302 lines
18 KiB
Markdown
302 lines
18 KiB
Markdown
---
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title: "Extraventricular Obstructive Hydrocephalus"
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docid: "a0886d4c-f504-4165-bb52-2400e2385f68"
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authors:
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- key: "a25c450b-3d34-4f64-bba3-cc0834813df6"
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value: "Miral D. Jhaveri, MD, MBA"
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breadcrumbs:
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-
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name: "Brain"
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slug: "brain"
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treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a"
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-
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name: "Diagnosis"
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slug: "diagnosis"
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treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8"
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-
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name: "Anatomy-Based Diagnoses"
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slug: "anatomy-based-diagnoses"
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treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629"
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-
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name: "Ventricles and Cisterns"
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slug: "ventricles-and-cisterns"
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treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592"
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-
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name: "Hydrocephalus"
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slug: "hydrocephalus"
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treeNodeId: "9ce86e3b-fab6-4657-9e51-5f47bb1a51b5"
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-
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name: "Extraventricular Obstructive Hydrocephalus"
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slug: "extraventricular-obstructive-hydro-"
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treeNodeId: null
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category: "Brain"
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documentVersionId: "244d0107-0a80-48b5-96b6-e095c226c02a"
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imageCount: 9
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lastUpdated: "08/20/20"
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pageDescription: "Extraventricular Obstructive Hydrocephalus"
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pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus, Extraventricular Obstructive Hydrocephalus"
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pageTitle: "Extraventricular Obstructive Hydrocephalus | STATdx"
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enhancedTitle: "Extraventricular Obstructive Hydrocephalus"
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type: "DX"
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references: true
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breadcrumbs:
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- "Brain"
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- "Diagnosis"
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- "Anatomy-Based Diagnoses"
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- "Ventricles and Cisterns"
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- "Hydrocephalus"
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- "Extraventricular Obstructive Hydrocephalus"
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---
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# KEY FACTS
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- ## Terminology
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- Extraventricular obstructive hydrocephalus (EVOH): Enlarged ventricles due to obstruction located outside ventricular system
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- Synonym: "Communicating" hydrocephalus
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- ## Imaging
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- Impaired absorption of CSF distal to 4th ventricle outlet foramina
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- Ventricular size varies with duration of obstruction
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- All ventricles enlarged with no intraventricular obstructive cause
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- Lateral, 3rd, and 4th ventricles dilated
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- ± periventricular white matter interstitial edema
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- ± abnormal density/intensity of cisternal CSF ± leptomeningeal enhancement
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- ## Top Differential Diagnoses
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- Intraventricular obstructive hydrocephalus
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- Ventricular enlargement 2° to parenchymal loss
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- Normal-pressure hydrocephalus
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- ## Pathology
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- Hemorrhage → fibrosis/obstruction of subarachnoid space
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- Most common cause of EVOH
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- Other etiologies include suppurative meningitis, neoplastic or inflammatory exudates
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- Subarachnoid hemorrhage (SAH), exudates may fibrose/occlude subarachnoid space, reduce CSF pulsations
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- ## Clinical Issues
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- Headache, papilledema
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- Nausea, vomiting, diplopia (cranial nerve palsy)
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- ## Diagnostic Checklist
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- EVOH: Generalized ventricular enlargement with abnormal density/intensity in basal cisterns ± leptomeningeal enhancement
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# TERMINOLOGY
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- ## Abbreviations
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- Extraventricular obstructive hydrocephalus (EVOH)
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- ## Synonyms
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- "Communicating" hydrocephalus
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- ## Definitions
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- Enlarged ventricles due to obstruction located outside ventricular system
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# IMAGING
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- ## General Features
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- ### Best diagnostic clue
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- Lateral, 3rd, and 4th ventricles all dilated
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- ± abnormal density/intensity of cisternal CSF ± leptomeningeal enhancement
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- ### Location
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- Obstruction distal to 4th ventricle outlet foramina
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- ### Size
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- Bifrontal horn to intracranial diameter ratio > 0.3
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- Temporal horn width > 3 mm
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- ### Morphology
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- All ventricles enlarged
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- Generally proportionate, symmetrical increase
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- No intraventricular obstructive cause
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- ## CT Findings
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- ### NECT
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- Variable ventricular dilatation ± basal cisterns effaced
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- If subarachnoid hemorrhage (SAH), look for hyperdense CSF
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- ### CECT
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- Look for sulcal/cisternal enhancement
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- ## MR Findings
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- ### T1WI
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- "Dirty" CSF, ventricular dilatation
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- ### T2WI
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- Dilated ventricles ± periventricular white matter interstitial edema
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- Effacement of cortical sulci
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- Hypointense CSF-SAH, exudates
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- ### FLAIR
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- ± periventricular white matter interstitial edema better delineated than T2
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- ### T1WI C+
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- ± enhancement of basal cisterns/sulci
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- Meningitis, carcinomatosis, etc.
