Hydrocephalus after aneurysmal subarachnoid hemorrhage

Hydrocephalus after aneurysmal subarachnoid hemorrhage

Epidemiology

Hydrocephalus complicates the clinical course of greater than 20% of patients with aneurysmal subarachnoid hemorrhage 1) 2) , and its onset can be acute, within 48 hours after SAH, or rarely chronic, occurring in a delayed fashion weeks and even months after the hemorrhage.

Etiology

The etiology of hydrocephalus following aSAH has yet to be fully elucidated, but is likely to include the following: obstruction of CSF flow within the basal cisterns and/or ventricles by clotted blood, diminished absorption at the arachnoid granulations, and inflammation 3) 4) 5) 6).

Na et al. found that higher sodium, lower potassium, and higher glucose levels were predictive values for shunt-dependent hydrocephalus from postoperative day (POD) 1 to POD 12-16 after subarachnoid hemorrhage. Strict correction of electrolyte imbalance seems necessary to reduce shunt-dependent hydrocephalus. Further large studies are warranted to confirm the findings 7).

Data suggest that the volume of the third ventricle in the initial CT is a strong predictor for shunt dependency after aSAH 8).

Diagnosis

Early recognition of its signs and symptoms and accurate interpretation of computed tomography (CT) studies are important for the management of patients with SAH. Clinically, a poor neurologic grade has the highest correlation with an increased incidence of hydrocephalus. Radiographically, the bicaudate index on CT studies has emerged as the best marker of this condition. Although further studies are needed to understand the complex pathophysiology of this condition, hydrocephalus after SAH can be treated effectively using current technology 9).


Most readmissions after aneurysmal subarachnoid hemorrhage (SAH) relate to late consequences of hemorrhage, such as hydrocephalus, or medical complications secondary to severe neurological injury. Although a minority of readmissions may potentially be avoided with closer medical follow-up in the transitional care environment, readmission after SAH is an insensitive and likely inappropriate hospital performance metric 10).

Data demonstrate that gender influences acute hydrocephalus development in a rat SAH model. Future studies should determine the role of estrogen in SAH-induced hydrocephalus 11).

Hydrocephalus might cause gradual obtundation in the first few hours or days; it can be treated by lumbar puncture or ventricular drainage, dependent on the site of obstruction

Aneurysmal subarachnoid hemorrhage (SAH) has been reported to induce an intrathecal inflammatory reaction reflected by cytokine release, particularly interleukin-6 (IL-6), which correlates with early brain damage and poor outcome.

Treatment

Hydrocephalus might cause gradual obtundation in the first few hours or days; it can be treated by lumbar puncture or ventricular drainage, dependent on the site of obstruction 12).

Outcome

Hydrocephalus is a common and potentially devastating complication of aneurysmal subarachnoid hemorrhage (SAH).

Hydrocephalus leads to prolonged hospital and ICU stays, well as to repeated surgical interventions, readmissions, and complications associated with ventriculoperitoneal shunts, including shunt failure and shunt infection. Whether variations in surgical technique at the time of aneurysm treatment may modify rates of shunt dependency remains a matter of debate 13).

Shunt dependency

The indication for and the timing of a permanent shunt operation in patients following acute hydrocephalus (HC) after subarachnoid hemorrhage (SAH) remains controversial because risk factors for chronic HC fail to predict permanent shunt dependency. The amount of cerebrospinal fluid (CSF) drained via an external ventricular drain (EVD) may predict shunt dependency.

Results suggest that the daily amount of external CSF drainage volume in the acute state of SAH might influence the development of HC 14).


CSF IL-6 values of ≥10,000 pg/ml in the early post-SAH period may be a useful diagnostic tool for predicting shunt dependency in patients with acute posthemorrhagic hydrocephalus. The development of shunt-dependent posthemorrhagic hydrocephalus remains a multifactorial process 15).

Reliable prognostic tools to estimate the case fatality rate (CFR) and the development of chronic hydrocephalus (CHC) in aneurysmal subarachnoid hemorrhage (SAH) are not well defined.

