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Poland Syndrome
Thursday, 28 July 2005
Tuesday, 24 October 2006

What

Poland syndrome is a congenital abnormality in which one side of the body, specifically the chest and hand, are not formed correctly. Besides from this physical defect, most children with Poland syndrome are perfectly healthy.

Who

The incidence of Poland syndrome is 1 in 30,000 live births. Boys are affected 2-3 times more than girls. The disease seems to be sporadic, but there have been familial cases reported.

Signs and Symptoms

A child with Poland syndrome has the signs of the disorder at birth, but the diagnosis might be missed if those physical signs are mild. Children with this disorder have a malformed chest where the upper chest muscle (pectoralis major) and the nipple on one side is missing. The right chest is involved more often than the left chest, accounting for 75% of all reported cases. Children with Poland syndrome might have some abnormality of the hand (on the same side of the chest abnormality), where the fingers are short and/or fused together (symbrachydactyly). In some children, the rib cage on the same side might not form correctly. At puberty, children with this disorder will have very few or lack of axillary (arm pit) hair on the affected side. Some girls might have no breast or an underdeveloped breast on that side.

Possible Causes

The cause of Poland syndrome is said to be due to decreased blood flow to the chest, arm and hand region of the fetus in utero. It is hypothesized that the subclavian artery (the artery supplying blood to the areas mentioned above) is pinched or narrowed during the 6th week of fetal life, when the chest, arm and hand are developing. Not having enough blood may cause these regions to fail to develop fully.

Diagnosis

The diagnosis can be made by physical exam or a CT scan of the chest. An X-ray of the chest and hand will also help the doctor learn more about the problems involving the rib cage and fingers of the child, if there are any.

Treatment

Parents of children with Poland syndrome with hand problems often opt for hand surgery when the children are young. Most patients seek plastic or reconstructive surgery of their chest in their teens or adult years. A surgeon might use part of a chest/back muscle of the patient called lattisimus dorsi to replace the missing muscle in front. Sometimes a silicone implant can also be used. It is often suggested that girls with Poland syndrome wait until after breast development before they seek surgery.

Prognosis

Since Poland syndrome is mostly a cosmetic problem, the patients often lead very healthy lives. For those with severe problems, surgery is a very successful treatment and the patients normally can expect a full recovery.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

Poland Syndrome Support
Great website with chat forum for parents, teens, kids and adults with Poland syndrome.

About .com
Great website with a link to a support group for Poland syndrome.

Poland’s Syndrome
Great website with a chat forum where people talk about their experience with the disorder as well as their surgery.

Italian Poland Syndrome Association (Associazione Italiana Sindrome di Poland)
Great website (in Italian) that gives an overview of the disorder as well as chat forum and research information.

Google Search for Poland Syndrome

References and Sources

Ferraro, G. Poland syndrome: Description of an Atypical Variant. Aesth. Plast. Surg. 29:32–33, 2005 http://www.springerlink.com/media/320ENVUGWKCB0YLPGW97/Contributions/J/3/8/M/J38MU1T43738W052.pdf OMIM. Poland syndrome. http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=173800 Russo, Jose. Breast development and anatomy. http://www.utdol.com/application/topic.asp?file=breastcn/10391&type=A&selectedTitle=1~2