Prepectoral Reconstruction Using Alloderm Matrix in a Patient With Unilateral Poland Syndrome: A Case Report and Review of Literature

Abstract

Poland syndrome is characterized by unilateral hypoplasia of the breast, chest wall deformities, and arm and hand deficits. The syndrome has a large spectrum with degrees of severity and presentation. This congenital condition has been well reported in literature with a broad range of options for surgical correction. We report a 15-year-old female with typical features of Poland syndrome and the reconstructive efforts undertaken to preserve pectoralis function and a normal chest wall while creating a natural-looking breast with the prepectoral method of breast reconstruction using acellular dermal matrix.

Introduction

Originally described by Alfred Poland in 1841, Poland syndrome is a congenital anomaly with extremely variable clinical manifestations. The spectrum includes hypoplasia of the breast and nipple, scarcity of the subcutaneous tissue, lack of pectoralis minor muscle and absence of the costosternal portion of pectoralis major, deformity or aplasia of ribs II to IV or III to V or the costal cartilages, alopecia in the mammary or axillary region, and ipsilateral brachysyndactyly. ¹ The diagnostic criteria now include hypoplasia or aplasia of the pectoralis major muscle and at least one other ipsilateral defect. ² While the exact pathophysiology of Poland syndrome is unknown, the most widely accepted theory is the hypoplasia of the subclavian artery or its branches during the sixth week of gestation while the upper limb bud and chest wall are in development. ^1,3,4

Most cases of Poland syndrome are sporadic, with a <1% risk of reoccurrence in the same family. ^1,2 In patients with familial Poland syndrome, an autosomal dominant with incomplete penetrance pattern has been demonstrated. ² The reported incidence ranges from 1/7000 to 1/100 000, with a higher prevalence in the male population (2:1 to 3:1 ratio). In males, the right side is affected more than twice as often as the left, while females show an equal presentation in left- and right-sided presentation. ¹ Presentation is usually unilateral, but recent literature has shown patients with bilateral features of Poland syndrome. ²

Impairment in function in patients with Poland syndrome is typically mild. Due to this, patients usually present late and for aesthetic concerns. ² Common operative indications include depression of the chest wall, paradoxical movement of the chest wall, hypoplasia or aplasia of the female breast, reconstruction of the male chest for lack of the pectoralis major and axillary fold, and upper limb or hand deformities causing functional deficits. When planning surgical correction of the female chest and breast, both the degree of muscular deficiency and chest wall deformity must be considered to determine the best approach. ¹

Case Report

The patient is a 15-year-old female with Poland syndrome. She had normal menarche and right breast development at the age of 12. She is an otherwise healthy female with no medical illnesses. She had no breast development on her left chest wall, including absence of breast mound and nipple and areolar complex (Figure 1). She had a normal chest wall structure. The patient had little subcutaneous tissue and thin skin on the affected side. There was clinical evidence of the pectoralis major muscle having mild hypoplasia but full function.

Figure 1.

Preoperative appearance.

The decision was made to proceed with surgical reconstruction. Due to the lack of breast tissue present, she required multiple step reconstruction starting with a tissue expander, followed by implant reconstruction and potential nipple areolar reconstruction. The first surgery was planned to place an expander in the standard subpectoral space. However, at the time of surgery, it was noted that pectoralis major muscle was severely hypoplastic and was only present starting at the third rib space. In order to preserve full function of the muscle, an intra-operative decision was made to use prepectoral reconstruction. A through-and-through skin suture technique was used to tack a full sheet of acellular dermal matrix (ADM, Alloderm) in the subcutaneous space covering the full left chest from second rib superiorly, sternum medially, and eighth rib inferiorly. A 300 mL Allergan MV tissue expander was placed underneath this and filled to 75 mL.

