Absence of the pectoralis major muscle. Signs and treatment of Poland syndrome in girls and boys. Signs of congenital malformation: incomplete and complete forms

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Poland syndrome

Accordingly, in French and German literature, however, it is named after the English medical student Alfred Poland, who in 1841 published a partial description of this deformation that he encountered at the section. A complete description of deformation with all its components appeared in the literature only in 1895.

Each patient has different components of Poland syndrome, which occurs sporadically, with a frequency of 1 in 30,000 - 1 in 32,000 newborns and is rarely familial. Damage to the hands varies in extent and severity. It is assumed that abnormal migration of embryonic tissues forming the pectoral muscles, hypoplasia of the subclavian artery, or intrauterine damage play a role in the etiology of Poland syndrome. Not a single theory has yet found reliable confirmation, however.

Deformation of the chest wall in Poland syndrome varies from mild hypoplasia of the ribs and costal cartilages on the affected side to aplasia of the anterior part of the ribs and all costal cartilages (Fig. 15-8). Thus, according to one study, among 75 patients with Poland syndrome, 41 had no deformation of the chest wall, 10 had hypoplasia of the ribs without local areas of depression, 16 had deformation of the ribs with retraction, and in 11 of them the retraction was significant. And finally, rib aplasia was detected in 8 cases.35 It is important to note that no correlation was found between the degree of deformation of the hand and chest.

Rice. 15-8. Abnormalities of the chest in Poland syndrome. A, Most often, the rib cage is completely normal, only the pectoral muscles are absent.
B, Retraction of the affected side of the chest wall with rotation and (often) retraction of the sternum. Often there is a keeled deformity on the opposite side.
C, Hypoplasia of ribs on the affected side, but without significant depression. This option usually does not require surgical correction.
D, Aplasia of one or more ribs is usually associated with retraction of adjacent ribs on the affected side and rotation of the sternum.

Rice. 15-9. A, A transverse incision is made below the nipple and between them, in girls - at the site of the fold that subsequently forms under the mammary glands.
B, Schematic representation of the deformity with sternal rotation, cartilage retraction on the affected side, and keeling on the opposite side.
C, In rib aplasia, the endothoracic fascia is located directly beneath the thinned subcutaneous layer and pectoral fascia. Elevate the pectoral muscle on the “healthy” side along with the pectoral fascia (if present) on the affected side. Subperichondral resection of the costal cartilages is performed as shown by the dotted line. In rare cases, resection is performed to the level of the second costal cartilage.
D, A transverse corrective wedge sternotomy is performed below the second costal cartilage. After suturing the sternum with a thick silk thread, both posterior displacement and rotation of the sternum are eliminated.
F, For rib aplasia, split rib grafts are taken from the opposite fifth or seventh ribs and reinforced on the affected side with metal sutures medially into the previously created sternum and laterally to the corresponding ribs. The ribs are split as shown in the figure to maximize their mechanical strength.

Rice. 15-10 Computed tomogram of a 24-year-old patient with Poland syndrome. Depression of the ribs on the affected side (solid line) and a calcified deformity on the opposite side (hollow arrow) are visible. The mammary gland on the affected side is hypoplastic.

Hello, Alexander!

Most likely, you talked about a congenital disease such as Poland syndrome, or the absence of the pectoral muscle. It occurs in both men and women. Treatment is surgical only, and is more often carried out in women with subsequent installation of implants. In men, if there is no pathology of the ribs, the operation is performed only for cosmetic reasons. Most often, muscle flaps of the dorsal muscle are used, but in this case you need to be prepared for the fact that there will be a scar under the armpit and minimal asymmetry of the back. With the current development of medicine, the scar can be easily removed. There are no other serious consequences of the operation, and after 2-3 days the patient goes home, having received recommendations from the attending physician. If the operation is done well - with preservation of the nerves in the transplanted muscle, etc., then after recovery it can be trained, but, of course, one must understand that training will still not give one hundred percent results.

The non-surgical route is the use of silicone prostheses, selected individually for each patient, and for men too. Now such prostheses are being manufactured that are very successfully used in practice. There is also a technique that involves introducing a special gel to create volume. You should consult directly with a plastic surgeon to assess the need for surgery, undergo a professional medical examination, and receive professional recommendations.

This is about the operation. But I would like to say something else. First of all, to calm you down, Alexander. Believe me, Poland syndrome is not a reason for suicide! Try to accept yourself as you are. And start relationships with girls. Because a normal, intelligent woman understands perfectly well that some defect in the body is nothing. And the one for whom only an ideal body is important... Do you need to connect your life with such a person? Look online for information about Nik Vucic. This man has no legs or arms - he was born this way. However, he created his own profitable business and recently got married. His example helped many people not to lose heart, to believe in themselves, and to avoid unforgivable actions. You are young, you have everything ahead of you.

Many people with Poland syndrome successfully engage in sports and achieve excellent results! Especially for you, I attach a photo of one of them. Exercising is useful, be sure to go to the gym, don’t quit, just distribute the load wisely.

Be confident in yourself and don't be afraid of anything. Live, because there is only one life. Don't let a cosmetic defect deprive you of its joys.

All the best, Veronica.

The defect is unilateral, most often observed on the right. The severity of the disorders can vary greatly. The diagnosis is made based on clinical data, radiography, MRI and other studies. Treatment tactics are determined individually.

Poland syndrome

Poland syndrome is a fairly rare congenital malformation. It is detected in one out of thousand. newborns. For the first time, partial descriptions of this pathology were made by Frorier and Lallemand in the first half of the 19th century, but the disease was named after the English medical student Poland, who also created a partial description of the defect in 1841. Occurs sporadically, family predisposition has not been proven. It is characterized by wide variability - in different patients there are significant differences both in severity and in the presence or absence of certain defects.

It is assumed that the cause of this anomaly is a violation of the migration of embryonic tissues from which the pectoral muscles are formed. There are also theories linking Poland's syndrome to intrauterine damage or hypoplasia of the subclavian artery. None of these theories has yet received reliable confirmation.

Pathological anatomy of Poland syndrome

The main and most constant component of this complex of developmental defects is hypoplasia or aplasia of the pectoral muscles, which can be supplemented by other signs. There may be slight underdevelopment or absence of costal cartilage. In some cases, on the affected side, not only muscles, fatty tissue and costal cartilages are completely absent, but also the bony part of the ribs. Other possible signs of Poland syndrome include brachydactyly (shortening of the fingers) and syndactyly (fusion of the fingers) on the affected side. Sometimes there is a decrease in the size of the brush or its complete absence.

