Unmasking Ehlers-Danlos Syndrome Through Reduction Mammaplasty: An Illustrative Case and a Systematic Literature Review.

Ehlers-Danlos syndrome (EDS) is characterized by joint hypermobility, skin hyperextensibility, and tissue fragility, which may worsen symptomatic macromastia. Concerns about elevated surgical risk have historically discouraged elective procedures in EDS patients; however, postoperative complications may themselves prompt an EDS diagnosis, as illustrated by our case. This relationship prompted the conduct of a systematic review examining outcomes of reduction mammaplasty in EDS. A 37-year-old woman underwent bilateral inferior pedicle reduction mammaplasty for symptomatic mammary hyperplasia. Persistent intraoperative oozing resulted in postoperative anemia (hemoglobin decline from 13.3 g/dL to 10.4 g/dL). Additional complications included delayed wound healing, hypertrophic scarring, and transient left upper extremity weakness. Hematologic evaluation was unremarkable. Clinical assessment revealed joint hypermobility (Beighton score ≥5/9), positive Walker and Steinberg signs, skin hyperextensibility, easy bruising, and menorrhagia, leading to a clinical diagnosis of hypermobile EDS. A systematic review of PubMed, Scopus, and Google Scholar (December 1950 to June 2025) was performed and included all study designs. Extracted data included demographics, interventions, complications, revision rates, and satisfaction. Two independent reviewers assessed quality using Joanna Briggs Institute (JBI) critical appraisal tools. Of the 115 identified records, the following four studies met the inclusion criteria: one case report, one case series, one retrospective cohort study, and one clinical reference. All reported cases involved patients with pre-existing EDS diagnoses and demonstrated successful outcomes with symptomatic improvement and few complications. The cohort study consisted of multiple elective procedures, including reduction mammaplasty, and reported fewer complications in EDS patients compared to matched controls, but did not report outcomes specific to the reduction mammaplasty group. The clinical reference emphasized meticulous operative planning. This systematic review with an illustrative case underscores the need for further research and updated clinical guidance. Although existing literature largely challenges the view of EDS as a surgical contraindication, our case demonstrates complications consistent with known EDS-related risks and suggests a potential role for plastic surgeons in identifying undiagnosed EDS.