Spondylocostal Dysostosis Associated with Split Spinal Cord and Other Malformations

Abstract

Spondylocostal dysostosis is a very rare combination of complex vertebra and rib malformations, accompanied occasionally by other disorders. A 3-year-old girl presented kyphoscoliosis, foot deformities, gate disturbance, and urinary incontinence. The CT and MRI examination revealed kyphosis and scoliosis with a double curve, some absent, broadened, bifurcating and fused ribs, hemivertebrae, butterfly and cleft vertebrae in thoracic and lumbar region, sporadic cleft or absent vertebral arches or pedicles, and hypoplastic sacrum with a cleft of the S2 vertebra. Spina bifida occulta extended from T10 to T11, and from L3 to the end of the sacrum. Two hemicords, separated by a bony septum and surrounded by their own dural tubes (type I), were present from the level of T9 to the conus medullaris. Filum terminale was thick and duplicated. Syringomyelia was present in the thoracic cord from T5 to T8. Finally, a small meningocele was seen at the T10–T11 level, and a subcutaneous lipoma in the thoracolumbar region. To our knowledge, such a combination of vertebra, rib, and cord malformations, including the mentioned additional disorders, has never been reported.

Jarcho-Levin syndrome with diastematomyelia and a dorsal dermal sinus

“…Our case fits type 2 diastematomyelia, in which the 2 hemicords are seen in a single dural sac with no separating septum between them. To date, only ten cases of JLS with true diastematomyelia have been reported in the English literature and this is the 11th reported case ( Table 1 ) [ [3] , [4] , [5] , [8] , [9] , [10] , [11] , [12] , [13] , [14] ]. In addition, most of the previously reported cases were type I malformations, making the current finding of type 2 split cord interesting [ 4 , 14 ].…”

Scoliosis

“…Finally, in children with congenital scoliosis, CT can provide a more detailed analysis of vertebral malformations. 27…”