How should we treat long-standing overt ventriculomegaly in adults (LOVA)? A retrospective cohort study.

Long-standing overt ventriculomegaly in adults (LOVA) is a heterogenous group of conditions with differing presentations. Few studies have evaluated success rates of available surgical treatments, or ascertained the natural history. There is a need to assess the efficacy of both endoscopic third ventriculostomy (ETV) and ventriculoperitoneal shunt (VPS) as first-line treatments. We conducted a retrospective, single-centre study of adults with LOVA at a tertiary neurosurgery centre in England, UK, aiming to identify presentation, management strategy, and outcome following treatment. A total of 127 patients were included (mean age 48.1 years, 61/127 male). Most patients were symptomatic (73.2%, n = 93/127, median symptom duration 10 months). The most common symptoms were gait ataxia, headache, and cognitive decline (52.8%, 50.4%, and 33.9%, respectively). Fourteen patients had papilloedema. Ninety-one patients (71.7%) underwent surgery (84 ETV, 7 VPS). Over a median follow-up of 33.0 months (interquartile range [IQR] 19.0-65.7), 82.4% had a clinical improvement after surgery, and 81.3% had radiological improvement. Clinical improvement rates were similar between ETV and VP shunt groups (82.1% vs 85.7%, p = 0.812). Surgical complication rates were significantly lower in the ETV group than the VP shunt group (4.8% vs 42.9%, p < 0.001). Of the patients treated surgically, 20 (22.0%) underwent further surgery, with 14 patients improving. This study demonstrates the efficacy of ETV as a first-line treatment for LOVA.

Utility of intracranial pressure monitoring as a diagnostic tool in pediatric ventriculomegaly.

OBJECTIVE: Intracranial pressure (ICP) monitoring is commonly utilized for identifying pathologic ICP in cases of traumatic brain injury; however, its utility in hydrocephalic children has not been elucidated. Although patients with typical (pressure-active) hydrocephalus present with clear signs and/or symptoms and the need for cerebrospinal fluid (CSF) diversion is often clear, others may have arrested or pressure-compensated hydrocephalus with pathologic ICP elevation masked by ambiguous signs or are completely asymptomatic. Without treatment these pathologic ICP elevations may affect neurologic development or crescendo over time leading to neurological decline. The purpose of this study is to investigate the utility of ICP monitoring as a diagnostic tool in this relatively common patient population and identify ventriculomegaly patients with and without pathologic ICP, thus improving accuracy of identifying those with and without surgical needs. METHODS: 36 patients (≤ 17 years old) underwent 41 inpatient ICP recording sessions between 2016 and 2022 and were retrospectively reviewed. This included patients with a history of severe, nonprogressive ventriculomegaly and normal fundoscopic examinations lacking traditional signs and symptoms concerning for elevated ICP. Nighttime pathological plateau waves were defined as sustained elevations of ICP ≥ 2x baseline for a duration of ≥ 5 minutes. RESULTS: The mean age of patients was 5.5 years old (range 0-17 years old). 46.3% of patients had prior endoscopic third ventriculostomy (ETV), 14.6% had prior ventriculoperitoneal shunt (VPS), and 39% were without prior surgical intervention. Roughly half (51.2%) of patients had congenital ventriculomegaly while other patients had ventriculomegaly due to other pathologies such as germinal matrix hemorrhage/intraventricular hemorrhage (GMH/IVH) (29.3%), stroke (4.9%), cerebral infections/meningitis (2.4%), or unknown etiology (12.2%). The average procedure time was 19.1 ± 10.5 minutes, and mean length of stay was 2.8 ± 0.7 days. Pathologic ICP was demonstrated in 12 cases (29.3%), 4 (33.3%) of which were asymptomatic. Pathologic ICP was found in 7 of 19 (36.8%) in the prior ETV group, 1 of 6 (16.7%) in prior shunt group, and 4 of 16 (25%) in the non-surgical group (p = 0.649). Among those with pathologic ICP, 6 (50%) cases received an ETV, 5 (41.7%) cases underwent VPS placement, and 1 (8.3%) case underwent a VPS revision. There were no infectious complications or cases of hemorrhage. 4 patients required repositioning of the ICP monitor due to dislodgement. CONCLUSION: Inpatient ICP monitoring is a safe and effective diagnostic tool for evaluating the presence of pathologic ICP in severe, persistent non-progressive ventriculomegaly. The use of ICP monitoring may aid in identifying patients with pressure-compensated hydrocephalus who demonstrate pathologic ICP where surgical intervention may be warranted, while preventing unnecessary CSF diversion in those without pathology.