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- 3D CISS/FIESTA
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- Exquisitely delineates CSF spaces and helps to exclude intraventricular obstruction
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- ## Imaging Recommendations
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- ### Best imaging tool
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- MR with T1WI C+
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- 3D CISS/FIESTA
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- ## Other Modality Findings
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- Isotope cisternography may show ventricular reflux, stasis (EVOH)
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# DIFFERENTIAL DIAGNOSIS
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- ## Intraventricular Obstructive Hydrocephalus
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- Global/focal enlarged ventricles due to obstruction proximal to 4th ventricle outflow
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- ## Ventricular Enlargement Secondary to Parenchymal Loss
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- Neurodegenerative disease, cerebritis, hypoxia/ischemia
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- Diffuse/focal enlargement of sulci, cisterns
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- [Normal-Pressure Hydrocephalus](/document/normal-pressure-hydrocephalus/ba3f857d-58de-4f21-8463-1631b4cb9972)
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- Ventricular enlargement with normal CSF pressure
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- Sulci normal/minimally enlarged
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- Progressive dementia, gait disturbance, incontinence
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# PATHOLOGY
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- ## General Features
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- ### Etiology
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- Obstruction to CSF flow at level of basal cisterns or arachnoid villi
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- Also reduced CSF pulsations reduce venous resorption of CSF
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- SAH: Most common cause of EVOH
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- Other etiologies include suppurative meningitis, neoplastic inflammatory exudates
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- All lead to subarachnoid scarring, reduced CSF pulsations
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- ## Gross Pathologic & Surgical Features
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- Generalized ventricular dilatation
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- SAH, exudates in basal cisterns & convexity sulci
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- Meningeal fibrosis, arachnoid adhesions
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# CLINICAL ISSUES
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- ## Presentation
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- ### Most common signs/symptoms
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- Headache, papilledema
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- Nausea, vomiting, diplopia (cranial nerve palsy)
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- ## Natural History & Prognosis
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- Usually progressive unless shunted and primary cause treated
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- ## Treatment
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- CSF diversion (shunt); directed to primary cause
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# DIAGNOSTIC CHECKLIST
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- ## Consider
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- EVOH: Generalized ventricular enlargement with abnormal density/intensity in basal cisterns ± leptomeningeal enhancement
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94b03593-3810-49dd-a698-ad9f46f7ba1f
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## References
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# Selected References
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1. [Capone PM et al: Neuroimaging of normal pressure hydrocephalus and hydrocephalus. Neurol Clin. 38(1):171-83, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31761057%5Bpmid%5D)
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1. [Farb R et al: Hydrocephalus and CSF disorders. Diseases of the brain, head and neck, spine 2020-3: Diagnostic Imaging. Cham (CH): Springer; 11-24, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32119247%5Bpmid%5D)
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1. [Fowler JB et al: Ventriculoperitoneal shunt. Treasure Island (FL): StatPearls Publishing, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=29083724%5Bpmid%5D)
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1. [Roth J et al: The added value of magnetic resonance imaging cisternography and ventriculography as a diagnostic aid in pediatric Hydrocephalus. Pediatr Neurosurg. 54(3):165-72, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30870836%5Bpmid%5D)
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1. [Fink KR et al: Imaging of nontraumatic neuroradiology emergencies. Radiol Clin North Am. 53(4):871-90, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26046515%5Bpmid%5D)
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1. [Flannery AM et al: Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 1: introduction and methodology. J Neurosurg Pediatr. 14 Suppl 1:3-7, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25988777%5Bpmid%5D)
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1. [Grunwald IQ et al: Aneurysmal SAH: current management and complications associated with treatment and disease. J Invasive Cardiol. 26(1):30-7, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24402809%5Bpmid%5D)
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1. [Krähenbühl AK et al: Endoscopic temporal ventriculocisternostomy: an option for the treatment of trapped temporal horns. J Neurosurg Pediatr. 11(5):568-74, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23521153%5Bpmid%5D)
