Delay E, Nachaoui H, Frobert P
Unité de chirurgie plastique et reconstructrice, Centre Léon Bérard, 28, rue Laënnec, 69373 Lyon cedex 08, France; Cabinet, 50, rue de la République, 69002 Lyon, France.
Unité de chirurgie plastique et reconstructrice, Centre Léon Bérard, 28, rue Laënnec, 69373 Lyon cedex 08, France.
Ann Chir Plast Esthet. 2022 Nov;67(5-6):358-373. doi: 10.1016/j.anplas.2022.07.020. Epub 2022 Aug 19.
Breast and thoracic deformities of Poland syndrome is a rare malformation known to be difficult to treat. Numerous descriptions of surgical corrections have been published but none achieved to correct severe cases before description of lipomodeling technique. The aim of this article is to present thoraco-mammary deformity of Poland syndrome, corrections techniques already available and therapeutical indications in primary and secondary cases. Constant anomaly of Poland syndrome is agenesis of sterno-costal part of pectoralis major muscle but other muscular anomalies can be associated. Skin and glandular anomalies present with a fine skin and an absent or hypoplasic subcutaneous fat with a glandular hypoplasia of various degree. Osteo-cartilaginous anomalies can be associated in very severe cases. Clinical sign of Poland syndrome is forced adduction manoeuvre highlighting pectoralis major agenesis. Functional impact of the deformity is low but psychological and psychosocial implications can be very important, supporting an early surgical correction. Therapeutic means are various and accurate descriptions are given in this article: thoracic bony reconstruction, thoracic implant made of silicone elastomer, breast implant, skin expansion, latissimus dorsi pedicled flap, free flaps, breast lipomodeling, Breast-pectoralis flap. Principles of each technique are described and balanced with their actual use in this malformation. Indications have been completely modified these last years due to lipomodeling contribution which represented a huge step in this deformity treatment. In our practice, if autologous reconstruction with lipomodeling is possible, we choose this solution at first. In case of severe thoracic deformity, a silicone elastomer implant made with the help of computed assisted conception can be an important adjunct, mainly by thin young man. In secondary cases, if implant is well tolerated, we found logical to stay in the same reconstruction path and do one or two sessions of lipomodeling in order to improve reconstruction. If implant tolerance is low and skin very thin at risk of exposure, we do recommend a conversion of implant reconstruction to autologous reconstruction. In conclusion, thoraco-mammary deformities of Poland syndrome are rare and hard to treat and should be managed by well trained and experimented surgeons. Breast lipomodeling is a huge step in the treatment of these deformities and should be regarded, in our opinion, as first line treatment if fat deposits are sufficient. In case of low fat provisions or in the thin young man, composite techniques should be used with silicone elastomer implant.
波兰综合征的乳房和胸廓畸形是一种罕见的畸形，已知治疗困难。已经发表了许多关于手术矫正的描述，但在脂肪建模技术出现之前，没有一种方法能够矫正严重病例。本文的目的是介绍波兰综合征的胸-乳腺畸形、现有的矫正技术以及在原发性和继发性病例中的治疗指征。波兰综合征的恒定异常是胸大肌胸骨-肋骨部分发育不全，但也可能伴有其他肌肉异常。皮肤和腺体异常表现为皮肤细腻、皮下脂肪缺失或发育不全，伴有不同程度的腺体发育不全。在非常严重的病例中，可能伴有骨-软骨异常。波兰综合征的临床体征是强迫内收动作，突出显示胸大肌发育不全。畸形的功能影响较小，但心理和社会心理影响可能非常重要，这支持早期手术矫正。治疗方法多种多样，本文给出了准确描述：胸廓骨重建、硅橡胶弹性体制成的胸廓植入物、乳房植入物、皮肤扩张、背阔肌带蒂皮瓣、游离皮瓣、乳房脂肪建模、乳房-胸大肌皮瓣。描述了每种技术的原理，并与它们在这种畸形中的实际应用进行了权衡。由于脂肪建模的贡献，近年来适应证已经完全改变，脂肪建模在这种畸形治疗中代表了巨大的进步。在我们的实践中，如果可以进行脂肪建模的自体重建，我们首先选择这种解决方案。对于严重的胸廓畸形，借助计算机辅助构想制作的硅橡胶弹性体植入物可能是一种重要的辅助手段，主要适用于瘦年轻人。在继发性病例中，如果植入物耐受性良好，我们认为继续采用相同的重建方法并进行一到两次脂肪建模以改善重建是合理的。如果植入物耐受性差且皮肤非常薄有暴露风险，我们确实建议将植入物重建转换为自体重建。总之，波兰综合征的胸-乳腺畸形罕见且难以治疗，应由训练有素且经验丰富的外科医生进行处理。乳房脂肪建模是治疗这些畸形的巨大进步，在我们看来，如果脂肪储备充足，应将其视为一线治疗方法。在脂肪供应不足或瘦年轻人的情况下，应使用复合技术结合硅橡胶弹性体植入物。
