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

As mentioned above, patients with Poland’s syndrome may present with various degrees of breast hypoplasia. There may be accompanying nipple–areola hypoplasia and abnormal placement of the nipple–areola complex. Each case has its own unique features and a highly individualized approach to surgical intervention is needed. Will optimal breast symmetry require single side or bilateral breast surgery? Can the procedure be done in a single stage or is more than one operation necessary to achieve the best possible result? Although staged reconstruction is the preferred method of implant breast reconstruction in the post-mastectomy patient, most congenital breast reconstruction procedures are not ‘covered’ by health insurance in the United States and therefore the cost of a multiple (two) stage procedure becomes an important factor.

Pitfalls and How to Correct

Most reconstructions for congenital and developmental breast problems are performed with the placement of a breast implant. In my practice bilateral breast surgery is done in the majority of cases (Figs 40.3–40.5). If there is sufficient thickness of the soft tissue envelope on the side of the patient’s hypoplastic breast an implant can be placed beneath this tissue and on top of the ribs to improve breast appearance and symmetry. In cases of congenital breast deformity the goal is improvement in breast appearance not perfection. I continually reinforce this to the patient and the patient’s family. The patient pictured in Figure 40.3 had been treated in a one-stage procedure with a unilateral implant breast reconstruction as a teenager. As she matured her right breast became larger than her left and she requested a revision procedure. An implant exchange that included anterior, superior and lateral capsulotomies coupled with placing a much larger implant improved her breast appearance.

Fig. 40.3 A This 26-year-old patient had undergone a single stage reconstruction of her left breast at age 16 with the placement of a 400 cc silicone gel breast implant. She has a dimensionally and volumetrically smaller left breast with a Baker III capsular contracture. B This was addressed with anterior, superior and lateral capsulotomies and exchange for a 600 cc high profile silicone gel breast implant with a substantially increased diameter. She is seen 6 months following the revision surgery.

Fig. 40.4 A, B This 16-year-old patient presented with severe hypoplasia of the left breast as a prominent feature of her Poland’s syndrome. She has pronounced ptosis of the opposite right breast. She is shown following staged reconstruction of the left breast with a tissue expander and implant placement and a contralateral right mastopexy for symmetry on C anteroposterior and D lateral view.

Fig. 40.5 A This 17-year-old patient presented with severe right breast hypoplasia with nipple hypoplasia and superior malposition of the right nipple as a feature of her Poland’s syndrome. B She underwent the subcutaneous placement of a tissue expander beneath the skin and soft tissues of the right breast to expand the soft tissue envelope and increase the distance from the clavicle to the nipple. A latissimus dorsi muscle flap was harvested using two small incisions on the back and single incisions in the axilla and inframammary region of the breast. C, D The muscle was transposed to the chest through a high axillary tunnel and placed beneath the expanded soft tissue envelope and was anchored to the parasternal area and inferior aspect of the expanded breast envelope.E An implant was placed beneath the muscle to provide the necessary volume to the breast reconstruction producing an immediate and F long-term improvement of the breast appearance at 3 years postoperative follow up. G, H This produced an excellent change in the breast appearance at a 3 year follow up after surgery.

This case courtesy of Julian J. Pribaz MD.

In cases where there are abnormalities in both breasts bilateral breast surgery is often needed to optimize breast appearance and symmetry. Such a case is illustrated in Fig. 40.4. This patient with Poland’s syndrome presented at age 16 with an extremely hypoplastic left breast and the ‘forme fruste’ of a constricted breast deformity on the right side. Due to the virtual absence of breast development on her left side (the affected side) and the need for considerable breast volume and dimension, reconstruction of the breast in stages was ideal. We initially achieved shaping and molding of the soft envelope of the left breast area with the preliminary placement of a tissue expander which was followed at a second stage by the placement of an implant with a contralateral mastopexy (Fig. 40.4C–F).

Postoperative Care

An elegant use of the latissimus dorsi for reconstruction of severe Poland’s syndrome is illustrated in Figure 40.5. This patient presented with severe breast and nipple hypoplasia and a deficiency of skin in the upper pole of the right breast with a superiorly displaced nipple–areola complex (Fig. 40.5A). In this case the quantitative deficiency of the covering soft tissue envelope was addressed first. This involved the subcutaneous placement of the expander (Fig. 40.5B) to expand the overlying breast skin thereby allowing the nipple–areola complex to be moved inferiorly. When a sufficient amount of skin had been generated, the latissimus dorsi muscle was transposed and an implant can be placed beneath the muscle to complete the reconstruction of the skin envelope, subcutaneous tissue and breast. (Fig. 40.5C, D) A breast implant was placed beneath the latissimus dorsi muscle to provide the necessary volume for a very good breast reconstruction (Fig. 40.5E–H) which was maintained at a 3-year follow up after surgery.

Summary

The breast appearance varies widely in Poland’s syndrome thereby demanding a highly individualized approach for each patient. The patients are analyzed for the degree of breast asymmetry. Not uncommonly a procedure on the opposite breast in the form of a breast augmentation, mastopexy or augmentation mastopexy is preferable to optimize symmetry.

The quantitative and qualitative features of the soft tissue envelope in the affected breast are studied. Implant reconstruction predominates for practical and fiscal reasons. In these cases a dimensional approach is helpful in that topography will guide the selection of the appropriate implant of tissue expander. This works well in patients with mild to moderate breast hypoplasia. For more significant tissue deficiencies an implant reconstruction may be done in two stages, with tissue expander placement initially to shape and mold the overlying tissues with the subsequent placement of an implant.

Breast reconstruction with autologous tissue from the back in the form of a latissimus dorsi muscle flap has had a role in the reconstruction of patients with severe soft tissue defects as we have illustrated. In the future the use of free microvascular tissue transfer in the form of muscle-sparing perforator flaps will find an increased role in the treatment of these young patients.