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

BACKGROUND: Low-pressure hydrocephalus (LPH) is a rare phenomenon characterized by a clinical picture consistent with elevated intracranial pressure (ICP) and ventricular enlargement, but also a well-functioning shunt and low or negative ICP.

OBJECTIVE: To report our experience in evaluating this challenging problem.

METHODS: Patients with LPH were identified from several sources, including institutional procedural databases and personal case logs. Electronic medical records were reviewed to collect demographic, clinical, surgical, and radiographic data to determine the presence of LPH. Each patient's clinical course, including presentation, management, and outcome, is reported.

RESULTS: Thirty instances of LPH were identified in 29 patients. Eleven cases (37.9%) of LPH were after lumbar puncture (LP), and 19 cases (62.1%) occurred without any preceding spinal procedure. Among the post-LP patients, conservative measures alone were successful in 3 cases (27%); lumbar blood patch was successful in 2 cases (18%); and 6 cases (55%) required external cerebrospinal fluid (CSF) drainage. Of the spontaneous cases, 5 patients did not receive the full spectrum of treatment because of terminal prognosis. Of the remaining 14 patients, 11 (78.6%) required external CSF drainage. Post-LP patients required fewer days of external CSF drainage (median, 4 [range, 0-12] vs median, 11 [range, 0-90]) and had a shorter hospital stay (median, 2 [range, 2-16] vs median, 8 [range, 0-26]).

CONCLUSION: 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.