Absence of the pectoral muscle in men. Poland syndrome: choice of surgical tactics. The result of the first stage of surgical correction of Poland syndrome

Description:

Poland syndrome is a congenital malformation that includes absence of the pectoralis major and minor muscles, syndactyly (fusion of the fingers), brachydactyly, atelia (absence of the nipple of the mammary gland) and/or amastia (absence of the mammary gland itself), deformation or absence of several ribs, absence of hair in the armpit and a decrease in the thickness of the subcutaneous fat layer. It is named after the English medical student Alfred Poland, who in 1841 published a partial description of this syndrome. The incidence of Poland syndrome is 1:30000-1:32000 newborns, it is always unilateral in 80% of cases on the right. More common in men than women.

Causes of Poland syndrome:

The cause of Poland syndrome has not been fully elucidated. It is assumed that it may be based on hereditary, infectious, chemical, radiation, mechanical and other factors that act during a certain period of time during the intrauterine development of the human body.

Symptoms of Poland syndrome:

Absence of the pectoralis major muscle, underdevelopment (or complete absence) of the mammary gland or nipple on the affected side, as well as absence of hair in the armpit on the side on which the pathology is observed.

Treatment for Poland syndrome:

Poland syndrome affects all structures of the chest wall: the sternum, ribs, muscles, subcutaneous fat and spine. Due to the need for complex reconstruction of the chest, Poland syndrome can only be treated surgically.

Aplasia of the pectoral muscle most often manifests itself as Poland syndrome. Let's try to figure out what it is. Poland syndrome or musculocostal defect is in most cases a genetic pathology. If it is present, then the entire chest is involved in the process, and in most cases the pectoralis major muscle is also affected, while in eighty percent of cases it is affected on the right side. This syndrome is often observed in combination with various pathologies of the spine and chest, pectoral cartilage, ribs, and there may also be a pathology of subcutaneous fat. There are cases when there is a need to involve other specialists in treatment, in addition to narrow thoracic surgeons. For example, very often there is a need for cardiologists, since pathology of the chest is combined with pathology of the heart, as well as the lungs and pleura. The specialized medical literature describes quite a lot of cases in which Poland's syndrome is also combined with fused fingers. This syndrome is diagnosed through a simple visual examination, as well as a special x-ray examination. Treatment of Poland syndrome is exclusively surgical. Moreover, in most cases, the patient needs not one, but several complex operations, with the help of which the problems that the patient has are gradually solved. It is very important to correctly diagnose and determine the position of the skeletal system of the chest relative to the organs, the position of the organs in the chest relative to each other. First of all, every patient with Poland syndrome needs to restore the bone frame of the chest. In cases where there is significant damage to the chest, there is a pathology of the ribs that cannot be corrected simply by changing their position, there is a need to transplant the ribs from the bottom up, for example, that is, using autotransplantation techniques. At one of the subsequent stages, it is necessary to reconstruct the external aesthetics of the chest - often this is a very important point for the patient, because the abnormal shape of the chest affected his life, usually this has a great psychological effect, patients begin to improve only due to their beliefs, that everything is already fine. At this stage, the pectoral muscles are restored, they are replaced in the presence of aplasia; in female patients there is a need to have one or even two mammary glands endoprosthetized. Surgical intervention almost always has a very good effect; it also includes correction of the costal arches, although they usually recover themselves over time. Poland syndrome is a complex defect; damage to the skeletal system and internal organs is very individual for each individual patient. The severity of the lesions also varies. Therefore, there is no specific optimal age for surgery.

Poland syndrome(costomuscular 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 syndrome is additionally characterized by underdevelopment of the upper limb on the affected side in the form of its shortening or syndactyly. The defect is most often found on the right; left-sided Poland syndrome is often accompanied by some form of reverse position of internal organs.

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

Methods for closing a costomuscular chest wall defect can be divided into three groups: bone, muscle and alloplasty of the 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 a periosteal pedicle into the chest wall defect. The surgical options we use for wide (a) and narrow chests (b). The use of these techniques allows you to reliably strengthen the chest wall.

Muscular plastic surgery of the costomuscular defect of the chest wall (by moving the latissimus dorsi muscle on a vascular pedicle) turned out to be ineffective. In all cases, atrophy of the displaced muscle occurs and the result of the operation is nullified. The best cosmetic results of correction of Poland syndrome were obtained using modern alloplastic materials. This type of surgery is performed only on adult patients. In men, to correct Poland syndrome, we use monolithic, textured pectoral silicone implants, which allow us to obtain a good chest configuration.

