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$Unique_ID{BRK04115}
$Pretitle{}
$Title{Poland Syndrome}
$Subject{Poland Syndrome Poland Syndactyly Poland Anomaly}
$Volume{}
$Log{}
Copyright (C) 1987, 1989 National Organization for Rare Disorders, Inc.
440:
Poland Syndrome
** IMPORTANT **
It is possible the main title of the article (Poland Syndrome) is not the
name you expected. Please check the SYNONYMS listing on the next page to
find alternate names and disorder subdivisions covered by this article.
Synonyms
Poland Syndactyly
Poland Anomaly
General Discussion
** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only. It should not be used for diagnostic or treatment
purposes. If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources
section of this report.
Poland Syndrome is a congenital developmental disorder which is thought
to be genetic. Major symptoms occur on one side of the body and include
absence of chest muscles and/or ribs, underdevelopment of the arm, wrist and
hand, and shortened, webbed fingers. Function of the affected arm may be
limited.
Symptoms
Poland Syndrome is characterized by an abnormal development of one side of
the chest, including the arm and hand of the same side. One or more chest
muscles are partially or completely absent with underdevelopment or absence
of the breast and/or nipple. The cartilage and ribs in the area are absent
or abnormally developed. The hand, arm, forearm, and wrist are also
underdeveloped, and the fingers are shortened and webbed. An unusually deep
crease is present in the palm of the hand. The right side of the body is
usually the site of the abnormality.
Causes
The exact cause of Poland Syndrome is not known. Some medical researchers
believe it is genetic, although the mode of transmission is not understood.
Other researchers believe the disorder occurs sporadically, perhaps as a
mutation. Symptoms may develop due to developmental arrest during the
seventh to eighth week of fetal life characterized by failure of attachment
of chest muscles to the embryonic chest wall.
Affected Population
Poland Syndrome affects males and females in equal numbers. In a Canadian
study, researchers found the incidence to be one in 32,000 live births. The
name of the syndrome should not suggest that the illness primarily affects
Polish children; rather it was named for the doctor that identified it in
1921.
Therapies: Standard
Treatment of Poland Syndrome involves reconstructive surgery to replace the
absent chest muscles. Implants may produce good cosmetic results. The hand
abnormalities may be surgically corrected and ribs may be grafted into place
with good results.
Therapies: Investigational
This disease entry is based upon medical information available through
October 1987. Since NORD's resources are limited, it is not possible to
keep every entry in the Rare Disease Database completely current and
accurate. Please check with the agencies listed in the Resources section
for the most current information about this disorder.
Resources
For more information on Poland Syndrome, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
The National Arthritis and Musculoskeletal and Skin Diseases Information
Clearinghouse
Box AMS
Bethesda, MD 20892
(301) 495-4484
For genetic information, contact:
March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
(914) 428-7100
Alliance of Genetic Support Groups
35 Wisconsin Circle, Suite 440
Chevy Chase, MD 20815
(800) 336-GENE
(301) 652-5553
References
EARLY RECONSTRUCTION OF POLAND'S SYNDROME USING AUTOLOGOUS RIB GRAFTS
COMBINED WITH A LATISSIMUS MUSCLE FLAP: J.A. Haller, Jr., et al.; J Pediatr
Surg (August 1984, issue 19(4)). Pp. 423-429.
POLAND'S SYNDROME: CORRECTION OF THORACIC ANOMALY THROUGH MINIMAL
INCISIONS: P. Santi, et al.; Plast Reconstr Surg (October 1985, issue
76(4)). Pp. 639-641.
EARLY CORRECTION OF THE THORACIC DEFORMITY OF POLAND'S SYNDROME IN
CHILDREN WITH THE LATISSIMUS DORSI MUSCLE FLAP: LONG-TERM FOLLOW-UP OF TWO
CASES: H. Anderl, et al.; Br J Plast Surg (April 1986, issue 39(2)). Pp.
167-172.