Preysyrinx state and shunt dysfunction: an under recognized entity?

摘要

Presyrinx state is a relatively recently recognized condition. Prompt identification of this condition and appropriate treatment leads to reversal of the radiological and clinical findings with a good prognosis. Failure to identify this condition in a timely fashion leads to fully established syringomyelia. To date, presyrinx state has not been described as a feature of shunt dysfunction. One such rare case is being reported. A 14-year-old boy who was shunted for postmeningitic hydrocephalus at the age of 2 years and subsequently underwent shunt revision presented for routine follow-up and was found to have shunt dysfunction. CT scan showed panventriculomegaly and MRI studies showed panventriculomegaly with a “presyrinx” state extending from the fourth ventricle up to the thoracic region. Shunt revision led to prompt radiological reversal of the presyrinx state. This case is being reported to highlight the hitherto unreported association of shunt dysfunction and presyrinx state. The literature on presyrinx state is being reviewed with reference to the 20 cases reported earlier.