The defective side may also show absence of a mammary gland (amastia), absence of a nipple (ately), and absence of hair in the armpit. In 80% of cases, a complex of defects is detected on the right side. With the left-sided version of Poland's syndrome, a reverse arrangement of internal organs is sometimes found - from dextracardia, in which the heart is on the right, and the rest of the organs remain in their place, to a mirror arrangement, in which a reverse (mirror) localization of all organs is observed.

With the left-sided version of the disease, the normal location of the heart and severe hypoplasia of the half of the chest, the heart remains poorly protected from external influences and can sometimes be located directly under the skin. In such cases, there is an immediate danger to the patient's life, since any blow can cause serious injury and cardiac arrest. In other cases, the consequences are not so serious and can range from deterioration in respiratory and circulatory functions due to deformation of the chest to a purely cosmetic defect due to the absence of muscles and/or the mammary gland.

Symptoms and diagnosis of Poland syndrome

Symptoms of Poland syndrome, as a rule, are clearly visible even to a non-specialist and are usually detected by parents in the first days of the baby’s life. Characterized by asymmetry of the chest, absence or insufficient development of muscles and underdevelopment of subcutaneous fatty tissue on one side. If the defect is located on the side of the heart, in the absence of ribs, you can observe the heartbeat just under the skin. In girls during puberty, the breast on the affected side does not grow or grows behind. In some cases (with muscle hypoplasia in the absence of other defects) in boys, Poland syndrome is diagnosed only in adolescence, when after playing sports, patients consult a doctor because of the asymmetry that has arisen between the “pumped up” normal and hypoplastic pectoral muscles.

There are four main options for the formation of the chest in Poland syndrome. In the first option (observed in most patients), the structure of the cartilaginous and bone parts of the ribs is not damaged, the shape of the chest is preserved, the anomaly is detected only at the level of soft tissues. In the second option, the bone and cartilaginous part of the ribs are preserved, but the chest has an irregular shape: on the affected side there is a pronounced retraction of the cartilaginous part of the ribs, the sternum is rotated (turned semi-sideways), and a keeled deformity of the chest is often detected on the opposite side.

The third option is characterized by hypoplasia of the costal cartilages while the bone part of the ribs is preserved. The chest is somewhat “skewed”, the sternum is slightly tilted towards the painful side, but no gross deformation is observed. In the fourth option, the absence of both cartilaginous and bone parts of one, two, three or four ribs (from the third to the sixth) is detected. The ribs on the affected side sink, and pronounced rotation of the sternum is detected.

To confirm the diagnosis and determine treatment tactics for Poland syndrome, a number of instrumental studies are carried out. Based on chest radiography, the severity and nature of pathological changes in bone structures are judged. To assess the condition of the cartilage and soft tissues, the patient is referred for MRI and CT of the chest. If secondary pathological changes in internal organs are suspected, consultations with a cardiologist and pulmonologist are prescribed, and external respiratory function tests, ECG, EchoEG and other studies are carried out.

Treatment of Poland syndrome

Treatment of Poland syndrome is surgical, usually begins at an early age, and is performed by thoracic and plastic surgeons. The scope of treatment measures depends on the presence and severity of certain pathological changes. Thus, in case of severe pathology with absence of ribs and deformation of the chest, a series of staged surgical interventions may be required to ensure the safety of internal organs and normalization of external respiration. And in the case of an isolated absence of the pectoral muscle and a normal shape of the chest, the only purpose of the operation is to eliminate the cosmetic defect.

If it is necessary to correct hand abnormalities (for example, to eliminate syndactyly), traumatologists and orthopedists are involved. If there is pathology in the internal organs, patients are referred to cardiologists and pulmonologists. The goal of treating the underlying pathology is to create optimal conditions for the protection and functioning of internal organs, restore the normal shape of the chest and restore normal anatomical relationships between soft tissues.

The first and main part of the treatment of Poland syndrome is the elimination of bone deformity and replacement of the rib defect. Several methods of thoracoplasty are used. If the defect is located on the right side and two or three ribs are missing, the underlying ribs are transposed. If four ribs are affected, a musculoskeletal flap is transplanted, “cut” from the healthy half of the patient’s chest. If necessary, a corrective wedge-shaped sternotomy is performed (removal of a section of the sternum to correct its shape and position from oblique to straight).

Currently, along with the use of the patient's own tissues, surgical interventions are increasingly being performed using implants made from special inert synthetic materials. In some cases, in preschool age, a special mesh is installed in the area of ​​the rib defect, which protects the internal organs without interfering with the normal growth of the ribs - this tactic helps reduce the likelihood of secondary deformations of the chest caused by uneven growth of the patient’s operated and non-operated ribs.

For isolated muscle defects, the serratus anterior or rectus abdominis muscle is transplanted. It is also possible to replace the pectoral muscles with a custom-made silicone graft. In women, reconstructive mammoplasty becomes an important part of treatment - elimination of a cosmetic defect resulting from underdevelopment or absence of the mammary gland. To restore the anatomical relationships between soft tissues, movement of the latissimus dorsi muscle is used, and some time after the wound has healed, a silicone breast prosthesis is installed.

Poland syndrome

Poland syndrome is an abnormal development of the chest due to the absence or underdevelopment of the pectoral muscles. These pathologies form during fetal development and can affect not only muscles, but also bones. In medical practice, there have been cases where one or more ribs were missing or they were underdeveloped. In addition, this syndrome is accompanied by other signs: shortening of the arm on the injured side of the body, an underdeveloped or even absent hand, complete or partial absence of fingers. Externally it manifests itself in the form of asymmetry of the upper body.

Reasons for development

In the course of research, it was found that Poland syndrome occurs due to a violation of the movement of embryonic cells that form muscles. This syndrome is not inherited. There are also theories that say that the cause of this anomaly may be intrauterine trauma or hypoplasia of the subclavian artery, but the reliability of this data has not been officially confirmed.

Symptoms

Poland syndrome is a rare defect; according to statistics, approximately 1 child in 32 thousand newborns is born with it. Parents can notice this pathology in their child almost immediately after birth. Externally, it manifests itself in the form of asymmetry of the chest; the absence of muscle and subcutaneous fat on the damaged side of the chest is felt to the touch. If the defect is located on the left side of the chest, then the pulsation of the heart muscle will be visible to the naked eye.

Anatomically, the pectoralis major muscle consists of three parts:

In addition, Poland syndrome includes symptoms such as shortening of the arm, syndactyly, that is, fusion of the fingers, absence of the nipple or the entire mammary gland on the damaged side of the breast.

Poland syndrome is also characterized by the pathological structure of the subclavian artery and vessels. The diameter of the artery exceeds the norm, which leads to disruption of blood flow in the abnormally developed half of the chest, due to a decrease in the resistance of the vessel walls.

