in residual volume (RV). A mechanical disadvantage limiting respiratory muscle function might be causing the air trapping. Axis deviation on an electrocardiogram (EKG) indicates a leftward heart deviation. In this patient group, 16% had Arrhythmias such as first-degree heart block, right bundle branch block, and Wolff-Parkinson-White.

Cardiopulmonary exercise testing may show cardiopulmonary limitations that are not apparent at rest. Echocardiography is recommended to check for cardiac compression, valvular abnormalities, and myocardial function. A patient with pectus excavatum may have a left heart deviation and conduction abnormalities.

The Haller index (HI) is the gold standard for determining the severity of a pectus excavatum deformity. It is the ratio of the transverse diameter to the anteroposterior diameter. The readings are from a computed tomography (CT) scan; the typical value is 2.5 or less. Measurements greater than 3.2 are deemed serious. A restrictive pattern on pulmonary function tests is four times more prevalent in patients with a Haller Index greater than 7.

MRI, especially breath-hold MRI, has been used to examine the morphology of thoracic abnormalities prior to surgery for pectus excavatum. Recently introduced techniques such as oculo-electronic plethysmography (OEP) can show that the depressed portion of the sternum and adjacent chest wall do not move with respiration and there is a reduction in lung volume, providing a better understanding of the functioning of the lungs of patients with pectus excavatum deformity.

Management of Pectus Excavatum

The extensive excision and chest wall rebuilding conducted for asphyxiating thoracic dystrophy (Jeune's condition) served as the foundation for early surgical repair of pectus excavatum. The current approach is to postpone the repair until pubertal development has occurred, and the procedure has been refined to allow for limited cartilage resection. The most prevalent reason for surgical correction is impaired cardiopulmonary function, not cosmesis. It is critical that surgical correction take place following the child's pubertal growth spurt. 

Ravitch conducted the first reported surgical correction of pectus excavatum in 1949. Ravitch conducted a subperichondrial excision of all malformed costal cartilages and the xiphoid process, combined with a transverse sternotomy, based on the aggressive chest wall resection used to treat asphyxiating thoracic dystrophy (Jeune's syndrome). Six years later, Rehbein and Wernicke changed the method by implanting a variety of metallic bars longitudinally and/or transversely to support the sternum.

This repair can be addressed either a median longitudinal incision along the sternum or, in female patients, using a submammary skin incision. Patients return one year later to have the implanted bars removed. The Ravitch treatment has been modernized by the use of orthopedic metal plates and screws. These devices may be customized to the patient's defect and do not require a second surgical operation for hardware removal.

Surgical correction of the funnel chest deformity

Nuss and Kelly's minimally invasive procedure entails inserting a metal bar beneath the sternum at the most depressed zone and most everted costal line on both sides of the chest. The retrosternal bar is inserted thoracoscopically through 3cm incisions along the mid-axillary lines. Skin flaps are raised and tennelled up to the most-everted intercostal gaps previously found. Under thoracoscopic guidance, a metal introducer is used to slice a retrosternal plane between the anterior pericardium and the posterior sternal table.

The guide is exteriorized through the incision on the left side in order to be linked to a titanium rod and then removed. The retrosternal curve var is turned to drive the concave side rearward, pressing the sternum ventrally. To keep the bar from moving, metallic stabilizers and/or subperiostal cable wires are utilized. Although the initial stabilization time was two years, it is currently more typical to leave the ban in place for three years.

Additional Ravitch and Nuss technique changes have been documented. After mobilizing the pectoralis muscles, the Leonard modification to Ravitch's operation involves excision of the lower costal cartilage while leaving the perichondirum in tact. Robicsek discusses posterior sternum stabilization following transverse osteotomy with Marlex mesh fixation to costal cartilage remains.

Erlangen's modification of the Nuss method is defined by minimal cartilage resection, with transternal implantation of an elastic metal bar through stitch incisions. Intraoperative tensiometry is used to reduce cartilage excision.

The magnetic mini-procedure pulls the sternum anteriorly by using magnetic forces between a sternal magnet and another on a recommended brace worn by the patient. Lacquet's modification is the only one that avoids the use of prosthetic material by repairing the deformity with a sternochondroplasty. For asymmetric flaws, this technique appears to outperform the Nuss approach.

Negative pressure applied to the thorax provides a non-surgical option to pectus excavatum. A vacuum bell is placed to the chest wall defect, and the patient applies negative pressure using a handpump. While long-term results are unclear, this may be a feasible alternative in the future for the management of less severe abnormalities. Furthermore, this treatment might be appropriate for younger symptomatic kids for whom prepubertal surgical correction is not required.

What are the long-term results of operation for pectus excavatum?

