Background:
Split cord malformations (SCMs) are uncommon congenital anomalies. They can be divided into Type 1 (bony septum and two separate dural sheaths) and Type 2 (fibrous septum and a single dural sheath).[1,2,4] Rarely,SCM can be associated with orthopedic anomalies (mostly seen in older children), such as scoliosis, followed by kyphosis, and/or both.[1-3] Conversely, patients with congenital scoliosis have 40% risk of congenital underlying neural deformities, with 16.3% attributed to SCM.[1,4] Those with thoracic or lumbosacral SCM may have congenital tethering lesions, predominantly consisting of intradural lipomas.[3,4] Surgery is optimally performed in two stages: first, removal of the spinal septum and untethering of the cord, and second, correction of the kyphosis, scoliosis, and/or both.[1-5]
Case Description:
In this video, a 44-year-old female initially presented after having progressively developed kyphoscoliosis since childhood. Three months before presentation, she had developed increased left lower extremity pain with hypoesthesia followed 1 month later by the onset of the left lower extremity monoplegia (0/5). The computed tomography and magnetic resonance imaging studies revealed a SCM at the L3 level with bony and membranous septum, accompanied by an intradural lipoma, and tethered cord. She underwent a two- stage procedure; first, removal of the bony and membranous septum, resection of the intradural lipoma, and untethering of the cord; second, she had correction of the kyphoscoliosis. Postoperatively, although the pain and sensory deficits improved, the left lower extremity monoplegia remained.
Conclusion:
SCM is a rare cause of spinal deformity. If left untreated, the associated neurological deficits may progress. Treatment should include a two-staged approach; first, the bony and membranous septum should be removed followed by lipoma resection and untethering the cord with adequate cord decompression, while second, a fusion may be performed to address attendant kyphoscoliosis.
Keywords:
Intradural lipoma, Kyphoscoliosis, Split cord malformation, Tethered cord
Staged corrective surgery for complex congenital scoliosis and split cord malformation