With this anomaly, there are the following options for the formation of the chest:

1. An anomaly in the development of muscle and fat tissue, while the development of bone and cartilage structures is not impaired.
2. Chest deformity. The bones and cartilage of the costal part are preserved, but deformed. The chest is usually subject to keeled deformation.
3. Anomaly of the development of soft tissues and cartilage in the absence of a violation of the development of the bone structure of the ribs.
4. Underdevelopment or complete absence of the bone part of the ribs with impaired development of muscles and cartilage.

Very often, Poland syndrome manifests itself in the form of a small cosmetic defect and becomes noticeable only in adolescence, when girls begin to develop mammary glands, and in boys, the affected side of the breast does not acquire a prominent, pumped-up shape, unlike the healthy half of the chest. Poland syndrome does not affect female fertility (the ability to conceive children).

Diagnostics

In addition to external signs, the following methods are used to diagnose Poland syndrome:

• X-ray examination. Allows you to determine the presence of pathology in the development of the bone structure of the ribs;
• computed tomography and magnetic resonance imaging. Used to determine abnormalities in the development of muscle tissue and cartilage;
• consultations and examinations by a cardiologist and pulmonologist. They are carried out if there is suspected damage to the internal organs of the chest.

Treatment

To treat this condition, only surgery is used, however, if the defect is only cosmetic and does not affect the health and functioning of internal organs, then the operation is voluntary. Depending on the severity of the anomaly, several operations may be performed:

1. Surgery to restore the bone structure of the ribs,
2. Surgery to restore the shape of the chest (elimination of deformity),
3. Surgery to restore muscle tissue, breast implantation, nipple formation,
4. An operation to eliminate syndactyly, underdevelopment of the hand and other related defects.

Restoration of the ribs is carried out by transplanting parts of the ribs from the healthy side of the chest or by implanting titanium implants. If an operation to restore the bone part of the ribs is performed at an early age, then a special dense mesh is installed as the costal wall, which does not interfere with the development of the ribs, but performs a protective function for the heart and lungs. This is due to the fact that there is a possibility of repeated deformation of the chest due to the uneven development of healthy and transplanted ribs.

When restoring soft tissues, a transplant of part of the latissimus muscle, serratus anterior muscle, or rectus abdominis muscle is used. In some cases, silicone implants are used.

After the operation, a course of antibiotics is prescribed. The use of traditional methods of improving metabolism is not prohibited, but this can only be done after consultation with your doctor.

The prognosis after surgery is very favorable. Elimination of a cosmetic defect that does not affect the functioning of internal organs and the hand increases the patient’s self-esteem, and in difficult cases, the operation allows the patient to live a full life. The vast majority of patients do not experience discomfort or additional health problems after surgery.

Unfortunately, there is no way to prevent the occurrence of Poland syndrome. However, if parents do not abuse bad habits, lead a healthy and active lifestyle, undergo timely examinations and the necessary treatment, this increases the possibility of having a healthy child.

Poland syndrome: causes and treatment options

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Problems with physique or body structure come in different forms. Today we want to tell our readers about Poland syndrome, which is one of the pathologies of the body structure. Our questions were answered by a thoracic surgeon, Ph.D. – Vladimir Aleksandrovich Kuzmichev.

- Vladimir Aleksandrovich, it’s probably best to start with a story about what Poland syndrome is? How does its presence manifest itself in human physiology and the functioning of its body?

Poland Syndrome is a pathology that externally manifests itself in the form of partial or complete absence of the pectoral muscles, which is complemented by other signs. For example, underdevelopment of the upper limbs, when there is a reduced size of the hand, there may be fusion or shortening of the fingers, or there may be a complete absence of the hand, i.e. There is asymmetry with the other hand. There may be underdevelopment of the latissimus dorsi muscle. And, most importantly from the point of view of the functioning of the body, the absence or underdevelopment of the ribs on the affected side. As a rule, this concerns the 3rd and 4th ribs, which are either completely absent or underdeveloped. Or the cartilage does not fully fit the sternum. Also on the defective side there is some degeneration of adipose tissue. A more common symptom of Poland syndrome is underdevelopment of two parts of the pectoralis major muscle. The pectoralis major muscle consists of three parts: the subclavian part, the sternal part and the costal part. As a rule, the sternum and costal parts are missing. Or the pectoralis major muscle is completely absent. All other signs are rarer, so patients with Poland syndrome often have a completely full-fledged hand.

- Is the affected side always the same or is there variability?

- Where did the name of the syndrome come from? Is it somehow connected with Poland?

No, in this case, the name of the phenomenon was formed on behalf of an English doctor who, as a student, worked in a morgue as an orderly and saw this feature on one of the bodies. He examined her in detail and described the signs. The name was formed from his name.

- How to diagnose the presence of this disease?

In this case, the diagnosis is very simple - based on the patient’s appearance. It must be said that this condition is diagnosed at an early age by the characteristic condition of the pectoral muscle in the shoulder area. If the pectoral muscle is absent, then the armpit has a very specific appearance. It is in such cases that parents ask what to do.

- But really, what should parents do if they discover such a defect in their child? What scares them most?

Firstly, the child's appearance, the absence of a muscle, as well as the beating of the heart just under the skin, if there are absence of ribs, are of concern.

- Does the discovery of the syndrome leave any imprint on the child’s upbringing in terms of limiting his activity or prohibiting any types of activities?

In most cases, patients may not pay attention to their syndrome; they develop well, despite the absence of the pectoral muscle, and the motor activity of the arm is compensated. There are situations when patients with the absence of the pectoral muscle are actively involved in sports, including high achievements. When there is no involvement of the chest, the problem is purely cosmetic and should be addressed jointly by plastic and thoracic surgeons.

- Is the problem expressed in the same way in men and women? Are there any differences?

Yes, solving a cosmetic problem has different meanings for men and women. In the case of women, there is underdevelopment of the mammary gland, while in men the nipple may simply be underdeveloped. In medicine, this is called Amazon syndrome, because, as is known, these girls were active in archery and impaired the growth of one of the mammary glands. Since the mammary glands play an important aesthetic and functional role for women, they have more frequent visits to the doctor to correct the defect.

- Does the absence of one of the mammary glands in women somehow affect the overall hormonal levels or motherhood?

No, it doesn’t, the problem is of an aesthetic nature. The woman is absolutely healthy.

- What are the methods to correct this defect? Do they always require surgery?

The method of solving the problem is surgery, which can be performed using various techniques depending on the set of symptoms of the disease. For example, in women, the latissimus muscle can be moved from the back forward to create a muscular frame and muscle layer, which subsequently makes it possible to implant a silicone prosthesis to create breasts. Or more complex options are used, for example, a rectus abdominis muscle transplant. Everything is decided depending on each specific case.

- Do the indications for surgery differ depending on the combination of symptoms?

