Atlantoaxial limited dorsal myeloschisis: A report of two cases and review of literature

Abstract Introduction Limited dorsal myeloschisis (LDM) is a rare form of spinal dysraphism that is characterised by a distinctive fibroneural stalk connecting the spinal cord to the overlying skin lesion. The skin lesions associated with LDM can appear benign clinically and careful evaluation with an MRI scan is essential for diagnosing LDM and to differentiate this entity from other forms of spinal dysraphism and benign causes of skin lesions. Research question There is a lack of reported atlantoaxial LDM in the literature. We sought to report the clinical presentation, radiological features and surgical management of the first two reported atlantoaxial LDM. Material and methods Clinical findings and radiological images of the two cases of atlantoaxial LDM that underwent surgical intervention at our institution were retrieved from the medical notes, operative records and imaging system. Results Both cases of atlantoaxial LDM (C0-1 and C1-2 respectively) underwent successful resection of the overlying cutaneous lesions and stalks to release the tethered spinal cords. Discussion The surgical management of LDM have been associated with good outcomes and consists of resecting the fibroneural stalk close to the underlying cord, releasing the tethered spinal cord and removing the overlying cutaneous lesion. Conclusion These are the first two reported cases of atlantoaxial LDM in the literature. We aim to raise awareness of this pathological entity and highlight the importance of establishing the correct diagnosis to guide definitive management, and report the favourable neurological outcome in these cases despite the rostral location.