Intracranial hypertension

Update on intracranial hypertension and hypotension

Authors: Hoffmann, Jan; Goadsby, Peter J.

Purpose of review: The aim of this article is to review recent findings on the pathophysiology of idiopathic changes in intracranial pressure. The review will focus on idiopathic intracranial hypertension (IIH) and spontaneous intracranial hypotension (SIH).

Recent findings: Substantial evidence indicates that IIH is associated with delayed absorption of cerebrospinal fluid (CSF). Stenoses of the transverse sinus are common in IIH, but their clinical significance has not been entirely clarified. Despite the observed efficacy of endovascular treatment in some IIH patients, a correlation between the extent of observed stenoses and the clinical course of the disease could not be demonstrated. The underlying cause of SIH is a spontaneous CSF leakage into the epidural space. Conservative treatment and the epidural blood patch remain the treatment of choice for this rare syndrome.

Summary: Recent clinical evidence indicates that IIH is probably a result of CSF outflow abnormality rather than of CSF production. Further research is needed to elucidate the causes of elevated intracranial pressure and the mechanism leading to visual loss. Prospective randomized clinical trials are needed to clarify a possible therapeutic potential of endovascular treatment. Research efforts on SIH should focus further on associated connective tissue disorders predisposing to CSF leaks.

Delayed intracranial hypertension after cranial vault remodeling for nonsyndromic single-suture synostosis

Authors: Cetas JS, Nasseri M, Saedi T, Kuang AA, Selden NR.

Object Delayed intracranial hypertension may occur after cranial vault remodeling for synostosis and may result in visual loss and developmental delay. Delayed intracranial hypertension is relatively common in children with syndromic, multisuture synostosis, but the incidence is poorly defined in children with single-suture nonsyndromic synostosis. This study evaluates the frequency of reoperation for delayed intracranial hypertension after single-suture synostosis repair. Methods Patients who had undergone cranial vault remodeling for nonsyndromic single-suture synostosis and were treated at a single tertiary pediatric hospital between July 2000 and December 2010 were analyzed for the occurrence of delayed intracranial hypertension and reoperation for cranial vault remodeling. Results Eighty-one patients with clinical follow-up of at least 3 years were analyzed from a total of 156 consecutive patients. The average patient age at the initial operation was 9.1 months. Five (6.2%) of 81 patients presented with delayed clinical and ophthalmological signs and symptoms of intracranial hypertension following initial cranial vault reconstruction, confirmed indirectly in each case by CT findings and directly by intracranial pressure monitoring. These 5 patients underwent repeat cranial vault reconstruction. Conclusions Calvarial growth restriction and intracranial hypertension occur sporadically following primary cranial vault reconstruction for single-suture nonsyndromic cranial synostosis. In this series, delayed intracranial hypertension occurred only in male patients who underwent primary repair of isolated sagittal synostoses at an age less than or equal to 5 months.

Long-term patency of venous sinus stents for idiopathic intracranial hypertension

Authors: Ducruet AF, Crowley RW, McDougall CG, Albuquerque FC.

BACKGROUND: Recent reports suggest that placement of a venous sinus stent improves symptoms in selected patients with idiopathic intracranial hypertension (IIH). We report our evaluation of the long-term patency of venous stents placed for IIH.
METHODS: We retrospectively reviewed our clinical database for 30 patients (mean age 33±10 years) with IIH who underwent placement of 36 venous sinus stents between October 2006 and December 2012. Relevant clinical, demographic and radiographic data were extracted after review of these records. All patients underwent retrograde venography to confirm a >50% stenosis and a trans-stenosis pressure gradient. Follow-up catheter angiography was performed beginning 3 months after the procedure.
RESULTS: Follow-up imaging was available for 23 (77%) of the 30 patients (mean 23 months). For seven patients, angiographic follow-up of >2 years (mean 45 months) was available. All stents remained patent with mild (<25%) in-stent stenosis observed in four patients. In five patients, however, we observed a narrowing of the sinus proximal to the stent. Although no patient underwent repeat stent placement for persistent or recurrent symptoms, cerebrospinal fluid diversion was performed in five cases.
CONCLUSIONS: Venous sinus stent placement has emerged as a promising treatment option for the subgroup of patients with IIH with a pressure gradient across a stenotic venous sinus. We observed long-term patency of all stents placed in this patient population. Further prospective investigation is necessary to improve our understanding of the phenomenon of sinus narrowing upstream of a patent stent and to establish definitively the long-term clinical efficacy of venous sinus stent placement for IIH.