Successful conservative management of a large acute epidural hematoma in a patient with arrested hydrocephalus: A case report.

Background: Arrested hydrocephalus is a condition wherein congenital hydrocephalus spontaneously ceases to progress due to a balance between production and absorption of cerebrospinal fluid. These patients rarely present with pressure symptoms so conservative treatment may be instituted. There are, however, little data on the long-term outcomes of these patients and how they present in the presence of other intracranial pathologies as they transition into adulthood. We aim to add to the growing knowledge about the management of patients with arrested hydrocephalus who have sustained traumatic hematomas. Case Description: To the best of our knowledge, we present the only reported case of a 34-year-old female with arrested hydrocephalus who sustained an acute epidural hematoma secondary to a fall and underwent a conservative management. She was asymptomatic except for mild headache that started on the 3rd day postinjury and was thus treated conservatively with favorable outcomes. A review of literature showed that adults with arrested hydrocephalus may develop intracranial hematomas after head injuries despite them manifesting with little or no symptoms. The hydrocephalus may have provided them with a form of internal decompression thus delaying symptomatology. Conclusion: Clinicians should be vigilant as these patients will present with either delayed or completely without neurologic symptomology. Tailored and individualized management of other intracranial pathologies should be adapted in this subset of patients.

Diagnostic Nuances and Surgical Management of Arrested Hydrocephalus.

Hydrocephalus is characterized by the increased volume of cerebrospinal fluid (CSF) with enlarged cerebral ventricles. In nearly 50% of the patients, if left untreated, the balance between CSF production and absorption is achieved, resulting in arrested hydrocephalus (AH). However, 15% of them who are diagnosed as arrested can progress over a period of time. Importantly, a large fraction of patients with hydrocephalus in India, may not have access to tertiary level care. Therefore, both progressive hydrocephalus and insidious progression of AH with related mortality and morbidity could be higher in India. The pathophysiology behind AH and insidious progression of AH are poorly established. Unfortunately, there are no established clinical or radiological parameters identifying or predicting AH from progressive hydrocephalous. Diagnosis is often based on a combination of neurological, psychometric, and magnetic resonance imaging (MRI) findings. Invasive monitoring of intracranial pressure (ICP) and telemetric ICP measurement is increasingly helping surgeons to detect insidious progressive AH in the early stages. In patients with AH, surgery may not be always necessary and a conservative approach is often adopted. On the contrary, AH that becomes progressive may require intervention. Surgical intervention should not be delayed and endoscopic third ventriculostomy (ETV) is preferable over shunt placement. Importantly, comprehensive counseling and the appropriate selection of patients are pivotal in improving outcomes and reducing complications.

Arrested Hydrocephalus Complicated by Growing Skull Fracture - A Case Report with Review of Literature.

Growing skull fracture (GSF) is an extremely uncommon entity and accounts for less than 1% of the skull fractures. This type of fracture is commonly seen in children of less than 3 year of age and two third of them occur in less than one year of age. Occurrence of GSF is higher in infancy and early childhood because of rapid growth of brain and skull take place in initial two year of the life. Dural tear is most common etiological factor that leads to growing skull fracture. Growing skull fracture with arrested hydrocephalus is a rare association and has been described only once in literature. We hereby, are reporting a case of one year child presented with gradual progressive head enlargement with progressive subgaleal swelling over left parietal region. patient sustained head injury 4 month back due to fall from bed. After all relevant radiological examinations, cyst excision and water tight dura closure was done. Patient improved and till last follow up there was not any recurrence of cyst.

Atlantoaxial Subluxation Secondary to Unstable Os Odontoideum in a Patient With Arrested Hydrocephalus Due to Congenital Aqueductal Stenosis: A Case Report.

