Choroid plexus hyperplasia

Choroid plexus hyperplasia

Choroid plexus hyperplasia (CPH), also known as villous hypertrophy of the choroid plexus, is a rare benign condition that is characterized by bilateral enlargement of the entire choroid plexus in lateral ventricles without any discrete masses. This can result in overproduction of CSF and communicating hydrocephalus.

Despite the current knowledge about hydrocephalus, we remain without a complete understanding of the pathophysiology of this condition. glymphatic system (GS) could be more important than the conventional concept of reabsorption of CSF in the arachnoid villi, therefore GS could be a new key point, which will guide future investigations 1).

Histology shows an increased number of normal-sized cells.

This is best diagnosed by MRI which demonstrates a diffuse enlargement and homogeneous enhancement of choroid plexuses in a patient with communicating hydrocephalus 2).

It is a rare condition that may necessitate unusual treatment paradigms.

Although some authors recommend choroid plexus excision or coagulation, ventriculoatrial shunt insertion is a simple and effective treatment modality in cases of diffuse villous hyperplasia of the choroid plexus 3).

It can be seen in trisomy 9p where coexisting congenital heart disease additionally may complicate the therapeutic approach 4).

At 20 months of age, a Caucasian girl with trisomy 9 and a family history of an older brother and twin sister having the same syndrome displayed signs of congenital hydrocephalus due to increasing head circumferenceMagnetic resonance imaging revealed enlarged lateral ventricles and a prominent choroid plexus and the girl was treated with a ventriculoperitoneal shunt, which 2 days later had to be replaced with a ventriculoatrial shunt as cerebrospinal fluid production greatly exceeded the ability of the patient’s abdominal absorptive capability. At 16 years of age, the patient was diagnosed with cardiomyopathy and diminished ejection fraction. Some months later, she was admitted to the neurosurgical ward showing signs of shunt dysfunction due to a colloid cyst in the third ventricle. Cystic drainage through endoscopic puncture only helped temporarily. Revision of the shunt system showed occlusion of the ventricular drainage, and replacement was merely temporary alleviating. Intracranial pressure was significantly increased at around 30 mmHg, prompting externalization of the drain, and measurements revealed high cerebrospinal fluid production of 60-100 ml liquor per hour. Thus, endoscopic choroid plexus coagulation was performed bilaterally leading to an immediate decrease of daily cerebrospinal fluid formation to 20-30 ml liquor per hour, and these values were stabilized by pharmaceutical treatment with acetazolamide 100 mg/kg/day and furosemide 1 mg/kg/day. Subsequently, a ventriculoperitoneal shunt was placed. Follow-up after 1 and 2 months displayed no signs of hydrocephalus or ascites.

High cerebrospinal fluid volume load and coexisting heart disease in children with trisomy 9p may call for endoscopic choroid plexus coagulation and pharmacological therapy to diminish the daily cerebrospinal fluid production to volumes that allow proper ventriculoperitoneal shunting 5).


A 1-year-old patient was diagnosed with communicating hydrocephalus; ventricle peritoneal shunt (VPS) is installed and ascites developed. VPS is exposed, yielding volumes of 1000-1200ml/day CSF per day. MRI is performed showing generalized choroidal plexus hyperplasia. Bilateral endoscopic coagulation of thechoroid plexus was performed in 2 stages (CPC) however the high rate of CSF production persisted, needing a bilateral plexectomy through septostomy, which finally decreased the CSF outflow.

New knowledge about CSF physiology will help to propose better treatment depending on the cause of the hydrocephalus. The GS is becoming an additional reason to better study and develop new therapies focused on the modulation of alternative CSF reabsorption. 6).


In these patients, intractable ascites can occur after a ventriculoperitoneal (VP) shunting operation. However, shunt-related hydrocele is a rare complication of VP shunting. Previous reports have indicated catheter-tip migration to the scrotum as a cause of hydrocele. Here, we present the first documented case of choroid plexus hyperplasia that led to intractable ascites after shunting and a resulting hydrocele without catheter-tip migration into the scrotum 7).


