Management of 2-Stage Breast Reconstruction in Ehlers-Danlos Syndrome: A Case Report.

Background: Ehlers-Danlos syndrome (EDS) refers to a group of heritable connective tissue disorders (HCTDs). Clinical hallmarks of EDS include tissue fragility, joint hypermobility, and skin hyperextensibility. One of the consequences of tissue fragility is abnormal wound healing and scar formation, posing potential challenges for surgeons treating these patients. There are limited previous reports of EDS patients undergoing mastectomy and/or breast reconstruction, and none wherein the patient had diagnoses of both vascular EDS (vEDS) and classical EDS (cEDS). Case: A 41-year-old female was referred to the plastic surgery clinic for breast reconstruction consultation after diagnosis of left breast lobular carcinoma in situ (LCIS). She has a past medical history of cEDS and vEDS with associated pectus carinatum, thoracic root dilation, and hypermobile joints. After shared decision making with the patient and her breast surgeon, it was decided the patient would benefit from bilateral prophylactic mastectomies with immediate 2-stage tissue expander (TE) reconstruction. Results: The patient reported here had an unremarkable postoperative course. Her complications were limited to more than average bleeding during the first stage of reconstruction, which was easily managed with meticulous intraoperative hemostasis, and a small uncomplicated submuscular seroma 1week postoperative. She had no complications following TE to implant exchange and continues to heal well. Conclusions: This case report documents a case in which a patient with both cEDS and vEDS had an unremarkable surgical and postoperative course following bilateral prophylactic mastectomies with 2-stage TE reconstruction.