TUBEROUS BREAST AND POLAND SYNDROME: AN UNDERESTIMATED ASSOCIATION?

Abstract

Aim of the study: Both Poland Syndrome(PS) and Tuberous breast(TB) are anomalies affecting the breast, but they are considered mutually exclusive. Our aim was to determine the possible coexistence of PS and TB and to discuss TB management when associated with PS.

Methods: 51 female adolescent PS patients were studied in our center between 2010 and 2023. Among these, we evaluated those who developed TB after puberty. PS diagnosis was made based on the hypoplasia or absence of pectoralis major muscle detected clinically and confirmed radiologically. Breast anomaly on PS side was classified with Thorax Breast and Nipple(TBN) classification: B1 correspond to breast hypoplasia, B2 to breast aplasia. TB diagnosis was made with a clinical evaluation after puberty. TB were classified according to Groulleau classification.

Main results: Among 51 post-pubertal PS females, we identified 23 (45%) who developed TB. In 3 cases (13%) TB was bilateral, in 20(87%) contralateral to PS affected side. Age at time of the first surgical procedure was 16 years. Patients were treated with breast implants, fat grafting, rigottomy, local flaps or a combination. TB treatment was performed simultaneously with PS breast reconstruction when feasible, or after it.

Conclusions: TB incidence in our series is almost twice than in general population. This is the first study demonstrating and quantifying this association. TB deformity must be considered while defining PS surgical path adolescents. Fat grafting is the first surgical option to treat both anomalies. A multidisciplinary approach is needed to minimize number of surgeries and maximize cosmetic results.