1) , 6)

Paez-Nova M, Andaur K, Campos G, Garcia-Ballestas E, Moscote-Salazar LR, Koller O, Valenzuela S. Bilateral hyperplasia of choroid plexus with severe CSF production: a case report and review of the glymphatic system. Childs Nerv Syst. 2021 Nov;37(11):3521-3529. doi: 10.1007/s00381-021-05325-2. Epub 2021 Aug 19. PMID: 34410450.
3)

Iplikcioglu AC, Bek S, Gökduman CA, Bikmaz K, Cosar M. Diffuse villous hyperplasia of choroid plexus. Acta Neurochir (Wien). 2006 Jun;148(6):691-4; discussion 694. doi: 10.1007/s00701-006-0753-1. Epub 2006 Mar 8. PMID: 16523225.
4) , 5)

Henningsen MB, Gulisano HA, Bjarkam CR. Congenital hydrocephalus in a trisomy 9p gained child: a case report. J Med Case Rep. 2022 May 27;16(1):206. doi: 10.1186/s13256-022-03424-5. PMID: 35619116.
7)

Hori YS, Nagakita K, Ebisudani Y, Aoi M, Shinno Y, Fukuhara T. Choroid Plexus Hyperplasia with Intractable Ascites and a Resulting Communicating Hydrocele following Shunt Operation for Hydrocephalus. Pediatr Neurosurg. 2018;53(6):407-412. doi: 10.1159/000492333. Epub 2018 Aug 29. PMID: 30157489.

Shunt for Idiopathic normal pressure hydrocephalus treatment

Shunt for Idiopathic normal pressure hydrocephalus treatment

• Early shunt surgery can significantly improve the clinical symptoms and prognosis of patients with idiopathic normal pressure hydrocephalus (iNPH). • Structural imaging findings have limited predictiveness for the prognosis of patients with iNPH after shunt surgery. • Patients should not be selected for shunt surgery based on only structural imaging findings 1).


Clinical decisions regarding Shunt for Idiopathic normal pressure hydrocephalus treatment should be individualized to each patient, with adequate consideration of the relative risks and benefits, including maximizing a healthy life expectancy 2).

see Ventriculoperitoneal shunt for idiopathic normal pressure hydrocephalus.

see Lumboperitoneal shunt for idiopathic normal pressure hydrocephalus.


1)

Chen J, He W, Zhang X, Lv M, Zhou X, Yang X, Wei H, Ma H, Li H, Xia J. Value of MRI-based semi-quantitative structural neuroimaging in predicting the prognosis of patients with idiopathic normal pressure hydrocephalus after shunt surgery. Eur Radiol. 2022 Apr 30. doi: 10.1007/s00330-022-08733-3. Epub ahead of print. PMID: 35501572.
2)

Nakajima M, Kuriyama N, Miyajima M, Ogino I, Akiba C, Kawamura K, Kurosawa M, Watanabe Y, Fukushima W, Mori E, Kato T, Sugano H, Tange Y, Karagiozov K, Arai H. Background Risk Factors Associated with Shunt Intervention for Possible Idiopathic Normal Pressure Hydrocephalus: A Nationwide Hospital-Based Survey in Japan. J Alzheimers Dis. 2019 Mar 11. doi: 10.3233/JAD-180955. [Epub ahead of print] PubMed PMID: 30883349.

Ventriculostomy related infection risk factors

Ventriculostomy related infection risk factors

Ventriculostomy related infection risk factors include prior brain surgerycerebrospinal fluid fistula, and insertion site dehiscence. Walek et al. from Rhode Island Hospital found no significant association between infection risk and duration of external ventricular drainage placement 1).


A total of 15 supposed influencing factors includes: age, age & sex interactions, coinfection, catheter insertion outside the hospital, catheter type, CSF leakage, CSF sampling frequency, diagnosis, duration of catheterization, ICP > 20 mmHg, irrigation, multiple catheter, neurosurgical operation, reduced CSF glucose at catheter insertion and sex 2).


In a large series of patients, ventriculostomy related infection (VRI) was associated with a longer ICU stay, but its presence did not influence survival. A longer duration of ventriculostomy catheter monitoring in patients with VRI might be due to an increased volume of drained CSF during infection. Risk factors associated with VRIs are SAH, IVH, craniotomy, and coinfection 3).


A retrospective cohort study strengthens a growing body of works suggesting the importance of inoculation of skin flora as a critical risk factor in ventriculostomy related infections, underscoring the importance of drain changes only when clinically indicated and, that as soon as clinically permitted, catheters should be removed 4).


Associated with a longer ICU stay, but its presence did not influence survival. A longer duration of ventriculostomy catheter monitoring in patients with VAI might be due to an increased volume of drained CSF during infection. Risk factors associated with VAIs are subarachnoid hemorrhage (SAH), intraventricular hemorrhage IVH, craniotomy, and coinfection 5).

