Squamous cell peritonitis associated with hydrometrocolpos in a multimalformed newborn

We present the case of a 1 day-old newborn with extensive squamous cell peritonitis resulting from vaginal atresia with hydrometrocolpos and squamous cell reflux through the genital system, a combination rarely recognized in the literature. Delivery was preceded by ultrasound diagnosis of oligohydramnios and "large bladder," the latter representing the dilated proximal vagina. Additional findings included paraesophageal hiatus hernia containing squames, urethral atresia, absence of vulva, lung hypoplasia, mild hypoplasia of the corpus callosum, and short umbilical cord with single umbilical artery (SUA). The possible relationship of the SUA with the constellation of infradiaphragmatic malformations is discussed.