Poland syndrome treatment. Costomuscular defect (Poland syndrome). Funnel chest plastic surgery was performed by S.S. Rudakov. and Ph.D. Korolev P.A.

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

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

Anatomy of Poland syndrome

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

The main anatomical features of this syndrome are:

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

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

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

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

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

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

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

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

Symptoms

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

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

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

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

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

Diagnostics

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

So, the ribs can have the following changes:

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

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

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

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

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

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

• 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
• Complete absence of the mammary gland bud, including the nipple, on the affected side.

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

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

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

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

Treatment and surgery

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

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

• Medical
• Cosmetic.

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

The group of cosmetic indications includes the following:

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

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

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

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

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

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

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

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

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

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

Which doctor treats

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

Milanov N.O.

Chausheva S.I.

Alyautdin S.R.

Kraskovsky F.Ya.

Annals of Plastic Reconstructive and Aesthetic Surgery, No. 2, 2014.

Introduction.

Poland syndrome is a rare congenital disease, the population frequency of which is 1 case per 30 - 100 thousand people,The ratio of male to female reaches 2:1-3:1.In 75% of cases, Poland syndrome occurs on the right side.

Poland syndrome is a complex of birth defects that includes absence of the corticosternal portion of the pectoralis major and minor muscles, syndactyly, brachydactyly, atelia and/or amastia, absence of costal cartilage or multiple ribs (usually 2-5), absence of axillary hair, and decreased thickness subcutaneous fat layer (2.12). In addition to the presence of an aesthetic defect in the chest area, Poland syndrome is often accompanied by asthma, pain, disruption of the cardiovascular and respiratory systems, limitation of movements, and frequent infectious diseases of the lower respiratory tract (20,21). Lallemand L.M. was one of the first to describe individual symptoms of this syndrome. (1826) and Frorier R. (1839). However, it is named after the English medical student Alfred Poland, who in 1841 published a more detailed description of this pathology. A complete description of the syndrome in the literature was first published by Thompson J. in 1895 (14).

Poland syndrome is characterized by significant polymorphism and all its manifestations are rarely found in one patient. J . Thomson in 1895 and Bing in 1902 described in detail the underdevelopment of the pectoralis major muscle and membranous unilateral syndactyly. Syndactyly is the most common symptom accompanying defects in the development of chest tissue in this pathology, in connection with which in 1962 Clarkson proposed the term “Poland syndactyly” (14). Later, Lauros described that patients with Poland syndrome lack the sternocostal portion of the pectoralis major muscle and have hypoplasia of the pectoralis minor muscle, rib abnormalities, and in rare cases, pulmonary herniation.

Less than 1% of patients with Poland syndrome have a family history of a dominant autosomal trait (15,16). The cause may be an interruption or reduction in blood flow from the thoracic artery or one of its peripheral branches during the sixth week of pregnancy. Depending on the timing and intensity of the blood supply disturbance, Poland syndrome has varying degrees of severity (14,15). Thus, the literature describes in detail the clinical observation of a patient with Poland syndrome, who had atelia, while the pectoralis major muscle was normally developed and only the underdevelopment of the serratus anterior muscle, the above was identified using electromyography data (14).

Methods for correcting aesthetic defects in Poland syndrome are based on the desire to create symmetry by increasing the volume of tissue on the affected side. Methods for installing a silicone endoprosthesis or expanding with subsequent replacement of it with an endoprosthesis were among the first to be proposed (22,23,24).

In the absence of vascular pathology on the affected side, rotational flaps with axial circulation were used (17). To replenish the volume of underdeveloped or completely absent pectoralis minor and major muscles, a flap is moved from the latissimus dorsi muscle (LMS) to the position of the pectoralis major muscle on the vascular-muscular pedicle. If Poland's syndrome is accompanied by vascular compromise on the affected side, this may be an indication for the use of a free latissimus dorsi flap transfer on the contralateral side (1), a superior and inferior gluteal flap transfer (9) or, in rare cases, the use of DIEP (7). It must be taken into account that when the latissimus dorsi muscle rotates, its atrophy develops over time (11). The problem of extensive scars in the donor area can be resolved using endoscopic video technology (6,8).

The least labor-intensive method of correction using only a silicone prosthesis subsequently had complications in the form of dislocation, protrusion and deformation associated with resorption of the ribs (3).