The risk of infection increases with increasing duration of catheterization and with repeated insertions. The use of local antibiotic irrigation or systemic antibiotics does not appear to reduce the risk of VAI. Routine surveillance cultures of CSF were no more likely to detect infection than cultures obtained when clinically indicated. These findings need to be considered in infection control policies addressing this important issue 6).


An increased risk of infection has been observed in patients with subarachnoid or intraventricular hemorrhage, in patients with concurrent systemic infections as well as with longer duration of catheterization, cerebrospinal (CSF) leakage, and frequent manipulation of the EVD system 7) 8) 9).

Many studies have been conducted to identify risk factors of EVD-related infections. However, none of these risk factors could be confirmed in a cohort of patients. Furthermore they not show any difference in infection rates between patients who were placed in single- or multibed rooms, respectively 10).


Interestingly no risk factor for EVD-related infection could be identified in a retrospective single center study 11).


1)

Walek KW, Leary OP, Sastry R, Asaad WF, Walsh JM, Horoho J, Mermel LA. Risk factors and outcomes associated with external ventricular drain infections. Infect Control Hosp Epidemiol. 2022 Apr 26:1-8. doi: 10.1017/ice.2022.23. Epub ahead of print. PMID: 35471129.
2)

Sorinola A, Buki A, Sandor J, Czeiter E. Risk Factors of External Ventricular Drain Infection: Proposing a Model for Future Studies. Front Neurol. 2019 Mar 15;10:226. doi: 10.3389/fneur.2019.00226. eCollection 2019. Review. PubMed PMID: 30930840; PubMed Central PMCID: PMC6428739.
3)

Bota DP, Lefranc F, Vilallobos HR, Brimioulle S, Vincent JL. Ventriculostomy-related infections in critically ill patients: a 6-year experience. J Neurosurg. 2005 Sep;103(3):468-72. PubMed PMID: 16235679.
4)

Katzir M, Lefkowitz JJ, Ben-Reuven D, Fuchs SJ, Hussein K, Sviri G. Decreasing external ventricular drain related infection rates with duration-independent, clinically indicated criteria for drain revision: A retrospective study. World Neurosurg. 2019 Aug 2. pii: S1878-8750(19)32121-7. doi: 10.1016/j.wneu.2019.07.205. [Epub ahead of print] PubMed PMID: 31382072.
5)

Bota DP, Lefranc F, Vilallobos HR, Brimioulle S, Vincent JL. Ventriculostomy-related infections in critically ill patients: a 6-year experience. J Neurosurg. 2005 Sep;103(3):468-72. PubMed PMID: 16235679.
6)

Arabi Y, Memish ZA, Balkhy HH, Francis C, Ferayan A, Al Shimemeri A, Almuneef MA. Ventriculostomy-associated infections: incidence and risk factors. Am J Infect Control. 2005 Apr;33(3):137-43. PubMed PMID: 15798667.
7)

Camacho E. F., Boszczowski Í., Basso M., Jeng B. C. P., Freire M. P., Guimarães T., Teixeira M. J., Costa S. F. Infection rate and risk factors associated with infections related to external ventricular drain. Infection. 2011;39(1):47–51. doi: 10.1007/s15010-010-0073-5.
8)

Kim J.-H., Desai N. S., Ricci J., Stieg P. E., Rosengart A. J., Hrtl R., Fraser J. F. Factors contributing to ventriculostomy infection. World Neurosurgery. 2012;77(1):135–140. doi: 10.1016/j.wneu.2011.04.017.
9)

Mayhall C. G., Archer N. H., Lamb V. A., Spadora A. C., Baggett J. W., Ward J. D., Narayan R. K. Ventriculostomy-related infections. A positive epidemiologic study. The New England Journal of Medicine. 1984;310(9):553–559. doi: 10.1056/NEJM198403013100903.
10)

Hagel S, Bruns T, Pletz MW, Engel C, Kalff R, Ewald C. External Ventricular Drain Infections: Risk Factors and Outcome. Interdiscip Perspect Infect Dis. 2014;2014:708531. Epub 2014 Nov 17. PubMed PMID: 25484896; PubMed Central PMCID: PMC4251652.
11)

Hagel S, Bruns T, Pletz MW, Engel C, Kalff R, Ewald C. External ventricular drain infections: risk factors and outcome. Interdiscip Perspect Infect Dis. 2014;2014:708531. doi: 10.1155/2014/708531. Epub 2014 Nov 17. PubMed PMID: 25484896; PubMed Central PMCID: PMC4251652.
WhatsApp WhatsApp us
%d bloggers like this: