Plastic Surgery and Poland Syndrome
“The female form is one of God’s greatest expressions of artistry in the universe. The effects of breast reconstruction, and the restoration of form lost, has the capacity to transcend the techniques themselves, providing a reclaiming of beauty and physical harmony, a return to wholeness that is greater than the sum of the individual efforts. I continue to be humbled by the impact this work has, both on the women I am privileged to take care of and those that are near and dear to them.”
Dr. Frank DellaCroce
Co-Founder, Center For Restorative Breast Surgery
and St. Charles Surgical Hospital
Living the Life of Inspiration,
Living with Poland Syndrome.
Living with a rare congenital condition that causes disfigurement can be both isolating and depressing. P.S. You are Loved is a collection of inspirational stories from real people all over the world that are living and thriving with a rare birth defect called Poland Syndrome.
Poland Syndrome (also known as Poland’s Syndrome, Poland sequence, or Poland anomaly) is a collection of developmental defects that can affect structures of the chest, breast, arm, and hand. It is present at birth and falls under the category of “congenital” birth defects, although it often goes undiagnosed until later in life since the severity of the effects varies from person to person. It is this range of defects and the fact that the manifestations may be more profound in the hand than the chest or more profound in the breast than the upper extremity, that makes Poland Syndrome a somewhat ethereal condition when it comes to diagnosis. When syndactyly (webbed fingers) is present, it is fairly obvious and noticed early on but when the major impact of the syndrome is confined to the breast, young women may not be accurately diagnosed until later in life as they enter puberty, if then. This is of particular significance because the effect on the breast and chest wall for these women can be considered the congenital equivalent of a radical mastectomy. “Radical” mastectomy is a long ago abandoned standard of surgical treatment for breast cancer. The procedure involved the complete removal of the breast tissue, its associated skin, as well as, the underlying pectoral muscle. Modern mastectomy is more conservative since surgeons have learned, over time, that removal of the pectoralis muscle does nothing to improve cure rates and radical excavation of structures in the chest wall significantly impacts function, healing, and reconstructive outcome potential.
Since Poland syndrome is a congenital defect that is represented by a RANGE of impacts, it is important to realize that the manifestations may be a breast that is simply smaller than the other, to complete absence of the breast, its nipple, and the underlying pectoral muscle (producing the aforementioned “congenital radical mastectomy”). Deformities of the ribs and skeletal chest wall are often also present and can present some of the most challenging reconstructive components for the attending plastic surgeon (Figure 1).
Figure 1: Poland Syndrome left breast with complete absence pectoralis muscle, and underdeveloped left upper extremity
Consultation with a Board Certified Plastic Surgeon is recommended for those seeking improvement in the balance of their breast size and shape. It may be worth considering a visit with a plastic surgeon, who is well experienced in the care of women with Poland Syndrome, even well before surgical correction is contemplated. This may also be time well spent for young women, who are entering puberty and beginning to see early signs of a problem on their affected side. The reassurance of a sensitive, caring surgeon for a young girl affected by Poland Syndrome can be a source of comfort and reassurance that, should she ever become ready to address it, there are procedures available to help her. This can serve to validate, encourage, and empower her during that delicate developmental stage of her physical and emotional self. While many young women will be satisfied in their early-mid teenage years to have this information alone, some may choose early intervention. It is important for the family to be on the same page with respect to any early treatment in these situations and for the patient to be sufficiently mature mentally and emotionally to make an informed decision with the help of her care team. The goal of an early approach is to reduce impediments to social integration to reduce the fear of a swimsuit or locker room changes for gym class. Beyond that, the progressive pursuit of symmetry may require intermittent surgical adjustments as development runs its course. For those who prefer to wait for surgical correction/reconstruction until they are older and their development is complete, the benefit may be fewer surgical adjustments. The preferred timing of surgical reconstruction is absolutely an individual decision and should be based on that preference. There is no “always” in terms of the best way things should proceed in that regard.
With respect to the available reconstructive methods that modern, sophisticated, plastic surgery has at its disposal, the news is good……with one critical qualifier. White coats don’t guarantee proficiency and diplomas don’t guarantee expertise. We often seem to spend more time making sure the Yelp reviews and Zagat write-ups, of a restaurant we’re considering, meet our desired level of quality than we do researching the doctors who give us medicines and apply scalpels to our bodies. It is essential that you do your own homework regarding the doctor you choose to correct your impact from Poland Syndrome. Now, with that out of the way, let’s review some of the basic surgical approaches for the restoration of breast volume and shape.
Implant reconstruction often forms the initial approach towards the correction of Poland Syndrome. In the younger patient, a small implant, followed by steps towards a larger one over time, as the opposite breast develops, may make sense. For others, if the opposite breast is much larger than the affected breast, tissue expansion to stretch the skin before the definitive implant is placed may be necessary. Implant reconstruction in Poland Syndrome is challenged by the lack of tissue, skin, and chest muscle that would ordinarily provide coverage for the implant. Since the tissue coverage over the implant is generally thin in the Poland patient, silicone implants may provide better texture to the touch compared to saline filled implants. Newer, shaped implants may provide added benefit depending on the exact individual shaping needs present. The most common complication following implant reconstruction for Poland Syndrome is capsular contracture. Although the overall incidence is relatively low, those with Poland Syndrome will tend to show the effects of a tightening capsule around an implant more than those with thicker tissue coverage.
When implants prove unsuccessful or are an undesirable individual choice, sophisticated microsurgical tissue transplantation using excess fat from the tummy or hip region may be used to recreate the volume and shape of the breast with living fat. The “Stacked DIEP” (deep inferior epigastric perforator) and “APEXcm” (abdominal perforator engineered vascular exchange) procedures, pioneered at the Center For Restorative Breast Surgery, allow rebuilding of the breast with one’s own natural tissue (Figure 2).
Figure 2: Before and after DIEP flap reconstruction Poland deformity right breast and chest wall
The APEX modification, allows the blood supply of a conventional tissue flap to be increased beyond the natural anatomy, without sacrificing muscle in the abdomen, to maximize long-term softness in the new breast. This is an important consideration since a rebuilt breast, at the expense of functional disability, is a historic tradeoff that is no longer necessary. The pioneering advances that allow stacking of multiple fatty layers may be of particular benefit for those affected by Polands who have thinner tummies but would like to avoid an implant or replace an unsatisfactory one (Figure 3 and 4). Tissue reconstructions tend to have a more natural look and texture, but require specialized techniques and a surgical team that is dedicated to the art and science of breast reconstruction.
Figure 3: Two layered DIEP flaps stacked and inset into position to restore breast volume and shape
Figure 4: Poland syndrome right breast before and after perforator flap replacement of previously placed implant that was encased in capsular scar
In summary, Poland syndrome effects in the breast may be addressed with several different options. An individual consultation with an expert in these procedures may allow an individualized recommendation tailored to your individual need. No two patients with this syndrome are the same and a surgeon well experienced with all the various options should be positioned to provide a recommendation set that is logical, easy to understand, with a full incorporation of the personal needs and goals of the woman he/she is entrusted to care for.
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