Review Question - QID 4551

Figure D demonstrates symbrachydactyly which is most commonly associated with Poland's syndrome.

Poland's syndrome is a rare birth defect characterized by underdevelopment or absence of the chest muscle in conjunction with ipsilateral symbrachydactyly. Poland syndrome most often affects the right side of the body, and occurs more often in males than in females.

Ireland et al. reviewed 43 consecutive cases of Poland's syndrome, and reviewed the relevant literature up to that point. The authors state that the clinical features are variable but always include congenital aplasia and syndactyly, and the right side is affected more than the left. They also note that 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.

Van Heest summarizes normal formation and growth of the upper limb as a basis for understanding malformation, with the goal of providing a basic understanding of the evaluation necessary for appropriate counseling and referrals for treatment of the child with hand and upper extremity congenital deformities.

Incorrect Answers:
Answer 1: Figure A demonstrates the classic deformity associated with Apert's syndrome, which commonly consists of complex syndactyly.
Answer 2: Figure B demonstrates the lymphedema characteristic of amniotic band syndrome.
Answer 3: Figure C demonstrates radial club hand which can be associated with VATER syndrome.
Answer 5: Figure E shows and example of thumb polydactyly which is associated with a variety of syndromes, but not typically Poland's.