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.

A rare anomaly of the intrauterine formation of the body structure, which mainly consists of hypoplasia of the sternal and costal parts of the pectoralis major muscle or its complete absence. It bears the name of an English surgeon who, back in his student years, described a specimen with a costomuscular defect that turned up to him while working part-time in a morgue. A. Poland was not the first; before him, individual cases had already received attention in France and Germany at the beginning of the 19th century, but it was his publication that marked the beginning of a serious study of this congenital pathology. At the turn of the 20th century, J. Thompson published a complete description of this disease. Since then, about 500 such cases have been described in the world medical literature.

, , , , , ,

ICD-10 code

Q65-Q79 Congenital anomalies [malformations] and deformations of the musculoskeletal system

Epidemiology

Morbidity statistics indicate that congenital costomuscular anomalies, expressed to varying degrees, occur on average in one newborn out of 30 thousand or slightly more children born alive. More often, boys are born with such developmental defects.

Up to 80% of deformities in Poland syndrome are right-sided. The disturbances are expressed to varying degrees, and there is no correspondence between the severity of the anomalies in the formation of the chest and hand.

, , , , , ,

Causes of Poland syndrome

The reasons for the birth of children with this anomaly remain hypothetical to this day. The mode of inheritance and the gene that transmits this pathology are not determined, but there are descriptions of rare family histories of Poland syndrome. Recessive inheritance is assumed. It is believed that the probability of transmission of the disease from a sick parent to their children is approximately 50%. Most cases are single. Risk factors for the birth of children with this anomaly are external and internal teratogenic effects on the embryo during the formation and development of organs and systems. There are several hypotheses explaining the etiology and pathogenesis of this costomuscular defect, but none of them is fully confirmed. The most likely assumption is that some unfavorable factor provokes insufficiency of embryonic blood supply in the sixth week of pregnancy, when the subclavian artery is formed. This causes its underdevelopment (narrowing of the lumen) and insufficient blood supply, which leads to local hypoplasia of soft tissues and bones. The extent of the lesion is determined by the degree of damage to the artery and/or its branches.

Symptoms of Poland syndrome

The first signs of this congenital anomaly are visually noticeable already in infancy by the characteristic appearance of the pectoral muscle and axilla. And in the presence of hypoplasia of the hand - from birth.

The symptom complex of the syndrome is as follows:

• unilateral insufficient development of the pectoralis major muscle or its fragments, most often the sternum and costal muscles;
• on the same side - hypoplasia of the hand: shortened, fused fingers or aplasia; insufficient development of the mammary gland or its absence, atelia; thinning of the subcutaneous fat layer; lack of armpit hair; structural anomalies of cartilaginous/bone rib tissue or their complete absence (usually III and IV).

The presence of all the features described in the second part is not mandatory; they can be combined with the first in a variety of options.

In addition to those listed, it is extremely rare that there may be structural anomalies of the latissimus dorsi muscle, causing asymmetry of the torso, hypoplasia or aplasia of the pectoralis minor muscle, abnormal development of the scapula and clavicle, a pectus with a funnel-shaped deformity, curvature of the spine and costal hump.

With the left defective side, transposition of the internal organs is often observed, in particular, the heart is displaced to the right. With a normal location of the heart in combination with the absence of ribs, it is practically unprotected and its beating is noticeable under the skin.

Poland syndrome in children is usually noticeable from birth, but in some cases of minor defects it becomes apparent around age three.

Based on location, defects in the structural elements of the chest are divided into deformations of the anterior, posterior and lateral walls.

Poland syndrome in girls during puberty, even in the mildest degree, makes itself felt by the fact that the breast on the defective side does not form or lags behind in development and is located noticeably higher than on the normal side. In mild cases of the disease in boys, the syndrome is sometimes detected quite late, in adolescence, when it is not possible to “pump up” the muscle on the defective side.

Poland syndrome in women does not affect hormonal levels and the ability to conceive a child.

In most cases, Poland syndrome is a cosmetic defect: most often the pectoral muscle is deformed or absent, there is no chest defect and there is a full-fledged hand. The motor functions of the upper limb are preserved, and nothing prevents such patients from intensively engaging in sports.

