Multiple intradural extramedullary Müllerian duct cysts: illustrative case.

Background: Müllerian duct cysts (MDCs) are rare congenital anomalies. They arise from Müllerian duct remnant (MDR) and usually present as prostatic utricle cyst or pelvic mass. The authors report their experience with an extremely rare case of multiple intradural extramedullary MDCs.

Observations: The patient, a 31-year-old female, presented with a 6-month history of progressive numbness, weakness, and sensory deficit in her lower extremities. After neurological examination at the authors' hospital, an MRI study was performed, revealing multiple intradural cystic lesions from C6 to L3, extending over 17 vertebral segments. Preoperative differential diagnoses included hydatid cysts, arachnoid cysts, enterogenous cysts, tuberculosis, and infection. Seven extramedullary noncommunicating transparent cysts were totally removed by surgery, with significant improvement of the neurological symptoms. Pathological examination showed MDCs.

Lessons: This is the first reported case of compressive myelopathy caused by multiple intraspinal subdural extramedullary MDCs. Regular imaging examinations and laboratory tests do not provide valuable information for differential diagnosis. Careful preoperative cyst localization and complete microsurgical resection are essential for a good outcome. https://thejns.org/doi/10.3171/CASE2511.