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Defining Poland’s Syndrome

Poland’s syndrome is described as a malformation involving the under-development or complete absence of the chest muscle on one side of the body. In addition, there is often webbing of the fingers on the same side as the chest wall anomaly. Although Poland’s syndrome can affect either side, it is three times more likely on the right side of the body.

Poland’s syndrome is listed as a rare disease by the US National Institutes of Health. It is not hereditary, and for reasons not yet understood by medical professionals, it is more frequent in men than women.

What causes Poland’s Syndrome

The exact cause of Poland’s syndrome is unknown, although some clinicians speculate that it may involve a lack of blood flow through the arteries at a critical point during fetal growth around the 46th week of life. It is thought that the diminished blood flow may be responsible for the malformation as at that time the fetal fingers and chest muscles would be in development. However, there is not yet any clear proof, or concrete clinical studies proving this suggestion.

Clinical Features of Poland’s Syndrome

There are many physical features of Poland’s syndrome. However, the main feature is absent chest muscle on one side of the body. Other chest muscles located in proximity and on the same side may also be missing. In women, the nipple and breast on that side may also be totally absent. For both sexes, the fingers will display webbing of various degrees and will be shortened. There may also be absence of the underarm hair on the affected side.

The severity of the syndrome varies in each person born with Poland’s syndrome; therefore, additional features of the syndrome will vary. Additional deformities of the hands and arms include abnormal bone of the lower or upper arm, arms that are not symmetrical, and fused or missing fingers. Deformity of the shoulder blade is also often seen. Gastrointestinal and liver problems are also experienced, as well as dextrocardia, in which the heart is instead on the right side of the body.

Plastic and Reconstructive Surgery of Chest

Today, the outcome for those born with Poland’s syndrome is excellent. For many, Dr. Agha will reconstruct the breast, nipple, and can utilize the existing muscles to rebuild the chest. If there is not enough muscles present for rebuilding, results can be obtained by taking muscle from other parts of the body. If reconstructive surgery is attempted, it can be performed as early as the age of 13 for boys, but should be postponed until breast development is complete in girls.

 

Nipple Inversion Correction:

Birth deformities of the nipple are a leading motivator for women to seek out Plastic and Reconstructive surgery. A nipple deformity such as inverted nipple can be an instigator for low self-esteem, leading women to eventually seek out a qualified plastic surgeon. One of the most common nipple deformities is nipple inversion which can affect approximately 5% to 15% of women.

Nipple inversion is defined as having a nipple that has grown inward, rather than outward, from the breast mound. An inverted nipple is also commonly referred to as “invaginated.” Occasionally a woman will have nipples that are only inverted partially; these generally do not point outward from the breast as much as a typical nipple would. In some women, the inverted nipple does not project outward or inward, giving the breast a flat, and almost nipple-less appearance. Others may have inverted nipples which retract so far into the breast mound that they create a hollow or dimple in the breast itself. Nipple inversion can affect one or both breasts, and can vary in degree of severity. Nipple inversion is not harmful, although complications can arise during for breastfeeding.

Cause for Immediate Attention

A new change in nipple form and shape is of concern since it may signify a breast cancer. So if any of the following are a new change to the nipple area, an appointment with a physician must be made immediately for further testing.

Breast cancer may be present if the nipple inversion is new and

  • The nipple seems to pucker; rather than have the traditional slit shape
  • The nipple cannot be pulled out into the normal shape as it was before
  • The nipple has a change in color or texture, or appearance of ulcers or scaliness
  • If any lump or irregularity can be felt behind the nipple

Any discharge from an inverted nipple must also be evaluated by a physician. Breast cancer may be present if the discharge of the nipple:

Tests positive for blood in the secretion

  • The secretion comes from only one duct
  • The discharge is expressed from the nipple without squeezing it
  • The nipple has a change in color or texture, or appearance of ulcers or scaliness
  • Is present in any woman aged 60 or over

Treatment for Inverted Nipples

Although inverted nipples are not in of themselves harmful, they do cause discomfort during nursing, as well as making a woman self-conscious. A number of non-surgical methods have been described to treat nipple inversion. However, most non-surgical methods, such as manual stimulation, are only temporarily successful. Dr. Agha offers several procedures that can permanently correct for nipple inversion, nipple asymmetry, as well as nipple reconstruction.

 

Chest Wall Deformity: Pectus Excavatum

Pectus excavatum, otherwise known as sunken or funnel chest, is a birth anomaly of the chest wall. In this defect, several ribs and the sternum grow inward and produce a caved-in look of the chest wall.

Of all congenital chest wall defects, pectus excavatum is the most common, appearing in 90% of those with a chest wall abnormality. Other deformities of the chest wall include pectus carinatum, cleft sternum, pentalogy of Cantrell, asphyxiating thoracic dystrophy, and spondylothoracic dysplasia. The condition is normally noticed at birth; however, the chest’s appearance will worsen as bone development, particularly in the teen years, grows rapidly.

Pectus excavatum occurs predominantly in males, and is estimated to occur in approximately 1 in 400 births. Self-esteem issues are frequently experienced with those born with pectus excavatum, particularly in teen patients.

Cause and Description

In this condition, 4 to 5 ribs on both sides of the sternum are typically affected, as the growth of the cartilage and bone grows abnormally in the chest wall. In addition, the abnormality can be quite asymmetrical, with the right and left sides differing. Unfortunately, the reason why the bone and cartilage grows abnormally is not understood, and no known genetic defect has been found as the cause of pectus excavatum. For reasons also not yet known, this condition is associated with Marfan syndrome (a condition in which the connective tissue is defective and does not function as it should) and Poland’s syndrome (a malformation on one side of the chest wall).

Symptoms associated with pectus excavatum include the inability to take deep breaths, shortness of breath, pain in the anterior chest wall, and lack of stamina during any exertion. A variety of respiratory complications are also experienced. Anxiety and other emotional problems can also surface in those with pectus excavatum, including loss of motivation and exhibiting feelings of low self-esteem.

Treatment Options

Reconstructive surgery of the chest wall is an option for correcting pectus excavatum. The results of such operations are very successful and can help to give the patient a certain quality of life that he or she did not possess before. Not only does the patient experience an immediate raise in their self-esteem, but the physical improvements in breathing and stamina are immeasurable. Most individuals report an increase in ability to engage in sports and hobbies and have been able to enjoy strenuous activities that they could not in the past.

More about Dr. Siamak Agha

Dr. Siamak Agha is a board certified plastic surgeon who performs a range of aesthetic procedures for the skin, face, body and breasts. Over the years, he has built a reputation as a top liposuction, abdominoplasty and breast implants Newport Beach surgeon. In addition, he performs delicate facial procedures such as face lift, nose surgery and eyelid lift. To learn more or to schedule a consultation, contact his practice today at 949-644-2442.

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