Graeb Score or LeRoux score improve the prediction of shunt dependency and in parts of CFR in aneurysmal SAH patients therefore confirming the relevance of the extent and distribution of intraventricular blood for the clinical course in SAH 16).

Case series

One-hundred and fifty-two patients who had undergone an operation for SAH were enrolled in this study. Clinical data, radiological data, and procedural data were investigated. Procedural data included the operating technique (clipping vs. EVT) and the use of additional procedures (no procedure, lumbar drainage, or EVD). Delayed hydrocephalus was defined as a condition in which the Evan’s index was 0.3 or higher, as assessed using brain computed tomography more than 2 weeks after surgery, requiring shunt placement due to neurological deterioration.

Of the 152 patients, 45 (29.6%) underwent surgical clipping and 107 (70.4%) underwent EVT. Twenty-five (16.4%) patients developed delayed hydrocephalus. Age (p = 0.019), procedure duration (p = 0.004), and acute hydrocephalus (p = 0.030) were significantly correlated with the incidence of delayed hydrocephalus. However, the operation technique (p = 0.593) and use of an additional procedure (p = 0.378) were not significantly correlated with delayed hydrocephalus incidence.

No significant difference in the incidence of delayed hydrocephalus was associated with operation technique or use of an additional procedure in patients with SAH. However, delayed hydrocephalus was significantly correlated with old age, long procedural duration, and acute hydrocephalus. Therefore, they recommend that additional procedures should be discontinued as soon as possible 17).

2017

Winkler et al. conducted a retrospective review of 663 consecutive patients with aSAH treated from 2005 to 2015 by open microsurgery via a pterional or orbitozygomatic craniotomy by the senior author (M.T.L.). Data collected from review of the electronic medical record included age, Hunt and Hess grade, Fisher grade, need for an external ventricular drain, and opening pressure. Patients were stratified into those undergoing no fenestration and those undergoing tandem fenestration of the LT and MoL at the time of surgical repair. Outcome variables, including VP shunt placement and timing of shunt placement, were recorded and statistically analyzed. RESULTS In total, shunt-dependent hydrocephalus was observed in 15.8% of patients undergoing open surgical repair following aSAH. Tandem microsurgical fenestration of the LT and MoL was associated with a statistically significant reduction in shunt dependency (17.9% vs 3.2%, p < 0.01). This effect was confirmed with multivariate analysis of collected variables (multivariate OR 0.09, 95% CI 0.03-0.30). Number-needed-to-treat analysis demonstrated that tandem fenestration was required in approximately 6.8 patients to prevent a single VP shunt placement. A statistically significant prolongation in days to VP shunt surgery was also observed in patients treated with tandem fenestration (26.6 ± 19.4 days vs 54.0 ± 36.5 days, p < 0.05). CONCLUSIONS Tandem fenestration of the LT and MoL at the time of open microsurgical clipping and/or bypass to secure ruptured anterior and posterior circulation aneurysms is associated with reductions in shunt-dependent hydrocephalus following aSAH. Future prospective randomized multicenter studies are needed to confirm this result 18).


181 participants with a mean age of 54.4 years. Higher sodium (hazard ratio, 1.53; 95% confidence interval, 1.13-2.07; p = 0.005), lower potassium, and higher glucose levels were associated with higher shunt-dependent hydrocephalus. The receiver operating characteristic curve analysis showed that the areas under the curve of sodium, potassium, and glucose were 0.649 (cutoff value, 142.75 mEq/L), 0.609 (cutoff value, 3.04 mmol/L), and 0.664 (cutoff value, 140.51 mg/dL), respectively.

Despite the exploratory nature of this study, we found that higher sodium, lower potassium, and higher glucose levels were predictive values for shunt-dependent hydrocephalus from postoperative day (POD) 1 to POD 12-16 after subarachnoid hemorrhage. Strict correction of electrolyte imbalance seems necessary to reduce shunt-dependent hydrocephalus. Further large studies are warranted to confirm our findings 19).

