Imperforate Hymen

The hymen is a thin membrane of stratified squamous epithelium circumscribing the vaginal introitus. When the hymen does not spontaneously rupture during neonatal development, it is referred to as an imperforate hymen. An imperforate hymen can present with obstructive symptoms of the female genital and urinary tracts during the perinatal, pediatric, or adolescent years and is a rare cause of primary amenorrhea. Infants with an imperforate hymen may develop a mucocele from endogenous maternal estrogen stimulation.  Many patients remain asymptomatic until menarche, when they present with cyclic abdominal pain and amenorrhea. Hematocolpos or hematometra, the accumulation of menstrual blood in the vaginal or uterine cavities, can create a pelvic mass identified on physical exam as a blue, bulging perineal mass. This mass may cause mechanical obstruction of the urinary tract, leading to urinary retention, hydronephrosis, or acute renal injury. Corrective hymenectomy may be delayed until puberty unless the child suffers from urgent complications of the pelvic mass, prompting emergent surgery to preserve renal function, treat an infection, or minimize infertility. Surgical treatment focuses on prevention of the recurrence of imperforate hymen. Pediatricians, gynecologists, and pediatric urologists encounter patients with this diagnosis in their practice. A multidisciplinary approach to evaluation and treatment is optimal for the patient.