In 1998, the omentum mobilization technique was used to correct severe hypomastia and aplasia of the pectoral muscles and adequately cover the implant. Although this technique has been repeatedly modified in recent years, labor intensity and numerous complications in the form of suppuration and tissue atrophy do not allow this method to be fully applied (18, 19).

Some authors, when savingThe sternoclavicular part of the pectoralis major muscle suggests its relocation to eliminate the defect of the axillary flexure and the immediate installation of a silicone implant (13).The use of endoscopic techniques to rotate the latissimus dorsi muscle and simultaneous arthroplasty allows one to avoid extensive scars in the donor area (25,26).

In men with Poland syndrome, correction is achieved by liposuction or only a silicone implant on the affected side (10).

A modern approach to the problem of correcting soft tissue defects in Poland syndrome involves the use of autologous fat (4) and silicone implants (5).

Despite the existence of numerous techniques, none of them can satisfy all the desires of the surgeon and patients; each has its own disadvantages and advantages. Today, the use of TDL in combination with immediate or delayed endoprosthetics provides optimal sustainable aesthetic results. Achieving symmetry is one of the most important tasks when eliminating defects caused by Poland syndrome.

Materials and methods.

We performed breast reconstruction for Polandau syndrome in 12 patients, 4 of them (33.4%) had left-sided Poland syndrome, and 8 (66.6%) had right-sided syndrome. In 8 (67%) patients, along with the absence of the mammary gland, there was underdevelopment of the upper limb, in 3 (25%) patients, hypoplasia of the 3, 4, 5 ribs, in 1 (8%) rib atresia. Of the 12 patients, in 8 (66.6%) we observed complete absence of not only the pectoralis minor and major muscles, but also breast tissue. In 1 (8.3%) patient, against the background of the absence of pectoral muscles, an insignificant volume of the mammary gland (size A) was observed, which made it possible to use for reconstruction only a section of the latissimus dorsi muscle without a skin island, and in 1 (8.3%) this made it possible to use only endoprosthesis. In 4 (33.6%) patients out of 12, breast reconstruction was performed using a simultaneous reversal of the thoracodorsal flap and installation of an endoprosthesis. In 6 (50%) patients, the latissimus dorsi muscle was simultaneously rotated and an expander was installed, followed by its replacement with an endoprosthesis (Figure No. 1).

Figure 1. Frequency of use of various methods of breast reconstruction for Poland syndrome.

Of the 12 patients, in 5 (41.6%) we performed reduction mammoplasty to achieve symmetry after correction of Poland's syndrome; in 7, relative symmetry was achieved only by reconstructing the defective gland.

In all clinical observations, asymmetry of the SAH was noted, but only 2 patients asked to restore the symmetry of the SAH.

Surgical tactics.

The main factor determining surgical tactics for Poland syndrome is the degree of tissue deficiency in the area of ​​the reconstructed mammary gland and the choice of the optimal method of correction.

The absence of breast tissue due to underdevelopment or aplasia of the pectoral muscles in most cases leads to the need to use a musculocutaneous flap for reconstruction.

Rotation of flaps based on the latissimus dorsi muscle with a skin with an island (TDL) or without an island (DS) is the most acceptable. Using this technique, we avoid complications associated with free flap transplantation, facilitate the postoperative period, and an easily hidden scar is formed.

Indications for the use of a full thoracodorsal flap (TDF) are not only aplasia or underdevelopment of the pectoralis major and minor muscles, but also the complete absence of breast tissue (size 0). The presence of breast tissue (size A or more) allows the use of only a flap based on the latissimus dorsi muscle (LMS) without a skin component.

In some clinical observations, when the volume of the contralateral breast exceeded the volume of C, we rotated the BMS area and installed an expander, and only after obtaining the required volume replaced it with an endoprosthesis. Of course, when choosing surgical tactics, we took into account the patient’s desire for a certain breast size and her attitude towards the presence of scars in the donor and recipient areas.

After achieving the desired result on the affected side, if necessary, we perform reduction mammoplasty or mastopexy on the contralateral side. The final stage is the formation of the nipple-areolar complex in the reconstructed mammary gland - recreating the nipple with local tissues and forming the areola using tattooing or free skin grafting.

Thus, several main stages can be identified in the correction of Poalnd syndrome.

1 stage. Elimination of soft tissue defects using various types of reconstruction.

Axillary defect in combination with skin deficiency, rotation of the TDL with simultaneous installation of a prosthesis or

expander.

