Poland's Syndrome

Abstract

Seven cases of Poland's syndrome (absence of the sternal por tion of the pectoralis major with ipsilateral upper extremity anomalies) are compared with 48 previously reported cases.

Poland syndrome associated with renal agenesis

“…Absence of pectoralis muscles most often occurs in association with symbrachydactyly of the ipsilateral hand. The ipsilateral deformity of thumb is not frequently associated with Poland syndrome [10]. The elevation of the hemidiaphragm, or the observed developmental delay in our patient, is also not a primary feature of Poland syndrome.…”

“…In one previously reported case, Mace et al [10] described an association of the ipsilateral renal aplasia in an 18-month-old boy with a typical left-sided Poland anomaly. Miller and Miller [16] described a 9-year-old boy with absent right pectoralis major muscle and an undescended left testis.…”

“…Renal anomalies associated with Poland syndrome have been reported infrequently [10,11], and rarely in the pediatric literature [12,13]. We describe an infant with Poland syndrome who also had ipsilateral aplasia of the kidney.…”

A case with Poland's syndrome and athelia

“…The syndrome is characterised by congenital aplasia of the sternocostal head of the pectoralis major muscle associated with ipsilateral hand deformities [6]. This rare entity is referred to as Poland's syndrome or Poland's syndactyly [3,4,7]. The former term seems to be more appropriate since multiple abnormalities of the upper extremity and the chest wall are usually associated with the syndactyly.…”

“…The former term seems to be more appropriate since multiple abnormalities of the upper extremity and the chest wall are usually associated with the syndactyly. Less frequently reported features of Poland's syndrome include the absence of digits, brachidactyly, hypoplasia of wrist and/or hand, hypoplasia of forearm, hypoplasia of arm, thoracic cage defects, herniation of lung, axillary web or band, absence of nipple and some genitourinary abnormalities [6,7]. Congenital absence of the breast (amastia) and of the nipples (athelia) is an extremely rare anomaly which occurs more frequently in females [2,4,8,9].…”