Poland's syndrome or rib muscle defect. Chest deformities. Poland syndrome. Causes of Poland's syndrome

Poland syndrome(rib-muscular defect of the chest) is a combination of aplasia of the pectoralis minor muscle, hypoplasia of the sternal part of the pectoralis major muscle and hypoplasia of the cartilaginous sections of the 3rd, 4th and 5th costal cartilages. In girls, among other things, there is a sharp underdevelopment or complete absence of the mammary gland. True Poland's syndrome is additionally characterized by underdevelopment of the upper limb on the side of the lesion in the form of its shortening or syndactyly. The defect is most often found on the right, the left-sided Poland syndrome is often accompanied by one form or another of the reverse location of the internal organs.

It is believed that the basis Poland syndrome lies congenital underdevelopment of the vessels of the axillary artery. The inheritance of this developmental defect is not typical. The clinical significance of Poland's syndrome is due to the paradox of inhalation (pulmonary hernia), which often manifests itself in early childhood, cosmetically disadvantageous asymmetry of the chest and scoliosis caused by the asymmetry of the pectoral muscles. This is also an indication for surgical correction.

Methods for closing a rib-muscular defect of the chest can be divided into three groups: bone, muscle and alloplasty of a chest wall defect. The purpose of bone grafting is to strengthen the chest wall to repair a pulmonary hernia. It is practiced to move free bone grafts (ribs) or split ribs on the periosteal pedicle into the defect of the chest wall. The operation options we use for wide (a) and narrow chest (b). The use of these techniques allows you to reliably strengthen the chest wall.

Muscular plastic surgery of the rib-muscular defect of the chest wall (by moving the latissimus dorsi muscle on the vascular pedicle) proved to be ineffective. In all cases, atrophy of the displaced muscle occurs and the result of the operation is reduced to nothing. The best cosmetic results of Poland's syndrome correction were obtained using modern alloplastic materials. This type of surgery is performed only on adult patients. In men, for the correction of Poland's syndrome, we use monolithic, textured pectoral silicone implants, which allow us to obtain a good configuration of the chest.

- this is a complex of malformations, including the absence of a small and / or large pectoral muscle, a decrease in the thickness of the layer of subcutaneous fatty tissue in the chest area, the absence or deformation of several ribs, the absence of a nipple or mammary gland, shortening of the fingers, complete or incomplete fusion of the fingers, and also the absence of hair in the armpit area. The defect is unilateral, more often observed on the right. The severity of violations can vary greatly. The diagnosis is made on the basis of clinical data, radiography, MRI and other studies. Treatment is usually surgical - thoracoplasty, correction of the defect with grafts, cosmetic interventions.

ICD-10

Q79.8 Other malformations of the musculoskeletal system

General information

Poland's syndrome is a fairly rare congenital malformation. It is detected in one of 30-32 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, familial 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.

Causes

Experts in the field of thoracic surgery, traumatology and orthopedics suggest 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 with intrauterine damage or hypoplasia of the subclavian artery. None of these theories has yet received reliable confirmation.

Pathogenesis

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

On the defective side, the absence of the mammary gland (amastia), the absence of the nipple (atelia) and the absence of hair in the armpit can also be detected. In 80% of cases, a complex of defects is detected on the right side. With the left-sided variant of Poland's syndrome, the reverse arrangement of the internal organs is sometimes found - from dextrocardia, in which the heart is on the right, and the remaining organs remain in place, to a mirror arrangement, in which there is a reverse (mirror) localization of all organs.

With a left-sided variant of the disease, a normal location of the heart and severe hypoplasia of 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 life of the patient, since any blow can cause serious injury and cardiac arrest. In other cases, the consequences are not so serious and can vary from a deterioration in respiratory and circulatory functions due to chest deformity to a purely cosmetic defect due to the absence of muscles and / or breasts.

Symptoms

The symptoms of Poland's syndrome, as a rule, are clearly visible even to a non-specialist and are usually detected by parents in the first days of a baby's life. The asymmetry of the chest, the absence or insufficient development of muscles and the underdevelopment of subcutaneous adipose tissue on the one hand are characteristic. 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 diseased side does not grow or lags behind in growth. In some cases (with muscle hypoplasia in the absence of other defects) in boys, Poland's syndrome is diagnosed only in adolescence, when, after playing sports, patients go to the doctor because of the asymmetry that has arisen between the "inflated" normal and hypoplastic pectoral muscles.

