Amniotic band syndrome associated with limited dorsal myeloschisis: a case report of an unusual case and review of the literature.

Amniotic band syndrome (ABS) has been known since ancient times. Descriptions in modern medicine have occurred since the mid-nineteenth century. The association of the amniotic band syndrome with tethered cord is rare. It was generally thought to be incompatible with life. Of late, with better imaging, there have been case reports of amniotic bands causing cord tethering in neurosurgical literature, but its association with limited dorsal myeloschisis has hitherto not been described. We report a case of amniotic band syndrome (ABS) associated with a cervical limited dorsal myeloschisis (LDM) in a child of 2 and a half years old. The management of LDM in ABS is essentially the same as in isolated cases.

Acute onset of postoperative syringohydromyelia.

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.

Missed limited dorsal myeloschisis: an unfortunate cause for recurrent tethered cord syndrome.

AIM: This study aims to highlight the recurrent tethered cord syndrome in relation to the relatively new pathological entity of limited dorsal myeloschisis (LDM) and to mention the difficulties faced by the paediatric neurosurgeon in developing countries with reference to LDM which was not recognized at initial presentation. MATERIALS AND METHODS: We present three cases of recurrent tethered cord syndrome who were operated early in life (not by paediatric neurosurgeons) as meningoceles and who were then subsequently referred to the senior author as they presented with clinical signs of re-tethering of the cord. RESULTS: The first child of 1 year of age represented with cervicodorsal swelling 10 months after primary surgery for a cervical meningomyelocele done elsewhere on the second day of life. The second was a girl of 3 years of age who was operated at birth and presented with severe brachialgia and neck pain after 2 years. The third was a 19-year-old girl operated at birth presenting with spastic paraparesis and also hand weakness associated with kyphosis. All the three patients improved symptomatically and neurologically after redo surgery. CONCLUSION: 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.