2016

The study is designed to determine the efficacy of lamina terminalis fenestration on the reduction of SDH after aneurysm clipping.

METHODS/DESIGN: A total of 288 patients who meet the inclusion criteria will be randomized into single aneurysm clipping or aneurysm clipping plus FLT in the Department of Neurosurgery, West China Hospital. Follow-up was performed 1, 3, 6, and 12 months after aneurysm clipping. The primary outcome is the incidence of SDH and the secondary outcomes include cerebral vasospasm, functional outcome evaluated by the modified Rankin Scale and Extended Glasgow Outcome Scale, and mortality.

DISCUSSION: The FISH trial is a large randomized, parallel controlled clinical trial to define the therapeutic value of FLT, the results of which will help to guide the surgical procedure and resolve the long-puzzled debate in the neurosurgical community.

CONCLUSIONS: This protocol will determine the efficacy of FLT in the setting of aneurysmal subarachnoid hemorrhage 20).

2003

Seven hundred eighteen patients with aneurysmal subarachnoid hemorrhage who were treated between 1990 and 1999 were retrospectively studied, to identify factors contributing to shunt-dependent hydrocephalus. With these data, a stepwise logistic regression procedure was used to determine the effect of each variable on the development of hydrocephalus and to create a scoring system.

Overall, 152 of the 718 patients (21.2%) underwent shunting procedures for treatment of hydrocephalus. Four hundred seventy-nine of the patients (66.7%) were female. Of the factors investigated, the following were associated with shunt-dependent hydrocephalus, as determined with a variety of statistical methods: 1) increasing age (P < 0.001), 2) female sex (P = 0.015), 3) poor admission Hunt and Hess grade (P < 0.001), 4) thick subarachnoid hemorrhage on admission computed tomographic scans (P < 0.001), 5) intraventricular hemorrhage (P < 0.001), 6) radiological hydrocephalus at the time of admission (P < 0.001), 7) distal posterior circulation location of the ruptured aneurysm (P = 0.046), 8) clinical vasospasm (P < 0.001), and 9) endovascular treatment (P = 0.013). The presence of intracerebral hematomas, giant aneurysms, or multiple aneurysms did not influence the development of shunt-dependent hydrocephalus.

The results of this study can help identify patients with a high risk of developing shunt-dependent hydrocephalus. This may help neurosurgeons expedite treatment, may decrease the cost and length of hospital stays, and may result in improved outcomes 21).

2000

In 138 patients with ruptured cerebral aneurysms operated on within 48 to 72 hours after subarachnoid hemorrhage, an external ventricular drainage catheter was inserted before craniotomy and was used intermittently during the first week after surgery. In 51 patients, intracranial pressure (ICP) was measured intraoperatively. The majority of patients showed increased ICP intraoperatively irrespective of the preoperative Hunt and Hess grade and the amount of subarachnoid blood accumulation or intraventricular blood clot. Intraoperative drainage of cerebrospinal fluid allowed easy access for aneurysm dissection by making the brain slack in more than 90% of patients. Postoperative ICP measurements revealed that significant brain swelling did not occur in the majority of patients. In 7 patients, persistently elevated ICP (greater than 20 mm Hg) was recorded. Nine patients (8%) developed shunt-dependent hydrocephalus; all of these patients had suffered an intraventricular hemorrhage. Measurements of the volumes of cerebrospinal fluid drained did not allow prediction of shunt-dependent hydrocephalus 22).

1987

The incidence and clinical aspects of acute hydrocephalus were examined in 200 patients with recently ruptured intracranial aneurysms. The following conclusions were reached: Acute hydrocephalus is an important complication of aneurysmal subarachnoid hemorrhage that occurs in approximately 20% of all cases and exhibits an incidence that tends to parallel clinical grade (Grade I, 3%; Grade II, 5%; “Good” Grade III, 21%; “Bad”Grade III, 40%; Grade IV, 42%; Grade V, 26%). Impaired consciousness leading to a general downgrading of clinical status was the predominant clinical finding (93%), but neither this nor other nonspecific signs of increased intracranial pressure were distinguishable from the effects of the precipitating hemorrhage. The computed tomographic signs of acute hydrocephalus are distinctive and consist of selective ballooning of the frontal horns, rostral-caudal enlargement of the cerebral ventricles, and a halo of periventricular hyperdensity (edema) that evolves in sequence with ventricular changes. The treatment of choice is external ventricular drainage, which results in prompt and often dramatic improvement in approximately two-thirds of the patients 23).

