Bilateral congenital amazia: a case report and systematic review of the literature.

BACKGROUND: Congenital breast anomalies present challenging management decisions to the plastic surgeon. One must consider the optimal age of reconstruction as well as the ideal surgical technique. Amazia, a very rare condition characterised by a complete lack of breast tissue in the presence of a nipple areolar complex (NAC), is one such congenital breast anomaly. METHODS: A comprehensive systematic review of the literature was performed to examine the various approaches to reconstruction of congenital breast anomalies. From this review, the data compiled included patient demographics and operative details, including type of reconstruction, treatment of the contralateral breast and treatment of the NAC. A case of bilateral amazia is also reported. RESULTS: Of 178 articles, 13 ultimately met the inclusion criteria and 54 individual patient reconstructions were identified from these papers. At the time of reconstruction, the patients were in the range of 13-54 years, with an average age of 27.6 years. Prosthetic and autologous reconstructions were equally represented (19 patients each, 35.2%; Table 2). Autologous reconstruction with prosthesis was slightly less common (15 patients, 27.8%). One patient was reconstructed using autologous lipo-augmentation only. Of the 36 cases in which the approach to the NAC was addressed, most (66.7%) were not reconstructed. CONCLUSIONS: Amazia is a very rare congenital anomaly of the breast. This systematic review of the literature highlights the need for better reporting and examination of this type of data to allow for future study and to better advise on decision making regarding the timing of reconstruction, surgical technique and the approach to the NAC.