A funnel chest is a funnel-shaped deformity of the thoracic wall resulting from impaired formation of the cartilage connections between the sternum and ribs. Men are slightly more likely to be affected by a funnel chest than women, with a ratio of 3:1.
What is a funnel chest?
A funnel chest (pectus excavatum) is a visible deformity of the thoracic wall that usually affects the 4th to 7th ribs in the lower sternum and, unlike the so-called keel or chicken chest, manifests in childhood. Maldevelopments in the cartilaginous connections between ribs and sternum cause a funnel-shaped depression of the anterior thoracic region, which can be symmetrical or asymmetrical in shape. Through this funnel, internal thoracic organs such as the heart can be displaced, depending on the specific manifestation, and in extreme forms can lead to serious cardiopulmonary (heart and lungs related) alterations, which cause reduced lung ventilation. In addition, a funnel chest may be associated with kyphoscoliosis or other conditions resulting from postural deformities (lumbalgia, dorsalgia).
Causes
The exact causes of funnel chest have not yet been conclusively determined. In most cases, however, the condition is due to genetic factors that lead to soft rib cartilage as a result of impaired cartilage metabolism. Accordingly, in about 35 to 37 percent of those affected, there is a first-degree relative with this disease in the family. However, the specific gene that triggers the manifestation of a funnel chest has not yet been deciphered. In addition, funnel chest is associated in some cases with certain syndromes such as Marfan syndrome. Funnel chest may also be observed after certain diseases (pleural nipple), as a sequelae of surgical procedures on the diaphragm or chest wall, and as a result of greatly increased alcohol consumption during pregnancy (fetal alcohol syndrome).
Symptoms, complaints, and signs
Funnel chest is already clearly recognizable at birth. Symptomatic is a chest that is pushed inward. In most cases, there are no other noticeable symptoms during the first years of life. It is not until puberty and increasing adulthood that a number of signs related to the congenital malformation appear. Puberty in particular brings with it a psychological burden for people with a funnel chest. Those affected feel ashamed of their body and withdraw. Social interaction with peers suffers. In addition, the deformity increases in growth, which intensifies the psychological symptoms. Affected individuals do not perform as well physically compared to other peers. This extends to their entire life. Even small stresses can lead to breathing difficulties and shortness of breath. In the developmental phase to manhood or womanhood, the heart sometimes cannot expand further due to the limited corpus. In old age, heart problems can then be expected on a regular basis. It has also been proven that the heart may squeeze other organs. Affected persons basically have to accept movement restrictions during their whole life. A bad posture often develops in the form of a rounded back. This not infrequently results in damage to the intervertebral discs.
Diagnosis and progression
A funnel chest can usually be diagnosed on the basis of the externally visible funnel-shaped depression in the area of the chest wall, which usually occurs in the first year of life and increases until the end of growth. Complete evaluation of the deformity is performed using computed tomography of the chest wall. Pulmonary function tests such as spirometry or bodyplethysmography can detect airway impairment. Cardiodiagnostic procedures (echocardiogram) provide information about involvement of the heart (mitral valve prolapse). In addition, X-ray examination of the spine should exclude other underlying diseases (keel chest, Harrenstein deformity). In most cases, a funnel chest has a mild manifestation and a good course. However, if left untreated, a funnel chest can lead to deformities of the spine and disorders of the heart or lungs as a result of poor posture.
When should you go to the doctor?
In the case of a funnel chest, a doctor should always be contacted. This disease must be examined and treated by a medical professional in any case, so that it does not lead to further complications or discomfort. In this disease, early diagnosis with subsequent treatment has a very positive effect on the further course of the disease and can also prevent further complications. As a rule, self-healing does not occur. A doctor should be consulted if the affected person suffers from a malformation of the breast. The breast is strongly pressed inwards, which can usually be seen with the naked eye. Furthermore, breathing difficulties or, in severe cases, even shortness of breath may indicate funnel chest. Heart pain also points to this disease. If funnel chest is not treated, it usually leads to restrictions in the movement of the affected person. In the case of a funnel chest, an orthopedist or a general practitioner can be consulted. Further treatment is then usually surgical. Since the disease can also lead to depression or other psychological complaints, a visit to a psychologist is usually also necessary.
Treatment and therapy
In the case of a funnel chest, the therapeutic measures depend on the specifically present manifestation of the deformity. For example, mild deformities without clinical or psychological impairment are usually treated within the framework of physiotherapeutic measures to correct postural defects (postural gymnastics). An indication for surgical intervention is derived from the psychological distress caused by the funnel chest as well as the degree of deformity, and in severe cases the conservative measures are complemented by surgical correction of the deformity. In most cases, the correction is performed by the minimally invasive surgical procedure according to Nuss. For this purpose, a customized U- or C-shaped metal arch is implanted through an opening in the rib cage through two smaller incisions in the area of the armpits. By raising the arch, the rib cage is brought into its regular position and space is created for proximal organs such as the heart. In adolescents, the implant remains in the patient’s body until the end of growth in order to prevent the formation of another funnel. In addition, the open corrective procedure according to Ravitch – Welsh – Rehbein is occasionally used, in which the affected ribs are modeled or removed via a horizontal (in men) or vertical (in women) incision in the rib cage and substituted by implants in order to correct the funnel chest. A purely cosmetic surgical procedure is to fill the depression with silicone implants. In addition, long-term treatment with a suction or vacuum bell is a relatively new alternative treatment option, which is intended to raise the sternum regularly and reduce the funnel accordingly. However, long-term studies on this treatment option for funnel chest are not yet available.
Prevention
It is not possible to prevent a funnel chest caused by genetic factors. However, physical therapy, especially strengthening of the back and chest muscles, will help minimize the risk of organ involvement. In addition, excessive alcohol consumption should be avoided during pregnancy to prevent fetal alcohol syndrome, which can cause funnel chest along with other malformations.
Follow-up
Depending on the severity of the funnel chest, surgical treatment is performed. Aftercare of the wound, including removal of stitches, is provided by the attending physician. For up to six weeks after surgery, it is advisable not to exercise and, if possible, to lie on your back while sleeping. During this time, the intake of painkillers is usually reduced. This is also regulated by the doctor. At the end of the six weeks, the patient can start physiotherapy. The patient can also slowly start doing sports again. However, the intensity and amount of sports should be discussed with the attending physician. However, certain types of sports such as boxing are not recommended for the long term in order to avoid damage to the chest area. It is also advantageous if the patient does not carry heavy loads for at least three months after the operation. After about a year, follow-up treatments are necessary only as needed. An exception to this is the removal of the stirrups three years after the actual operation.The removal is done by another surgery. Patients with a funnel chest may also experience psychological distress, which can be treated by a professional therapist.
What you can do yourself
Regardless of the conservative treatment of funnel chest, which nowadays usually takes place surgically or by means of suction cups, it is recommended to correct incorrect postures such as a hollow back or drooping shoulders by means of physiotherapy. Those affected must make a conscious effort to adopt a natural posture in order to improve their posture in the long term. Regular training and the resulting successes also boost the self-confidence of those affected. After an operation, patients must take care of themselves. The wound must be professionally treated and carefully monitored, as there is a risk of inflammation or bleeding. Funnel chest does not necessarily require treatment. Affected persons who decide against therapy should take advantage of therapeutic options to make it easier to deal with the deformity of the chest. Attending a support group and contacting other sufferers can also be useful if the funnel chest is a significant burden. In most cases, parents must also ensure that the child develops healthily despite the deformity. Exclusion and bullying can occur, especially during puberty. When these situations occur, parents of affected children need to talk to teachers and other parents.