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

The third variant is characterized by hypoplasia of the costal cartilages with the preservation of the bony part of the ribs. The chest is somewhat "skewed", the sternum is slightly tilted to the affected side, but no gross deformation is observed. In the fourth variant, the absence of both the cartilaginous and bone parts of one, two, three or four ribs (from the third to the sixth) is found. The ribs on the affected side sink in, a pronounced rotation of the sternum is revealed.

Diagnostics

To confirm the diagnosis and determine the tactics of treating Poland's syndrome, a number of instrumental studies are carried out. On the basis of 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 the internal organs are suspected, consultations with a cardiologist and a pulmonologist are prescribed, a study of the function of external respiration, ECG, EchoEG and other studies is carried out.

Treatment of Poland's syndrome

Treatment is operative, usually started at an early age, by plastic and thoracic surgeons. The volume of therapeutic measures depends on the presence and severity of certain pathological changes. So, with a gross pathology with the absence of ribs and deformity of the chest, a number of phased surgical interventions may be required to ensure the safety of internal organs and normalize external respiration. And in the isolated absence of the pectoral muscle and the normal shape of the chest, the only purpose of the operation is to eliminate a cosmetic defect.

If necessary, to correct hand anomalies (for example, to eliminate syndactyly), traumatologists and orthopedists are involved. In the presence of pathology from 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.

An English pathologist described this syndrome 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's 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, the mode of inheritance of Poland's syndrome has not yet been established. But it was found that if one of the parents has this pathology, the risk of its occurrence in a child is almost 50%.

Anatomy in Poland syndrome

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

The main anatomical features in this syndrome are:

Lack 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 the female)
Aplasia of the ribs, that is, their absence, especially pronounced in the anterior chest
Change in the shape of the bone skeleton of the anterior chest.

Quite often, with Poland's syndrome, violations 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 hand 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 can be fused with each other. The fusion is most often observed at the level of the skin, while the bones are not affected.

Other anatomical manifestations of Poland's syndrome are somewhat less common, namely:

Underdevelopment of the latissimus dorsi muscle, leading to the asymmetry of its lateral parts
Absence or incomplete development of the pectoralis minor muscle
Funnel chest
costal hump
- rachiocampsis
Underdevelopment of the scapula and collarbone.

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's syndrome is also manifested by the abnormal structure of the subclavian artery - its diameter on the side of the lesion is greater than normal. Therefore, prerequisites are created for the violation of arterial blood flow on the affected side of the chest, as the overall resistance of the vascular wall decreases, which is necessary for the normal exchange of gases and nutrients between blood and tissues.

Anatomical features in Poland's syndrome are also in the abnormal structure and location of the internal organs. Depending on their severity, the general condition of the patient 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
Expansion of the heart boundaries
Rotate the heart clockwise
Underdevelopment of the lungs on the side of the lesion
Abnormal structure of the kidneys.

Symptoms

The clinical manifestations of Poland's syndrome follow directly from the anatomical features observed in this pathology. Therefore, these patients have a characteristic appearance:

Asymmetry of the anterior chest
Soft tissues on the side of the lesion are sunken
Absence of the anterior wall in the axilla when there is no pectoral muscle
With aplasia of the pectoral muscle, the mammary gland is underdeveloped, it seems to be sunken
Usually there is no hair in the armpit
If you take your hand 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 chest wall defect becomes even more pronounced, so the ribs begin to shine through, which has an extremely ugly appearance.

In women, the asymmetry of the chest becomes especially pronounced, as the normal structure of the mammary glands changes. On the side of the lesion, the latter has a reduced size, while it is located higher than healthy. 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. Extremely rarely, amastia, that is, the complete absence of the mammary gland, can also be noted. This variant of Poland's syndrome presents particular difficulties in surgical treatment.

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

Heart rhythm disorders
Feeling of tightness in the chest
Intermittent pain in the region of the heart
Dyspnea
Tendency to edema in the presence of an abnormal structure of the kidneys.