1985

Hydrocephalus, defined as a bicaudate index above the 95th percentile for age, was found in 34 (20%) of 174 prospectively studied patients with subarachnoid hemorrhage (SAH) who survived the first 24 hours and who underwent computerized tomography (CT) scanning within 72 hours. The occurrence of acute hydrocephalus was related to the presence of intraventricular blood, and not to the extent of cisternal hemorrhage. The level of consciousness was depressed in 30 of the 34 patients. Characteristic clinical features were present in 19 patients, including a gradual obtundation after the initial hemorrhage in 16 patients and small nonreactive pupils in nine patients (all with a Glasgow Coma Scale score of 7 or less). In the remaining 15 patients (44%), the diagnosis could be made only by CT scanning. After 1 month, 20 of the 34 patients had died: six from rebleeding (four after shunting), 11 from cerebral infarction (eight after an initial improvement), and three from other or mixed causes. Only one of nine patients in whom a shunt was placed survived, despite rapid improvement in all immediately after shunting. The mortality rate among patients with acute hydrocephalus was significantly higher than in those without, with the higher incidence caused by cerebral infarction (11 of 34 versus 12 of 140 cases, respectively; p less than 0.001). Death from infarction could not be attributed to the extent of cisternal hemorrhage, the use of antifibrinolytic drugs, or failure to apply surgical drainage, but could often be explained by the development of hyponatremia, probably accompanied by hypovolemia 24).

1984

Seventeen patients suffering from SAH and/or intraventricular hemorrhage were studied; all were admitted in Grades II to V according to Hunt and Hess. Eleven patients had a proven aneurysm. The ICP, monitored via an intraventricular catheter, was above 15 mm Hg (2 kPa) during part of the monitoring period in all patients. B-waves at 1/min were noted in all patients. Resistance to outflow of CSF was determined by the following techniques: 1) bolus injection; 2) constant-rate steady-state infusion; or 3) controlled withdrawal (“inverse infusion”). Resistance to outflow of CSF was increased in all patients, ranging from 11.5 to 85 mm Hg/ml/min. The ICP was linearly correlated with outflow resistance. Four (50%) of the eight survivors required a shunt. Neither the presence of hydrocephalus on admission, nor the level of ICP, nor the magnitude of resistance to outflow of CSF was clearly related to the requirement of a permanent CSF diversion 25).

References

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Wilson CD, Safavi-Abbasi S, Sun H, Kalani MY, Zhao YD, Levitt MR, et al: Meta-analysis and systematic review of risk factors for shunt dependency after aneurysmal subarachnoid hemorrhage. J Neurosurg 126:586–595, 2017
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Yamada S, Nakase H, Park YS, Nishimura F, Nakagawa I: Discriminant analysis prediction of the need for ventriculo- peritoneal shunt after subarachnoid hemorrhage. J Stroke Cerebrovasc Dis 21:493–497, 2012
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Auer LM, Mokry M. Disturbed cerebrospinal fluid circulation after subarachnoid hemorrhage and acute aneurysm surgery. Neurosurgery. 1990 May;26(5):804-8; discussion 808-9. PubMed PMID: 2352599.
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Dorai Z, Hynan LS, Kopitnik TA, Samson D: Factors related to hydrocephalus after aneurysmal subarachnoid hemorrhage. Neurosurgery 52:763–771, 2003
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Massicotte EM, Del Bigio MR. Human arachnoid villi response to subarachnoid hemorrhage: possible relationship to chronic hydrocephalus. J Neurosurg. 1999 Jul;91(1):80-4. PubMed PMID: 10389884.
6) , 24)

