Images in Neurosurgery

17-Year-Old Female with Congenital Scoliosis and Type I Diastematomyelia

The patient is a 17-year-old female with congenital deformity who underwent at T8-T12 anterior fusion elsewhere at the age of six. The patient presents now with mild back pain and a predominantly cosmetic concern. Her neurological exam was positive only for positive sagittal imbalance and left-sided coronal offset, with the left leg longer than the right. Imaging revealed multiple congenital abnormalities including C6-7 Klippel-Feil anomaly, butterfly vertebrae of T12, L1, L2, L3 and S1 with fused left hemivertebrae of L1 and L2. The patient had type 1 diastematomyelia with a bony septum at L2-4, tethering the cord. The operative plan devised was to first detether the cord, resect the osseous septum and reconstruct the two hemi-sacs, reserving the correction of the kyphoscoliotic deformity for a second stage. Surgery was conducted successfully and the patient returned to her home country to re-evaluate her cosmetic concerns after recovery. 

Xray of scoliosis patient

Figure 1. Scoliosis x-rays lateral (A) and AP (B) projections. (C) Coronal CT with axial sections (D-F) showing patients previous thoracic fusion at T8-12 and lumbar butterfly and hemivertebrae. (C-F) highlighting the bony septum present from L2-4 as well as the abhorrent anatomy above and below this level.

Image of initial extradural exposure and bony septum

Figure 2. Intraoperative photos (rostral on the left in all photos) showing
(A) initial extradural exposure and bony septum, (B) dissection of dura away from bony septum, (C) intradural exposure after repair of ventral dural defect showing two separate spinal cords and cauda equina roots, (D) final dorsal dural repair with a patch graft.

Post-operative T2 MRI coronal (A) and axial (B) section showing removal of the bony septum and joining of the two cords in a single dural sac.

Figure 3. Post-operative T2 MRI coronal (A) and axial (B) section showing the removal of the bony septum and joining of the two cords in a single dural sac.

Submitted by: Bledi C. Brahimaj, MD, Ricardo B. Fontes, MD and John E. O’Toole, MD
Affiliations: Rush University Medical Center