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1. [McAllister JP 2nd: Pathophysiology of congenital and neonatal hydrocephalus. Semin Fetal Neonatal Med. 17(5):285-94, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22800608%5Bpmid%5D)
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1. [Feng F et al: Evaluation of radionuclide cerebrospinal fluid scintigraphy as a guide in the management of patients with hydrocephalus. Clin Imaging. 33(2):85-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19237049%5Bpmid%5D)
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1. [Yamada S et al: Visualization of cerebrospinal fluid movement with spin labeling at MR imaging: preliminary results in normal and pathophysiologic conditions. Radiology. 249(2):644-52, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18936318%5Bpmid%5D)
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1. [Greitz D: Paradigm shift in hydrocephalus research in legacy of Dandy's pioneering work: rationale for third ventriculostomy in communicating hydrocephalus. Childs Nerv Syst. 23(5):487-9, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=-1%5Bpmid%5D)
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1. [ter Laan M et al: Improvement after treatment of hydrocephalus in aneurysmal subarachnoid haemorrhage: implications for grading and prognosis. Acta Neurochir (Wien). 148(3):325-8; discussion 328, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16328775%5Bpmid%5D)
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1. [Greitz D: Radiological assessment of hydrocephalus: new theories and implications for therapy. Neurosurg Rev. 27(3):145-65; discussion 166-7, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15164255%5Bpmid%5D)
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1. [Kehler U et al: Extraventricular intracisternal obstructive hydrocephalus--a hypothesis to explain successful 3rd ventriculostomy in communicating hydrocephalus. Pediatr Neurosurg. 38(2):98-101, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12566844%5Bpmid%5D)
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1. [Biedert S et al: [Extraventricular obstructive hydrocephalus.] Fortschr Neurol Psychiatr. 62(11):405-8, 1994](http://www.ncbi.nlm.nih.gov/pubmed/?term=7829027%5Bpmid%5D)
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## Images
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### Selected Images
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*Axial NECT shows acute subarachnoid hemorrhage <img src='img/arrows/CC.png'/> in the basal cisterns and early extraventricular obstructive hydrocephalus with dilatation of all the ventricles and subtle periventricular hypodensity <img src='img/arrows/CO.png'/> due to interstitial edema.*
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*Axial NECT shows acute subarachnoid hemorrhage <img src='img/arrows/CC.png'/> in the basal cisterns and early extraventricular obstructive hydrocephalus with dilatation of all the ventricles and subtle periventricular hypodensity <img src='img/arrows/CO.png'/> due to interstitial edema.*
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*Axial FLAIR MR images (top) in a patient with breast carcinoma leptomeningeal metastasis shows dilatation of the ventricles <img src='img/arrows/CS.png'/> with mild periventricular interstitial edema <img src='img/arrows/CO.png'/>. Axial T1 C+ MR (bottom) shows extensive leptomeningeal enhancement <img src='img/arrows/CC.png'/> along the cerebellar folia.*
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*Axial T2 MR in a 21-year-old patient with a remote history of meningitis shows chronic "compensated" extraventricular communicating hydrocephalus with marked dilatation of the lateral <img src='img/arrows/CC.png'/> and 3rd ventricles <img src='img/arrows/CS.png'/>.*
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*Sagittal T1 MR in the same patient shows a patent widened cerebral aqueduct <img src='img/arrows/CC.png'/> and foramen of Magendie <img src='img/arrows/CS.png'/> with dilatation of the 4th ventricle <img src='img/arrows/CO.png'/>. In longstanding "compensated" hydrocephalus, there is no periventricular interstitial edema around the ventricles, as in this case.*
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### Additional Images
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*Axial T1WI C+ MR demonstrates subtle leptomeningeal enhancement in the left sylvian fissure <img src='img/arrows/CO.png'/> in this patient with tuberculous meningitis. There is mild dilatation of the lateral ventricles <img src='img/arrows/CS.png'/> due to extraventricular obstructive hydrocephalus.*
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*Coronal T1WI MR in a toddler with rapid head growth for 4 months shows enlarged ventricular trigone on the left and enlarging subarachnoid spaces at an age when they should be shrinking. MR venography showed occlusion of both transverse sinuses.*
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*Axial NECT shows hyperdense material in the basal cisterns <img src='img/arrows/BS.png'/> and sylvian fissures <img src='img/arrows/BO.png'/> in acute subarachnoid hemorrhage. There is early dilatation of the ventricles <img src='img/arrows/CO.png'/> with mild periventricular edema <img src='img/arrows/CS.png'/> due to interstitial edema.*
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*Axial NECT shows acute subarachnoid hemorrhage in the basal cisterns <img src='img/arrows/CC.png'/> and sylvian fissures <img src='img/arrows/CS.png'/>. There is early extraventricular obstructive hydrocephalus with mild periventricular hypodensity <img src='img/arrows/CO.png'/> due to interstitial edema.*
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*Axial T1WI C+ MR shows extensive leptomeningeal enhancement of the basal cisterns in neurosarcoidosis <img src='img/arrows/CS.png'/>. Notice the early communicating hydrocephalus with the dilated 3rd ventricle <img src='img/arrows/CC.png'/> and temporal horns <img src='img/arrows/CO.png'/>.*
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