An 18-month-old female child presented to the neurology outpatient department with complaints of weakness of both lower limbs and urinary incontinence for 4 days. The child had been toilet trained at the age of 12 months. The onset of weakness was acute in nature and non-progressive. There was no history of trauma to the spine, no history of fever, and no history of muscular dystrophies. The child was a full-term normal vaginal delivery at the hospital with no intrapartum or postpartum complications. Developmental milestones were age-appropriate and immunized per the Indian Academy of Pediatrics schedule. There was no history of marriage consanguinity. General and other systems examinations were found to be normal. MRI of the spine was requested to look for the cause of her weakness.
MRI spine with standard pre- and post-contrast sequences sagittal TSE T1, TSE T2, STIR, HASTE T2, MEDIC, Axial T1, T1 FS, and TSE T2, Coronal STIR, and MR myelograms was included in the protocol. IV contrast was administered, and multiplanar post-contrast imaging was done. The MR images revealed: Abnormal intramedullary bunch of flow voids with a sac-like structure measuring 9.0 mm × 10.5 mm × 15 mm (AP × TR × CC) was seen extending from D9 to D10 Intravertebral disk level to mid D11 vertebral body level. Associated cord edema was seen extending superiorly till the D1 vertebral level and till conus medullaris inferiorly (Fig. 1).
Based on MRI findings, the child was diagnosed with a spinal arteriovenous malformation type 2 with intra- and extramedullary components of the vascular sac and evidence of intramedullary hematoma.
Digital subtraction angiography (DSA) was done, which showed hypertrophied intercostal artery with a large intramedullary and extramedullary tangle of vessels. Given complex anatomy, the neurosurgeon and neuro-intervention team took the decision of targeted endovascular embolization.
The child was taken up for endovascular embolization under general anesthesia in a separate sitting after appropriate counseling of the parents and obtaining informed consent from them. A diagnostic spinal vessel angiogram revealed an abnormal hypertrophied feeder from the left D8 intercostal artery supplying the AV Fistula with an aneurysmal sac. Superselective cannulation of the feeding artery was done using a flow-directed microcatheter (Marathon, EV3), and 2 ml of 30% NBCA glue (n-butyl cyanoacrylate) was injected at the fistulous site. Complete obliteration of the AV fistula and intramedullary aneurysmal sac was achieved (Fig. 2).
Post-embolization MRI was done, and it revealed a significant reduction in nidus and obliteration of sac. There was also resolution of the cord edema (Fig. 3). On follow-up, the child gradually improved over the next 1 year and could walk with assistance and regain complete bladder control. At 36 months, the patient could walk with support, regained complete bladder control, and residual wriggling gait was present.