POLAND SYNDROME IN A FEMALE PATIENT RECONSTRUCTED BY ENDOSCOPICALLY ASSISTED TECHNIQUE

INTRODUCTION

Poland syndrome comprises a congenital unilateralabsence of the sternal part of pectoralis major muscle, ipsilateralsymbrachydactyly, and occasionally other malformationsof the anterior chest wall and breast (7). The currenttheory on the etiology of Poland syndrome is hypoplasiaof the subclavian artery, caused by kinking of the arteryduring the 6th week of gestation: the stronger the interaction,the more severe the pathology. Although the conditionis more frequent among males, they seldom seek therapy,especially with less severe forms. On the other hand,female patients request breast symmetry, which is complicatedby lack of anterior chest wall muscle and the anterioraxillary fold. Hester and Bostwick first described a techniqueconsisting of immobilizing the ipsilateral latissimusdorsi muscle, transposing it anteriorly to cover a breastimplant and moving the latissimus insertion anteriorly onthe humerus to establish an anterior axillary fold (4).Although this approach gives a pleasing aesthetic result, itrequires a large incision on the back for muscle harvestingand an incision on the submammary fold for positioningthe muscle and the implant. The associated large scars arenot usually well accepted, so a minimally invasive techniqueshould always be proposed to the patient as an alternativeprocedure (1).

We report a two-stage endoscopically assisted technique,as a modification of the Hester and Bostwick technique (4), in a female patient with Poland syndrome.

CASE REPORT

A 28-year-old woman presented with unilateral breast and chest wall asymmetry (Fig. 1). The sternal head of the left pectoralis major muscle was absent with associated lack of the anterior axillary fold. Interestingly, the clavicular head of the pectoralis major was hypertrophic exaggerating the chest wall anomaly. The breast was severely hypoplastic and the nipple-areola complex was underdeveloped. No hand anomaly was present. Contralateral breast presented ptosis grade I according to Regnault classification. Physical examination revealed normal contraction of the ipsilateral latissimus dorsi muscle.

The patient was a suitable candidate for Hester and Bostwick (4) technique to recreate the anterior axillary fold, to augment the breast volume, and restore the anterior chest wall contour. In order to minimize the associated large scars a two-stage endoscopically assisted technique was planned. The first operation involved an endoscopically assisted approach to dissect and transfer anteriorly the ipsilateral latissimus dorsi muscle. The patient was placed in the left lateral decubitus position. A vertical 5-cm incision was made in the midaxillary line. The dissection of the thoracodorsal vessels and the division of the thoracodorsal nerve were performed under direct visualization without  the use of an endoscope. Then a 5-mm, 30º angled endoscope attached to an endoretractor (KARL STORZ GmbH & Co.KG, Germany) was inserted through the skin incision. The superficial surface of the muscle was initially dissected, and this was followed by dissection of the undersurface. To complete the flap harvest an additional 4-cm incision above the iliac crest was used to facilitate access to the caudal part of the muscle. The muscle was transected at its origin from the iliac crest and the spine. Large perforators were ligated with an endoscopic clip applier. The muscle’s humural insertion was divided under direct visualization and was reattached to the periosteum of the acromion. A subcutaneous dissection was then performed endoscopically at the anterior chest wall to expose the lateral border of the sternum, the lateral border of the clavicular head of pectoralis major muscle and its undersurface. The lateral border of the latissimus dorsi muscle was sutured to the caudal border of the clavicular head of pectoralis major muscle and the caudal border of the muscle to the periosteum of the sternum. A bean-shaped tissue expander (McGhan Medical Corporation, Dusseldorf) was placed under the lattisimus. Percutaneous expansion was begun 2 weeks postoperatively, and the expander was filled to a volume 10% greater than that which seemed necessary (Fig. 2). The total expansion time was 12 weeks. Then the expander was partially deflated and after 2 additional weeks without further expansion, the second stage was performed.

The second operation was performed with the patient in the supine position. The same 5-cm incision in the midaxillary line was used to remove the expander and to insert the permanent cohesive silicone implant (McGhan Medical Corporation, Style 410). The postoperative recovery was uneventful (Fig. 3).

