What is Pectus Excavatum (Funnel Chest) ?
It is the most common type of chest wall deformities or malformations. It is characterized by sinking of the chest wall inwards. It occurs in one in every 300-400 live births. It is 4 times more common in males than in females. Also, family history of 37% of the patients has this disease. With the severe relocation of the chest wall through the back, sinking may occur on the anterior wall of the right ventricle of the heart. Furthermore, in severely sunken situations, the heart is usually relocated through the left.
Pektus ekskavatum örneği
Pektus ekskavatum örneği
Pektus ekskavatum örneği
Pektus ekskavatum örneği
Causes
It is resulted from abnormal growth of the costal cartilage junction between the rib and the sternum (breastbone). Although over growth of the ribs causes this sinking, exact cause is unknown. Ribs composed of two parts: costal bone and costal cartilage. Costal cartilage of the irregularly growing rib pulls the breastbone backwards.
Signs
Although signs appear during the childhood, typically they become prominent during the growth period and adolescence. Whether pectus excavatum will cause significant symptoms and signs is still questionable. Signs can be determined by a healthcare professional based on the patient’s complaints. Presence of the symptoms in patients is variable. Chest and back pain are the most common and occur almost in every patient. These are musculo-skeletal pains. Another common complaint is the shortness of breath due to effort. Bad posture is also a factor in this type of pains and development of shortness of breath. Most common signs can be listed as below:
Pain
It usually occurs in the chest and back region of the body. The pain usually comes and goes and may deteriorate following some activities or exercises. It may get worse during rapid growth periods. Bad posture or posture disorders may trigger the pain.
Shortness of breath
Shortness of breath or the feeling of shortness of breath is usually experienced or worsen following some activities and exercise with big efforts. Most of the physicians think that there is a pronounced association between sunken chest wall and symptoms like decreased lung function and shortness of breath. On the other hand, normal results are obtained in some patients when the pulmonary and respiratory functions are measured at least in some simple tests (spirometry). However, particularly some measurements done in patients observed during exercising showed the correlation between shortness of breath by effort and decrease in the pulmonary reserve. Exercise tolerance was observed to increase in patients following surgical correction.
Cardiac problems
In serious forms, increase in tachycardia complaints is seen. Also, cardiac arrhythmia and cardiac murmur may be detected. Inwards sinking of the chest wall leads to physically relocation of the heart which is posterior and slightly left to the sternum which in turn causes the above-mentioned symptoms. Severe forms may even affect the cardiac functions. Some studies showed that surgical correction could improve cardiac functions.
Fainting
Although rare, in severe cases syncope (temporary fainting or loss of consciousness) may develop as a result of shortness of breath. It may occur during exercise or other conditions requiring effort. It is thought to result from impaired blood flow due to the pressure to the heart from the sunken sternum.
Psychosocial effects
The most important symptom that the patients with pectus excavatum complain about is the psychological effect due to the abnormal look of the chest wall. Serious loss of self-confidence, avoiding from activities such as swimming, negative psychological effects due to physical appearance can be seen in patients. This may even impede the patient's socialization.
Diagnosis
Diagnosis of Pectus excavatum (funnel chest) is usually done by physician based on the physical examination and complaint of the patient. Generally, physical examination by a physician who is experienced in pectus deformities is adequate for the diagnosis. No specific blood test is available. However, sometimes radiological imaging (X-ray) can help to determine the severity of the disease and detect the associated problems like scoliosis of the spine. For the problems that it may cause in the heart, the most useful radiological test is the tomography. With this imaging method, not only the relocation of the heart but also the extent of the sternal rotation (severity of the sinking) resulted from pectus asymmetry could be examined in a more precise manner. This imaging method is important particularly for the patients for whom surgery is planned. MR is an imaging method that can be safely use in children without a radiation concern. Furthermore, other tests like echocardiography (ultrasound assessment of the heart) and pulmonary function tests are not required unless the signs are severe and another underlying reason such as “Marfan syndrome” is suspected.