However, there are other, more traumatic types of this pathology. The consequences and complications of such cases are somewhat more serious. Depending on the severity of the deformities, the patient may develop respiratory and hemodynamic disorders. In cases of complete absence of the costochondral framework, a pulmonary hernia is usually found, and respiratory distress manifests itself from birth.

In more rare cases of left-sided pathologies in combination with the absence of ribs with a normal disposition of organs, the heart appears directly under the skin. The life of such a patient is constantly in danger associated with possible injury and cardiac arrest.

A child with a pronounced chest wall defect usually has hemodynamic problems due to decreased systolic and increased diastolic arterial blood pressure combined with increased venous pressure. Such children are characterized by increased fatigue, asthenic syndrome, and they may lag behind their peers in physical development.

Manifestations of Poland syndrome also concern the structure of the subclavian artery and/or its branches, which creates conditions for a disorder of arterial blood flow on the side of the defect.

Some anatomical anomalies in the structure and location of vital internal organs are observed. The degree of their severity can significantly complicate the patient’s condition. This is a deviation of the heart from its normal location in one direction or another, up to transposition, expansion of its borders or rotation clockwise, hypoplasia of the lung and kidney on the defective side.

Stages

There are four stages of chest formation in this disease.

The first is typical for most known cases, when only soft tissues are abnormally developed, and the shape of the chest and the structure of the cartilaginous and bone parts of the ribs are normal.

The second is when the deformations affect the chest: the defective side, while the bone and cartilaginous parts of the ribs are preserved, is slightly depressed in the area of ​​the costal cartilages, the sternum is turned semi-sideways, and on the opposite side there is often a protruding (keeled) part of the chest.

At the third stage, the structure of the bony part of the ribs is preserved, but the cartilaginous part is underdeveloped, the chest is asymmetrical, the sternum is sloping towards deformation, but no gross anomalies are detected.

The fourth stage is characterized by the absence of the cartilaginous and bone parts of ribs from one to four (from III to VI). On the defective side there is a depression in place of the missing ribs, the sternum is noticeably turned out.

Nevertheless, at any stage of the formation of the elements of the structure of the chest, the state of the child’s body can be normal (compensated), with periodic improvements (subcompensated) and with increasing deterioration in the functioning of the internal organs and skeletal system (decompensated). It depends on the individual characteristics of the organism, the pace of development, comorbidity and lifestyle.

, , , ,

Diagnosis of Poland syndrome

Costomuscular congenital pathology is determined visually, the doctor palpates the patient and prescribes radiography. This is usually sufficient to identify the extent and type of chest lesion. Computer and magnetic resonance imaging can provide a more accurate picture of the disease.

An ultrasound examination of the subclavian artery is also prescribed to determine its diameter, an ultrasound examination of the brain and other instrumental diagnostics as indicated.

To assess associated anatomical defects, consultation with a cardiologist and electrocardiography, ultrasound examination of the heart, bicycle ergometry, echocardiography and Dopplerography of the great vessels are necessary.

In case of respiratory disorders, a consultation with a pulmonologist is required, who may prescribe a study of the functional state of the lungs, for example, spirography.

Tests for this disease are usually within normal limits, provided there are no concomitant pathologies.

Differential diagnosis

Treatment of Poland syndrome

This pathology is subject to surgical treatment. Often, according to indications, it begins in early childhood. Sometimes several surgical interventions may be required, for example, in case of a severe chest defect or costal aplasia, to ensure the safety of the heart or normalize the respiratory system. Such operations are performed in thoracic surgery departments. Their goal is to form the best protection for internal organs, ensure their normal functioning, eliminate curvatures of the chest, restore it and recreate the natural anatomical relationship of soft tissues.

The main and most difficult stage of surgical treatment of this syndrome is the elimination of curvature of the skeletal elements of the chest and replacement of missing ribs. Various thoracoplasty techniques are used. In the case of the right-sided location of the defect and the absence, for example, of the 3rd and 4th ribs, a split of the 2nd and 5th ribs is made. The deficiency of four ribs is corrected by transplanting part of the ribs taken from the healthy side of the patient’s chest. In modern medical practice, preference is given to titanium implants for patient tissue transplantation.

For preschoolers, a dense mesh is placed in the area of ​​the rib defect that protects the internal organs and does not interfere with the further development of the ribs, since costal plastic surgery in children can lead to secondary curvature of the skeletal elements of the chest caused by the uneven formation of the child’s healthy and operated ribs.