van Gijn J, Hijdra A, Wijdicks EF, Vermeulen M, van Crevel H. Acute hydrocephalus after aneurysmal subarachnoid hemorrhage. J Neurosurg. 1985 Sep;63(3):355-62. PubMed PMID: 4020461.
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Na MK, Won YD, Kim CH, Kim JM, Cheong JH, Ryu JI, Han MH. Early variations of laboratory parameters predicting shunt-dependent hydrocephalus after subarachnoid hemorrhage. PLoS One. 2017 Dec 12;12(12):e0189499. doi: 10.1371/journal.pone.0189499. eCollection 2017. PubMed PMID: 29232410; PubMed Central PMCID: PMC5726740.
8)

Pinggera D, Kerschbaumer J, Petr O, Ortler M, Thomé C, Freyschlag CF. The Volume of the Third Ventricle as a Prognostic Marker for Shunt Dependency After Aneurysmal Subarachnoid Hemorrhage. World Neurosurg. 2017 Dec;108:107-111. doi: 10.1016/j.wneu.2017.08.129. Epub 2017 Sep 1. PubMed PMID: 28867328.
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Germanwala AV, Huang J, Tamargo RJ. Hydrocephalus after aneurysmal subarachnoid hemorrhage. Neurosurg Clin N Am. 2010 Apr;21(2):263-70. doi: 10.1016/j.nec.2009.10.013. Review. PubMed PMID: 20380968.
10)

Greenberg JK, Washington CW, Guniganti R, Dacey RG Jr, Derdeyn CP, Zipfel GJ. Causes of 30-day readmission after aneurysmal subarachnoid hemorrhage. J Neurosurg. 2016 Mar;124(3):743-9. doi: 10.3171/2015.2.JNS142771. Epub 2015 Sep 11. PubMed PMID: 26361278.
11)

Shishido H, Zhang H, Okubo S, Hua Y, Keep RF, Xi G. The Effect of Gender on Acute Hydrocephalus after Experimental Subarachnoid Hemorrhage. Acta Neurochir Suppl. 2016;121:335-9. doi: 10.1007/978-3-319-18497-5_58. PubMed PMID: 26463971.
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van Gijn J, Kerr RS, Rinkel GJ. Subarachnoid haemorrhage. Lancet. 2007 Jan 27;369(9558):306-18. Review. PubMed PMID: 17258671.
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Winkler EA, Burkhardt JK, Rutledge WC, Rick JW, Partow CP, Yue JK, Birk H, Bach AM, Raygor KP, Lawton MT. Reduction of shunt dependency rates following aneurysmal subarachnoid hemorrhage by tandem fenestration of the lamina terminalis and membrane of Liliequist during microsurgical aneurysm repair. J Neurosurg. 2017 Dec 15:1-7. doi: 10.3171/2017.5.JNS163271. [Epub ahead of print] PubMed PMID: 29243978.
14)

Hayek MA, Roth C, Kaestner S, Deinsberger W. Impact of External Ventricular Drainage Volumes on Shunt Dependency after Subarachnoid Hemorrhage. J Neurol Surg A Cent Eur Neurosurg. 2016 Jul 22. [Epub ahead of print] PubMed PMID: 27448196.
15)

Wostrack M, Reeb T, Martin J, Kehl V, Shiban E, Preuss A, Ringel F, Meyer B, Ryang YM. Shunt-Dependent Hydrocephalus After Aneurysmal Subarachnoid Hemorrhage: The Role of Intrathecal Interleukin-6. Neurocrit Care. 2014 May 20. [Epub ahead of print] PubMed PMID: 24840896.
16)