Yes, I would say that the indication for surgery in men is the absence of ribs, which affects the vulnerability of internal organs, including the heart. For men, eliminating a cosmetic defect is completely impossible. Thus, the displaced back muscle does not become full-fledged, and the back suffers, from where a piece of the muscle is taken. In the future, the back will also become slightly asymmetrical. In other words, there cannot be full-fledged cosmetics, but there is a violation of the body’s functions. More often for men, implantation of silicone prostheses is used, which are made from plaster or paraffin casts for each patient individually.

- In men, if there is no manifestation in the form of underdeveloped fingers on one hand, is the presence of the syndrome diagnosed only when undressed? Or does the shape of the sternum also affect the appearance in clothing?

No, the absence of the pectoral muscle is not noticeable in clothes. The presence of the syndrome in this case is diagnosed only when undressed. That's why many men don't turn to a doctor for help. The absence of the pectoral muscle is not noticeable under clothing, however, many of them are embarrassed to undress, since it is quite obvious when undressed.

- But with muscle building, the asymmetry of the chest probably becomes more obvious?

- At the same time, the muscle is not trained or built up?

Absolutely right. However, if it was possible to fully move the muscle from the back along with the nerves, etc., then some training is possible, but still it will not be complete.

- What is the reason for the absence of the pectoral muscle?

It is believed that the root of the problem is the underdevelopment of the clavicular artery, which does not give off full branches in this area.

- Can’t you influence the artery?

The formation of the artery occurs in the uterine state. Therefore, at this stage it is impossible to influence its development.

- If people adapt, does it make sense to operate on them?

There are no serious indications for surgery, that’s absolutely certain. The operation is indicated only if there is pathology of the ribs. It’s a different matter for women, when surgery is performed for subsequent breast implantation and significantly improves aesthetic perception.

- Does a muscle transplant from the back have any effect on the spine?

No, the latissimus dorsi muscle is connected only to the shoulder girdle, it is not connected to the spine in any way. The only thing that will happen is a certain scar in the armpit (the site of muscle collection and the transplant site are the same scar) and some asymmetry.

- Could there be a negative effect from the operation, any irreversible damage?

No. The latissimus dorsi muscle suffers a little, but there are no other serious consequences.

- How long does the operation take? How complex is it?

This is a rather delicate operation, since the isolation of the muscle must take place while preserving the vascular and nerve bundles, followed by the formation of a fixation site. It should only be done by trained professionals. The operation lasts two hours. During the operation, endoscopic techniques are used to harvest and fix the muscle. But this is not a purely endoscopic operation.

- How is the rehabilitation of patients going?

Quite quickly, after 2-3 days the patient can leave the clinic. There will be movement restrictions for about a month.

- What exactly are the restrictions? Do I need to wear compression garments?

Nothing special is needed.

- Is the treatment method different for adults and children?

- If the heart beats under the skin, is it dangerous to live until the age of 18?

This problem is relevant, here you can think about additional meshes that strengthen the body. But contacts and competitive sports are not desirable for such patients.

- Can they fall without consequences or sleep on their stomachs?

They can sleep on their stomach. Only impacts to this area should be avoided.

- Are there cases when a person has missing ribs, but you do not advise him to undergo surgery?

There are patients who survived safely to adulthood, for example, their parents did not pay attention. Then we weigh the pros and cons. This depends on the individual wishes of the patient.

On the one hand, it is better to provide additional protection. On the other hand, if the patient is used to sparing himself and avoids playing sports, then in general he can live quite well with this condition.

- The operation to correct the ribs is performed differently than a muscle transplant from the back. What is it?

Let's talk about the ribs separately. There are different methods for operating ribs; you can use an extra anatomical technique, when the rib is placed diagonally as an obstacle. This is where the creativity of reconstructive surgery comes into play. The established practice of performing surgery in the absence of ribs is to replace them. This may involve transplanting part of the ribs from the opposite side, or using a technique for splitting existing ribs and creating new contacts with the sternum. But in general, there is no ideal method.

- Is it carried out equally in men and women, in adults and children?

In women, everything is solved easier, because the defect of the absence of ribs can be hidden by a breast implant, it can slightly cover the heart. If the operation is performed on children, then the growth of the ribs may be disrupted in the future. If a child has a problem with the ribs, but a symmetrical chest, then by performing rib plastic surgery, we can lead to disruption of the growth of the ribs. I have observed patients who had this operation in childhood and, as a result, now have problems due to impaired growth of the ribs on one side. Maybe if they weren't touched, the chest would develop more correctly.

- What age do you consider the most optimal for a child? To both avoid harm and protect?

This problem is very relevant. It cannot be said that in medicine it has been completely resolved; it is constantly being discussed. Previously, it was believed that cleft ribs should be done at 3-4 years of age, but now a later age is recommended. By school age a frame should be made. But it’s hard to say what to choose: limit yourself to a dense mesh for this period of growth or do splitting ribs or transplanting ribs. Let's look at the situation.

Nowadays, during such an operation, they try to use implants rather than their own tissues. Let's say the problem is protecting the heart, that is, the absence of muscles and ribs, in which case we can consider the use of special synthetic materials that will replace and strengthen the chest wall. These can be special meshes or artificial ribs, which are made of titanium and allow you to install sufficient protection without injuring healthy ribs.

- Why do healthy ribs “catch”?

To the sternum from above. Previously, when the 3rd and 4th ribs were missing, the 2nd and 5th ribs were split. So, 2 was used to partially replace the third, and 5 was used to partially replace the fourth. As a result, instead of two defective ribs, four defective ribs were obtained. Directly this may be a good result, but in the long term we ended up with suffering healthy ribs.

- There is no material that increases over time, i.e. not titanium, but something more mobile?

No, there are no such technologies, maybe something will be invented in the future. Titanium is lightweight and does not ring the metal detector. A person with such ribs does not experience any stress.

- Are rib replacement and muscle transfer operations different from the point of view of patient recovery?

Yes, after transposition of the ribs, the patient remains in the hospital for at least two weeks, and scar formation occurs within several months.

After rib surgery, there are restrictions on activity. You can raise your arms and sleep on your stomach. But you should avoid hitting the operated area.

- Is this disease transmitted genetically?

Poland syndrome is considered a genetic pathology and can therefore be passed on to children.

- Summarizing everything you said, it turns out that if we are talking about aesthetics, then it is better for men and women to differentiate the decision, and if the function is affected, then surgery is important, but you need to choose the right age in order to protect the internal organs now and not harm them in the future. There is a logic of medical indicators. It is worth living with this problem until you are very old, but avoiding collisions.

Yes, that's exactly right

- Thank you, Vladimir Alexandrovich.

Make an appointment with Kuzmichev V.A. You can call:

In-person appointments are conducted in Moscow.

