Poland Syndrome Treatment & Management

Updated: Jul 03, 2018
  • Author: Bradon J Wilhelmi, MD; Chief Editor: James Neal Long, MD, FACS  more...
  • Print
Treatment

Surgical Therapy

In female teenagers, abnormal breast development can influence the patient's psychological growth. However, if the reconstruction is performed too early, asymmetry may result with continued growth of the breasts. In this case, a secondary revision procedure may be required once breast development is completed, but this compromises the overall result. When the young female patient experiences intense anxiety associated with the breast deformity, one may consider early treatment with a temporary expander, planning for additional reconstruction once breast development has been completed.

During adolescence, a subcutaneous tissue expander can be placed in the affected side. The expander is placed in the subcutaneous plane, since the pectoralis muscle is absent. This expander can be inflated at intervals to rival or match the development of the unaffected breast. This expanded breast does not exactly match the uninvolved side but may help the patient look more symmetrical. Placement of the expander during breast development allows for expansion of the skin to accommodate the eventual permanent implant and latissimus muscle. Moreover, this expansion of the breast skin may enlarge the hypoplastic nipple-areola complex (NAC) often present in these patients. Tissue expansion also can correct the eccentric and elevated NAC observed in patients with Poland syndrome through strategic expander placement to lower it. [13] Tissue expansion can be performed with a pure temporary tissue expander that is eventually replaced with a permanent implant or with an permanent implant expander.

If the patient has mild breast asymmetry, breast reconstruction with an implant expander can be considered in a one-stage procedure. As described above, an implant expander is a permanent implant with a removable remote port. The implant expander can be incrementally expanded postoperatively until the desired size is achieved to match the other breast. Then, the port of the expander can be removed through a separate incision while the permanent implant is left in place. The most popular implant expander is the Becker implant, which contains silicone gel and saline. The saline component of this implant allows for adjustable expansion.

Occasionally, with long periods of implant inactivity between expansions, a capsule contracture may develop, which can restrict expansion. An open capsulotomy may be required to release the capsule and allow for further tissue expansion and or implant repositioning. Finally, upon completion of breast development (when the patient is aged 18-19 y), the tissue expander can be removed and the breast can be reconstructed with the latissimus muscle transposed over a permanent implant.

The latissimus muscle can be used to correct the absence of the axillary line, correct infraclavicular flattening, and provide subcutaneous filler to cover the edge of the implant, thus preventing or minimizing rippling. Occasionally, a de-epithelialized skin paddle may be required with the latissimus muscle to reconstruct the axillary line. If the nipple is absent the skin paddle of the latissimus muscle can be used to reconstruct a nipple, and the areola can be reconstructed with tattooing.

The latissimus muscle is harvested through a small axillary incision and a transverse incision in the back, which can be concealed in the bra line. The muscle is transposed anteriorly over the breast implant and sutured to the pectoralis fascia superiorly, medially, and inferiorly. The use of the latissimus is saved until the completion of breast development and tissue expansion; if used earlier over the tissue expander, the muscle is attenuated with expansion. If skin is required with muscle harvest, the back incision can be modified to recruit as much skin as needed, making sure to still be able to close the back primarily.

When striving to achieve breast symmetry at the final operation, treatment of the contralateral breast by reduction, mastopexy, or augmentation may be indicated. Furthermore, over time, the patient may develop a unilateral contracture to the breast prosthesis, resulting in loss of symmetry. Accordingly, long-term symmetry may be optimized by placement of an implant in the unaffected breast, as well. If the unaffected breast is excessively large, it may require a reduction or mastopexy as an adjunct to implant insertion.

If tissue expansion does not correct nipple-areola asymmetry, additional procedures may be needed to correct nipple and areolar size and location. Consider nipple-sharing composite grafts if the unaffected nipple is of adequate size, or consider nipple reconstruction with local flaps. Areolar discrepancies can be corrected with crescent excisions, strategic tattooing, or relocation by transposing through a new skin opening and skin closure of old location. These nipple-areolar reconstruction procedures are usually performed at a separate stage after the maturation from the initial reconstructive procedure.