For axillary defects with sufficient skin and breast tissue, it is possible to use BMS with an endoprosthesis or only an endoprosthesis.

2 stage. Correction of the contralateral mammary gland.

3 stage. Reconstruction of the SAC.

Clinical examples.

Patient Z., 18 years old, was admitted with a diagnosis of Poland syndrome on the right. Complaints upon admission about contour deformation of the chest on the right and underdevelopment of the mammary gland. Upon examination at the time of admission: there is asymmetry of the mammary glands due to underdevelopment of the right mammary gland, the skin over it is of normal color, the nipple-areola complex is reduced in size, of normal color. Locally: the pectoralis major muscle on the right is not palpable, palpation of the right mammary gland is painless. The left mammary gland is healthy, corresponds to size B, painless on palpation, soft-elastic consistency, without pathological formations.

Main stages of the operation:

After preliminary marking, in the projection of the latissimus dorsi muscle. Performed the main stage of the fence, turning the area of ​​the widest

back muscles figure No. 2,3.

Fig. No. 2. Flap harvesting stage.

Rice. No. 3. Stage of flap fitting for rotation.

Then the muscle flap was rotated and placed in the area of ​​the anterior chest wall, Figure No. 4.

Fig.4. Rotated muscle pocket.

We fix the muscle to the anterior wall of the chest along the lateral edge of the sternum, cranially at the level of 2 ribs, caudally at the level of 8-9 ribs.

Endoprosthesis figure No. 5 was placed in the formed pocket.

Fig.5. Submuscular pocket with endoprosthesis.

Fig.6. Final view of the reconstructed right breast.

In some clinical observations, even maintaining slight asymmetry meets the patient’s requirements, although the surgeon suggests the possibility of optimal symmetry. This patient (Figure 7a) was fitted with a round prosthesis measuring 325cc (Figure 7b,c)

Fig. No. 7a. view of the patient before surgeryFig. No. 7b. view of the patient after surgery

Fig. No. 7c. View of the patient after surgery in the “hands behind the head” position.

In the patient's position, standing with her hands behind her head, we noted close to ideal symmetry. Although at the first stage we were unable to achieve complete symmetry, the patient refused further correction of asymmetry.

Clinical example No. 2.

Patient O., 19 years old. Diagnosis: Poland syndrome on the left.

From the anamnesis, from childhood he notes a contour deformation of the chest on the left, and from puberty, underdevelopment of the mammary gland.

Correction of Poland syndrome in this patient took several stages:

Stage 1: turning the ShMS, we completed the turn of the ShMS and installed an expander with a volume of 400cc,

Stage 2: replacement of the expander with an anatomically shaped prosthesis with a volume of 355cc after 6 months

Stage 3: reduction mammoplasty of the right breast.

Stage 4: reconstruction of the SAH (Figure 8).

Rice. No. 8 -a before surgery, after the first stage 8b of reversing the BMS and installing an expander, after the second 8c, replacing the expander with an endoprosthesis, and after 3 months of reduction mammoplasty on the right 8 d.

In our opinion, we achieved the optimal possible aesthetic result for this patient.

Thus, restoring symmetry in Poland syndrome is a multi-stage procedure. The main task is to create an adequate pocket for the endoprosthesis with a sufficient amount of covering tissue. The choice of surgical tactics depends on the patient’s anatomical data - the degree of soft tissue damage, the size and shape of the healthy mammary gland.

Literature:

1. Avcı G., Mısırlıoglu A., Eker G., Ako¨ Z T. Mild Degree Of Poland’s Syndrome Reconstruction With Customized Silicone Prosthesis. //Aesth. Plast. Surg. 2003. Vol. 27. P. 112-115.

2. Baban A, Torre M, Bianca S, Et Al. Poland Syndrome With Bilateral Features: Case Description With Review Of The Literature. Am J Med Genet A. 2009;149A(7):1597-1602

3. Beer G.M., Kompatscher P., Hergan K. .Poland's Syndrome And Vascular Malformations.// Br. J. Plast. Surg. 1996. Vol. 49. P. 482-844

4. Borschel G.H., Izenberg P.H., Cederna P.S. . Endoscopically Assisted Reconstruction Of Male And Female Poland Syndrome. // 2002. Vol. 109. P.1536-1543.

