Purpose: Limited dorsal myeloschisis (LDM) is characterized by a fibroneural stalk linking the skin lesion to the underlying spinal cord. On account of the external skin lesion, all LDMs are either flat (nonsaccular) or saccular, and a human tail-like cutaneous appendage has not been reported. Methods: In our 14 LDM patients, 2 had tail-like appendages. We retrospectively analyzed the relationship between the appendage and the LDM tract from the clinicopathological findings of these 2 patients. Results: Preoperative magnetic resonance imaging including three-dimensional heavily T2-weighted images demonstrated an intradural tethering tract, but failed to reveal the precise communication with the appendage. However, surgery revealed the extradural and intradural slender stalk, starting at the base of appendage and running through the myofascial defect. Histological examination demonstrated that there was a tight anatomical relationship between the fibroadipose tissue of the appendage and the fibrocollagenous LDM stalk. Conclusion: When there is potential for an LDM stalk in patients with an appendage, a meticulous exploration of the stalk leading from an appendage is required. Clinicians should be aware of possible morphological variations of skin lesions associated with LDM.
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