Poland's syndrome: a review of 23 cases

Abstract.

In 1841, Sir Alfred Poland described congenital deficiency of the pectoralis major muscle associated with brachysyndactyly. In this report a description of the variety of Poland's syndrome is presented by reviewing 23 patients treated in three different hospitals in The Netherlands. Sixteen patients were subjected to a meticulous physical examination and the medical records of all patients were reviewed. There were 15 males and 8 females in our group. Nineteen patients had right-sided Poland's syndrome, four left-sided. Two patients were related to each other (mother–daughter). The severity of the syndrome varied from mild hypoplasia of the pectoralis major muscle and mild hypoplasia of the hand to aplasia of the pectoralis major and serratus anterior muscles, rib and sternal defects, aplasia of the breast, and complete aplasia of the radius and hand. Thirteen patients required surgery for hand, chest, or breast deformities, ranging from 1 to 8 operations per patient. This study shows that Poland's syndrome is an extremely varied condition, which appears to involve an entire upper quadrant. The milder forms may easily be overlooked and do not require surgical intervention. The more severe cases may require hand, chest wall, and/or breast reconstruction. In general, expert specialist attention in a multidisciplinary setting is required for patients with this condition.