Breast Reconstruction in Patients with Poland Syndrome

James D. Namnoum

Introduction

In 1841, while completing a preceptorship in anatomy at Guy’s Hospital in London, Alfred Poland described the characteristics of a cadaver with notable unilateral chest wall and upper extremity deformities involving the pectoralis major and minor, serratus, and external oblique muscles (1). This syndrome, later named after him by Clarkson (2)—although, according to Ravitch (3), it had been described earlier in separate reports by Lallemand and Foriep—includes a variety of findings of varying combinations and degrees of severity. Classically, the deficiency or absence of the sternocostal portion of the pectoralis major muscle with some degree of breast involvement is the hallmark feature of Poland syndrome. However, other muscles of the thorax and abdomen and underlying bony skeleton may also be affected in the thoracic component of this syndrome. The various hand and upper extremity anomalies found in frequent association are beyond the scope of this discussion (4).

Etiology and Incidence

Most cases of Poland syndrome occur spontaneously; however, a familial form of inheritance has been described, as well as a case of bilateral involvement (5,6). The incidence ranges from 1 to 20,000 to about 1 to 30,000 births (7,8). Poland syndrome has been observed primarily in males and appears to generally affect the right side (9). Several theories abound regarding possible etiologies, but no causation has been established (10,11,12,13,14).

Classification

Conceptually, patients with Poland syndrome can be organized into three categories based on their physical findings (Table 121.1).

Mild Poland Deformity

These patients demonstrate the mildest form of the Poland deformity (Fig. 121.1). Typically, these patients show hypoplasia of the breast, nipple-areolar complex, and pectoralis major muscle that may only be appreciated radiographically (15). In this respect it may be a different entity than the anterior thoracic syndrome described by Spear et al. (16).

Moderate Poland Deformity

The classic deformity seen in Poland patients includes hypoplasia of the affected breast and nipple-areolar complex, as well as absence of the sternocostal portion of the pectoralis major muscle (Fig. 121.2). Some portion of the sternoclavicular portion of the pectoralis muscle is present in most cases, although it may be rudimentary. On frontal view, the patients demonstrate marked breast asymmetry, with a small, elevated nipple-areolar complex. Absence of the anterior axillary fold and deficient soft tissue “fill” in the infraclavicular region are common features (Fig. 121.3).

Severe Poland Deformity

The most challenging deformity to reconstruct is characterized by absence of the breast and at times nipple-areolar complex, absence of the pectoralis muscle, and absence or marked deformities of the ribs and sternum. Tight skin of the chest and axillary webbing may be seen (Fig. 121.4).

Treatment

Treatment of the Poland patient is indexed to the severity of the deformity. The goal, as in all breast surgery, is to create a breast that is symmetric with the opposite side. Options for treatment include tissue expanders, permanent breast implants, and autologous tissues, free and pedicled, with or without implants (17,18,19,20,21,22,23,24,25,26,27). Adjunctive procedures such as the use of fat transfer to the breast and chest or acellular dermal matrix help to refine the results and may extend the application of certain treatment options.

Only gold members can continue reading. Log In or Register to continue