Diagnostics

Diagnostic search for Poland's syndrome can reveal various variants of this pathology. They are divided into several groups. To carry out such a differentiation, as a rule, an objective (palpation and visual examination) and x-ray examination is sufficient. This classification is necessary to determine the surgical tactics of patient care.

So, edges 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 impression on them
The shape of the ribs has been changed
There are bone defects on the ribs.

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

Underdevelopment of the pectoralis major muscle
Missing some parts
The 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, the thickness of the subcutaneous fat layer is less than normal
The skin is thin, and the subcutaneous fat layer is absent.

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

The gland is of normal size and occupies a typical location
The gland is reduced in size
Complete underdevelopment of the mammary gland and violation of the attachment site - lateral or vertical dystopia
The complete absence of the rudiment of the mammary gland, including the nipple, on the side of the lesion.

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 complete development of the nuclear structures of the facial nerve. Similar signs of these two syndromes are:

syndactyly
Finger shortening
Underdevelopment of the pectoral muscles
Breast defect.

However, the distinguishing features that are characteristic only for the pathology described by Möbius are:

Masculinity of the face
Open mouth
wide open eyes
The absence of facial wrinkles even when the baby is crying and some others.

Treatment and operation

The main place in the treatment of Poland's syndrome, or a congenital rib-muscular 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 should be noted that there is a certain risk of complications of surgical intervention. So, its frequency, depending on the complexity of the clinical case, ranges from 10 to 20%.

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

Medical
Cosmetic.

Medical indications are conditions in which the normal functioning of a person is disturbed due to the presence of a direct threat to his life. These include lung hypoplasia due to compression, a violation 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 inspiration
The 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, hemoblastosis and other diseases, when the risk of surgery is very high.

The main methods for replacing an existing defect and aplasia are as follows:

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

The difficulty here lies in the fact that own donor techniques are quite traumatic, as they lead to damage to those parts of the body from which the flap is taken for plastic surgery. In addition, the achieved contour effect is not always preserved 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 with the help of the latissimus dorsi muscle. However, the problem here is that with Poland's syndrome, the latter is also often not fully developed.

Recently, new corrective methods have been used to solve the above problems. In many ways, they are 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 in Poland's syndrome is especially indicated in the following cases:

Defects in one or two ribs
Funnel chest of the first degree with normal cardiorespiratory parameters
Funnel chest of the second degree, if the patient refuses radical surgery
Various options for the absence of the pectoralis major muscle
Underdevelopment of the breast
Asymmetric chest.

Usually, surgery 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 defect of the ribs and restoring the normal structure of the bone skeleton 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, with the help of endoprostheses (especially in women), myoplasty is performed. At the same time, the structure of the fingers of the affected upper limb can be corrected - they lengthen and the skin fold between them is separated.

Which doctor treats

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

The defect is unilateral, more often observed on the right. The severity of violations can vary greatly. The diagnosis is made on the basis of clinical data, radiography, MRI and other studies. Tactics of treatment is determined individually.

Poland syndrome

Poland's syndrome is a fairly rare congenital malformation. Revealed in one of the 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, familial 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 with intrauterine damage or hypoplasia of the subclavian artery. None of these theories has yet received reliable confirmation.

Pathological anatomy in Poland syndrome

Symptoms and diagnosis of Poland's syndrome

To confirm the diagnosis and determine the tactics of treating Poland's syndrome, a number of instrumental studies are carried out. On the basis of 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 the internal organs are suspected, consultations with a cardiologist and pulmonologist are prescribed, a study of the function of external respiration, ECG, EchoEG and other studies is carried out.

Treatment of Poland's syndrome

Treatment of Poland syndrome is surgical, usually begins at an early age, is carried out by thoracic and plastic surgeons. The volume of therapeutic measures depends on the presence and severity of certain pathological changes. So, with a gross pathology with the absence of ribs and deformity of the chest, a number of phased surgical interventions may be required to ensure the safety of internal organs and normalize external respiration. And in the isolated absence of the pectoral muscle and the normal shape of the chest, the only purpose of the operation is to eliminate a cosmetic defect.