Surgery for pectus excavatum

Costomuscular defect (Poland syndrome)

Poland syndrome, or costomuscular defect, is a genetically determined complex of developmental defects, including the absence of the pectoralis major and/or minor muscles, partial or complete fusion of the fingers (syndactyly), their shortening (brachydactyly), absence of the nipple (ately ) and/or the mammary gland itself (amastia), deformation or absence of several ribs, a decrease in the thickness of the subcutaneous fat layer and lack of hair in the armpit area.

This birth defect occurs twice as often on the right side. If the left side of the chest is affected, organ transposition often occurs.

The skin on the affected side may be too thin, without fatty tissue. Very rarely, there may be a defect in the development of the scapula or forearm bones on the affected side. In some cases, congenital defects of the kidney and spine have been described in patients with Poland syndrome. Mental development does not suffer with this syndrome.

The individual components of this syndrome were first described by Lallemand (1826) and Frorier (1839), but it was named after Alfred Poland, an English medical student who in 1841 gave a partial description of this deformity. A complete description of the costomuscular defect was published by Thompson 54 years later, in 1895.

In the vast majority of cases (about 80%), Poland syndrome is right-sided. The left-sided variant is sometimes associated with a reverse arrangement of internal organs, ranging from dextracardia, when only the heart is localized on the right, and up to the full form of the reverse (mirror) arrangement of internal organs. Deformation of the chest in this case varies from mild hypoplasia to congenital absence (aplasia) of costal cartilage or even entire ribs on the side corresponding to the lesion.

In the diagnosis of Poland syndrome, X-ray diagnostic methods are used, as well as computed tomography and magnetic resonance imaging. An ultrasound may also be performed. These methods help assess the presence of changes in the anatomy of internal organs and the affected area, which is necessary for surgeons to perform reconstructive interventions.

Treatment of Poland syndrome is surgical and is aimed, firstly, at eliminating the rib defect and restoring the integrity of the bone frame, secondly, it should eliminate the existing retraction inside the half of the chest, and, thirdly, create normal anatomical relationships of soft tissues, including nipple modeling, breast prosthetics in women and muscle plastic surgery in men.

The result of the first stage of surgical correction of Poland syndrome

(elimination of pectus excavatum)

An 18-year-old man with grade 2 VDHA, asymmetrical shape, aplasia of the pectoral muscles and mammary gland (right).

Photos before surgery and results 7 months after surgery

One year after surgery, a pectoral implant is planned to be installed (right)

Funnel chest plastic surgery was performed by Dr. med. Rudakov S.S. and Ph.D. Korolev P.A.

Technically, these are very complex operations that are performed at an early age. As a rule, a whole series of interventions is performed, since it is almost impossible to obtain a good result at once - this is usually associated with an unjustified increase in the duration of the intervention and an increase in its traumatic nature.

According to the plan, the bone deformity is first eliminated and the rib defect is replaced, and only then the transition to the next stages is carried out. Sometimes, in severe cases, autologous rib transplantation may be necessary. In case of hand anomalies (fused fingers), orthopedic surgeons are involved, in case of problems with internal organs, relevant specialists are involved.

Today, several options for thoracoplasty are used. For example, in case of right-sided Poland syndrome and primary damage to two or three ribs, transposition of the underlying ribs is performed to close the bone defect of the chest. In case of agenesis of three ribs, it is possible to perform a modified Sulamaa operation, and in the absence of four ribs (from the third to the sixth), autotransplantation of a musculoskeletal flap is performed, removed from the contralateral, healthy side using microsurgical techniques. In the isolated absence of the pectoralis major muscle, in some cases it is replaced with the serratus anterior muscle on a neurovascular pedicle.

Make an appointment for a consultation (operation) with a thoracic surgeon, Ph.D. Korolev P.A. Can:

Types of rib anomalies. Pathologies of the chest. Read more.

Treatment of VDHA for Marfan syndrome. They operated on Prof. Rudakov S.S. and Ph.D. Korolev P.A. Read more.

Photo of the results of surgery for carinatum deformity. They operated on Prof. Rudakov S.S. and Ph.D. Korolev P.A. Read more.

PHOTO of the patient before and after correction of Poland's syndrome. They operated on Prof. Rudakov S.S. and Ph.D. Korolev P.A. Read more.

The method of treating VDHA, developed by MD Rudakov S.S. and Ph.D. Korolev P.A. surgery using cosmetic incisions with fixation of the sternocostal complex with a metal plate with shape memory effect. Read more.

Treatment of keeled chest deformity. Thoracoplasty according to M. Ravitch. Photos before and after surgery. More details

The chest is a natural shield that covers the vital structures located underneath it - the heart, lungs and large vascular trunks, which must be protected from various types of injuries and damage. Read more.

Make an appointment with a doctor:

An English pathologist described this syndrome back in 1841. However, there are still isolated cases in the medical literature (only 500 cases have been published worldwide since the first description of this pathology), so there are certain difficulties in diagnosing this syndrome. According to the classification, it belongs to the group of non-hereditary syndromes with the presence of multiple congenital malformations.

An epidemiological analysis of the described 50 worldwide cases of Poland syndrome shows that the frequency of occurrence between the sexes is absolutely the same. This confirms the hypothesis that this pathology is in no way linked to the sex chromosome. However, to date, the type of inheritance of Poland syndrome is unknown. But it has been revealed that if one of the parents has this pathology, the risk of its occurrence in the child is almost 50%.

Anatomy of Poland syndrome

The reasons for the development of this syndrome are not completely known. However, they are associated with disruption of the normal development of the musculoskeletal system during the period of embryonic ontogenetic development. This is what determines the nature of the anatomical changes that occur.

The main anatomical features of this syndrome are:

• Absence of pectoral muscles on one side of the chest
• Complete absence or insufficient development of the mammary gland (this symptom leads to severe psychological trauma for women)
• Aplasia of the ribs, that is, their absence, especially pronounced in the anterior chest
• Change in the shape of the bone frame of the anterior chest.

Quite often, with Poland syndrome, disorders of the normal structure of the upper limb are also diagnosed. They are expressed in the fact that there is a change in the structure of the arm on the same side as the affected half of the chest. Synbrachydactyly is also often determined, that is, underdevelopment of the fingers (shortening), despite the fact that some may be fused to each other. Fusion is most often observed at the skin level, while the bones are not affected.

Somewhat less frequently, other anatomical manifestations of Poland syndrome are detected, namely:

• Underdevelopment of the latissimus dorsi muscle, leading to asymmetry of its lateral parts
• Absence or incomplete development of the pectoralis minor muscle
• Funnel chest
• Costal hump
• Scoliosis – curvature of the spine
• Underdevelopment of the scapula and clavicle.

