Low-pressure Hydrocephalus in Children: A Case Series and Review of the Literature

Abstract

This study represents the largest series of LPH. Although its pathophysiology remains a mystery, there are a variety of management options. Multiple procedures and a protracted hospital stay are often required to successfully treat LPH.

Low-Pressure Hydrocephalus and Shunt Malfunction Following a Lumbar Puncture in an Adult Reversed by an Epidural Blood Patch

“…[1][2][3] The symptoms perceived are identical to those usually observed in the more commonly seen high-pressure hydrocephalus, with headaches, nausea, cranial neuropathies, ataxia, and obtundation. [2][3][4] McCullough 1 reported a series of patients who would develop symptomatic ventriculomegaly only when in the upright position, despite low or normal pressures and patent shunts. Subsequently, several case series of shunted patients with LPH occurring either spontaneously or following cerebrospinal fluid (CSF) loss were published.…”

“…7 LPH may either occur spontaneously or as a result of CSF egress from the skull base, spinal intradural procedures, or lumbar punctures. [2][3][4][5]10,11 Patients usually present with symptoms similar to those seen in the more prevalent high-pressure hydrocephalus. Headaches, nausea, cranial neuropathies, disturbances in mental status, and decreased consciousness are often seen.…”

“…28,29 The indications for an epidural blood patch have become broader and now include spontaneous intracranial hypotension, post-lumbar puncture paradoxical herniation, treatment of cranial nerve dysfunction following lumbar puncture or spinal anesthesia, among others. 25,28,[30][31][32][33] Smalley et al 2 were the first to report an epidural blood patch for the management of post-lumbar puncture LPH in 2 children, but to this date this approach has not been reported in an adult.…”

“…Recumbency and cervical wrapping have been successful in the management of post-lumbar puncture LPH, but in most cases external ventricular drainage with subsequent revision of the shunt system has been necessary. 2,3,9 An epidural blood patch was effective in 2 children with shunt malfunction due to post-lumbar puncture LPH, 2 but this management strategy in an adult, to our knowledge, has not been published. In addition, we provide further thoughts on the interaction between the CSF spaces and the cerebrospinal venous system in the pathophysiology of post-lumbar puncture LPH.…”

“…Subsequently, several case series of shunted patients with LPH occurring either spontaneously or following cerebrospinal fluid (CSF) loss were published. [2][3][4][5][6][7] Egress of cerebral subarachnoid CSF from the lumbar theca with a subsequent decrease in ICP has been suggested as the cause of post-lumbar punctures LPH. 4 More recently, the hypothesis that cerebral venous overdrainage plays a major role in the pathogenesis of LPH has emerged, 8 although its connection to spinal CSF loss has not been contemplated yet.…”