If necessary, to correct anomalies of the hand (for example, to eliminate syndactyly), traumatologists and orthopedists are involved. In the presence of pathology from 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's syndrome is the elimination of bone deformity and the replacement of a defect in the ribs. There are several methods of thoracoplasty. With right-sided localization of the defect and the absence of two or three ribs, the underlying ribs are transposed. When 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 performed using implants made from special inert synthetic materials. In some cases, at preschool age, a special mesh is installed in the area of the defect of the ribs, which protects the internal organs without interfering with the normal growth of the ribs - this tactic reduces the likelihood of secondary deformities of the chest due to the uneven growth of the patient's operated and non-operated ribs.

With 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, an important part of the treatment is reconstructive mammoplasty - the elimination of a cosmetic defect resulting from the underdevelopment or absence of the mammary gland. To restore the anatomical relationships between the soft tissues, the movement of the latissimus dorsi muscle is used, and some time after the wound has healed, a silicone prosthesis of the mammary gland is installed.

Poland syndrome

Poland's syndrome is an anomaly in the development of the chest due to the absence or underdevelopment of the pectoral muscles. These pathologies are formed during fetal development and can affect not only muscles, but also bones. In medical practice, there were cases when 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 damaged side of the body, underdeveloped or even absent hand, complete or partial absence of fingers. Outwardly, 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's 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 intrauterine trauma or hypoplasia of the subclavian artery may be the cause of this anomaly, but the reliability of these data has not been officially confirmed.

Symptoms

Poland syndrome is a rare defect, according to statistics, about 1 child in 32 thousand newborns is born with it. Parents can notice this pathology in a child almost immediately after birth. Outwardly, 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's 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's syndrome is also characterized by the pathological structure of the subclavian artery and blood vessels. The artery in diameter exceeds the norm, which leads to impaired 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:

Anomaly in the development of muscle and adipose tissue, while the development of bone and cartilage structures is not disturbed.
Chest deformity. The bones and cartilages of the costal part are preserved, but deformed. The chest is usually subject to keeled deformation.
Anomaly in the development of soft tissues and cartilage in the absence of a violation of the development of the bone structure of the ribs.
Underdevelopment or complete absence of the bone part of the ribs in violation of the development of muscles and cartilage.

Very often, Poland's syndrome manifests itself in the form of a small cosmetic defect and becomes noticeable only at a transitional age, when the mammary glands begin to develop in girls, and in boys the affected side of the chest does not acquire a raised, inflated shape, unlike the healthy half of the chest. Poland's 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's syndrome:

x-ray examination. Allows you to determine the presence of a 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 with suspicion of damage to the internal organs of the chest.

Treatment

For the treatment of this condition, only surgical intervention 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 can be performed:

Surgery to restore the bone structure of the ribs,
Surgery to restore the shape of the chest (elimination of deformity),
Surgery to restore muscle tissue, breast implantation, nipple shaping,
Surgery 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 the operation to restore the bone part of the ribs is performed at an early age, then a special dense mesh is installed as the rib wall, which does not prevent 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 uneven development of healthy and transplanted ribs.

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

After the operation, a course of antibiotics is prescribed. It is not prohibited to use folk methods to improve metabolism, but this can be done only after consulting with your doctor.

The prognosis after surgery is very favorable. Elimination of a cosmetic defect that does not affect the functioning of the 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's syndrome. However, if parents do not abuse bad habits, lead a healthy and active lifestyle, undergo examinations and necessary treatment in a timely manner, this increases the possibility of having a healthy child.

Poland Syndrome: Causes and Treatment Options

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Problems with the physique or body structure are different. Today we want to tell our readers about Poland's syndrome, which is one of the pathologies of the body structure. Our questions were answered by a thoracic surgeon, Ph.D. - Vladimir Alexandrovich Kuzmichev.

- Vladimir Alexandrovich, it is probably best to start with a story about what Poland's syndrome is? How is its presence manifested in human physiology and the functioning of his body?

