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Congenital gynecologic anomalies result from interruption of embryologic development of the female reproductive tract. The anomalies may be hymenal, vaginal, cervical, or uterine. The impact of these anomalies is variable: some are asymptomatic, incidental findings that require no intervention, others require simple surgical management, while some complex anomalies may require a multidisciplinary approach with extensive surgical expertise for optimal outcomes. Uterovaginal anomalies may occur in isolation or in association with other malformations, such as renal anomalies. The origin, presentation, evaluation and treatment of these conditions are reviewed here.

Anhydramnios in the setting of severe malformations of the fetal kidney and urinary tract is associated with a high incidence of stillbirths and life-threatening complications, including severe pulmonary hypoplasia, umbilical cord compression, and perinatal asphyxia. To prevent such adverse outcomes, some centers in the United States and elsewhere are offering amniotic fluid restoration for women diagnosed with anhydramnios in the setting of fetal renal malformations. The procedures include infusions of amniotic fluid substitutes (normal saline), percutaneously or through an amnioport-an implanted system for serial or continuous infusion of normal saline to maintain the desired amniotic fluid volume. The procedures are intended to prevent pulmonary hypoplasia and allow the pregnancy to progress closer to term gestation, enabling postnatal renal management, including long-term dialysis and renal transplantation. However, these procedures have not been recommended as standard of care by the professional societies because there are many knowledge gaps, including few data on short-term and long-term renal outcomes. The available diagnostic methods do not provide reliable prognostic information, and the current maternal and fetal interventions have not been standardized. To address these unresolved issues and to propose a research agenda, the Eunice Kennedy Shriver National Institute of Child Health and Human Development and the National Institute of Diabetes and Digestive and Kidney Diseases invited a panel of experts to a workshop in August 2016. This report provides a summary of that meeting.