A nine month old male child was brought with the history of a gradually increasing swelling in the lower back in the midline since birth. There is no weakness of lower limbs or complaints related to bowel/urinary bladder function. On examination there is a cystic swelling located in the midline in the posterior lumbosacral region. There is no tuft of hair on it.
The MRI of the brain and the spinal cord shows the lateral ventricles and the third ventricle to be dilated. (Evan’s index 0.58). The posterior fossa is small; its contents crowded, The cerebellar tonsils and vermis have herniated through the foramen magnum. The fourth ventricle is compressed ; it is elongated and caudally displaced. The corpus callosum is thin and displaced upwards. There is a defect in the posterior elements between L5 and S2 vertebrae with posterior herniation of a CSF filled sac that is about 5 cm in size. It contains the spinal cord and nerve fibres.