Surgical correction of severe curvature of the sternum is carried out using a wedge-shaped sternotomy.

If there is underdevelopment of the hand, prompt assistance is provided by orthopedists and traumatologists.

In the first stage of Poland syndrome, the only purpose of surgery is to eliminate the cosmetic defect. If there is a defect in the pectoral muscles, the normal anatomical relationship is restored either using the patient’s muscle tissue (part of the serratus anterior muscle or rectus abdominis muscle can be used for this) or with a silicone prosthesis. For men, individual silicone prostheses are more desirable, since muscle transplantation does not provide a full cosmetic effect and instead of one muscle defect, two appear. However, the choice of surgical method is always determined depending on the specific case.

For women, the latissimus muscle is moved from the back forward, simultaneously creating a muscular frame and layer. After waiting for healing, reconstructive mammoplasty is performed.

These operations are considered pure; preventive drug therapy is prescribed individually, taking into account the volume of the operation, the presence of implants, drug tolerance, age and concomitant diseases of the patient. The minimum amount of drug prophylaxis includes preoperative antibiotic therapy (prescribed an hour before the start of the operation and ends no later than a day or two later), anesthesia and sedation, restoration of intestinal function and drainage of the pleural cavity (if thoracoplasty is performed). The most used in the prevention of bacterial complications are cephalosporin antibiotics of the second and third generations.

Cefuroxime– ß-lactam bactericide with a wide range of activity, the mechanism of action is to interrupt the synthesis of the bacterial cell wall. Antagonist of strains of gram-positive and gram-negative bacteria, including those resistant to synthetic penicillins - ampicillin and amoxicillin. After intramuscular administration of 0.75 g of the drug, the maximum serum level is observed after no more than an hour, intravenous - after 15 minutes. The required concentrations remain for more than five and eight hours, respectively, they are fixed in bone and soft tissues, and skin. Eliminates completely within 24 hours. Contraindicated in case of sensitization to other cephalosporins, with caution in case of allergy to penicillin drugs.

If you are allergic to cephalosporins, you may be prescribed Vancomycin. This drug is used only intravenously over an hour (0.5 g after six hours or 1 g after twelve). For patients with renal dysfunction, the dose is adjusted.

Before prescribing an antibiotic, a sensitivity test is usually done so as not to complicate the operation. Side effects, except allergic reactions, can be neglected with short prophylaxis.

On the first day after surgery, pain relief is carried out with narcotic analgesics. For example, Promedolom, an opioid pain reliever that significantly increases the pain threshold, inhibits reactions to stimuli, calms and speeds up the process of falling asleep. It is used in the form of injections, subcutaneous and intramuscular. The highest dose is 160 mg per day. The body occasionally reacts to the administration of this drug with nausea, dizziness, and the development of euphoria.

On the second day after surgical treatment, the narcotic drug is alternated with a non-narcotic drug, then Promedol is prescribed only before bedtime.

In the postoperative period, before spontaneous bowel movements, patients are given hypertonic enemas, the consumption of sweets and carbonated water is excluded, Prozerin, which stimulates the secretory function of the glands of the gastrointestinal tract, as well as sweat and bronchial glands, tones the smooth muscles of the intestines and bladder, as well as skeletal muscles. Contraindicated in case of sensitization, thyrotoxicosis, severe pathologies of the cardiovascular system. Take orally half an hour before meals, the daily dose (no more than 50 mg) is divided into two or three doses.

Drugs that improve blood circulation in tissues and supply them with oxygen are also prescribed: Riboxin, Solcoseryl, Actovegin - improving metabolic processes, blood microcirculation and healing of postoperative wounds.

To monitor the pleural cavities in the early postoperative period, ultrasound examination is used to detect blood and transudate there and perform pleural drainage.

After the rib transplant surgery, the patient is observed in the hospital for approximately 14 days, the recovery period lasts several months. After surgery, it is recommended to limit physical activity and avoid pushing or hitting the surgical area for a year.

Muscle plastic surgery is a less traumatic operation; the patient is usually discharged after two or three days; physical activity must be limited for a month.