5. Borschel G.H., Costantino D.A., Cederna P.S. . Individualized Implant-Based Reconstruction Of Poland Syndrome Breast And Soft Tissue Deformities.// 2007. Vol. 59. P. 507-514.

6. Coll Anthropol. 2011 Dec;35(4):1303-5.Endoscopically Assisted Latissimus Dorsi Flap Harvesting And Breast Reconstruction in Young Female With Poland Syndrome. Veir Z Dujmović A , Duduković M, Mijatović D,Cvjeticanin B,Veir M.

7. Costa S.S., Blotta R.M., Mariano M.B., Meurer L., Edelweiss A. Laparoscopic Treatment Of Poland’s Syndrome Using The Omentum Flap Technique. //Clinics (Sao Paulo). 2010. Vol. 65. P. 401-406.

8. Copper R.A., Johnson M.S.: Mammographic Depiction Of Poland’s Syndrome. //Br. J. Radiol.1990. Vol. 6. P. 302-303

9. Costa SS. Tratamento Cirúrgico Da Síndrome De Poland Com Omento Transposto Por Videolaparoscopia. //In: Pedrini JLRA, Penczek F, Editors. XI Congresso Brasileiro De Mastologia. Foz Do Iguaçu-Paraná: XI Congresso Brasileiro De Mastologia; 1998. P. 186

10. Fekih M, Mansouri-Hattab N, Bergaoui D, Et Al. Correction Of Breast Poland's Anomalies. About Eight Cases And Literature Review. Ann Chir Plast Esthet. 2010;55(3):211-218.

11. Ferraro G.A., Perrotta A., Rossano F., D’Andrea F. Poland Syndrome: Description Of An Atypical Variant. //Aesth. Plast. Surg. 2005. Vol. 29. P. 32-33,

12. Fokin A.A., Robicsek F. Poland_S Syndrome Revisited.//Ann. Thorac. Surg. 2002. Vol.74. P. 2218-2225.

13. Kaguraoka H, ​​Ohnuki T, Itaoka T, Et Al. Degree Of Severity Of Pectus Excavatum And Pulmonary Function In Preoperative And Postoperative Periods.J Thorac Cardiovasc Surg. 1992;104:1483-1488.

14. Longaker M.T., Glat P.M., Colen L.B., Siebert J.W. .. Reconstruction Of Breast Asymmetry In Poland"s Chest-Wall Deformity Using Microvascular Free Flaps.// 1997. Vol. 99. P. 429-436.

15. Liao H.T., Cheng M.H., Ulusal B.G., Wei F.C. . Deep Inferior Epigastric Perforator Flap For Successful Simultaneous Breast And Chest Wall Reconstruction In A Poland Anomaly Patient. // 2005. Vol. 55. P. 422-426.

16. Marks M.W., Argenta L.C., Izenberg P.H., Mes L.G. Management Of The Chest-Wall Deformity In Male Patients With Poland’s Syndrome.// Plast. Renconstr. Surg. 1991. Vol.87. P. 674-678

17. Mavanur A, Hight DW. Pectus Excavatum And Carinatum: New Concepts In The Correction Of Congenital Chest Wall Deformities In The Pediatric Age Group. Conn Med. 2008;72(1):5-11.

18. Martinez-Ferro M., Fraire C., Saldaña L., Reussmann A., Dogliotti P. . Complete Videoendoscopic Harvest And Transposition Of Latissimus Dorsi Muscle For The Treatment Of Poland Syndrome: A First Report. // 2007. Vol. 17. P. 108-113.

// Costomuscular defect (Poland syndrome)

Costomuscular defect (Poland syndrome)

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

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

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

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

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

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

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

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

(elimination of pectus excavatum)

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

Photos before surgery and results 7 months after surgery

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

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

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

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

Lung resection without thoracotomy.

We have introduced lung operations using endoscopic equipment. These operations avoid thoracotomy incisions. We have developed a video-assisted technique for lung resection without the use of expensive staplers. In this case, a classic, standard lung resection is performed. The postoperative period after such operations is much easier compared to standard operations. Hospitalization times are also reduced.

Radical treatment of portal hypertension.

In the Department of Thoracic Surgery, mesenterioportal anastomosis operations for extrahepatic portal hypertension were performed for the first time. These operations are aimed at restoring physiological blood flow through the portal vein. The uniqueness of these operations lies in the complete restoration of physiological and anatomical relationships in the portal system while completely eliminating the threat of bleeding from varicose veins of the esophagus. Thus, seriously ill children turn into practically healthy children.
A fundamentally new treatment method
funnel chest deformity.

