Multiple intradural extramedullary Müllerian duct cysts: illustrative case

Area of Science:

• Neurology
• Pathology
• Surgical Oncology

Background:

• Müllerian duct cysts (MDCs) are rare congenital anomalies originating from Müllerian duct remnants (MDRs).
• Typically presenting as prostatic utricle cysts or pelvic masses, MDCs are seldom found within the spinal canal.
• This report details an exceptionally rare instance of multiple intradural extramedullary MDCs.

Purpose of the Study:

• To document an extremely rare case of multiple intradural extramedullary Müllerian duct cysts.
• To describe the clinical presentation, diagnostic challenges, and surgical management of this condition.
• To emphasize the importance of complete surgical resection for favorable outcomes.

Main Methods:

• A 31-year-old female patient with progressive lower extremity neurological deficits underwent MRI.
• MRI revealed extensive intradural cystic lesions spanning multiple vertebral segments.
• Surgical excision of seven extramedullary cysts was performed, followed by pathological confirmation as MDCs.

Main Results:

• The patient presented with numbness, weakness, and sensory deficits in the lower extremities over six months.
• Preoperative differential diagnoses were broad, including infectious and neoplastic etiologies.
• Complete microsurgical resection of the MDCs resulted in significant neurological symptom improvement.

Conclusions:

• This is the first reported case of compressive myelopathy due to multiple intraspinal subdural extramedullary MDCs.
• Standard imaging and laboratory tests offer limited diagnostic value for differentiating these cysts.
• Accurate preoperative localization and complete microsurgical resection are critical for successful treatment and patient recovery.