Funnel chest is a congenital and often familial condition. It is more common in males than females, with a reported male to female ratio of 4:1, and is a dominant trait. The incidence of funnel chest in those with a family history is 2.5 per 1,000, while in those without a family history, the incidence of funnel chest is only 1.0 per 1,000. The cause of funnel chest is unknown and is thought to be genetically related. Funnel chest is a progressive lesion that may be present at birth, but often becomes more pronounced after a few months or even years and is detected by parents. The external appearance is characterized by a sunken anterior chest, anteriorly extended shoulders, a slight hunchback and a prominent epigastrium.
Symptoms and signs
In infancy, the symptoms of compression of the funnel chest are often unnoticed. Some have inspiratory stridor and a sternal aspiration depression, but the cause of the airway obstruction is often not detected. Children are often thin and immobile, prone to upper respiratory tract infections, limited mobility, panic attacks, shortness of breath and dyspnea during activity. In addition to the thoracic deformity, the child often has a special body shape such as mild hunchback and abdominal protrusion.
Funnel chest is a congenital malformation in which the sternal body (especially the root of the saber) and its corresponding 3rd to 6th ribs are sunken inward, resulting in the anterior chest wall resembling a funnel, with the heart displaced by pressure and the lungs restricted in movement due to the thoracic malformation, which affects the cardiopulmonary function of the child. Upper respiratory tract and lung infections often occur, and even heart failure. The symptoms become more obvious after the age of 3, with concave chest, convex belly, wasting and poor growth. In funnel chest, the rib alignment slope is larger than normal, and the ribs are depressed sharply from the top to the bottom, making the front and back closer, and in severe cases, the deepest depression of the sternum can reach the spinal surgery. In young patients with funnel chest, the deformity is often symmetrical, but with age, the deformity gradually becomes asymmetrical, the sternum tends to rotate to the right, the depression of the right rib cartilage tends to be deeper than the left, and the right breast development is worse than the left. The posterior thorax is mostly flat back or round back, and scoliosis gradually worsens with age; scoliosis is less likely to occur when the patient is younger, and is more pronounced after puberty. The funnel chest deformity compresses the heart and lungs, and the heart is mostly displaced to the left side of the chest. Children often present with a distinctive frail posture: forward neck, rounded cut shoulders, and a cankered abdomen. The sternal body is most deeply depressed at the saber junction. There is a familial tendency or concomitant congenital heart disease.
Disease etiology and pathology
It is more common in males than females, with a reported male to female ratio of 4:1, and is a dominant trait. The incidence of funnel chest in those with a family history is 2.5 per 1,000,
The incidence of funnel chest in people without family history is only 1.0 per 1,000. The cause of funnel chest is unknown and is thought to be genetically related.
It is thought that this deformity is due to the uncoordinated growth of the ribs, with the lower part being smaller than the upper part, squeezing the sternum backward; it is also thought that it is due to the attachment of the diaphragm fibers to the lower part of the sternal body and the saber process in front, which pulls the sternum and saber process backward when the central tendon of the diaphragm is too short.
Funnel chest is a deformity in which the sternum, rib cartilage and part of the rib cage are depressed toward the spine to form a funnel shape. In most of the funnel chest, the sternum starts from the level of the second or third rib cartilage backward to a point slightly above the saber process as the lowest point.
(1) Effects on physiology
A mild funnel chest can be asymptomatic, while a more severe deformity will compress the heart and lungs, causing varying degrees of damage to cardiopulmonary function. It affects the respiratory and circulatory functions, with reduced lung capacity, increased functional residual air volume, and reduced activity tolerance. Young children often have recurrent respiratory infections with cough and fever, and are often diagnosed with bronchitis or bronchial wheeze. In older age, because the heart is compressed, cardiac blood output cannot meet the needs during exercise, and the myocardium is hypoxic, dyspnea, rapid pulse, palpitations, and even pain in the precordial region after activity often occur, and some patients can also develop arrhythmias, as well as systolic murmurs.
