Surgical correction of Polanda syndrome
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The causes of the development of the disease
Statistics shows that men face this condition 3 times more often than women.
The reasons that cause Poland syndrome include:
- Hereditary predisposition
- Village of blood supply to the chest in the early stages of the development of the embryo
- The effect of harmful factors on the fetus in the first trimester of pregnancy (medicines, infections, radiation)
- stress or injury in the mother during pregnancy
- Smoking while gesturing a child
Most doctors believe that the cause is a combination of genetic characteristics and external influences.
In addition, they distinguish two key forms of Poland's syndrome:
- Full form - is a rare and complex condition that includes thoracic disorders and brush defects.
- Incomplete form - manifests itself only in the form of a chest underdevelopment.
Depending on the level of complexity, thoracic defects are also divided into simple lesions and complex ones. In the first case, only a deficiency of soft tissues is revealed, and in more severe cases, bone anomalies are added to soft tissue defects.
Symptoms of the disease
Poland syndrome is characterized by various symptoms:
- Muscular features. Slow development of the pectoral, back, and abdominal muscles
- Bone features. Underdevelopment or deformation of the ribs and cartilage, causing a noticeable curvature of the chest
- Skin changes. Usually on the affected side there is thinning of the subcutaneous fat layer, lack of hair in the chest and armpit area
- Breast abnormalities. Manifest through underdevelopment of the mammary gland, abnormalities in the position and size of the nipple or its complete absence
In some cases, anomalies in the development of the hand may be present, for example, shortening of the phalanges or absence of fingers. There are also cases where congenital anomalies are accompanied by tumors of the hematopoietic system and thrombocytopenia.
Classification
In orthopedics, it is customary to use the Foucras classification to describe Poland syndrome:
- First degree. Slight curvature of the chest and asymmetry of the mammary glands with normal skeletal development.
- Second degree. Moderate deformities due to the absence of the pectoralis major muscle and ribs. The mammary gland may be completely absent on the affected side.
- Third degree. Severe deformities of the chest and significant underdevelopment or absence of several pectoral muscles.
Most cases (75%) of Poland syndrome affect the right side of the body, while bilateral involvement of the pectoral muscles is rare.
Diagnosis of Polandaa syndrome
Modern diagnostic methods make it possible not only to identify the presence of the disease, but also to determine the degree of its severity.
To establish a diagnosis, the following research methods are used:
- X-ray - allows you to detect changes in the structure of the ribs, thoracic vertebrae and determine the location of internal organs.
- Computed tomography of the thoracic region - provides detailed information about the condition of tissues and bone structures.
- ECG and ultrasound examination of the heart – used to analyze the functioning of the cardiovascular system.
When it comes to treating Poland syndrome, traditional methods usually do not bring the desired effect. In most cases, surgery is recommended.
General information about the procedure
Methods of surgical correction
Surgical treatment depends on the specific manifestations of the disease and the needs of the patient. Surgical interventions are divided into two main categories: correction of fingers and plastic surgery on chest tissue.
The first group includes:
- Functured finger release is a procedure in which the surgeon separates the fused fingers to improve the function and appearance of the hand
- Increasing the length of the fingers of the hand is a method that allows you to lengthen too short fingers and restore their functionality
- Transplantation of fingers from the lower limb to the hand - in some cases it is possible to use a toe as a donor to replace a missing or underdeveloped finger on the hand
In addition to surgical treatment, doctors recommend psychological support for patients with Poland syndrome. Consultations with a psychologist help to gain self-confidence.
The main types of plastic surgery on chest tissue include:
- Plasty of the isolated soft tissue component of the chest - correction of soft tissue defects without changing the bone structure of the chest
- Step-by-step reconstruction of the chest frame for complex defects - methods include the use of implants and the patient's own tissue to restore normal shape and function of the chest
- Breast plastic surgery for women - correction of the shape and size of the breast using various methods, including the use of implants and autologous tissue
Sometimes treatment requires not one, but several surgical interventions, where each stage is aimed at eliminating the patient’s problems step by step.
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About the disease
Poland syndrome is a congenital anomaly that is characterized by disturbances in the development of the pectoral muscle. This rare syndrome was first documented in the 19th century by British scientist Alfred Poland. The disease was subsequently named after him.
Symptoms of the syndrome can manifest themselves in varying degrees: from chest asymmetry, which some patients notice only after reaching puberty, to more rare manifestations, such as curvature of the ribs or scoliosis.
To clarify the diagnosis, patients are recommended to undergo radiography and computed tomography. Treatment focuses on plastic and reconstructive surgeries to correct the shape of the chest and restore its function.