Summary/Key Points

Poland’s Syndrome or Poland Anomaly – Introduction

Poland’s syndrome is a congenital anomaly which is a constellation of unilateral abnormalities most frequently involving the chest wall musculature and ipsilateral upper extremity with syndactyly and brachydactyly being the most common clinical findings.

These findings were published in 1841 in the Reports of Guys Hospital in London by Sir Alfred Poland who did a prosection (laboratory dissection) of an upper extremity in a patient with syndactyly.¹ In 1962 a plastic surgeon named Patrick Clarkson also working in London at Guys Hospital encountered a series of three patients with hand abnormalities with accompanying breast hypoplasia. Clarkson reported these three cases² and also found the original hand specimen that had been dissected by Poland, which had been kept in the museum of Guys Hospital.

Poland’s syndrome is a condition with an incidence of approximately 1 in 30,000. It is three times more common in males than females, and the right side of the body is affected twice as often as the left. The etiology is unknown.

The Poland anomaly presents with many variations of chest wall abnormalities ranging from absence of the sternal head of the pectoralis major and minimal involvement of the breast to various degrees of breast and nipple–areola hypoplasia and nipple–areola malposition. In its extreme form there may be absence of ribs, the scapula, the latissimus dorsi muscle, and complete absence of the breast. This form is very rare.

Patient Selection

Indications

Clinical presentation

Patients with Poland’s syndrome are usually seen early in life for evaluation of upper extremity abnormalities. These are either syndactyly or synbrachydactyly and some element of upper extremity hypoplasia. A typical example of a young female patient is illustrated in Fig. 40.1.

Fig. 40.1 Young patient presenting at age 3 with Poland’s anomaly. Note the hypoplasia of the left upper extremity and typical chest wall appearance with absence of the anterolateral chest fold.

At this point in time decisions are made regarding the need for surgical intervention for the congenital hand problems. Most often this might include correction of the syndactyly.

Absence of the anterolateral axillary fold is apparent during early childhood but problems with breast development may not be apparent. The potential of problems with breast development is discussed with the parents and plans are made to periodically follow patients through their young teenage years and into late adolescence (Fig. 40.2)

Fig. 40.2 Adolescent with Poland’s anomaly. She is concerned about her hypoplastic left breast. Note differences in the nipple–areola complex position and size when compared to her opposite breast.

Alternatively, it is not uncommon for patients with breast development problems to present for the first time during the late phases of puberty for plastic surgery consultation (Fig. 40.2)

Breast Reconstruction Options – Operative Technique