(2) Effects on psychology
As the patient grows older, he or she gradually feels unattractive in appearance and is reluctant to let others look at his or her chest. The usual manifestations are low self-esteem, introversion, insecurity and even depression. This heart abnormality is more pronounced in older children. It has been reported in the literature that some children can be suicidal. Because he or she has a particularly low self-esteem is afraid to meet people, afraid to expose his or her chest naked, and then develops suicidal psychological tendencies. Therefore, this disease brings not only physical harm to children, but also more serious psychological harm. It may be that when he or she grows to a certain age, he or she suddenly realizes that he or she is not the same as other children. At this time, this psychological inferiority complex will become more and more serious, the pressure will become more and more. The vast majority of patients have psychological disorders, especially older children and adults. On the surface, patients appear to be introverted and non-verbal, but in fact have deep psychological reactions, manifested as idiosyncratic embarrassment reactions, a sense of shame, social anxiety, limited work ability, poor orientation; reduced tolerance for frustration and temptation; limited sociability. In severe cases, depression may occur. Therefore, the patient’s psychological disorder should not be ignored.
(3)Whether funnel chest has any effect on life expectancy
This may be due to the compression of the heart and lungs by the funnel chest and scoliosis, which impairs the respiratory and circulatory functions, resulting in shorter survival time and death before the age of 40.
The fact that funnel chest is often associated with other diseases and that compression of the heart and lungs is a fact should be considered detrimental to the survival and quality of life of patients. In a cause of death analysis of 62 patients with funnel chest who died in 112 years at the Eastern Virginia Medical School in the United States, funnel chest itself was not the cause of death, but the concomitant disease of funnel chest was the main cause of death of the patients.
The conditions requiring surgery were as follows.
1, age >3 years, all 4-24 years old can receive surgery, and the best age is 4-12 years old.
2.Moderate or severe to funnel chest deformity, Haller index >3.2 on CT examination.
3.Pulmonary function tests suggest restrictive or obstructive airway pathology, susceptibility to upper respiratory tract infection, reduced tolerance to strenuous activity, shortness of breath when running or climbing stairs.
4.The heart is displaced by pressure and the electrocardiogram shows myocardial damage.
5.People who have failed other surgical methods.
6.Adolescents who have a serious psychological burden and require correction of their appearance.
Funnel chest is sometimes combined with pulmonary dysplasia, Marfan syndrome, asthma and other diseases, which often become intolerable deformities when combined, and often require early surgical correction.
What are the treatment methods for funnel chest
1, funnel chest internal drug treatment is not effective. There are various surgical treatment options for funnel chest. Typical traditional surgeries are: sternal reversal and thoracic rib lift (Ravitch procedure). Both surgeries can correct the sternal depression of the funnel chest, but they are difficult for many children and parents to accept because of the trauma to the child (sternum and ribs have to be cut) and the lack of aesthetic wound (long incision in the middle of the chest). At present, this kind of surgery is still carried out in many hospitals.
2, in recent years a new minimally invasive way of funnel chest orthopedic surgery has emerged, the United States Virginia Children’s Hospital of King’s Daughter of Dr. Donald Nuss developed a minimally invasive surgery, has replaced the traditional surgery this minimally invasive surgery that NUSS surgery (thoracoscopic plate placement sternal lift, also known as minimally invasive funnel chest orthopedic surgery). In this procedure, a shaped alloy steel plate is passed through the back of the sternum through the intercostal space on one side and then through the intercostal space on the other side with the assistance of a thoracoscope, and the plate is flipped to lift the sternum to correct the deformity.
The advantages of this surgery are.
1.Small trauma, no need to break the sternum and ribs.
2.Aesthetic wound: only 2-3cm incisions are made on each side of the chest, and the scar is not obvious after healing.
3.Fast recovery after surgery: traditional surgery requires 2 weeks of lying down after surgery, but you can get up and move around on the 3rd day after this surgery. Because of its minimally invasive, fast recovery and safety features, more and more parents are now asking for this procedure for their children.