Delayed Intracranial Hypertension and Cerebral Edema in Severe Pediatric Head Injury: Risk Factor Analysis

Authors: Bennett Colomer C, Solari Vergara F, Tapia Perez F, Miranda Vasquez F, Horlacher Kunstmann A, Parra Fierro G, Salazar Zenkovich C.

Introduction: Diffuse brain edema has been described as a major cause of intracranial hypertension (IH) following traumatic brain injury (TBI), and several studies suggest that it may be more frequent in children than in adults. While most cases of IH following TBI are present from the beginning, several studies have described a subgroup of patients with delayed elevations in intracranial pressure (ICP). Methods: Retrospective review of severe pediatric TBI cases admitted to a single institution during a 6-year period. Patients were classified into three groups, based on the temporal evolution of ICP: patients who evolved without IH, patients who had IH at admission and patients with delayed IH. A risk factor analysis was performed to find differences between these groups. Results: 31 cases of severe pediatric TBI were analyzed. 13 patients were female and 18 male, with an average age of 8.9 years. 4 patients met the described criteria for delayed IH; the only significant risk factor was presence of edema at the initial brain CT (p = 0.008). 3 additional patients presented clinical deterioration after 48 h and signs of brain edema in the CT, after ICP monitoring had been discontinued. Conclusions: Late-onset IH is a relatively common clinical condition in the pediatric population with severe TBI (present in 13% of the cases in our series), and the presence of a Marshall III CT scan at admission is a significant risk factor for this condition. Pediatric patients may benefit from a more prolonged period of ICP monitoring than adults, and the lack of amelioration of brain edema at follow-up brain CT (even with normal ICP values) may be an indication that more prolonged monitoring is needed.

Fever and bulging fontanelle mimicking meningitis in an infant diagnosed with benign intracranial hypertension

Authors: Goldberg EM.

A previously healthy 7-month-old male presented to the emergency department with fever and a bulging anterior fontanelle. A computed tomographic scan of the head suggested mild communicating hydrocephalus. Lumbar puncture was performed, which revealed a normal cerebrospinal fluid (CSF) cell count and glucose concentration, but a markedly elevated opening pressure. DNA polymerase chain reaction for herpes simplex virus performed on CSF was negative; CSF bacterial cultures were without growth. DNA polymerase chain reaction for human herpes virus 6 was strongly positive in serum. Fever and bulging fontanelle resolved within 24 hours. A presumptive diagnosis of transient intracranial hypertension of infancy was made, a form of benign idiopathic intracranial hypertension that mimics the presentation of serious intracranial pathology.

Idiopathic Intracranial Hypertension: Relation Between Obesity and Visual Outcomes

Authors: Szewka, Aimee J. MD; Bruce, Beau B. MD; Newman, Nancy J. MD; Biousse, Valérie MD

Background: Increased body mass index (BMI) has been associated with increased risk of idiopathic intracranial hypertension (IIH), but the relationship of BMI to visual outcomes in IIH is unclear.

Methods: A retrospective chart review of all adult cases of IIH satisfying the modified Dandy criteria seen at our institution between 1989 and 2010 was performed. Demographics, diagnostic evaluations, baseline visit and last follow-up examination data, treatment, and visual outcome data were collected in a standardized fashion. Groups were compared, and logistic regression was used to evaluate the relationship of BMI to severe visual loss, evaluating for interaction and controlling for potential confounders.

Results: Among 414 consecutive IIH patients, 158 had BMI ≥40 (World Health Organization Obese Class III) and 172 had BMI 30–39.9. Patients with BMI ≥40 were more likely to have severe papilledema at first neuro-ophthalmology encounter than those with a lower BMI (P = 0.02). There was a trend toward more severe visual loss in 1 or both eyes at last follow-up among those patients with BMI ≥40 (18% vs 11%, P = 0.067). Logistic regression modeling found that 10-unit (kilogram per square meter) increases in BMI increased the odds of severe visual loss by 1.4 times (95% confidence interval, 1.03–1.91, P = 0.03) after controlling for sex, race, diagnosed hypertension, and diagnosed sleep apnea.

Conclusion: Our finding of a trend for severe papilledema and visual loss associated with increasing BMI suggests that very obese IIH patients should be closely monitored for progression of visual field loss.

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