The patient was referred to an emergency surgical facility for immediate exploratory due to suspicion of persistent Müllerian duct syndrome vs. other. During surgery a fluid filled possible pseudo uterus was found; that structure was attached to either testes through a hernia in the body wall. A rare congenital condition known as persistent Müllerian duct syndrome was believed to be the finding. Patient is a pseudohemaphrodite. Both testicles were removed as was the fluid filled structure. Surgery notes: two inguinal hernias, communication from each of the testicles to a fluid filled structure in the abdomen, 800 mls of purulent filled was extracted from the fluid filled structure and neuter was performed. The patient is expected to recovered well after surgery and was treated with Unasyn, buprenorphine, and continued I.V. fluids post-op. The fluid filled structure and both testicles were submitted for biopsy and cultures to the lab.
Pathology results confirmed the presence of a tubular organ- oviduct and uterus.Pathology comments: the finding of a tubular organ that histologically resembles different areas of oviduct and uterus is compatible with the clinical diagnosis of a persistent Müllerian duct syndrome.Testicles: testis azoospermia with plexus malformation and moderate lymphoplasmacytic duct efferent ductitis.