At the same time, there is a certain pattern - the absence of a direct correlation between the severity of the existing anatomical defects of the chest and the severity of the violation of the normal structure of the upper limb.

In addition to the cosmetic problem, Poland syndrome is also manifested by the abnormal structure of the subclavian artery - its diameter on the affected side is greater than normal. Therefore, the preconditions are created for disruption of arterial blood flow on the affected side of the chest, since the overall resistance of the vascular wall, necessary for the normal exchange of gases and nutrients between the blood and tissues, is reduced.

Anatomical features of Poland syndrome include the abnormal structure and location of internal organs. Depending on their severity, the patient’s general condition suffers, as vital organs may be involved. From this point of view, the following structural anomalies can be identified:

• Deviation of the heart in one direction or another
• Expanding the boundaries of the heart
• Turning the heart clockwise
• Insufficient development of the lungs on the affected side
• Abnormal kidney structure.

Symptoms

Clinical manifestations of Poland syndrome arise directly from the anatomical features observed in this pathology. Therefore, such patients have a characteristic appearance:

• Asymmetry of the anterior chest
• The soft tissues on the affected side are sunken
• Absence of the anterior wall in the axillary fossa when there is no pectoral muscle
• With aplasia of the pectoral muscle, the mammary gland is underdeveloped, it seems to be sunken
• There is usually no hair in the armpit area
• If you move your arm up, you can see the rudiment of the pectoral muscle. It looks like a connective tissue cord like a “stretched string”
• The shoulder girdle functions normally in these patients
• If the pectoralis minor muscle is also missing, then the existing defect in the chest wall becomes even more pronounced, so the ribs begin to show through, which has an extremely unsightly appearance.

In women, the asymmetry of the chest becomes especially pronounced, as the normal structure of the mammary glands changes. On the affected side, the latter has a reduced size, while it is located higher than the healthy one. There may also be deviations in one direction or another, that is, lateral displacements. But according to statistics, they are less common than vertical ones. It is extremely rare to experience amastia, that is, a complete absence of the mammary gland. This variant of Poland syndrome presents particular difficulties in surgical treatment.

The presence of abnormalities in internal organs localized in the chest cavity leads to the appearance of additional clinical signs. We are talking about the following:

• Heart rhythm disturbances
• Feeling of disturbances behind the sternum
• Periodically occurring pain in the heart area
• Dyspnea
• Tendency to edema in the presence of abnormal kidney structure.

Diagnostics

A diagnostic search for Poland syndrome can reveal various variants of this pathology. They are divided into several groups. To make such a differentiation, as a rule, objective (palpation and visual inspection) and x-ray examination are sufficient. This classification is necessary to determine surgical tactics to help the patient.

So, the ribs can have the following changes:

• There are no changes in the ribs, that is, their structure is normal
• The ribs are underdeveloped, but there is no depression on them
• The shape of the ribs has been changed
• There are bone tissue defects on the ribs.

Regarding the pectoralis major muscle, the following options are distinguished:

• Underdevelopment of the pectoralis major muscle
• The absence of some parts
• Total absence of muscle bundles of this muscle.

The condition of the skin and the thickness of the subcutaneous fat layer is another criterion:

• The skin is normal, but the thickness of the subcutaneous fat layer is less than normal
• The skin is thin and there is no subcutaneous fat layer.

The last criterion is the mammary gland, its position and size:

Differential diagnosis is primarily carried out with Mobius syndrome, with which the described pathology is very similar. The latter is based on the lack of full development of the nuclear structures of the facial nerve. Similar signs of these two syndromes are:

• Syndactyly
• Shortening of fingers
• Underdevelopment of the pectoral muscles
• Breast defect.

However, the distinctive features that are characteristic only of the pathology described by Moebius are:

• Mask-like face
• Open mouth
• Eyes wide open
• Absence of facial wrinkles even when a child cries and some others.

Treatment and surgery

The main place in the treatment of Poland syndrome, or congenital costomuscular defect, is given to surgical methods. Their main task is to correct the existing aesthetic problem. However, in most cases, the proposed methods are extremely complex, so not all thoracic and plastic surgeons undertake this operation. In addition, it is worth noting that there is a certain risk of complications from surgical intervention. Thus, its frequency, depending on the complexity of the clinical case, ranges from 10 to 20%.

There are certain indications for surgical treatment of Poland syndrome. They are divided into two main groups:

Medical indications are conditions in which the normal functioning of a person is disrupted due to the presence of a direct threat to his life. These include hypoplasia of the lung due to compression, disruption of the normal location of the heart in the chest cavity. An important criterion in this case is a decrease in cardiorespiratory parameters, which are detected by functional diagnostic methods (spirography, Doppler, bicycle ergometry and others).

The group of cosmetic indications includes the following:

• Severe rib defects that lead to disruption of normal inhalation
• Funnel-shaped structure of the chest, corresponding to the second or third degree of this anomaly.

In practice, as a rule, there is a combination of medical and cosmetic indications. However, surgical treatment is contraindicated in severe diabetes mellitus, hematological malignancies and other diseases, when the risk of surgery is very high.

The main methods of replacing an existing defect and aplasia are the following:

• The rib defect is closed with own bone grafts
• Artificial creation of the mammary gland from skin and muscle flaps.

The difficulty here is that one’s own donor techniques are quite traumatic, as they lead to damage to those areas of the body from which the flap for plastic surgery is taken. In addition, the achieved contour effect is not always maintained for a long time, so there is a real need for repeated plastic surgeries.

Replacement of an underdeveloped or missing pectoral muscle in plastic surgery usually occurs using the latissimus dorsi muscle. However, the problem here is that with Poland syndrome, the latter is also often not fully developed.

Recently, new correction methods have been used to solve the above problems. They are largely associated with the intensive development of polymer chemistry in medicine. This made it possible to create unique endoprostheses of the mammary gland and chest wall.

Endoprosthetics for Poland syndrome is especially indicated in the following cases:

• Defects of one or two ribs
• Stage 1 funnel chest with normal cardiorespiratory parameters
• Pectus excavatum of the second degree, if the patient refuses radical surgery
• Various options for the absence of the pectoralis major muscle
• Breast underdevelopment
• Asymmetry of the chest.

Typically, surgical intervention is carried out in several stages, that is, first one operation, and after a while another (more surgical interventions may be required). The first operation is aimed at eliminating the rib defect and restoring the normal structure of the bone frame of the chest. As a result, this allows you to restore normal chest rigidity. The second operation involves improving the aesthetic result. To do this, the mammary gland is modeled, for example, using endoprostheses (especially in women), and myoplasty is performed. At the same time, the structure of the fingers of the affected upper limb can be corrected - they are lengthened and the skin fold between them is separated.