Poland Syndrome is a pathology that externally manifests itself in the form of a 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, 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 side of the lesion. 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, some degeneration of adipose tissue is observed. A more common symptom of Poland's syndrome is the underdevelopment of two parts of the pectoralis major muscle. The pectoralis major muscle consists of three parts: the subclavian part, the sternum part and the costal part. As a rule, the sternum and costal parts are absent. Or the pectoralis major muscle is completely absent. All other signs are rarer, so patients with Poland syndrome often have an absolutely complete hand.

- Is the side of the lesion always the same or is there variability?

Where did the name of the syndrome come from? Does it have anything to do with Poland?

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

- How to diagnose the presence of this disease?

In this case, the diagnosis is very simple - by the appearance of the patient. I must say that this condition is diagnosed at an early age according to the characteristic state of the pectoral muscle in the shoulder area. If there is no pectoral muscle, then the armpit has a very specific appearance. It is in such cases that parents turn to the question of what to do.

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

Firstly, the appearance of the child, the lack of muscle, as well as the beating of the heart right under the skin, if there is a lack of ribs, worries.

- Does the detection of the syndrome leave any imprints on the upbringing of the child in terms of limiting his activity or prohibiting any kind of activity?

In most cases, patients may not pay attention to their syndrome, they develop well, despite the absence of the pectoral muscle, 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 those with high achievements. When there is no chest involvement, the problem is purely cosmetic and should be addressed jointly by plastic and thoracic surgeons.

- Do men and women have the same problem? Are there any differences?

Yes, the solution to a cosmetic problem has a different meaning for men and women. In the case of women, there is an underdevelopment of the mammary gland, while in men, the nipple may simply be underdeveloped. In medicine, this is called the Amazon Syndrome, because, as you know, these girls were actively archery and disrupted the growth of one of the mammary glands. Since the mammary glands play an important aesthetic and functional role for women, there are more frequent visits to the doctor to correct the defect on their part.

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

No, it does not, the problem is aesthetic in nature. The woman is absolutely healthy.

- What are the methods of correcting this defect? Do they always require surgery?

The method of solving the problem is an operation that can be performed using various techniques, depending on the totality of the signs of the disease. For example, in women, there may be a movement of the latissimus dorsi muscle from the back forward with the creation of a muscular frame and a muscular layer, which subsequently makes it possible to implant a silicone prosthesis to create a breast. Or more complex options are used, for example, transplantation of the rectus abdominis muscle. Everything is decided depending on each specific case.

- 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 insecurity of internal organs, including the heart. For men, the elimination of a cosmetic defect is completely impossible. So, the displaced back muscle does not become full-fledged, while the back suffers, from where a piece of muscle is taken. In the future, the back will also become slightly asymmetrical. In other words, there can be no full-fledged cosmetics, but there is a violation of the body's function. 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 underdevelopment of fingers on one hand, is the presence of the syndrome diagnosed only when undressed? Or does the shape of the sternum leave an imprint on the appearance in clothes?

No, in clothes the absence of the pectoral muscle is imperceptible. The presence of the syndrome in this case is diagnosed only in the undressed form. Therefore, many men do not go to the doctor for help. The absence of the pectoral muscle is not noticeable under clothing, however, many of them are embarrassed to undress, as 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 does not train and does not build up?

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

- And 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-fledged branches in this area.

- It is impossible to influence an 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 for sure. The operation is indicated only if there is a pathology of the ribs. Another thing for women, when the operation is done for the subsequent implantation of the breast and significantly improves the aesthetic perception.

- Does a muscle transplant from the back affect the spine in any way?

No, the latissimus dorsi has a connection only with the shoulder girdle, it has nothing to do with the spine. The only thing that will be is a certain scar in the armpit (the place where the muscle was taken and the place of transplantation are the same scar) and some asymmetry.

- Can there be a negative effect from the operation, any irreversible violation?

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

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

This is a rather delicate operation, since the isolation of the muscle must take place with the preservation of the vascular and nerve bundle, followed by the formation of a fixation site. It should only be done by trained professionals. The operation takes two hours. During the operation, endoscopic techniques are used for the collection and fixation of the muscle. But this is not a purely endoscopic operation.

How is patient recovery 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 underwear?

Nothing in particular is needed.

- Is the method of treatment of adults and children different?

- If the heart beats under the skin, is it not dangerous to live until the age of 18?

