Prevalence:

• 1 in 250,000 births.

Ultrasound diagnosis:

• Presacral cystic mass in the pelvis of a female fetus, due to a common channel for urinary and genital tracts. The mass represents hydrometrocolpos, distended vagina and a compressed, anteriorly located bladder with varying degrees of urinary obstruction. The contents may be clear (urine) or turbid (hemorrhage). Ascites may result from escape of urine through the Fallopian tubes into the abdomen.
• Normal amount of the amniotic fluid.

Associated abnormalities:

• The incidence of chromosomal abnormalities or genetic syndromes is not increased.
• Other defects, mainly of the genitourinary tract (bicornuate uterus, cervical atresia, vaginal atresia or duplication, hydronephrosis, renal agenesis, multicystic kidneys) gastrointestinal tract (imperforate anus, esophageal atresia) and cardiovascular system, are often found.

Investigations:

• Detailed ultrasound examination.

Follow up:

• Ultrasound scans every 4 weeks to monitor the evolution of the condition.

Delivery:

• Place: hospital with neonatal intensive care and facilities for pediatric surgery.
• Time: 38 weeks.
• Method: induction of labor aiming for vaginal delivery.

Prognosis:

• Neonatal evaluation of the exact anatomical picture and investigation for other anomalies of the genital and urinary tracts. Reconstructive surgery is necessary. Most patients are continent and fertile, but about 50% require intermittent catheterization.

Recurrence:

• Isolated: no increased risk of recurrence.