Which doctor treats

Treatment of Poland syndrome is carried out jointly by doctors of two specialties - a plastic surgeon and a thoracic surgeon. An orthopedic surgeon is involved in cosmetic correction of brachysyndactyly.

A single center for making appointments with a doctor by phone.

Poland syndrome

Considering the location, two types of pathology can be distinguished:

There are also four types of chest development with this defect:

1. the cartilaginous and bone structure of the ribs is not disturbed, the shape of the sternum is not deformed, the deviation is manifested in a lack of soft tissues;
2. the ribs are in normal condition, but the chest cage on one side is turned half-sided, has a sunken cartilaginous region of the ribs, and on the other - a keeled deformity;
3. the costal cartilages are underdeveloped, but all the bones are in satisfactory condition, the sternum is skewed to the painful side;
4. severe displacement of the upper body, absence of one to four rib bones.

Causes

At the moment, the exact cause of Poland syndrome is not known to scientists. The most common explanation is a failure in the migration of cells in the embryonic period, from which muscle fibers are formed or deformation/hypoplasia of the subclavian artery occurs.

No less relevant is the option of exposure to hereditary, chemical, environmental, mechanical, radiation and infectious factors during the intrauterine development of an infant.

Symptoms

Characteristic signs indicating the presence of pathology:

• There is no pectoralis major or minor muscle (hypoplasia, possible aplasia);
• there is no nipple on the body;
• no mammary glands (amastia);
• asymmetry of the chest;
• lack of vegetation under the armpit of the affected side;
• with left-sided syndrome, abnormal placement of internal organs, dextracardia (the heart muscle is located on the right), and poor protection of the heart are sometimes observed, since it can be located directly under the skin;
• the heartbeat can be heard right under the skin;
• reduction in the level of subcutaneous fat;
• deformation or complete atrophy of the cartilaginous layer of the ribs, in some cases of bone formations;
• during puberty in girls, one breast grows slowly or does not grow at all, and in boys, differences in the development of muscle fibers appear when playing sports;
• fusion of fingers (syndactyly);
• abnormally short finger length (brachydactyly);
• the entire hand is small or may not exist at all.

If the heart is not protected by a natural corset and the required amount of tissue, a threat to human life arises, since any blow can provoke cardiac arrest, severe respiratory failure, and disruptions in the circulatory system.

Diagnostics

To confirm the diagnosis, you will need to consult a surgeon. During the examination, diagnostic methods are used:

• X-ray (shows the level of damage to bone structures);
• computer and magnetic resonance imaging (detect the condition of soft and cartilaginous tissues);
• ECG and echocardiography (done to check the functioning of the cardiac system).

Treatment

To treat the disease, you can make an appointment online with a thoracic surgeon. The best traumatologists and orthopedists will help you with hand defects. If there are problems with internal organs, pulmonologists and cardiologists are involved. Cosmetic restoration of normal appearance is performed by plastic surgeons.

The disease can be eliminated only through surgery, and sometimes a whole series of such procedures. Depending on the degree of impairment and the general well-being of the patient, the doctor selects a plastic surgery scheme and the appropriate age for its implementation.

To begin with, the integrity of the natural corset is restored, which usually involves the following: bone tissue transplantation from the healthy half of the patient’s body, transposition of the costal arches located below, removal and correction of a section of the sternum. Currently, various implants are actively used. Then the muscle fibers are reconstructed by transplanting healthy cells from other areas or using a custom silicone substitute. For girls, mammoplasty - imitation of mammary glands - becomes a mandatory procedure. In this case, silicone prostheses are used.

For preschool children, it is possible to install a special mesh that does not prevent further bone growth, but at the same time protects internal organs from external influences and additional symptoms.

Prevention

During pregnancy, a woman should monitor her health, not expose her unborn child to the harmful effects of external factors, and not use harmful substances and drugs.

Poland syndrome is a congenital pathology of the chest, shoulders and palms. The rare disease is diagnosed in 1 newborn out of 30,000. In 70% of cases, the anomaly is found in male infants, in 30% of cases - in female infants. The disease may be associated with dextracardia and mirror arrangement of internal organs. Pathology requires early diagnosis and early surgical intervention, which allows to achieve good results and gives the patient the opportunity to live a full life.

What it is

Poland syndrome is not a genetic disease. Scientists have not discovered the genes that are responsible for the transmission of pathology from parent to child. Doctors suggest that the anomaly is caused by a circulatory disorder in the vertebral and/or subclavian arteries at the 6th week of fetal development.

Poland syndrome is a right-sided pathology characterized by:

• underdevelopment of the chest muscles or their monolateral absence;
• defects of long bones, ribs, mammary glands or kidneys;
• anomaly of the upper limbs;
• pulmonary hernias and severe respiratory disorders.

In ICD-10, pathology is located in section Q65–Q79 “Congenital anomalies of the musculoskeletal system.” The syndrome was assigned the number Q79.8 “Other malformations of the musculoskeletal system.”

Types of disease

Depending on the location of the defect, the disease is conventionally divided into two types:

• right-sided – occurs in 80% of patients;
• left-sided - diagnosed only in 20% of cases.

Left-sided pathology is sometimes accompanied by a mirror arrangement of internal organs or dextracardia, in which the heart is located closer to the right side of the chest.

With a congenital defect, the costochondral frame and muscle corset can develop differently. Depending on the severity of the defect and its characteristic signs, four main stages are distinguished:

• Stage I – natural shape of the ribs, normal structure of cartilage and bone tissue, but there is a lack of muscle and subcutaneous fat layer;
• Stage II - the ribs are developed normally, but sink into the cartilage area, and on the other hand they have a keeled shape, the sternum is turned to the side;
• Stage III - the chest is severely skewed to the right or left due to underdeveloped cartilage tissue, the bones have a normal structure;
• Stage IV - the upper part of the body is greatly displaced, one to four ribs are missing.

Depending on the location, the pathology is divided into deformation of the lateral, posterior or anterior wall.

Causes of the anomaly

The causes of the defect have not yet been studied. Scientists put forward several theories about the origin of Poland syndrome. The most common is vascular. According to this theory, at the 6th week of intrauterine development in the embryo, blood circulation in the subclavian and/or vertebral region is disrupted and hypoplasia of a large artery or its branch occurs.

The diameter of the artery decreases, which leads to a decrease in blood flow speed and oxygen starvation of the tissues for which it is responsible. Tissue hypoxia is the main cause of underdeveloped muscles, mammary glands, subcutaneous tissue, skin and upper limbs. The smaller the diameter of the subclavian artery, the more severe the defect.

Other scientists believe that Poland syndrome occurs due to a failure in the migration of cells from which the rudiments of the muscle corset and osteochondral frame are formed in the embryo.