This problem is relevant, here you can think about additional nets that strengthen the body. But such patients do not want contacts, competitive sports.

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

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

- Are there cases when a person has no ribs, but you do not advise him to have an operation?

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

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

- The operation associated with the correction of the ribs is different than the transplantation of a muscle from the back. What is it?

Let's talk about ribs separately. There are different methods of rib surgery, you can use an extra anatomical technique when a rib is placed obliquely as an obstacle. This is where the creativity of reconstructive surgery takes place. The established practice of performing an operation in the absence of ribs is their replacement. This may be a transplant of part of the ribs on the opposite side, the technique of splitting existing ribs and creating new contacts with the sternum can be applied. But in general, there is no ideal method.

Is it the same for men and women, adults and children?

In women, everything is solved more easily, because the defect of the absence of ribs can be hidden by a breast implant, it can close the heart a little. If the operation is performed on children, then the growth of the ribs may subsequently be disturbed. If the child has a problem with the ribs, but a symmetrical chest, then performing rib plastic surgery, we can lead to disruption of the growth of the ribs. I had to observe patients who underwent such an operation in childhood and, as a result, now they have problems due to rib growth disorders on one side. Maybe if they had not been touched, the chest would have developed more correctly.

What age do you think is the best for a child? In order not to harm, and to protect?

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

Now, during such an operation, they try not to use their own tissues, but implants. Suppose the problem is to protect the heart, that is, the lack of muscles and ribs, in which case the use of special synthetic materials that will replace and strengthen the chest wall can be considered. These can be special meshes or artificial ribs, which are made of titanium and allow you to install sufficient protection without injuring healthy ribs.

- And what do healthy ribs “cling to”?

Above the chest. Previously, when ribs 3 and 4 were missing, ribs 2 and 5 were split. So, the 2nd went to a partial replacement of the third, and the 5th to a partial replacement of the fourth. As a result, four defective ribs were obtained instead of two defective ribs. This may be a good result immediately, but in the long run we got suffering healthy ribs.

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

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

- Are rib replacements and muscle grafts different in terms of patient recovery?

Yes, after transposition of the ribs, the patient stays in the hospital for at least two weeks, and the formation of scars occurs within a few months.

After surgery on the ribs, restrictions on activity are imposed. You can raise your arms and sleep on your stomach. But blows to the operated area should be avoided.

Is this disease genetically transmitted?

Poland syndrome is considered a genetic pathology, therefore it can be transmitted 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 the operation is important, but you need to choose the right age in order to protect the internal organs now and not harm in the future. There is a logic of medical indicators. With this problem it is worth living to a ripe old age, but avoiding collisions.

Yes, absolutely right

- Thank you, Vladimir Alexandrovich.

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Surgery for pectus excavatum

Rib-muscular defect (Poland's syndrome)

Poland syndrome (Poland), or a rib-muscular defect, is a genetically determined complex of malformations, 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 (atelia ) and / or the mammary gland itself (amastia), deformity or absence of several ribs, a decrease in the thickness of the subcutaneous fat layer and the absence 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 side of the lesion may be too thin, without fatty tissue. Very rarely, there may be a defect in the development of the scapula or bones of the forearm on the side of the lesion. In some cases, congenital defects of the kidney and spine have been described in patients with Polland's syndrome. Mental development in this syndrome does not suffer.

Separate 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 rib-muscular defect was published by Thompson 54 years later - in 1895.

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

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

The treatment of Poland's syndrome is surgical and is aimed, firstly, at eliminating the defect of the ribs and restoring the integrity of the bone frame, secondly, it must eliminate the existing retraction inside half of the chest, and, thirdly, to create normal anatomical relationships of soft tissues, including nipple modeling, breast prosthetics in women and muscle plasticity in men.

The result of the I-stage of surgical correction of Poland's syndrome

(elimination of funnel chest deformity)

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

Photos before surgery and the result 7 months after surgery

A year after the operation, a pectoral implant is planned (right)

Funnel-shaped chest plasty 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 practically impossible to get a good result at once - usually this is associated with an unjustified increase in the duration of the intervention and an increase in its trauma.

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

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

Varieties of anomalies of the ribs. Pathology of the chest. More.

