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Special Feature
December 1999

Picture of the Month

Arch Pediatr Adolesc Med. 1999;153(12):1305-1306. doi:

Figure 1. The left breast, areola, and nipple are absent.

Alfred Poland1 described unilateral absence of the pectoralis major, serratus anterior, and abdominal external oblique muscles on autopsy examination of a 27-year-old man who also had cutaneous syndactyly of the hand on the same side. The syndrome that carries his eponym has been expanded, but it most commonly refers to the association of a congenital unilateral absence of the pectoralis major muscle and syndactyly of the ipsilateral upper extremity.

The constitutive mark of the condition is the absence of the pectoralis major muscle, present in 100% of the cases.2 In a series of 599,109 live births, a unilateral pectoralis major muscle defect was found in 27 neonates, 12 of whom also had hypoplasia and/or syndactyly of the ipsilateral hand.3 The incidence of Poland syndrome is estimated to be approximately 1 in 20,000, with about 10% of patients with syndactyly demonstrating features of the syndrome.4 More than 75% of the defects associated with this syndrome are present on the right side.5