Three years postoperatively the patient was noted to have a well developed anterior axillary fold and adequate infraclavicular fullness (Fig. 4). The shape and volume of the reconstructed breast were pleasing, demonstrating reasonable symmetry with the contralateral breast. The midaxillary and back scars were virtually invisible. The patient was satisfied with the aesthetic outcome of her reconstruction and did not desire any further procedures to achieve greater nipple-areola symmetry.

DISCUSSION

The surgical challenges presented by Poland syndrome depend on clinical manifestations and may include repair of anterior axillary fold and infraclavicular hollowness when the pectoralis major muscle is absent, restoration of breast volume and shape, and reconstruction of nipple- areola complex. Frequently, female patients seek therapy for cosmetic reasons given that functional compromise is very rare, even in the most severe forms of the syndrome with rib involvement. Several strategies have been proposed to achieve the reconstructive goals in Poland syndrome. Custom-made prostheses (2), pedicled latissimus dorsi muscle transfer combined with an implant (9) or with rib grafts (3) have been referred to in the literature, with variable success. In more severe forms Poland syndrome may be associated with an absent or severly hypoplastic latissimus dorsi muscle precluding ipsilateral muscle transposition. In these cases free transfer of the contralateral latissimus dorsi muscle (5) or of the deep inferior epigastric perforator flap (6) have been proposed with fine results. However, these techniques require large incisions for both flap harvesting and flap inset.

In view of the fact that the goal of the treatment is aesthetic, associated large scars are not well accepted; therefore a minimally invasive technique is required as an alternative procedure. Santi et al. (8) first reported in 1985 correction of Poland syndrome through minimal incisions. The authors utilized a dorsal incision to raise the latissimus, and an axillary incision for latissimus muscle’s insertion reattachment and an anterior thoracic incision for muscle inset and implant insertion. Borschel et al. (1) further modified and improved Hester and Bostwick (4) technique using a two-stage endoscopically assisted approach. In the first operation they used an endoscopically assisted technique for placement of a tissue expander to increase the size of the skin envelope. The second operation was also performed endoscopically, to remove the tissue expander, place a permanent implant and transfer the ipsilateral latissimus dorsi muscle. The authors demonstrated reasonable breast symmetry in shape and volume, an anterior axillary fold, and adequate infraclavicular fullness.

In our clinical case we used the same approach as Borschel et al. (1), but in reverse order. The first stage involved endoscopically assisted dissection of the ipsilateral latissimus dorsi muscle, which was transferred anteriorly for chest wall and anterior axillary fold reconstruction. A tissue expander was placed under the transposed muscle to adjust the size of the breast accordingly.

That way the muscle followed the skin expansion, ensuring safe expansion and resulting in a controlled creation of a uniform anatomic envelope. Therefore the insertion of the permanent implant, in the second stage, had a more predictable aesthetic result. The long-term outcome of our approach was characterized by a welldefined anterior axillary fold and pleasing breast contour with adequate volume. The bean-shaped tissue expander smoothed the progress of the inframammary fold formation and facilitated reasonable symmetry with the contralateral breast. With the endoscopically assisted technique we have demonstrated a large reduction in incision length, which is a clear benefit to the patient. Most importantly, the scars were inconspicuous three years postoperatively.

The drawback of the procedure is the technical complexity associated with muscle harvesting and muscle inset at the anterior chest wall, but it is justified by the minor donor site morbidity.

In conclusion, the two-stage technically demanding endoscopically assisted reconstruction of Poland syndrome is rewarded by a fine aesthetic result and inconspicuous scars.

Presented at the 13th Congress of the International Confederation for Plastic, Reconstructive and Aesthetic Surgery (IPRAS), 10–15 August 2003, Sydney, Australia.

Address for correspondence:

Andreas Gravvanis, M.D., Ph.D., FEBOPRAS

10 Patroklou Str., Agia Paraskevi

15343, Athens

Greece

E-mail: gravvani@yahoo.com