Treatment of VDHK in Marfan's syndrome. Operated by Prof. Rudakov S.S. and Ph.D. Korolev P.A. More.

Photo of the results of the operation for keeled deformity. Operated by Prof. Rudakov S.S. and Ph.D. Korolev P.A. More.

PHOTO of the patient before and after the correction of Poland's syndrome. Operated by Prof. Rudakov S.S. and Ph.D. Korolev P.A. More.

The method of treatment of VDHK, developed by Dr. Rudakov S.S. and Ph.D. Korolev P.A. an operation from cosmetic incisions with fixation of the sternocostal complex with a metal plate with a shape memory effect. More.

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

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

Doctor's note:

Anatomy in Poland syndrome

The main anatomical features in this syndrome are:

Lack 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 the female)
Aplasia of the ribs, that is, their absence, especially pronounced in the anterior chest
Change in the shape of the bone skeleton of the anterior chest.

Other anatomical manifestations of Poland's syndrome are somewhat less common, namely:

Underdevelopment of the latissimus dorsi muscle, leading to the 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 collarbone.

Deviation of the heart in one direction or another
Expansion of the heart boundaries
Rotate the heart clockwise
Underdevelopment of the lungs on the side of the lesion
Abnormal structure of the kidneys.

Symptoms

The clinical manifestations of Poland's syndrome follow directly from the anatomical features observed in this pathology. Therefore, these patients have a characteristic appearance:

Asymmetry of the anterior chest
Soft tissues on the side of the lesion are sunken
Absence of the anterior wall in the axilla when there is no pectoral muscle
With aplasia of the pectoral muscle, the mammary gland is underdeveloped, it seems to be sunken
Usually there is no hair in the armpit
If you take your hand 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 chest wall defect becomes even more pronounced, so the ribs begin to shine through, which has an extremely ugly appearance.

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

Heart rhythm disorders
Feeling of tightness in the chest
Intermittent pain in the region of the heart
Dyspnea
Tendency to edema in the presence of an abnormal structure of the kidneys.

Diagnostics

So, edges 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 impression on them
The shape of the ribs has been changed
There are bone defects on the ribs.

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

Underdevelopment of the pectoralis major muscle
Missing some parts
The 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, the thickness of the subcutaneous fat layer is less than normal
The skin is thin, and the subcutaneous fat layer is absent.

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

syndactyly
Finger shortening
Underdevelopment of the pectoral muscles
Breast defect.

However, the distinguishing features that are characteristic only for the pathology described by Möbius are:

Masculinity of the face
Open mouth
wide open eyes
The absence of facial wrinkles even when the baby is crying and some others.

Treatment and operation

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

The group of cosmetic indications includes the following:

Severe rib defects that lead to disruption of normal inspiration
The funnel-shaped structure of the chest, corresponding to the second or third degree of this anomaly.

The main methods for replacing an existing defect and aplasia are as follows:

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

Endoprosthetics in Poland's syndrome is especially indicated in the following cases:

Defects in one or two ribs
Funnel chest of the first degree with normal cardiorespiratory parameters
Funnel chest of the second degree, if the patient refuses radical surgery
Various options for the absence of the pectoralis major muscle
Underdevelopment of the breast
Asymmetric chest.

Which doctor treats

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Poland syndrome

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

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

the cartilaginous and bone structure of the ribs is not broken, the shape of the sternum is not deformed, the deviation manifests itself in the lack of soft tissues;
the ribs are in a normal state, but the chest cell on one side is turned half-sided, has a sunken cartilaginous region of the ribs, and on the other, a keeled deformity;
the costal cartilages are underdeveloped, but all the bones are in a satisfactory condition, the sternum is skewed to the affected side;
a strong displacement of the upper body, the absence of one to four costal bones.

Causes

At the moment, the exact cause of Poland's syndrome is not known to scientists. The most common explanation is considered to be a failure in the migration of cells in the infancy, from which muscle fibers are formed or deformity / 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 the baby.

Symptoms

Characteristic signs indicating the presence of pathology:

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

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

Diagnostics

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

radiography (shows the level of violation of bone structures);
computer and magnetic resonance imaging (detect the condition of soft and cartilaginous tissues);
ECG and echocardiography (performed to check the functioning of the heart system).