For children with mild funnel chest, regular exercise and follow-up are recommended rather than blind surgery. In some children, the funnel chest will be corrected with exercise. If the funnel chest gradually worsens during regular review, surgery is needed at the right time.
In 2009, based on the original NUSS surgery, we adopted non-thoracoscopic extrapleural plate placement for sternal lift. No thoracoscope is needed to operate inside the chest cavity, avoiding stimulation of the lung and pleura and compression of the wall pleura, reducing the impact of surgery on respiratory circulation, reducing painful stimulation, maintaining the integrity of the pleural cavity reducing the chance of chest infection more in line with physiology and less trauma. The procedure is performed by simply inserting a tailor-made metal plate behind the sternum and pushing out the sternal recess and all inwardly deformed rib cartilage to perform the corrective surgery. The procedure can be completed by making tiny incisions of about 2 to 2.5 cm on both sides of the chest wall, and takes an average of 35 minutes to complete. In addition to improving cardiopulmonary function, it can also correct the position of the sternum and ribs and take into account the aesthetic appearance, which is especially important in girls.
Preoperative care for funnel chest
1.General preoperative preparation.
Increase and decrease clothes according to the temperature change to prevent cold and flu. Instruct the child with funnel chest to practice effective coughing, coughing and abdominal breathing, and to practice urination and defecation in bed. Prepare the skin in the operating area and keep the skin clean. Fast food and water for 8 hours before surgery to prevent aspiration pneumonia and asphyxia caused by anesthesia or vomiting during the operation. Keep the ward environment clean and quiet, ensure that the child sleeps, and give sedative and sleeping drugs as prescribed by the doctor if necessary.
2. Nutritional support.
Due to the compression of the heart, lungs and esophagus by the sternum, some children with funnel chest have delayed development, are thin and prone to respiratory infections and food reflux after eating. Before funnel chest surgery, assess the nutritional status of the child, explain the importance and necessity of preoperative nutritional support, and instruct the child to eat a high-protein, high-calorie, high vitamin diet: such as meat, eggs, milk, fresh fruits and vegetables. If necessary, give intravenous fluids to replenish energy and vitamins, and apply antibiotics and hemostatic drugs.
3.Psychological care.
Older children with funnel chest have heavy worries, mainly in the fear of surgery and anesthesia, worrying about the effect of orthopedic surgery on funnel chest, and the effect of surgery on study and life. Nurses should communicate with the children and their families in a timely manner to understand the psychological condition of the children and cooperate with the doctors in educating them about the disease. According to the age and psychological characteristics of different children, explain the necessity of funnel chest surgery, the brief process and postoperative effects. This can be done by introducing the situation of previous cases in our department and also with the help of promotional materials with pictures, photos and texts.
In general, patients are discharged when they can walk on their own without assistance.
Points to note for patients after discharge.
The following instructions can help the patient return to normal activity and reduce displacement of the orthopedic plate
1. Pay attention to the cleanliness of the incision and seek medical attention if there is any discomfort
2.Walk regularly and normally
3.Deep breathing exercise every morning and evening
4.In the first month after surgery, the back must be straight, do not bend, twist or roll
5.Do not lift heavy objects within two months after surgery
6.Don’t do confrontational sports (such as soccer and basketball) in March after surgery
As the implant needs to be kept in the patient’s body for more than 2 years, the following points need to be noted.
1.Post-operative stent for funnel chest is usually removed after 2-3 years post-operatively depending on the situation.
2.Funnel chest is regularly reviewed as an outpatient after surgery to understand changes in the condition.
3.If there is difficulty in breathing and chest pain after funnel chest surgery, it is necessary to consult a doctor immediately and take a frontal and lateral X-ray of the chest. If there is obvious deformation of the chest, follow up early to avoid direct collision of the chest.
4.No MRI (magnetic resonance imaging) examination of the chest and upper abdomen.
5.If cardiac defibrillation is needed, place the electrode plate in anterior-posterior position for cardiac shock.