Palang syndrome. Department of Thoracic Surgery. Radical treatment of portal hypertension

is a complex of developmental defects, including the absence of the pectoralis minor and/or major muscles, 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 lack of hair in the armpit area. The defect is unilateral, most often observed on the right. The severity of the disorders can vary greatly. The diagnosis is made based on clinical data, radiography, MRI and other studies. Treatment 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 syndrome is a fairly rare congenital malformation. It is detected in one out 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, family predisposition has not been proven. It is characterized by wide variability - in different patients there are significant differences both in severity and in the presence or absence of certain defects.

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 to intrauterine damage or hypoplasia of the subclavian artery. None of these theories has yet received reliable confirmation.

Pathogenesis

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

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

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

Symptoms

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

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

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

Diagnostics

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

Treatment of Poland syndrome

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

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

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

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

Causes of Poland syndrome

Abnormal migration of embryonic tissues forming the pectoral muscles, hypoplasia of the subclavian artery, or intrauterine injury are thought to play a role in the etiology. Not a single theory has yet found reliable confirmation, however.

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. Thus, according to one study, among 75 patients with Poland syndrome, 41 had no deformation of the chest wall, 10 had hypoplasia of the ribs without local areas of depression, 16 had deformation of the ribs with siltation, and in 11 of them the retraction was significant, and finally, In 8 cases, rib aplasia was detected. It is important to note that no correlation was found between the degree of deformation of the hand and chest

Treatment of Poland syndrome

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

Video:

Healthy:

Related articles:

1. The keeled chest is characterized by a protruding sternum and recessed ribs along its edges. Frequency ratio...
2. A keeled chest, which is a protrusion of the chest wall anteriorly, is less common than a funnel-shaped chest, and is...
3. The most common breast diseases in children are congenital malformations and tumors....

4095 0

Poland syndrome

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

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

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

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

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

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

// 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