Acute low-pressure hydrocephalus: a case series and systematic review of 195 patients

Abstract

OBJECTIVEAcute low-pressure hydrocephalus (ALPH) is characterized by clinical manifestations of an apparent raised intracranial pressure (ICP) and ventriculomegaly despite measured ICP that is below the expected range (i.e., typically ≤ 5 cm H2O). ALPH is often refractory to standard hydrocephalus intervention protocols and the ICP paradox commonly leads to delayed diagnosis. The aim of this study was to characterize ALPH and develop an algorithm to facilitate diagnosis and management for patients with ALPH.METHODSEMBASE, MEDLINE, and Google Scholar databases were searched for ALPH cases from its first description in 1994 until 2019. Cases that met inclusion criteria were pooled with cases managed at the authors’ institution. Patient characteristics, presenting signs/symptoms, precipitating factors, temporizing interventions, definitive treatment, and patient outcomes were recorded.RESULTSThere were 195 patients identified, with 42 local and 153 from the literature review (53 pediatric patients and 142 adults). Decreased level of consciousness was the predominant clinical sign. The most common etiologies of hydrocephalus were neoplasm and hemorrhage. While the majority of ALPH occurred spontaneously, 39% of pediatric patients had previously undergone a lumbar puncture. Prior to ALPH diagnosis, 92% of pediatric and 39% of adult patients had a ventricular shunt in situ. The most common temporizing intervention was subatmospheric CSF drainage. The majority of patients underwent a shunt insertion/revision or endoscopic third ventriculostomy as definitive ALPH treatment. Although the mortality rate was 11%, 83% of pediatric and 49% of adult patients returned to their pre-ALPH neurological functional status after definitive treatment. Outcomes were related to both the severity of the underlying neurosurgical disease causing the hydrocephalus and the efficacy of ALPH treatment.CONCLUSIONSALPH is an underrecognized variant phenotype of hydrocephalus that is associated with multiple etiologies and can be challenging to treat as it frequently does not initially respond to standard strategies of CSF shunting. With early recognition, ALPH can be effectively managed. A management algorithm is provided as a guide for this purpose.

Hidrocefalia aguda a presión negativa: propuesta de manejo y valor de la ventriculostomía temprana

“…Recientemente Keough et al realizaron una excelente revisión de 195 casos de hidrocefalia aguda a baja presión (43 propios), así como unas guías de manejo de la misma 8 . El protocolo diagnóstico-terapéutico que manejamos en nuestro hospital es el siguiente y se muestra en la figura 1.…”

“…Pocos artículos han descrito la utilidad de la ventriculostomía premamilar endoscópica (VPME) en el manejo de la HAPN. Aunque los resultados parecen ser satisfactorios en dichas series 4,5,8 , no queda claro qué pacientes pueden beneficiarse de esta técnica, ni el mejor momento para llevarla a cabo.…”

Negative-pressure hydrocephalus in the course of a complex postoperative intracranial pressure disturbance: illustrative case

“…The importance in treating the CSF leak has been implied; however, EVD, despite the negative pressure, has been the main choice, and eventually most cases are designated for permanent CSF shunt diversion. 1,6,12 In the literature, this phenomenon has been given several names, including "(acute) low-pressure hydrocephalus," "NePH," and "syndrome of inappropriately low-pressure hydrocephalus." The term for this condition should be integrated; even "low-pressure ventriculomegaly" can appropriately express the current status of knowledge.…”

“…ETV has also been reported as an alternative treatment because it restores the communication between intra-and extraventricular spaces. [5][6][7] However, because we understood that the main point of the treatment of NePH is correcting the negative transmantle pressure, we selected a blood patch to stop the CSF leak intervening on the pressure outside ventricles.…”

“…Etiology includes subarachnoid hemorrhage, posterior fossa tumor cases, or conditions after lumbar puncture, among others. [1][2][3][4] Negative-pressure external ventricular drainage (EVD), 1,3 endoscopic third ventriculostomy (ETV), 5,6 neck wrapping (or a cervical tourniquet), 1,7 intermittent shunt valve pressing, and enforced recumbency were applied to treat patients with poor outcomes. 4,[7][8][9] This report describes an adult patient with iatrogenic NePH whose management was successful and whose symptoms disappeared without requiring permanent cerebrospinal fluid (CSF) diversion.…”