Surgical Repair of Chest Wall and Pectoral Deformities in Poland Syndrome in Children
Downloads
Poland syndrome is a rare congenital condition characterized by absence or underdevelopment of the sternal head of the pectoralis major muscle on one side, often with ipsilateral breast and chest wall hypoplasia (though concomitant hand anomalies are beyond our scope). Severe deformities may also include absent ribs or asymmetric chest. 3D imaging (such as MRI) vividly demonstrates the unilateral chest depression and muscle absence. For example, the MRI above (right side views) shows a profound right anterior chest wall depression with no right pectoralis muscle visible; the left side (normal) is indicated by the dashed line. Such imaging is valuable in preoperative planning. Surgical correction is generally reserved for the more severe thoracic defects. In Romanini’s classification, only grades T2–T4 (the moderate-to-severe forms) typically warrant operative repair [1,2]. In practice, pediatric patients with significant chest asymmetry are referred for reconstructive surgery to restore chest contour and stability.
1. Torre, M., Palo, F., & Infant, M. (2019). Open-surgery repair of congenital malformation of the chest: indications, technical tips and outcomes. Pediatr Med (Pittsburg), 2(9), 1–13.
2. Lantzsch, T., Lampe, D., & Kantelhardt, E. J. (2013). Correction of Poland’s syndrome: case report and review of the current literature. Breast Care (Basel), 8(4), 259–262
3. Ioannidis, C. (2020). Poland syndrome: Chest wall and breast repair. HJOG: An Obstetrics & Gynecology International Journal, 19(2), 83–94.
4. Foucras, L., Guerin, N., Darsonval, R., & Roussillou, J. M. (2005). Poland syndrome: management of chest wall and breast deformities. Ann. Chir. Plast. Esthet., 50(6), 521–528.
5. Al-Qattan, M. M., & Hajjar, W. M. (2020). Reconstruction of an anterior chest wall defect in a child using a latissimus dorsi muscle–thoraco-lumbar fascia composite flap: a case report. Int. J. Surg. Case Rep., 72, 290–293.
6. Mahrhofer, M., Schoeller, T., Casari, M., Bachleitner, K., & Weitgasser, L. (2021). Development of a surgical treatment algorithm for breast reconstruction in Poland syndrome patients considering severity, sex, and BMI. Journal of Clinical Medicine, 10(19), 4515.
7. Arango Tomás, E., Baamonde Laborda, C., Algar-Algar, J., & Salvatierra-Velázquez, A. (2013). Chest wall reconstruction with methacrylate prosthesis in Poland syndrome. Arch. Bronconeumol., 49(10), 450–452.
8. Anderson, C. J., Moss, R. T., Nikolajczyk, K. V., Gilula, L. A., Graham, M. M., & Rothenberg, S. S. (2016). A technique for pediatric chest wall reconstruction using custom-designed titanium implants: description of technique and report of two cases. J. Child Orthop., 10(3), 1–5.
9. Goldsmith, I., Llewellyn-Evans, P., Goodrum, H., Warbrick-Smith, J., & Bragg, T. (2020). Chest wall reconstruction with an anatomically designed 3D-printed titanium ribs and hemi-sternum implant. 3D Print Med., 6(1), 26.
10. Rosen, S. D., Drebov, R. S., & Katsarov, A. (2016). Poland syndrome: use of the Vertical Expandable Prosthetic Titanium Rib (VEPTR) system before walking age – a case report. Surg. J. (N.Y.), 2(4), e91–e94.