BACKGROUND: In a small percentage of children born with congenital hydrocephalus, enlargement of the head and the presence of ventriculomegaly may halt and ultimately stabilize the condition designated as arrested hydrocephalus. Arrested hydrocephalus in children is typically due to congenital aqueduct stenosis, which can be described appropriately as a stasis existing within the channel between the third and fourth ventricles. Os odontoideum (OO) is an uncommonly occurring pathology at the craniovertebral junction. Although the clinical and radiologic features of its existence and the therapeutic options for its pathology have been widely discussed within the medical literature, its true etiology has been a source of divisive debate, proposing both a traumatic as well as a congenital mechanism. The etiology of OO has been heartily debated in the literature for several years as well. Most authors have come to support a posttraumatic causality. However, strong evidence exists to support a congenital origin to this rarely observed malformation. METHODS: Within this case study we present a 24-year-old woman with atlantoaxial subluxation that exists secondary to an orthotropic OO. The patient had a history of arrested hydrocephalus due to congenital aqueductal stenosis beginning in early childhood. She presented with normal intelligence and was neurologically without deficits before the occurrence of an atlantoaxial dislocation. Unfortunately, the pathology was initially misdiagnosed as a decompensation state of the arrested hydrocephalus, and after 8 months the patient became wheelchair bound. Following this unfortunate event the correct diagnosis was ultimately uncovered. Subsequently a C2-1 instrumentation procedure resulted in excellent alignment and fusion. CONCLUSIONS: To the best of our knowledge, this is the first example of an aqueduct stenosis in the setting of an existing OO, a combination that might be another clue in favor of a congenital etiology.

An algorithmic approach to the management of unrecognized hydrocephalus in pediatric candidates for intrathecal baclofen pump implantation.

BACKGROUND: Complications of intrathecal baclofen (ITB) pump implantation for treatment of pediatric patients with spasticity and dystonia associated with cerebral palsy remain unacceptably high. To address the concern that some patients may have underlying arrested hydrocephalus, which is difficult to detect clinically because of a low baseline level of neurological function, and may contribute to the high rates of postoperative cerebrospinal fluid leak, wound breakdown, and infection associated with ITB pump implantation, the authors implemented a standardized protocol including mandatory cranial imaging and assessment of intracranial pressure (ICP) by lumbar puncture prior to ITB pump implantation. METHODS: A retrospective case series of patients considered for ITB pump implantation between September 2012 and October 2014 at Seattle Children's Hospital is presented. All patients underwent lumbar puncture under general anesthesia prior to ITB pump implantation and, if the opening pressure was greater than 21 cmH2O, ITB pump implantation was aborted and alternative management options were presented to the patient's family. RESULTS: Eighteen patients were treated during the study time period. Eight patients (44.4%) who had ICPs in excess of 21 cmH2O on initial LP were identified. Eleven patients (61.1%) ultimately underwent ITB pump implantation (9/10 in the "normal ICP" group and 2/8 in the "elevated ICP" group following ventriculoperitoneal shunt placement), without any postoperative complications. CONCLUSIONS: Given the potentially high rate of elevated ICP and arrested hydrocephalus, the authors advocate pre-implantation assessment of ICP under controlled conditions and a thoughtful consideration of the neurosurgical management options for patients with elevated ICP.

Surgical management of arrested hydrocephalus: Case report, literature review, and 18-month follow-up.

OBJECTIVE: Arrested hydrocephalus is stable ventriculomegaly without evidence of neurologic deterioration or symptoms. Management of arrested hydrocephalus in asymptomatic adults is controversial, with little clinical data. This case highlights the potential for decompensation in adults with arrested hydrocephalus and reviews the literature regarding pathophysiology and management of this clinical entity. PATIENTS AND METHODS: A 39 year-old gentleman with arrested hydrocephalus incidentally found during work-up for new-onset seizure and managed conservatively for ten years presented with increasing headache, memory loss, gait instability and urinary and fecal incontinence. Stable massive triventriculomegaly was documented on serial brain imaging, and ophthalmologic exam revealed no papilledema. RESULTS: The patient underwent endoscopic third ventriculostomy with immediate post-operative improvement of headache, resolution of incontinence, and cessation of seizures. At 15 months after surgery, neuropsychiatric testing demonstrated improvement in visuomotor skills, problem solving, verbal fluency and cognitive flexibility compared to his pre-operative baseline. At 18 months after surgery he remained seizure free with full continence and significant improvement in headaches. CONCLUSION: Early recognition of arrested hydrocephalus and its potential for decompensation may prompt surgical treatment and prevent neurologic deterioration.