Other diseases may accompany Pectus excavatum. In cases with Marfan syndrome, sinking is more severe. Particularly, boys with scoliosis should be evaluated from this point of view. In addition to that, Tetralogy of Fallot (TOF: a congenital heart disease) and Mitral Valve Prolapse (mitral valve bulge) may rarely accompany the condition. In fact, pectus excavatum is more common in those with musculoskeletal disease and developmental abnormality. It may be seen together with connective tissue diseases (Ehlers-Danlos syndrome, Marfan syndrome, osteogenesis imperfecta and homocystinuria). Also, it is common in those with Down syndrome (mongolism), congenital heart disease (TOF etc.) and congenital diaphragmatic hernia. Lateral curvature of the spine (scoliosis) and hunching of the back (kyphoscoliosis) may accompany this condition in 5 to 26% of the patients.
Treatments
Vacuum Bell - Non Surcigal Method
Surgery is in the forefront for the treatment of Pectus excavatum. However, in selected patients “Vacuum Bell” treatment may give satisfactory outcomes. Patient is monitored by using this device regularly for 3 to 12 months. However, before deciding the Vacuum Bell treatment degree of sinking should be determined and if the patient is suitable for non-surgical methods, this therapy should be initiated. Vacuum treatment is a procedure that can be applied before 17-18 of age while the thoracic cage is still flexible. This procedure provides correction by applying suction (negative pressure) after the device is positioned on the pectus excavatum. The device should be used twice a day, in the morning and evening, for one hour for each application. Best outcomes are achieved in mild and moderate funnel chest cases. In a study conducted in the US, it was shown that best outcomes are achieved at the age of 13. When used regularly, results are obtained around Months 3-6. For a precise result, it may be required to continue to use the device for up to 9-12 months. Vacuum application is a treatment method that requires patience and stability.
Pectus Excavatum- Vacuum Bell Treatment Details
Nuss Procedure
Nuss Procedure is a closed surgical method successfully performed in our country by Prof. Dr. Mustafa Yuksel since 2005. Sunken breastbone is lifted forward by supporting posterior part of the sinking using a metal bar without cutting or splitting the cartilages. Before this method was developed, correcting surgeries for pectus excavatum used to lead to long hospitalization, longer duration of surgeries and prolongation of the recovery period. In 1998, the new technique developed by an American surgeon Dr. Donald Nuss allowed patients to be treated in a short time. In suitable cases, Nussoperation is an approach that should be preferred because of being less invasive with the thoracoscopic method compared to Ravitch operation which lasts at least 4 hours, its total duration of 15-20 minutes, esthetical incision and increased comfort of the patient and faster discharge times after the operation. Hundreds of patients have been treated by Prof. Dr. Mustafa Yüksel by using this method. In order to correct the sunken chest wall, a nickel-chromium alloy steel bar (or for patients with allergy, a custom-made titanium bar) is placed subcutaneously.
X-Ray after Nuss Surgery
Nuss Surgery - Before & After
Patients stay in the hospital for an average of 4-5 days after the surgery. No intervention is required other than relieving any pain that may occur during this process. He/she may need family support at home for up to 23 more weeks after discharge. Full return to school/work may take up to 1-1.5 months.
You can find the details of Pectus Excavatum NUSS Surgery HERE.
Open Surgery (Ravitch)
There are two methods preferred in surgery. One of them is the open surgery called the Modified Ravitch. Open surgery requires a vertical or horizontal incision on the chest wall. After the muscles under the mammary tissue are lifted, some part of the costal cartilages from the both sides of the breastbones is removed and the sternum is corrected. In addition to that, various plates can be applied under or on the sternumin order to avoid recurrence of the pectus excavatum. Duration of the open surgery is longer and a scar remains on the anterior chest wall. Some impairment in respiratory functions due to narrowing of the thoracic wall after removal of the costal cartilages. Chest wall is weaken and its stability and strength are decreased for a long time following open surgery.