Doctors are trying to understand what factors lead to hypoplasia of the subclavian artery and failures. Some believe that intrauterine injuries to the fetus are to blame. Others consider chemical, environmental, infectious and radiation causes. There is also a theory about genetic predisposition, but there is no actual evidence of all these hypotheses yet.

Symptoms

Characteristic signs of Poland syndrome include:

• unilateral underdevelopment (hypoplasia) of the pectoralis major muscle;
• unilateral aplasia (absence) of the pectoralis minor or major muscle, usually of the costosternal part;
• deformation of the costal cartilages;
• aplasia or underdevelopment of one breast;
• lack of cartilage and/or bone tissue;
• local baldness of the chest and armpit;
• thinning of the skin and subcutaneous tissue;
• deficiency of body fat;
• atelia – absence of one or two nipples at once;
• deformation of the back muscles, as well as the serratus anterior and external oblique muscles.

The pathology is often accompanied by abnormalities in the development of the hand and arm:

• syndactyly - fusion of several fingers;
• brachydactyly - too short and small fingers;
• lack of a brush;
• abnormally small hand size.

Patients with the left-sided type of the disease may lack ribs that are supposed to protect the heart. The organ is located under a thin layer of skin and subcutaneous tissue, so any, even minor, injury to the chest can provoke disruptions in blood circulation, respiratory failure and cardiac arrest.

In rare cases, newborns are missing almost the entire bone corset. The pathology leads to lung herniation and respiratory failure, which develops at a very early age.

Poland syndrome is diagnosed in the first days or months of life. If the baby is missing ribs, parents may notice a pulsating heartbeat. It is clearly visible through the thin skin. Pathology is also indicated by abnormal development of the scapula or collarbone, funnel-shaped or keeled deformity of the thoracic region, rib hump and unnatural curvature of the spine.

Patients with the first stage of the disease are diagnosed with Poland syndrome in adolescence or adulthood. Girls notice that one breast does not grow, and there is no hair in the armpit located on the side of the defect.

Young men pay attention to their peculiarity when they begin to actively engage in sports and pump up their pectoral muscles. They notice that one side of the chest does not develop, although the other increases in volume and becomes more prominent.

Poland syndrome in the first and second stages does not affect hormonal levels and does not prevent a person from playing sports. Patients with the third and fourth stages of pathology often have impaired blood circulation, problems with blood pressure, disruptions in the respiratory system, and reduced resistance to respiratory diseases.

Children with serious disabilities suffer from chronic fatigue and fatigue, increased irritability, frequent headaches and drowsiness. They often lag behind their peers in development due to decreased ability to work and cognitive functions.

A disorder of arterial blood flow leads to hypoplasia of the kidney and lung located on the defective side, as well as to heart pathologies: clockwise rotation of the organ, expansion of its boundaries, or deviation from the normal location.

Diagnosis of anomaly

The diagnosis of Poland syndrome is confirmed by the surgeon. First, the doctor conducts a visual examination of the chest, then prescribes several examinations:

• X-ray – reveals the degree and type of bone tissue damage;
• computed tomography or MRI - allows you to study the condition of soft tissues and cartilage;
• Ultrasound of the subclavian artery - determines its diameter, helps to identify the characteristics of blood circulation and blood supply to deformed muscles;
• electrocardiography – allows you to diagnose heart pathologies.

• Dopplerography of the great vessels;
• Ultrasound of the heart;
• echocardiography;
• bicycle ergometry.

If there are problems with the lungs or impaired respiratory function, the patient is referred to a pulmonologist and offered to undergo spirography, as well as other additional examinations.

An integrated approach to diagnosis allows doctors to determine the degree of deformation and assess the scope of reconstructive interventions aimed at restoring the chest and improving the patient’s quality of life.

Treatment of Poland syndrome

The congenital anomaly is treated surgically. The type of operation depends on the stage of the pathology.

Treatment of the first stage

Patients with hypoplasia or aplasia of the pectoral muscle are recommended to consult a plastic surgeon and undergo surgery to eliminate the cosmetic defect. Surgical treatment consists of two stages:

1. Creation of a muscular frame and muscle layer. Doctors use the latissimus dorsi muscle for the procedure, and less commonly, the rectus abdominis muscle.
2. Implantation of a silicone prosthesis and nipple formation.

The operation is performed on both men and women. Patients with a displaced muscle are not prohibited from playing sports. The transplanted organ will not be able to fully develop, but this does not prevent men and women from achieving success in various sports.

An operation to eliminate a cosmetic defect is performed after 18–20 years. In childhood and adolescence, resorting to surgical intervention is pointless. The transplant may slow down the development of the chest and worsen the defect.

A surgical solution to the problem at an early age is advisable only for syndactyly. The child will need to consult an orthopedic surgeon. The doctor will develop the optimal treatment strategy for Poland syndrome and reconstruct the fused fingers.

If the anomaly does not interfere with the patient, plastic surgery may not be performed. Transplantation of the latissimus dorsi muscle does not affect the spine, but leads to slight asymmetry and scar formation in the armpit, from where the material is taken, so before the procedure you should carefully consider all its pros and cons.

Treatment of the second and third stages

Severe chest deformity is an indication for Nuss thoracoplasty. The minimally invasive procedure is usually performed in early or teenage years, but is not contraindicated in adult patients with Poland syndrome.

The operation increases the volume of the pleural cavities, triggers regression of respiratory disorders and is recommended for heart diseases caused by pectus excavatum.

Surgical treatment is performed under general anesthesia. The doctor makes several small incisions through which introducers - metal plates - are inserted into the chest. Structures made of titanium or steel pass under the ribs and give the bone tissue the correct shape.

Chest deformation can also be corrected with silicone or hydrogel endoprostheses. Implants visually reduce the defect and protect the heart from injury.

Treatment of the fourth stage

If one or more ribs are missing, it is worth resorting to reconstructive surgery. There are three main treatment methods:

• installation of a mesh frame or titanium ribs to protect the heart;
• splitting of healthy ribs located above and below the deformed area;
• transplantation of ribs from a healthy part of the chest.

The first option is recommended for children of preschool and school age. The mesh frame and titanium plates do not interfere with the normal development of the chest, while splitting and transplantation lead to serious complications and problems in adulthood.

The second and third methods are recommended for adult patients with Poland syndrome, although they raise many questions. Often, splitting of the ribs leads to their weakening, which is why modern surgery gives preference to metal implants and synthetic frames that replace muscle tissue.

Surgical patients are allowed to sleep on their stomachs and raise their arms, but are advised to avoid hitting the surgical area.

There is no prevention for Poland syndrome. Neither giving up bad habits, nor a balanced diet for parents, nor playing sports can prevent pathology, but children with the anomaly can become full-fledged members of society and live to a ripe old age. The main thing is to promptly seek help from a surgeon and follow all the specialist’s recommendations.