Usually the disorder starts after the sternum grip and the first two sides. The disorder may be symmetrical or asymmetric accompanied by sternal rotation.
The incidence is from 1: 300 to 1: 400 births and is usually observed in the white race and in males. It is recognized in 90% of cases at birth, while in less than 5% it develops in adolescents. 37% of patients have a family history, in 26% the dysplasia is accompanied by scoliosis, while 1.5% have some form of congenital heart disease and a similar percentage of other musculoskeletal disorders.
People with Marfan syndrome have a high incidence of anterior chest wall dysplasia and mainly scaphoid.
“Infants usually have no symptoms, while older children complain of pain in the anterior chest wall or precardiac pain after prolonged exercise. Few patients complain of palpitations and shortness of breath, while few have syncope. The appearance of these symptoms accompanies the existence of transient nodular arrhythmias due to prolapse of the mitral valve.
The examination of individuals with scaphoid thorax consists of routine hematological and biochemical examination, plain chest radiography, spirometric examination, cardiac ultrasound (transthoracic or esophageal), orthopedic assessment of the presence and degree of scoliosis. and in case of severe form, the three-dimensional imaging with the help of computed tomography. In cases of onset suggesting Marfan syndrome, the test is completed with an ophthalmological and maxillofacial examination, but also with a genotype test.
Accurate evaluation is done with a chest CT scan where the Haller index is calculated, the value of which if it is above 2.5 is considered abnormal.
Studies of patients ‘cardiovascular function describe a significant improvement postoperatively, due to the movement of the sternum forward and the creation of more space for the heart, with improvement of the cardiothoracic index, and the working conditions of the heart, while the patients’ symptoms disappear in prolonged exercise. Finally, the disappearance of both arrhythmias and prolapse of the mitral valve postoperatively in patients with scapular thorax and scoliosis is reported.
Restoration of the scaphoid is undoubtedly surgical and aims to relieve the pressure of the thoracic structures by creating conditions for the normal development of the thoracic wall, preventing the development of cardiorespiratory dysfunctions and avoiding the development of psychological problems. The best age for surgical rehabilitation is between 5 and 10 years. It should be noted that the onset of symptoms is an indication for surgery at both younger and older ages, but the surgical technique varies depending on the age of treatment.
There are several techniques for correcting the scaphoid:
• The open technique of reversing Wada’s sternum, which is almost abandoned
• Endoscopic surgical rehabilitation with Nuss metal bar mounting
• The installation of silicone prefixes for aesthetics only eliminating the problem without restoring the correct anatomy.
• Ravitch’s technique, which is the most popular. The sternum is mobilized and fixed in a normal position, with a wide variety of different materials being used for its posterior support in its new improved position.
This describes both the use of metallic or plastic or synthetic materials and that of autologous materials such as bones or cartilage. Most of these materials present two main problems: on the one hand the lower than expected aesthetic result, and on the other hand the risk of both the displacement of the materials intrathoracically, and the need to remove them in a second year.
• The Robicsek technique, based on the Ravitch technique, in which the sternum is supported by a mesh. In the technique described and a modification of the Robicsek technique, the sternum is supported using a synthetic polytetrafluoroethylene (PTFE) implant that remains in place for life, without any burden to the patient and with excellent results.
Surgical rehabilitation, in specialized centers, is accompanied by zero mortality and minimal morbidity. The most serious distant complication of surgical rehabilitation is the recurrence of the scapular thorax accompanied by abnormal growth of the thoracic cage that occurs in a minimal percentage and always when the rehabilitation occurs in childhood, in very sick children with marpane features of weak muscular growth and chest diameter.
Writes:
Mr. Christoforos S. Kotoulas, Cardiac Surgeon,
Director of the Minimally Invasive Cardiac-Thoracic Surgery Clinic of the Metropolitan General
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