Reduction mammaplasty in an adolescent with Ehlers-Danlos syndrome: a case report

Abstract

Background: Hypermobile Ehlers-Danlos syndrome (hEDS) is a connective tissue disorder that results in joint hypermobility and tissue fragility. Symptoms of macromastia may be exacerbated in patients with concomitant hEDS. Surgeons are often hesitant to operate on patients with hEDS given their increased propensity to develop surgical complications, including wound dehiscence, severe bruising, and hematomas, but there is a paucity of literature actually describing surgical complications in these patients. This is further limited in the pediatric and adolescent populations. To our knowledge, this is the first report of breast reduction in an adolescent patient with any form of Ehlers-Danlos syndrome (EDS). Case Description: This case report describes an adolescent patient with hEDS who underwent bilateral breast reduction for symptomatic macromastia with an uncomplicated recovery. An 18-year-old female presented to the plastic surgery clinic with chronic back, neck, and arm pain, poor body image and low self-esteem, inability to participate in sports and social activities due to emotional distress and discomfort, poor posture, difficulty fitting into clothes, and inframammary intertrigo all related to large breast size. She previously failed physical therapy and other forms of conservative management. On physical examination, she had large, ptotic, heavy dense breasts with significant striae. The areola size was 7 cm on the left and 7.5 cm on the right, the breast footprint base was 13 cm on the left and 14 cm on the right, the nipple to inframammary fold distance was 11.5 cm on the left and 11.5 cm on the right, and the suprasternal notch to nipple distance was 30 cm on the left and 30.5 cm on the right. She was diagnosed with macromastia and underwent bilateral mammoplasty with Wise-pattern and inferior pedicles. The patient recovered uneventfully after the procedure and followed up postoperatively with both the physical medicine and rehabilitation and plastic surgery departments. She subjectively reported a reduction in neck and back pain. Conclusions: We propose that hypermobile EDS may not be a contraindication to surgery in adolescents and acceptable outcomes can be achieved.