Poland syndrome in British Columbia: incidence and reproductive experience of affected persons.

Abstract

Patients with Poland syndrome were ascertained through the British Columbia Health Surveillance Registry, through hospital records, and through practicing plastic and orthopedic surgeons. Of 44 patients who were ascertained, 28 had family histories taken and were examined. Physical findings were: absence of the sternal head of the pectoralis major in all patients, symbrachydactyly in most patients, and infrequent other associations such as ipsilateral undescended testis, Möbius syndrome, clubfoot, and submucous cleft palate. Family histories were "negative" in all cases. The 8 affected adults had 24 reportedly normal children. Mean paternal age was significantly higher than the paternal age in the population. The incidence was 1/32,000 livebirths in British Columbia. It was concluded that in the British Columbia population the syndrome is usually a sporadic event.

• Authors: B C McGillivray, R B Lowry
• Journal: American journal of medical genetics (Am J Med Genet) Vol. 1 Issue 1 Pg. 65-74 ( 1977) ISSN: 0148-7299 [Print] United States
• PMID: 205132 (Publication Type: Journal Article)

Topics

• Adult
• British Columbia
• Female
• Humans
• Male
• Maternal Age
• Paternal Age
• Pectoralis Muscles (abnormalities)
• Syndactyly (epidemiology, genetics)
• Syndrome