Correction of Poland Syndrome Breast Deformity

Introduction

Poland syndrome is a complex malformation syndrome and was first described by Alfred Poland in 1841 and in French literature by Lallemand in 1826. The diagnosis is clinical and the malformations are not life-threatening. The impact is mostly psycho-social and the malformations become more visible at puberty when the growing process is accelerated. The functional impact is minimal, even in hypoplasia or agenesis of several muscles.

Clinically, Poland syndrome presents with hypotrophy or agenesis of one or more muscles of one side of the thoracic wall or thoracic cage and sometimes the ipsilateral hand. The constant malformation encountered is agenesis of the sterno-costal insertions of the pectoralis major muscle. It can also be associated with complete agenesis of the pectoralis major, pectoralis minor, breast tissue and sometimes of the latissimus dorsi muscle or other muscles of the rotator cuff. When involving the breast, the skin is very thin with small or no amount of subcutaneous tissue and mammary gland agenesis or hypotrophy. In more severe cases, Poland syndrome can be associated with osseous and cartilaginous deformities of the chest wall on the affected side and also hand malformations.

In this chapter, we focus on the treatment of breast deformities in Poland syndrome. These deformities have an important psycho-social impact on young females and this is why they consult at a young age for a solution to their breast asymmetry. The breast deformity can be augmented by an underlying thoracic deformity. For the treatment of this syndrome numerous corrective surgical techniques have been developed: from bone and cartilage correction in cases with severe deformities, to implants for the chest wall, or breast implants and tissue expanders, fat transfer and flaps. These techniques can be used individually or they can be associated for a better aesthetic result.

Indications and Contraindications

Depending on the severity of the breast malformations present in patients with Poland syndrome, the patients can be classified into three major categories: mild, moderate and severe, according to the classification described by Foucras et al. ( Table 23.1 ).

For patients with mild breast deformity ( Fig. 23.1 ), our preferred correction technique is fat transfer or lipomodeling. It can also be corrected using a combination of lipomodeling and mammary implants of asymmetric size, when the patient desires breast augmentation. This technique requires several operations to achieve the desired volume and symmetry.

For patients with moderate breast deformity ( Fig. 23.2 ), our preferred approach has been latissimus dorsi transfer and breast implants or tissue expanders. In our team, we prefer to perform autologous reconstruction for these young patients and our preferred method is fat transfer. We use lipomodeling exclusively if the patient has enough fat deposits to allow four or five fat grafting sessions. If there are not enough fat deposits and we can perform only two lipomodeling sessions, we combine these with silicone implant reconstruction. The reconstruction can be obtained by combining lipomodeling with tissue expander and silicone breast implant. The purpose of lipomodeling in these cases is to increase tissue thickness for a better aesthetic result and better implant coverage.

For patients with severe breast deformity ( Fig. 23.3 ), if rib aplasia is present, rib reconstruction can be performed first, or personalized silicone implants created using a 3-D mould taken from the patient or a 3-D CT-scan reconstruction. The breast can be reconstructed using the previously described techniques. If the latissimus dorsi is hypoplastic as can sometimes be the case, other flaps can be used, such as the deep inferior epigastric perforator (DIEP) free flap and, if there is not enough abdominal laxity, superior gluteal artery perforator (SGAP) or inferior gluteal artery perforator (IGAP) flaps are good options.

Preoperative Evaluation and Special Considerations

The diagnosis in Poland syndrome is mainly clinical. Examination of the pectoralis major muscle is done by adduction against resistance.

The absence of the anterior axillary fold is a pathognomonic sign of pectoralis major agenesis. Thoracic wall deformities like pectus excavatum can also be observed if chondro-osseous deformities are present. The presence of latissimus dorsi muscle can be confirmed by adduction against resistance and by the existence of the posterior axillary fold.

Breast examination is also mainly clinical: the presence or absence of mammary tissue, the position of the nipple–areola complex, the presence or absence of the nipple–areola complex, and the skin and subcutaneous tissue thickness should be noted. It is also essential to analyze the contralateral breast: dimensions, base width, ptosis, hypertrophy.

The mild forms do not require any specific exams. A breast ultrasound can be performed before surgical treatment.

When treating severe deformities, a CT scan with 3-D reconstruction should be performed in order to have a complete analysis of all deformities and, if necessary, the scanner can be a useful tool in personalized chest wall silicone implant creation.

The algorithm that we use in our practice is:

• For mild cases (stage I):

  • fat grafting in several sessions (2–4 sessions) spaced by ~3 months (our first choice of treatment)

• For moderate cases (stage II):

  • fat grafting in several sessions (4 or more sessions)

  • lipomodeling in association with breast expanders and breast implants if the patient does not have enough fat deposits and she desires breast augmentation

  • short scar total autologous musculocutaneous latissimus dorsi flap in association with fat grafting

  • the pectoro-mammary flap in association with lipomodeling when the contralateral breast is hypertrophic and the patient desires autologous reconstruction without other donor sites, or when the latissimus dorsi is absent or hypotrophic

  • free flaps such as DIEP, SGAP, IGAP or transverse upper gracilis (TUG)

• For severe cases (stage III):

  • the thoracic wall can be corrected first if required with personalized silicone implants and afterwards the breast reconstruction is performed as shown for stage II deformities.

Surgical Techniques

Relevant Anatomy in the Poland Syndrome

The constant deformity is agenesis of the sternal insertions of the pectoralis major muscle. The most frequently described form of Poland syndrome is the association of breast and pectoralis major hypoplasia without upper limb malformations ( Fig. 23.4 ).

In regard to the muscular malformations, most authors describe agenesis of the inferior and middle pectoralis major fascicles. The clavicular fascicle of the pectoralis major muscle is usually normal. Seyfer et al. found that in 20% of cases the clavicular fascicle is absent and these patients present clinically with an infraclavicular depression and the absence of the anterior axillary fold.

There are other muscles that can be hypoplastic or absent: pectoralis minor, serratus anterior, latissimus dorsi (in about 10% of cases), intercostal muscles, rectus abdominis muscle on the affected side, external oblique muscle, deltoid muscle, trapezius muscle, the supraspinatus and infraspinatus muscles.

The skin on the affected side is usually thin with little or no subcutaneous tissue. This deformity is more frequent in men. The absence of axillary hair and sudoriferous glands can also be recorded in some patients.

Sometimes a bridle is present at the level of the anterior axillary fold, and it limits the abduction movement of the arm. The mammary gland is usually hypotrophic and it can also be atrophic or absent. The breast hypoplasia can be accentuated by the chest wall deformities.

Osteo-cartilaginous malformations and chest wall deformities are recorded in 20% of cases. Rib hypoplasia and sometimes aplasia can be found. Usually, the sternal insertions of the 2nd to 5th ribs are affected. In severe chest wall deformities, a pectus excavatum of the affected site can be observed.