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) 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 fetal 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 gland. Latissimus dorsi rotation is rarely used in boys, but in girls it can be successfully used when breast reconstruction is needed.

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Poland syndrome

Poland syndrome is a combination of abnormalities including absence of the pectoralis major and minor muscles, syndactyly, brachydactyly, atslia (absence of the nipple) and/or amastia (absence of the breast itself), deformed or absent ribs, absence of axillary bands, and decreased thickness subcutaneous fat layer (Fig. 15-7). This syndrome (its individual components) was first described in 1826 and in 1839.

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.

Surgery for Poland syndrome is required in only a small proportion of patients, usually for rib aplasia or a severely depressed deformity (Figure 15-9). Patients with significant retraction on the contralateral side often have a keeled deformity of the costal cartilages (Figure I5-8B), which can also be corrected intraoperatively.

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 strengthened 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.

Ravich used Teflon-coated split rib grafts for reconstruction. Other surgeons have used latissimus dorsi flaps in addition to rib grafts. In girls, it is important to correct the deformation of the chest before surgery due to hypoplasia or aplasia of the mammary gland, which allows for optimal conditions for subsequent surgical increase in the size of the gland (Fig. 15-10). Latissimus dorsi rotation is rarely used in boys, while in girls this operation can be successfully used when breast reconstruction is necessary.

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 calculus deformation on the opposite side (hollow arrow) are visible. The mammary gland on the affected side is hypoplastic.

K.U. Ashcraft, T.M. Holder

Poland syndrome is a rare disorder that appears at birth. Signs are extremely variable.

Classically characterized by absence (aplasia) of the chest muscles on one side of the body (unilateral) and abnormally short, webbed fingers on one side (ipsilateral). Patients usually have a unilateral absence of the breast or part of the pectoral muscle.

Affected persons may have:

• underdevelopment or absence of one nipple (including the dark area around the nipple [areola]);
• partial absence of armpit hair.

Poland syndrome in girls manifests itself as insufficient development or absence (aplasia) of one breast and subcutaneous tissue. In some cases, skeletal abnormalities are present, such as:

• insufficient development or absence of the upper ribs;
• elevation of the scapula (Sprengel deformity);
• shortening of the arm, with underdevelopment of the bones of the forearm.

The disease most often affects the right side of the body. It occurs more often in men than in women. The exact cause of the disorder is unknown.

Synonyms

• Poland Anomaly;
• Poland sequence;
• Poland Syndactyly;
• Unilateral defect of the muscles of the chest and syndicalium of the arm.

Symptoms are extremely varied, even when more than one family member is affected. For example, one brother had all the main features of the condition, while another had only the absence of the pectoral muscle.

The disorder is most often characterized by the absence of chest muscles on one side of the body and involvement of the arm on one side (ipsilateral). 75 percent – ​​deviations affect the right side of the body.

Most affected people lack the pectoral muscle.

Some people with Poland syndrome have unilateral absence of other regional muscles, such as the latissimus dorsi and chest wall, which extends from the ribs under the armpit to the shoulder blade.

Anomalies include:

• underdevelopment or absence of a darkened area around the nipple (areola);
• absence of a nipple;
• abnormal hair growth under the armpit.

Poland syndrome in girls manifests itself as insufficient development or absence of breasts and subcutaneous tissues.

Additional bone defects are present in some people:

• underdevelopment or absence of the upper ribs;
• costal cartilages, with the help of which the ribs are attached to the sternum;
• abnormal height or insufficient development of the scapula;
• limited movement of the arm on the affected side;
• Sprengel deformity.

Most people with Poland syndrome:

• arms on one side of the body are affected;
• some finger bones (phalanxes) are underdeveloped or missing, resulting in abnormally short fingers (brachydactyl);
• syndactyls, fusion of fingers, especially index and middle fingers.

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Sometimes there is an abnormal contraction of the arm, with insufficient development of the bones of the forearm.

Reasons

According to reports in the medical literature, the vast majority of cases occur incidentally for unknown reasons in the absence of a family history.

Family inheritance has sometimes been reported - the disease in mother and child, siblings born to healthy parents.

Familial cases may result from an inherited susceptibility to a particular abnormality (early interruption of blood flow) predisposing to the syndrome.

The disorder is sometimes called Poland sequence. "Sequence" refers to the pattern of malformations derived from a single abnormality.

The main drawback of the disease is impaired arterial development, which leads to reduced or interrupted blood flow during early embryonic growth.

For a group of conditions arising from disruption of blood flow through arteries (subclavian, vertebral and their branches), the terminology “Temporal sequence of arterial interruption” was proposed at the sixth week of embryonic development. Such conditions include Poland syndrome, Mobius syndrome, Klippel-Feil syndrome, and Sprengel deformity.

It is believed that the specific pattern of defects depends on the site and degree of reduced blood flow.

Affected populations

The disorder is named after the researcher (Pland A) who described the condition in 1841. Poland syndrome is three times more common in men. Incidence is from one in 10,000 to one in 100,000. (Incidence is the number of new cases over a certain period.)

As noted above, the disease affects the right side of the body in about 75 percent of cases.

In 1998, the first case was reported in which both sides of the body were affected (bilateral involvement). The researchers described a young girl with bilateral pectoralis absence, symmetrical pectoral deformity, and bilateral arm involvement.

Related violations

Symptoms of the following disorders are similar, and comparisons are useful for differential diagnosis:

There are known cases where Poland's signs occur in combination with (Poland-Mobius syndrome). They are characterized by facial palsy due to disruption of the sixth and seventh cranial nerves (Moebius), chest wall defects, or uterine defects (symbrachydactyls) commonly seen in Poland.

Mobius syndrome

Characterized by:

• “mask”, without expressed emotions on the face;
• inability to make certain eye movements;
• ptosis;
• limited mobility of the tongue;
• poor sucking, swallowing;
• speech disorders.

Additional signs include:

• clubfoot;
• various limb defects;
• moderate mental retardation.

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Mobius syndrome usually occurs randomly for unknown reasons. It may result from early disruption of arterial blood flow during embryonic development.

Hypogenesis of the oromandibular limbus

A term used to describe a group of rare conditions characterized by underdevelopment (hypogenesis) of the mouth, jaw (oromandibular), hands, and feet.

This group of face-limb malformations includes Moebius, Charlie M, and Hanhart syndromes. They occur sporadically and are characterized by extremely variable clinical signs.

Some researchers believe that these are overlapping disease variants, representing a spectrum of malformations caused by environmental or other factors. Oromandibular-Limbus, Moebius hyporeception is caused by early disruption of blood flow in the arteries due to various mechanical factors.

Charlie M syndrome

A rare condition characterized by certain distinctive deformities of the face and limbs. Characterized by:

• wide-set eyes (ocular hypertelorism);
• wide nose and small mouth;
• incomplete closure of the roof of the mouth (cleft palate);
• abnormally small jaw (micrognathia);
• facial paralysis;
• missing or tapered front teeth (incisors);
• asymmetrical deviations of fingers and toes.

Occurs randomly for unknown reasons.

Diagnostics

The diagnosis is usually confirmed at birth based on characteristic physical signs, careful clinical evaluation, and various specialized tests.

Tests include advanced imaging techniques, such as CT scans, which determine the extent of the effect on the muscles. X-ray examinations are used to identify abnormalities of the arms, forearms, ribs, and shoulder blades.

Treatment

Treatment for Poland syndrome targets specific symptoms. It requires the coordinated efforts of a group of specialists: Plastic surgery to restore the chest wall, transplantation of the rib in the proper place.