Functional müllerian tissue within the conus medullaris generating cyclical neurological morbidity in an otherwise healthy female

Purpose: Endometriosis is a common disease; however, ectopic müllerian tissue within the spine is a rare entity with the potential for producing significant neurological compromise. There are several postulated etiologies for this phenomenon, and only a few case reports are available in the world literature. Knowledge of this rare phenomenon is of paramount importance, since early diagnosis can lead to lessened neurological morbidity. Methods: In this manuscript, we present a case report, discuss gynecological and neurosurgical perspectives relating to the treatment strategies for managing this entity, and propose an alternative explanation for such an occurrence from a neurogenetic standpoint. Results: We present a case of spinal müllerianosis within the conus medullaris which was managed symptomatically for several years with an intracystic drain and subcutaneous reservoir. Over the years, it became clear that there was a cyclical presentation to her clinical malady, which at times was severe. Ultimately, she required surgical resection which aided in her diagnosis and subsequent treatment. Conclusion: Intraspinal müllerianosis is a rare location for an otherwise common disease in women and has the potential to create significant neurological morbidity by creating a mass lesion. Although the exact etiology remains unclear, the histogenic theories of embryologic origin appear most plausible. Treatment strategies for this condition may include hormonal therapy, obstetrical surgery, or open spinal surgery. This unusual and poorly understood disease should be considered in the differential diagnosis for intraspinal lesions presenting with hemorrhage in the clinical context of cyclical neurological symptoms.