Rehabilitation measures should promote rapid detoxification of the body and normalization of metabolic processes, improvement of blood microcirculation and tissue restoration in the surgical area. Patients are prescribed vitamins (C, A, E, group B, D3, calcium, zinc) that promote healing of skin, soft tissues and bones, strengthen the immune system and improve the general condition of the body. In the hospital, vitamins are administered intramuscularly and orally; upon discharge, the doctor may recommend taking vitamin-mineral complexes.

After surgical treatment, the circulatory and respiratory systems adapt well to the background of medicated sleep. From the first to the tenth day of treatment, patients are recommended breathing exercises, massage and physical therapy.

During the rehabilitation period, physiotherapeutic treatment is prescribed, aimed at increasing the tone of skeletal and respiratory muscles, developing correct posture and walking: electrophoresis, electro- and magnetic therapy, thermal treatment procedures (therapeutic mud, paraffin, ozokerite), hydrotherapy (swimming, hydromassage, pearl baths) .

Alternative Treatment

Traditional treatment can help in preparation for surgery and stimulate recovery processes in the body, saturating it not with synthetic, but with natural vitamins.

It is useful to consume vitamin-mineral mixtures that strengthen the immune system, improve blood circulation and accelerate the healing of postoperative wounds.

1. Grind in any way 500 g of cranberries, a glass of peeled Voloshsky nuts, four large apples with green skin, removing the core. Add half a glass of water and 500 g of sugar, put on low heat. Bring to a boil, stirring regularly. Store in a jar with a lid. Take a tablespoon several times a day.
2. Grind dried apricots, raisins, voloshka nuts, lemons in equal parts by any method, removing the seeds, but with the peel. Pour in the same amount of honey and mix well. Store in the refrigerator in a glass jar with a lid. In the morning, 30 minutes before meals, eat a tablespoon of this mixture.

It is good to drink freshly prepared juices from available vegetables and fruits (in winter - carrot, apple, citrus), they can be mixed, for example, the classic combination - carrot and apple. The course of juice therapy is continued for three weeks, week 1 - half a glass once a day in the morning before meals, week 2 - the same amount, but in the morning and before lunch, week 3 - again once a half glass. The course can be repeated, but not earlier than ten days later.

You can brew tea, both black and green, with dried pieces of citrus peel.

You can boost immunity and improve blood circulation, especially in winter and spring, when the body is deficient in vitamins, using an arsenal of herbal treatments. The following mixtures of medicinal herbs are drunk instead of tea at breakfast after meals.

1. Mix crushed nettle and lemongrass leaves (150 g each) with 50 g of sage. Brew a teaspoon of herbal mixture with boiling water in a thermos in a volume of 200 ml. After two hours, strain and add a teaspoon of honey.
2. Prepare a herbal mixture from crushed string grass, strawberry leaves, and chamomile flowers. Brew a tablespoon of the mixture with boiling water (250 ml), strain after 20 minutes.

Drink an infusion of dried red rowan, half a glass four times a day: brew ½ liter of boiling water, two tablespoons of berries, leave for at least a quarter of an hour, strain, you can add honey.

Homeopathy belongs to conservative methods of treatment and is unlikely to help correct congenital deformities, however, homeopathic medicines are quite capable of preparing for surgery or activating the recovery process. Arsenicum album, Arnica, Mancinella promote wound healing, Calcarea fluorica and Calcarea phosphorica promote bone fusion. Please note that homeopathic medicines must be prescribed by a homeopathic doctor.

Pharmacy homeopathic medicine Traumeel S can be used in the postoperative period, taking advantage of its high restorative qualities. The effectiveness of the drug is due to its ability to activate the chain of reactions of the immune system, stimulating the clone of Th3 lymphocytes. As a result, the balance of pro- and anti-inflammatory cytokines at the surgical site is normalized, which helps eliminate swelling, pain, and hyperemia. Restores impaired blood microcirculation and tissue functions.

Injections are made intramuscularly and intradermally, under the skin, using the method of biopuncture and homeosiniatry. The daily dosage for patients aged six years and older is 2.2 ml, for those under six years old – no more than 0.55 ml. In severe cases, adults can administer two ampoules during the day.

When the condition improves, they switch to the tablet form of the drug (sublingually): patients from the age of three can be prescribed a tablet in three doses, 0-2 years - half a tablet in three doses. To relieve acute conditions, the tablets are dissolved at intervals of a quarter of an hour. This procedure can be carried out within two hours (no more than eight doses).