Czorlich P, Ricklefs F, Reitz M, Vettorazzi E, Abboud T, Regelsberger J, Westphal M, Schmidt NO. Impact of intraventricular hemorrhage measured by Graeb and LeRoux score on case fatality risk and chronic hydrocephalus in aneurysmal subarachnoid hemorrhage. Acta Neurochir (Wien). 2015 Jan 21. [Epub ahead of print] PubMed PMID: 25599911.
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Eom TO, Park ES, Park JB, Kwon SC, Sim HB, Lyo IU, Kim MS. Does Neurosurgical Clipping or Endovascular Coiling Lead to More Cases of Delayed Hydrocephalus in Patients with Subarachnoid Hemorrhage? J Cerebrovasc Endovasc Neurosurg. 2018 Jun;20(2):87-95. doi: 10.7461/jcen.2018.20.2.87. Epub 2018 Jun 30. PubMed PMID: 30370242; PubMed Central PMCID: PMC6196142.
19)

Na MK, Won YD, Kim CH, Kim JM, Cheong JH, Ryu JI, Han MH. Early variations of laboratory parameters predicting shunt-dependent hydrocephalus after subarachnoid hemorrhage. PLoS One. 2017 Dec 12;12(12):e0189499. doi: 10.1371/journal.pone.0189499. eCollection 2017. PubMed PMID: 29232410.
20)

Tao C, Fan C, Hu X, Ma J, Ma L, Li H, Liu Y, Sun H, He M, You C. The effect of fenestration of the lamina terminalis on the incidence of shunt-dependent hydrocephalus after aneurysmal subarachnoid hemorrhage (FISH): Study protocol for a randomized controlled trial. Medicine (Baltimore). 2016 Dec;95(52):e5727. doi: 10.1097/MD.0000000000005727. PubMed PMID: 28033279; PubMed Central PMCID: PMC5207575.
21)

Dorai Z, Hynan LS, Kopitnik TA, Samson D. Factors related to hydrocephalus after aneurysmal subarachnoid hemorrhage. Neurosurgery. 2003 Apr;52(4):763-9; discussion 769-71. PubMed PMID: 12657171.
23)

Milhorat TH. Acute hydrocephalus after aneurysmal subarachnoid hemorrhage. Neurosurgery. 1987 Jan;20(1):15-20. PubMed PMID: 3808257.
25)

Kosteljanetz M. CSF dynamics in patients with subarachnoid and/or intraventricular hemorrhage. J Neurosurg. 1984 May;60(5):940-6. PubMed PMID: 6716162.

Hydrocephalus Classification

Hydrocephalus Classification

There is no international consensus on the classification of hydrocephalus, and there are various systems based on the age of onset, cerebrospinal fluid dynamics and anatomical area of accumulation, the levels of cerebrospinal fluid pressure and the presence of symptoms.

However, no definitive classification exists comprehensively to cover the variety of these aspects.

Wu et al. proposed a classification Based on Ventricular Pressure 1).


Walter Edward Dandy first described the basic mechanism and classification of hydrocephalus as:

Obstructive hydrocephalus or Non Obstructive hydrocephalus.

Despite advances in understanding of the underlying process, current classification systems still rely upon Dandy’s classification scheme 2).


The aim of a study was to evaluate the diagnostic utility of three-dimensional sampling perfection with application optimized contrast using different flip angle evolution (3D SPACE) sequence and Susceptibility Weighted Imaging (SWI) in hydrocephalus and to propose a refined definition and classification of hydrocephalus with relevance to the selection of treatment option.

A prospective study of 109 patients with hydrocephalus was performed with magnetic resonance imaging (MRI) brain using standardized institutional sequences along with additional sequences 3D SPACE and SWI. The images were independently read by two senior neuroradiologists and the etiopathogenesis of hydrocephalus was arrived by consensus.

With conventional sequences, 46 out of 109 patients of hydrocephalus were diagnosed as obstructive of which 21 patients showed direct signs of obstruction and 25 showed indirect signs. In the remaining 63 patients of communicating hydrocephalus, cause could not be found out in 41 patients. Whereas with 3D SPACE sequence, 88 patients were diagnosed as obstructive hydrocephalus in which all of them showed direct signs of obstruction and 21 patients were diagnosed as communicating hydrocephalus. By including SWI, we found out hemorrhage causing intraventricular obstruction in three patients and hemorrhage at various sites in 24 other patients. With these findings, we have classified the hydrocephalus into communicating and noncommunicating, with latter divided into intraventricular and extraventricular obstruction, which is very well pertaining to the selection of surgical option.

