Congenital Anomaly Of The Month: Poland’s Syndrome
Poland’s syndrome is a congenital disorder. The classic ipsilateral features of Poland syndrome include the following: absence of sternal head of the pectoralis major, hypoplasia and/or aplasia of breast or nipple, deficiency of subcutaneous fat and axillary hair, abnormalities of rib cage, and upper extremity anomalies. These upper extremity anomalies include short upper arm, forearm, or fingers (brachysymphalangism). (photo credit)
Additional features of Poland syndrome include the following: hypoplasia or aplasia of serratus, external oblique, pectoralis minor, latissimus dorsi, infraspinatus, and supraspinatus muscles; total absence of anterolateral ribs and herniation of lung; and symphalangism with syndactyly and hypoplasia or aplasia of the middle phalanges. (photo credit)
The name of this condition pays homage to Dr. Alfred Poland of Guy’s Hospital, who in 1841 described a case of these two deformities during the autopsy of a 27-year-old convict, but as this article points out he wasn’t the first to recognize the syndrome.
If you enjoy medical history, then you will enjoy this article. It explores the historical literature to reveal the progression of knowledge about this syndrome. Here is a quick summary of different investigators who contributed to the understanding of Poland’s syndrome. The article goes into more detail of each.
| 1826 | Lallemand is first to describe the absence of the pectoralis. |
| 1835 | Bell is the first to record the absence of the pectoralis |
| 1839 | Forlep is first to describe the paired absence of the pectoralis and ipsilateral syndactyly |
| 1841 | Poland is the second to describe the paired absence of the pectoralis and ipsilateral syndactyly |
| 1895 | Thomson is the first to document an understanding that the deformities accompanied one another |
| 1900 | Furst is the first to propose that the anomalies constituted a syndrome |
| 1902 | Bing is the first to present a case series of patients with the syndrome |
| 1940 | Brown and McDowell are the first to document a thorough review of the syndrome |
| 1962 | Clarkson is the first to propose the name “Poland’s Syndactyly” for the syndrome |
As the authors conclude:
Honoring physicians for notable achievements in the form of eponyms can be viewed as a harmless way to bring a little bit of warmth to an otherwise cold world of facts. The least we can do, though, is to recognize the contributions of those who endeavored to shape our current understanding of disease.
Perhaps if history took another course, Poland’s syndrome would instead be called Frolep’s syndrome or Furst’s syndrome. Or perhaps it might simply have been called pectoral-aplasia-dysdactylia syndrome
REFERENCES
Poland’s Syndrome: Current Thoughts in the Setting of a Controversy; Plastic & Reconstructive Surgery. 123(3):949-953, March 2009; Ram, Ashwin N. B.S.; Chung, Kevin C. M.D., M.S. (subscription required)
*This blog post was originally published at Suture for a Living*
Return to article »