Diagnostics and treatment of Herlyn–Werner–Wunderlich syndrome in a newborn girl

Abstract

Herlin-Werner-Vanderlich syndrome refers to the Müllerian duct anomalies, including uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. At the first stage of treatment, a reasonable intervention is opening the hemivaginal septum by extra-vaginal access under visual control or using vaginoscopy. We identified a rare variant of the defect identified, since the longitudinal septum of the hemivagina did not prolapse through the introinus, which required a complex diagnosis and surgical aid in the newborn. A significant volume of hydrometrocolpos was associated with the accumulation of urine from the right hypoplasmic kidney with an abnormal flow of the ectopic ureter into the cavity of the atresized vagina. It determined the need for surgical treatment for urgent indications. In this regard, a biochemical analysis of the fluid obtained after drainage of the hydrometrocolpos with the detection of the concentration of urea, creatinine, total protein, and cellular composition is mandatory.