Thoracoplasty according to Nuss. (treatment of children with pectus excavatum)

We have introduced a new method of thoracoplasty - according to Nuss. This operation is performed using two small incisions on the sides of the chest and does not require resection or intersection of the sternum or ribs. The postoperative period is much easier. An almost ideal cosmetic result is achieved. With this operation, unlike standard thoracoplasty, the volume of the chest increases to physiological levels.

Thoracic surgery is well equipped to care for the most severe group of children; it has the most modern operating room, equipped with a laminar flow system that eliminates infectious complications during the operation, and endoscopic equipment for bronchoscopy, thoracoscopy, and laparoscopy. Doctors have a variety of highly informative diagnostic methods at their disposal, including endoscopic, ultrasound, radioisotope, and radiation (radiography, computed tomography, angiography). On the territory of the hospital there is one of the largest Moscow laboratories for biochemical and microbiological research.

In childhood, there are both congenital diseases - defects and anomalies in the development of various organs, and acquired ones - inflammatory diseases, consequences of injuries and burns, as well as tumors. A wide variety of diseases require the doctor to have knowledge and skills in many areas of medicine, including vascular and plastic surgery, oncology, endocrinology, pulmonology and others.

The goal of treatment - to return the child to a normal, full life - can be achieved subject to a complete and comprehensive examination, treatment and postoperative observation of the child in a specialized department by highly qualified doctors.

We have accumulated extensive experience in performing endoscopic diagnostic and therapeutic procedures for foreign bodies of the trachea, bronchi and esophagus, and other pathological conditions and malformations of the esophagus, stomach and respiratory tract. Laser treatment, cryosurgery and the most modern electrosurgical instruments and devices are used.

Consultation, hospitalization and treatment in the department for all Russian citizens who have a compulsory health insurance policy, from the moment of birth until the age of 18, regardless of their place of permanent residence, are carried out under a compulsory health insurance policy.

A referral from your local health authority is not required.

Hospitalization of Russians over 18 years of age, as well as citizens of near and far abroad, is possible under the terms of voluntary health insurance.

In recent years, there has been a consistent trend towards an increase in the number of children admitted and operated on.
Most of the children who come to us have previously been operated on in other medical institutions.
Many operations and treatment methods were developed and applied in our country for the first time by the department’s staff.

Children under 3 years old have the opportunity to stay with their parents around the clock in boxed single and double rooms. Older children are accommodated in rooms for 6 people. The department treats children aged from the neonatal period to 18 years on the basis of a compulsory health insurance policy. Hospitalization of Russians over 18 years of age and foreigners is carried out under the terms of voluntary medical insurance. All rooms have oxygen and the ability to connect aspirators, as well as devices for respiratory therapy. The intensive care unit provides 24/7 monitoring of vital signs.


Thanks to the widespread introduction of low-traumatic and endoscopic technologies in the surgical treatment of children with various diseases of the thoracic and abdominal cavity, mediastinum and chest, most of them do not need to be transferred to the intensive care unit after surgery, but have the opportunity to stay with their parents in the intensive care ward, equipped with everything necessary for a comfortable stay in the postoperative period.


The department has a modern endoscopic room, where a wide range of diagnostic esophagoscopies, laryngoscopies, bronchoscopies and therapeutic endoluminal manipulations are performed: removal of foreign bodies of the esophagus and stomach, removal of foreign bodies of the trachea and bronchi, bougienage of the esophagus and trachea, etc. If necessary, we actively use laser and CRYO therapy (liquid nitrogen) in the treatment of diseases and malformations of the larynx, trachea and esophagus. All diagnostic and therapeutic procedures are archived on digital media.


The department has its own ultrasound room with an expert-level device. This expands the possibilities of non-invasive high-precision diagnostics. In addition, many manipulations are performed in our department under ultrasound control: puncture of cysts of the kidneys, spleen, liver, etc.
Every year, more than 500 operations (link to the operation report) of the highest degree of complexity and more than 600 studies and manipulations (link to the endoscopy report) under anesthesia are performed (bronchoscopy, biopsy, ultrasound-guided puncture, endoluminal operations on the respiratory tract and esophagus, etc.

Operating room of the thoracic surgery department