If the latissimus is absent as part of the Poland syndrome complex, other options for reconstruction include free latissimus muscle from the unaffected side, the transverse rectus abdominus muscle (TRAM) flap, Rubens flap, gluteus maximus flap, thigh flap, or free perforator TRAM or gluteus flaps. Because thoracic vascular anomalies also can be encountered in patients with Poland syndrome, assessment of recipient vessels with duplex or angiogram is required. [2] Another described reconstructive option is a customized prosthetic implant for the chest wall placed under the breast implant. [16, 17, 18]

The TRAM flap should not be considered for those who intend to become pregnant. The gluteus and Rubens flaps have short pedicles, often requiring grafts for microanastomoses, and result in significant donor site asymmetry. In general, these other reconstructive options provide less aesthetically acceptable results than the latissimus muscle for coverage over the implant; however, treatment must be individualized.

For male patients with Poland syndrome who have an intact latissimus muscle, consider reconstruction when they are aged 12-13 years. The ipsilateral latissimus muscle is harvested through a small incision in the back and axilla and transposed to fill the void of the absent pectoralis major muscle. The latissimus is folded along the sternal and inferior borders to resemble the contour of the pectoralis major muscle. The humeral insertion of the latissimus muscle must be detached, anteriorly transposed, and sutured to the bicipital groove of the humerus. Reconstruction with prosthesis also has been described for males but with disappointing results. If the latissimus is absent on the affected side, the contralateral latissimus can be used as a free flap to the axillary or internal mammary vessels after appropriate evaluation for recipient vessels with duplex or angiogram. For more information, see Pectoral Implants.

Next:

Intraoperative Details

See Surgical Therapy.

Previous
Next:

Postoperative Details

Insertion of the tissue expander or single-stage implant expander is usually performed on an outpatient basis. The patient's follow-up care is initially for wound evaluation. Then, patients return at 2 weeks for possible suture removal and the initial expansion, if wound healing is suitable. Tissue expansion then is performed at weekly to biweekly intervals until the affected breast matches the contralateral side. The affected breast is expanded further as the unaffected breast grows.

Patients with moderate to severe abnormalities may require 2-stage reconstruction with the latissimus muscle over the permanent implant. The second procedure, involving expander exchange to permanent implant and latissimus muscle reconstruction, involves placement of drains in the donor site and breast pocket. An elastic wrap (eg, Ace) is used to minimize potential space at the donor site and risk of hematoma or seroma. Drain care can be performed at home. These drains are removed when outputs are less than 25 mL over 24 hours. Expansion exercises of the implants can usually be initiated at 1-4 weeks to minimize risk for capsule contracture.

A study by Manzano Surroca et al indicated that the use of free autologous fat grafts a year or more after breast implant surgery may improve reconstructive results in females with Poland syndrome. In the study, 6 females with the syndrome underwent free autologous fat grafting 11-18 months after receiving breast implants, with the contralateral breast also treated in order to improve symmetry and volume. The investigators concluded that fat grafting permits correction of the anterior axillary fold and improves the projection and symmetry of the breasts. [19]

Previous
Next:

Complications

Any of the complications described for breast reconstruction with implants and autogenous tissue can be experienced in the reconstruction of patients with Poland syndrome, including seroma [16] or hematoma around the implant, implant displacement, implant deflation, implant defect, implant infection, implant exposure, requirement for removal of implant, contracture, breast asymmetry immediate or late, progressive change in symmetry, drooping of the breasts, loss of feeling of the breast or nipple, need for displacement mammography, difficulty with breast cancer surveillance, galactorrhea, symmastia, difficulty with breast feeding, nipple asymmetry, rippling, contour abnormalities, inability to guarantee bra size, painful unattractive scarring, flap loss, muscle atrophy, latissimus donor site seroma and hematoma, back asymmetry from latissimustransposition, and need for additional revisions. [20, 21, 22]

Previous
Next:

Outcome and Prognosis

With appropriate timing, procedure selection, preoperative evaluation, and preoperative discussion to give appropriate expectations, excellent results and high patient satisfaction can be achieved in the treatment of chest and breast deformities resulting from Poland syndrome. Through the early use of tissue expansion, breast growth can be achieved to rival or match the normal development on the unaffected side. Tissue expansion prepares the breast envelope for the autogenous reconstruction and permanent implant and can sometimes improve nipple and areolar irregularities as well. Finally, upon completion of breast development, the latissimus muscle can be used to reconstruct the axillary line, reconstruct the infraclavicular hollow, and provide implant coverage.

Previous