On initial follow-up, good healing was noted at the incision. The patient went on to have some lateral incision breakdown that required debridement and antibiotic treatment. Standard transcutaneous saline expansions were carried out. Once the expander reached 235 mL, the patient elected to undergo an expander implant exchange. At the time of expander implant exchange, it was noted that the ADM was completely incorporated and gave the patient excellent tissue quality and strength. The previous wound issues created a tight lower lateral band, and to obtain proper positioning, a partial capsulectomy was done on the medial and lateral inferior areas.

At last assessment, the breast reconstruction had good overall shape and size match to the natural breast. It was sitting about 1 cm higher than it should naturally, likely do to scarring and surgical healing (Figure 2). She was instructed to start implant and scar massage. The patient had full arm range of motion without any chest wall restrictions. She had no pain on arm or shoulder movement and no implant animation. The patient was given the options of nipple reconstruction or tattoo, as well as a scar revision and release. She was overall happy with her appearance and declined further revisions.

Figure 2.

Post-operative appearance.

Discussion

Developmental maturity of the female breast is achieved by 16 to 19 years of age. Most individuals affected by Poland syndrome present with a normally developed breast on the non-involved side and a small, abnormally shaped, or absent breast on the other. The nipple and areola is usually anatomically normal but displaced superiorly and small. On rare occasions, the nipple and areola may be completely absent. The amount of subcutaneous tissue varies depending on the body habitus of the patient and the penetrance and degree of the syndrome. ⁵

Initial attempts at reconstruction for these patients with simple breast prosthesis placed in a subcutaneous pocket resulted in a high incidence of capsular contracture, superior displacement of the nipple and areola (bottoming out), lack of contour, visibility, palpability, implant exposure due to skin breakdown, and continued concavity of the infraclavicular region. The use of tissue expanders allows for gradual expansion of the skin, soft tissue, and muscle to recreate a natural-appearing breast. ⁵ Regardless of whether an implant was used as a single stage, or a 2-stage reconstruction was done with expanders and implants, most complications resulted from insufficient soft tissue coverage. This realization lead to submuscular techniques. ^5,6 Since the final implant is placed beneath the pectoralis muscle in submuscular techniques, there is almost always implant animation with chest and hand motion. In one method of submuscular placement, the implant is placed under pectoralis major and the inferior origin of the muscle is not released. This results in inferior restrictions and ultimately prevents lower pole expansion, poor breast projection, and poor definition. If the inferior portion of the pectoralis major muscle is released, the reconstruction is covered partially by pectoralis major superiorly and the skin and subcutaneous tissue inferiorly. Without the inferior attachment point of pectoralis major, the muscle is free to migrate superiorly leading to “window shading.” This can result in a band across the implant that changes the shape and projection. The subcutaneous coverage of the inferior aspect of the implant causes the same problems seen with the original subcutaneous technique. ⁶

To improve the results of reconstruction, ADM has been used to complete the subpectoral pocket. A sling of ADM is sutured between the inframammary fold and the lower pole of the released pectoral muscle. The use of ADM in this manner addresses the issue of inferior pole coverage and allows for submuscular placement of the implant for a more natural breast contour. However, animation of the implant still remains. ⁷ The prepectoral technique of the reconstruction with ADM allows the pectoralis muscle to remain intact and give full coverage and support. This results in less pain, better subcutaneous tissue volume, no animation deformity, and possible decreased capsular contracture rates. ⁸

Conclusion

Many different approaches for reconstruction of the female breast in Poland syndrome are described in the literature (eg, pedicled or free rectus abdominus muscle, latisumuss dorsi pedicled muscle, submuscular implant placement, and autologous fat injection). ^{5,6,9 -11} With the multitude of options available, it is the severity of the malformation and patient preference that ultimately dictates the reconstructive technique. The reported patient had characteristic findings of Poland syndrome. These included left breast hypoplasia, lack of nipple areolar complex, and hypoplasia of the left pectoralis major muscle. In order to preserve fullness of the upper pole, limit implant animation, and maintain full chest wall muscle function, a prepectoral plane was chosen. This case illustrates the usefulness of this option of reconstruction in these types of cases.