Poland syndrome remains a complex malformed entity with various clinical presentations because of levels of atrophy (muscle, bone, skin, and breast). Many procedures have been proposed to correct the malformations. For some, the latissimus dorsi muscle flap remains the gold standard8 , 18 , 19 and is sometimes used in women with a breast prosthesis. Moreover, tissue expansion,2 transverse rectus abdominis flap,20 an omentum flap16 , 21 and thoracic remodeling surgery,22 alone or in combination, can be used. The use of perforator flaps has also been described, such as the free deep inferior epigastric perforator flap23 or pedicled thoracodorsal artery perforator flap.24 Nonetheless, some of these procedures have high morbidity, and case series are limited.
Fat-transfer techniques are highly assistive in minor malformation, resulting in significant improvement in patients, but in most deformations, the limits of this treatment are quickly exceeded.25 Moreover lipofilling has been used widely to allow additional corrective procedures or to prepare the tissue before placing a prosthesis and thus improve atrophy of the chest wall. We have been reluctant to practice this procedure near the mammary gland, but it seems well established and the neoplastic risk appears very low.26–29
Several classifications of Poland syndrome have been proposed. Some are descriptive, whereas others propose decision algorithms for selecting the best surgical procedure.2 , 6 , 8 , 18 However, no classification system appears to have been widely accepted. Because of the wide clinical variety of manifestations of Poland syndrome, it is very difficult to apply standardized management techniques. Thus, with a view toward simplification, we developed a standardized three-dimensional computer-aided design procedure for custom silicone prostheses.
Even in the presence of a rib or thoracic malformation, or an associated pectus excavatum, there is no evidence of cardiac or respiratory functional consequences of the thoracic malformations that occur in patients with Poland syndrome. Indeed, recent meta-analyses by Malek et al. and Guntheroth and Spiers on the pulmonary and cardiac consequences of the condition revealed no evidence of improvement after thoracic surgery to correct malformations.30 , 31 Thus, we developed a reconstruction procedure that prioritized cosmetic concerns.
It is necessary to be aware that an aesthetic result and perfect symmetry are extremely difficult to obtain in patients with Poland syndrome; this must be relayed to the patient from the outset. The technical and surgical requirements for reconstruction differ between men and women, and it seems that reconstruction with computer-aided design implants makes it possible to obtain satisfactory results in both sexes. Nevertheless, women often require implantation of a breast prosthesis; 41 percent of the cases in our series showed frequent mammary hypoplasia.
Our initial implant preparation technique using an external plaster mold yielded good results; however, barely detectable imperfections in terms of contour or volume were occasionally evident. This was attributable to the interposition of soft tissues, such as breasts in female patients or well-developed muscles in male patients, in an effort to camouflage the deformation, with occasionally asymmetric results. These tissues varied in thickness and location, which compromised the plaster molding. In addition, the implant was more likely to be visible or detectable by touch when the skin was thin but three-dimensional computer-aided design allowed for rendering of a more optimized prosthetic design. With three-dimensional computer-aided design, the contours of the implant were softened, and the volume was always underestimated by 15 to 20 percent to avoid the maximum contour when the skin was thin.
We experienced only two hematomas and two infections, secondary to lipofilling, as complications. The hematomas required reoperation, and the infections necessitated removal of the prosthesis and cessation of antibiotic treatment. Seromas formed in 20.6 percent of cases during the early postoperative period and were the most frequently observed minor complication. These seromas were treated with one or two aspirations during the first postoperative month but always resorbed. No patient developed a residual seroma after 3 months. Patients should be told to expect a seroma, and aspiration should be performed by a surgeon to avoid any risk of infection. No periprosthetic capsular contracture has been observed around the implants in the long term. Unlike breast implants filled with soft silicone gel, silicone elastomer implants are semirigid; thus, we assumed that they would neither retract nor contract. In the absence of a fracture, rupture, or degradation, the prosthesis is retained for the duration of the patient’s life.
Since 2007, we have completely abandoned other procedures, such as the latissimus dorsi approach, which over time produces atrophy (sometimes leaving behind dorsal sequelae) and apparently random results, but also isolated mammary prostheses that did not respond to the actual deformation. Finally, we have never obtained sufficient and symmetric results using the lipofilling procedure alone, as reported by La Marca et al.32; this often requires three to five procedures. The other negative aspect of lipofilling for men is that they are often young, thin, and muscular patients, and thus it is difficult to find an adipose tissue donor site.
The recent study of Baldelli et al. highlights that patients should be operated on during the period of growth to allow for proper body image stabilization and improved quality of life.7 With our technique, it is possible to operate on patients relatively early, (i.e., just after puberty). If the prosthesis becomes too small, it is always possible to redo it or to perform secondary lipofilling. The mean patient age in our series was 26 years, reflecting that a large proportion of patients do not undergo correction during childhood. Psychological difficulties often develop in adolescence or adulthood, motivating consultations. Such patients are frequently seen for the first time as adults, and often request surgery that is rapid and not disabling or associated with prolonged effects on work or sports activities. Although psychological and cosmetic considerations remain the main indications for treating Poland syndrome, few studies have explored patient satisfaction and aesthetic results, and none include any work done involving computer-aided design to create silicone implants. In the present study, both men and women considered that the outcomes of the computer-aided design implants were good to excellent. In terms of overall satisfaction, 76.9 percent of women and more than 80 percent of men rated they were “were satisfied” or “very satisfied,” demonstrating that this technique gives good results in both sexes.
No specific scale to evaluate the effect of Poland syndrome on quality of life exists; thus, we used the Medical Outcomes Study 36-Item Short-Form Health Survey, which is one of the most widely used and internationally well-validated scales.15 Significant improvements in social and emotional functioning were evident in both groups, and men reported significantly greater improvements in social functioning compared with women (p < 0.001 and p = 0.096). It is likely that social functioning would also show significant improvement in women with a larger patient series. This result demonstrates that reconstruction improved patient body image.7
The reported pain associated with implant placement was highlighted on the Medical Outcomes Study 36-Item Short-Form Health Survey (bodily pain). We have not encountered this complaint before, and the literature is silent on the topic. Patients reported that they could feel their prostheses during certain intense sporting activities, which may also be the case for breast implants. We believe that the medium- and long-term pain associated with others procedures, such as thoracic remodeling surgery, latissimus dorsi flap, transverse rectus abdominis muscle flap, or a mammary implant are rarely evaluated in most studies; thus, it was difficult to compare previous cases with those of our series. Pain reports are often unreliable in retrospective studies. The experience of the prosthesis during physical effort was described more as discomfort than as pain, and only one patient requested removal of the prosthesis for pain-related reasons.
All of our demographic and surgical data were collected prospectively, but the cosmetic self-evaluation and quality-of-life assessment were retrospective in nature, and thus accompanied by the limitations inherent in retrospective reports. To the best of our knowledge, this is the largest reported series of Poland syndrome corrections using computer-aided design custom silicone implants. We also present long-term follow-up data showing the stability of the implants and outcomes. Because the implants are custom-made and all are different, they are not required in Europe to carry the European Conformity marking as is the case for classic silicone gel mammary implants.
We managed patients with Poland syndrome on a strictly cosmetic basis. Custom implants designed using three-dimensional computed tomographic data afforded good to excellent cosmetic results in most patients, who were generally satisfied and enjoyed an improved quality of life, in both social and emotional terms. This procedure allows for the management of the majority of defects. Obtaining an excellent result remains difficult in patients with Poland syndrome, but three-dimensional computer-aided design has optimized our reconstructions. Nevertheless, certain associated procedures and secondary corrections remain indispensable for achieving an optimal result.
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