Arrested hydrocephalus and the presyrinx state

Abstract

Hydrocephalus is known to be associated with syringomyelia; however, the association between arrested hydrocephalus and syringomyelia has been reported only once before. This presyrinx state is a recently recognized condition that is known to resolve with proper treatment. This case report highlights a rare association between arrested hydrocephalus and the presyrinx state and outlines the implications for treatment and etiopathogenesis. A 6-year-old girl with arrested hydrocephalus presented with a recent history of difficulty in walking despite no antecedent trauma or infection. An examination revealed macrocrania and features of cervical myelopathy, and magnetic resonance (MR) imaging revealed panventriculomegaly with evidence of the presyrinx state on both T1- and T2-weighted images of the cervical spine. The patient underwent a procedure to insert a ventriculoperitoneal shunt that used a mediumpressure system. After the shunt was inserted, the patient's myelopathic symptoms gradually improved over a period of 6 months. Postoperative MR images obtained 1 year later revealed decompression of the ventricular system and complete resolution of the presyrinx state in the cervical spinal cord. Decompensation in arrested hydrocephalus is a well-known phenomenon. This case illustrates the concept that the presyrinx state may be one of the manifestations of decompensated arrested hydrocephalus. The importance of early recognition of this condition and its implications for the pathogenesis of syringomyelia are discussed.

Presyrinx Associated with Post-Traumatic Hydrocephalus Successfully Treated by Ventriculoperitoneal Shunt

“…Several clinical conditions are characterized by abnormal CSF flow dynamics, in which case a timely intervention is needed to restore normal CSF passage (TABLE 1). 3) Good clinical outcomes have been obtained with surgical decompression, ventriculoperitoneal shunting, and endoscopic fenestration 3111215)…”

“…Treatment of the hydrocephalus and increased ICP is the primary strategy before decompressive surgery is considered. An important aspect of presyrinx is that the radiological and clinical abnormalities are potentially reversible,51112) whereas in syringomyelia, a favorable prognosis can be difficult to achieve. Our patient likely already had an asymptomatic Chiari I malformation.…”

Syringomyelia as a presenting feature of shunt dysfunction: Implications for the pathogenesis of syringomyelia

“…[17] It has been previously shown that when a patient with arrested hydrocephalus decompensates, development of a presyrinx state might be one of the ways of decompensation and treatment of the decompensated arrested hydrocephalus can reverse the presyrinx state. [18] More recently, Camacho and colleagues have documented the development of communicating syringomyelia in a preterm infant with post hemorrhagic hydrocephalus. [15] More recently, Hagihara and Sakata reported a 13 year old boy with disproportionately large communicating fourth ventricle with syringomyelia in whom there was complete clinical and radiological improvement following placement of ventriculoperitoneal shunt.…”