Chellathurai et al., strongly suggest to include 3D SPACE and SWI sequences in the set of routine MRI sequences, as they are powerful diagnostic tools and offer complementary information regarding the precise evaluation of the etiopathogenesis of hydrocephalus and have an effective impact in selecting the mode of management 3).

Terms used

Acquired hydrocephalus

Adult hydrocephalus

Arrested hydrocephalus or Compensated hydrocephalus

Chronic hydrocephalus

Communicating hydrocephalus or Non obstructive hydrocephalus

Congenital hydrocephalus

External hydrocephalus

Focal hydrocephalus

Hydrocephalus Ex Vacuo

Idiopathic normal pressure hydrocephalus

Infantile hydrocephalus or Pediatric hydrocephalus.

Internal hydrocephalus

Non obstructive hydrocephalus or Communicating hydrocephalus

Normal pressure hydrocephalus for Idiopathic normal pressure hydrocephalus or Secondary normal pressure hydrocephalus.

Obstructive hydrocephalus.

Pediatric hydrocephalus or Infantile hydrocephalus

Secondary normal pressure hydrocephalus.

Unilateral hydrocephalus.


With the rare exception of hydrocephalus associated with overproduction of CSF in patients with choroid plexus papillomas (CPPs), all hydrocephalus is basically obstructive hydrocephalus. That the rare CPP causes hydrocephalus is not debated, but why it does so is the subject of some discussion. CPPs are known to lead to increases in the rate of CSF production and are known to cause hydrocephalus.

Normal CSF absorptive mechanisms can clear the amount of spinal fluid produced in the ventricular system at extremely high rates without producing ventriculomegaly. If CSF production and ICP increase substantially, ventricular size increases 4). When CSF flow is obstructed in the context of increased CSF production, there is a great tendency for ventriculomegaly or hydrocephalus to develop. CPPs, in themselves, can create the only pure form of communicating hydrocephalus. However, that these tumors tend to be large and to restrict CSF flow through the foramen of Monro or aqueduct of Sylvius, is more likely to account for the severity of hydrocephalus in this context 5).

When hydrocephalus is severe, especially in the very young, it may not be possible to define the point of CSF obstruction without introducing tracers into the CSF pathways. In patients treated early in life whose ventricles have become smaller with treatment, it is possible to determine the first site of obstruction to CSF flow on MRI or CT.

Patients with complex congenital anomalies such as hydrocephalus related to a Chiari II malformation and spina bifida often have multiple sites of obstruction 6) 7). It may not be possible to predict a second or downstream point of obstruction. In these patients, only one point may be obstructed or all of these sites may be restricted.

Based on a analyses from a mathematical modeling, of the work on the circuitry of CSF flow, and these potential sites of obstruction, Rekate et al., proposed a classification

It is generally assumed that endoscopic third ventriculostomy (ETV) is only effective for treating obstructive hydrocephalus, and many assume that obstructive hydrocephalus is synonymous with aqueductal stenosis. The growing number of reports on the efficacy of ETV for treating “communicating hydrocephalus” has generated considerable consternation 8).


The “Multi-categorical Hydrocephalus Classification” (Mc HC), was invented and developed to cover the entire aspects of hydrocephalus with all considerable classification items and categories.

Ten categories include “Mc HC” category I: onset (age, phase), II: cause, III: underlying lesion, IV: symptomatology, V: pathophysiology 1-CSF circulation, VI: pathophysiology 2-ICP dynamics, VII: chronology, VII: post-shunt, VIII: post-endoscopic third ventriculostomy, and X: others. From a 100-year search of publication related to the classification of hydrocephalus, 14 representative publications were reviewed and divided into the 10 categories.

