Intraoperative neurophysiological monitoring (IONM) in paediatric neurosurgeries is emerging over the past few years, and it is being widely used to decrease the risk of postoperative deficits. The same techniques of IONM used in adults can be used in paediatric patients with some modifications keeping in consideration their immature developing nervous system. Neurophysiology in children differs from that in adults. In paediatric patients, the IONM signals and responses differ according to the age of the child. The type of surgery and the neuronal pathways expected to be at risk, determine the type of IONM modality used. 1,2 Various factors like age, type and location of the lesion, preoperative neurological functional status should be considered while using IONM in paediatric patients. There are also immense challenges with anaesthesia maintenance in children undergoing IONM. Also, the IONM signals and responses are not as easily obtained as in adults due to many factors related to the immature nervous system. 3,4

The diagnosis of LDMs, especially in developing countries, is frequently missed. This needs to be tackled and addressed so that children with this subset of neural tube defects, who otherwise would normally have a very good outcome, may not be blighted and left scarred for life at an early age.

Syringohydromyelia is a frequent finding in cases of tethered cord syndrome. The classical teaching is that the development and progression of a syrinx is a chronic process. We present a case report of an acute onset syringomyelia in an infant, who underwent an excision of a lumbosacral transitional lipoma and detethering of the cord. Immediately after recovery, the infant was found to have flaccid paraplegia. An emergency magnetic resonance imaging revealed a large acute onset syringomyelia for which he underwent an emergency midline myelotomy and release of fluid from the syrinx. Though the eventual recovery was good, this made us re-visit our understanding of the concept of syringohydromyelia. The case details and a plausible hypothesis for the rapid development of the syrinx are presented.