The Journal of Bone and Joint Surgery, Vol 58, Issue 1 52-58, Copyright © 1976 by Journal of Bone and Joint Surgery, Inc
Poland's syndrome
DC Ireland, N Takayama and AE Flatt
Forty-three consecutive cases of Poland's syndrome were analyzed and the
relevant literature was reviewed. The syndrome is not hereditary and is of
unknown origin. It affects males more frequently than females. The clinical
features are variable but always include congenital aplasia and syndactyly.
The middle phalanges are hypoplastic or absent so that effectively there is
only one interphalangeal joint. The syndactyly is usually incomplete and
simple. It may involve all fingers and frequently includes the thumb, which
then lies in the same plane as the fingers. Poland's syndrome may also
include hypoplasia of the nipple and breast, hypoplasia of the upper ribs,
herniation of the lung, contracture of the anterior axillary web, and
elevated scapula. The arm and more frequently the forearm are hypoplastic.
The right side is more often affected than the left. Surgical treatment by
separating the syndactyly is recommended. In some cases a digit is removed
to produce a three-fingered hand. Surgery is initiated by the age of one
year and is completed by the time the child enters school, although
periodic revisions may be necessary. Although the hand remains hypoplastic
and functional capacity is limited by the inherent skeletal anomalies,
surgical treatment improves functional capacity and cosmetic appearance in
the majority of patients.
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