The Baumkuchen classification graph made from the round o’clock classification demonstrated the historical tendency of deviation to the categories in pathophysiology, either CSF or ICP dynamics.

In the preliminary clinical application, it was concluded that “Mc HC” is extremely effective in expressing the individual state with various categories in the past and present condition or among the compatible cases of hydrocephalus along with the possible chronological change in the future 9).

References

1)

Wu X, Zang D, Wu X, Sun Y, Yu J, Hu J. Diagnosis and Management for Secondary Low- or Negative-Pressure Hydrocephalus and a New Hydrocephalus Classification Based on Ventricular Pressure. World Neurosurg. 2019 Jan 4. pii: S1878-8750(18)32946-2. doi: 10.1016/j.wneu.2018.12.123. [Epub ahead of print] PubMed PMID: 30611954.
2)

Dandy WE, Blackfan KD. Internal hydrocephalus: an experimental, clinical and pathological study. Am J Dis Child. 1914;8(6):406-482.
3)

Chellathurai A, Subbiah K, Abdul Ajis BN, Balasubramaniam S, Gnanasigamani S. Role of 3D SPACE sequence and susceptibility weighted imaging in the evaluation of hydrocephalus and treatment-oriented refined classification of hydrocephalus. Indian J Radiol Imaging. 2018 Oct-Dec;28(4):385-394. doi: 10.4103/ijri.IJRI_161_18. PubMed PMID: 30662197; PubMed Central PMCID: PMC6319109.
4) , 5)

Rekate HL, Erwood S, Brodkey JA, Chizeck HJ, Spear T, Ko W, Montague F. Etiology of ventriculomegaly in choroid plexus papilloma. Pediatr Neurosci. 1985;12:196–201.
6)

Rekate HL. Neurosurgical management of the newborn with spinal bifida. In: Rekate HL, editor. Comprehensive Management of Spina Bifida. Boca Raton, FL, CRC Press; 1991. pp. 1–20.
7)

Rekate HL. Neurosurgical mangement of the child with spinal bifida. In: Rekate HL, editor. Comprehensive Management of Spina Bifida. Boca Raton, FL, CRC Press; 1991. pp. 93–112.
8)

Rekate HL. Selecting patients for endoscopic third ventriculostomy. Neurosurg Clin N Am. 2004;15:39–49. doi: 10.1016/S1042-3680(03)00074-3.
9)

Oi S. Classification of hydrocephalus: critical analysis of classification categories and advantages of “Multi-categorical Hydrocephalus Classification” (Mc HC). Childs Nerv Syst. 2011 Oct;27(10):1523-33. doi: 10.1007/s00381-011-1542-6. Epub 2011 Sep 17. Review. PubMed PMID: 21928018.

Pediatric Hydrocephalus

Pediatric Hydrocephalus

by Giuseppe Cinalli (Editor), M. Memet Özek (Editor), Christian Sainte-Rose (Editor)

Price: $569.05

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Since the first edition of this book, the impressive development of neuroendoscopy has dramatically changed the surgical approach to hydrocephalus, the main pathology pediatric neurosurgeons worldwide have to deal with. This revised and updated second edition, written by worldwide leaders in the field, fully reflects this progress: not only existing chapters have been reviewed whenever required, but new ones have been added thus taking into consideration every aspect of hydrocephalus, even when associated with the rarest pathologies. The general part include now more data on history, biomechanics, circulation and molecular basis. Special consideration for fetal surgery has been added, whereas the section on neonatal hydrocephalus has been further developed. Section 4, on the different pathologies associated with hydrocephalus, has been significantly expanded. and is now amazingly detailed, as well as the section on shunt treatment. Infections are now dealt with in two different chapters, and special attention to shunt complications (the nightmare of every Pediatric Neurosurgeon.) is paid in five different chapters. A very complete overview of the endoscopic treatment, that will surely draw the reader’s attention thanks to the wonderful full color images, is also included. Written by acknowledged experts in the field, this title is an indispensable tool for all those facing this pathology in their daily practice.

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