Treatment

For the treatment of the disease, you can make an appointment through the Internet for an appointment 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 is eliminated only through an operation, and sometimes a whole series of such procedures. Depending on the degree of violations and the general well-being of the patient, the doctor selects a scheme for plastic surgery and the appropriate age for its implementation.

To begin with, the integrity of the natural corset is restored, in which the following are usually used: bone tissue transplantation from the healthy half of the patient's body, transposition of the costal arches located below, removal and correction of the sternum. Currently, various implants are actively used. Then the muscle fibers are reconstructed by transplanting healthy cells from other areas or by an individual silicone substitute. For girls, mammoplasty, an imitation of the 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 take care of her health, not expose her unborn child to the harmful effects of external factors, and not use harmful substances and drugs.

Poland's syndrome is a combination of anomalies that includes the absence of the pectoralis major muscle, pectoralis minor muscle, syndactyly, brachydactyly, atelia (absence of the breast nipple) or amastia (absence of the breast), deformity or absence of ribs, hair in the armpit, decreased thickness of the subcutaneous fat layer. This syndrome (its individual components) was first described in 1826 and in 1839, respectively, in French and German literature, but named after Alfred Poland, an English medical student who published in 1841 a description of this disease that he encountered in the section. A complete description of the deformation with all its components appeared in the literature only in 1895.

Each patient has different components of Poland syndrome, which occurs sporadically, with an incidence of 1 in 30,000 to 1 in 32,000 newborns, and is rarely familial. Hand involvement varies in extent and severity.

Causes of Poland's syndrome

Abnormal migration of fetal tissues that form the pectoral muscles, hypoplasia of the subclavian artery, or intrauterine injury are thought to play a role in the etiology. No theory has yet been found, however, reliable confirmation

Deformation of the chest wall in Poland syndrome can be of varying degrees - from hypoplasia of the ribs to aplasia of the anterior part of the ribs. So, according to one study, among 75 patients with Poland's syndrome, 41 had no chest wall deformity, 10 had rib hypoplasia without local areas of depression, 16 had rib deformity with silting, and in 11 of them retraction was significant, and finally, 8 cases revealed rib aplasia. It is important to note that no correlation was found between the degree of hand and chest deformity.

Treatment of Poland's syndrome

Surgical treatment for Poland's syndrome is required in only a subset of patients, usually with aplasia of the ribs or deformity with severe compression. Patients with significant retraction on the contralateral side often have a carinated deformity of the costal cartilages, which can also be corrected during Ravich's Teflon-coated split rib grafts for reconstruction. Others used latissimus dorsi flaps in combination with rib grafts. In girls, it is important to correct the deformity of the chest before intervention for hypoplasia or aplasia of the mammary gland, which provides optimal conditions for a subsequent surgical increase in the size of the gland. Latissimus dorsi rotation is rarely used in boys, but in girls this operation can be successfully used when breast reconstruction is needed.

The article was prepared and edited by: surgeon

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Poland's syndrome or rib muscle defect. Chest deformities. Poland syndrome. Causes of Poland's syndrome

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Pathogenesis

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Treatment of Poland's syndrome

Anatomy in Poland syndrome

Symptoms

Diagnostics

Treatment and operation

Which doctor treats

Poland syndrome

Pathological anatomy in Poland syndrome

Symptoms and diagnosis of Poland's syndrome

Treatment of Poland's syndrome

Poland syndrome

Reasons for development

Symptoms

Diagnostics

Treatment

Poland Syndrome: Causes and Treatment Options

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Surgery for pectus excavatum

Rib-muscular defect (Poland's syndrome)

The result of the I-stage of surgical correction of Poland's syndrome

(elimination of funnel chest deformity)

Funnel-shaped chest plasty was performed by Dr. med. Rudakov S.S. and Ph.D. Korolev P.A.

Doctor's note:

Anatomy in Poland syndrome

Symptoms

Diagnostics

Treatment and operation

Which doctor treats

Poland syndrome

Causes

Symptoms

Diagnostics

Treatment

Prevention

Video:

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