Several studies have been conducted following pectus excavatum correction. The Ravitch technique has a very low recurrence incidence (1-2%). In terms of symptoms, the results may be judged based on the specific symptoms before to the operation:

1. Pain. Around 40-50% of all individuals who get the procedure due to discomfort improve.
2. Symptoms of exertion When patients believe they do not have the same endurance as other youngsters, almost 60% improve following restoration.
3. When this is the primary complaint, 95% of patients are satisfied with the outcome. A surgical scar will be evident, as with any procedure.

What can the patient do after going home from the operation?

Your child will be hospitalized for a few days following surgery to recuperate. The length of stay will be mostly determined by pain management. Physiotherapists will begin physical therapy, which is essential for promoting healing and mobility, regaining strength, expanding the lungs, promoting bowel function, and avoiding problems.

Your child will be seen at the general surgery clinic in 2-4 weeks after being discharged from the hospital. Your kid's surgeon or nurse practitioner will discuss pain management and activity limits, as well as any other information you may require to care for your child at home.

While wearing a Medical Alert bracelet following surgical repair of pectus excavatum is not required, it is strongly advised. Obtaining a Medical Alert bracelet might be discussed with you during your post-operative appointment. The bracelet's engraving should read "steel bar in chest, CPR greater force, cardioversion ant/post insertion."

The presence of a steel bar in the chest raises numerous typical concerns regarding daily activities. Here are a few things you should be aware of.:

• The bar should not set off metal detectors in the airport, although it may in rare circumstances, depending on the type of material (titanium bar is more likely to set off detectors, while stainless steel will not). If necessary, we can offer documentation for the bar.
• While the ban is in place, antibiotics are not necessary prior to dental treatments.
• While the bar is in place, your child can still get CPR if necessary. Because of the bar positioning, chest compressions should be performed with more force. With the bar in place, an AED can also be utilized; however, the paddle location will be different. One paddle should be put in front of the chest, and the other in rear.

Prognosis

With therapy, the prognosis of pectus excavatum is favorable. Patients with modest pectus excavatum who do not require surgical treatment have a great prognosis as well. Patients with moderate-to-severe pectus excavatum may develop cardiovascular impairment, lower exercise tolerance, diminished stamina, and adjustment difficulties as a result of the deformity's influence on body image and coping techniques. The disorder is not related with death.

Patients who had pectus excavatum corrected commonly reported an improvement in exercise stamina after surgery, with the effect seemingly unrelated to the exact surgical method utilized. The researchers hypothesized that increased exercise capacity is caused by an increase in anterior-posterior thoracic dimensions, which relieves strain on the cardiac chambers and, as a consequence, allows for better heart filling.

Many people with pectus excavatum are functionally asymptomatic. The extent of cardiopulmonary damage induced by lung compression and the level of cardiac displacement produced by the caved-in chest are debatable. Exercise tolerance is usually reported as abnormal, and in extreme cases, a restrictive pattern in the pulmonary function test might be detected.

Although cardiac function is often normal, mitral valve prolapse has been found in 20-60% of instances. Echocardiography usually demonstrates atrial compression and cardiac displacement. It may occasionally detect mitral or tricuspid regurgitation. Following operational treatment of the deformity, echocardiographic study revealed an improved cardiac index during exercise. It is unknown what the long-term health hazards are for people who are handled without surgery.

Complications

Minor pectus excavatum deformities are typically asymptomatic and so do not cause issues. More severe abnormalities, on the other hand, might have a deleterious influence on cardiopulmonary function, resulting in a number of symptoms:

• Chest pain
• Fatigue
• Exertional dyspnea
• Recurrent respiratory tract infections
• Asthma
• Palpitations
• Heart murmur (mitral regurgitation)
• Arrythmia
• Syncope

Complications are more frequently observed in individuals undergoing surgical treatment of the defect:

• Migration of the sternal bar
• Infection
• Pneumothorax
• Bleeding
• Cardiac laceration
• Chronic pain
• Recurrence

What effect does pectus excavatum have on the heart and lungs?

Normal daily activities have little effect on heart or lung function. Most toddlers and adults will have lung capacity that is somewhat below average but still within the normal range. However, the heart's capacity to pump adequately during hard activity may be abnormal. Several medical tests have found that the sternum can put enough pressure on the heart that it cannot fill with blood and pump it out as quickly as a normal chest. These same studies show that after surgical treatment of pectus excavatum, the heart's pumping function improves. However, there is usually no change in lung capacity after restoration.

Conclusion 

The most common congenital chest wall malformation is pectus excavatum, which is caused by posterior sternal depression. Boys are more affected than females by this illness. It is common from birth but becomes more obvious during periods of rapid development, such as puberty. The degree of the deformity and your child's symptoms will determine how you treat pectus excavatum. Surgery is not usually necessary.