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This report presents a case of a one-month three-day-old full-term female infant with hydrometrocolpos (HMC) and post-axial polydactyly whose first clinical sign was acute intestinal obstruction and hydroureteronephrosis, caused by compression of the structures due to the increasing size of the cystic-like pelvic mass. This is the first report of HMC with post-axial polydactyly complicated with acute intestinal obstruction in Peru. It raises importance on prenatal diagnosis, management and complications of HMC. Although it is rare, clinicians should have it as an option when discussing abdominal cystic masses in neonates to perform early management and avoid complications. Continuous follow-up should be carried out on patients presenting with HMC and post-axial polydactyly to assess for Bardet-Biedl syndrome, which could affect different systems in those patients long-term.

Hydrometrocolpos and Post-axial Polydactyly Complicated With Acute Intestinal Obstruction and Hydroureteronephrosis