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Idiopathic cerebrospinal fluid overproduction: case-based review of the pathophysiological mechanism implied in the cerebrospinal fluid production.

Trevisi G, Frassanito P, Di Rocco C - Croat. Med. J. (2014)

Bottom Line: We report a unique case of a male infant with Crouzon syndrome who presented with intracranial hypertension, caused by up to 4-fold increase in CSF daily production.Medical therapy failed to reduce CSF production and the patient underwent several shunting procedures, cranial expansion, and endoscopic coagulation of the choroid plexus.This article thoroughly reviews pertinent literature on CSF production mechanisms and possible therapeutic implications.

View Article: PubMed Central - PubMed

Affiliation: Paolo Frassanito, Pediatric Neurosurgery, Catholic University Medical School, Largo Agostino Gemelli 8, 00168 Rome, Italy, paolo.frassanito@gmail.com.

ABSTRACT
Cerebrospinal fluid (CSF) overproduction results from either CSF infection or choroid plexus hypertrophy or tumor, with only a single idiopathic case described so far. We report a unique case of a male infant with Crouzon syndrome who presented with intracranial hypertension, caused by up to 4-fold increase in CSF daily production. Conditions related to CSF overproduction, namely central nervous system infections and choroid plexus hypertrophy or tumor, were ruled out by repeated magnetic resonance imaging and CSF samples. Medical therapy failed to reduce CSF production and the patient underwent several shunting procedures, cranial expansion, and endoscopic coagulation of the choroid plexus. This article thoroughly reviews pertinent literature on CSF production mechanisms and possible therapeutic implications.

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Brain magnetic resonance imaging (MRI) after Gadolinium administration ruled out pathological conditions affecting the choroid plexuses (A),(B). Endoscopic pictures of the choroid plexus before (C) and during (D) the coagulation performed using the Thulium laser.
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Figure 3: Brain magnetic resonance imaging (MRI) after Gadolinium administration ruled out pathological conditions affecting the choroid plexuses (A),(B). Endoscopic pictures of the choroid plexus before (C) and during (D) the coagulation performed using the Thulium laser.

Mentions: A few months later, the child suffered again from symptoms of increased intracranial pressure. After surgical revision of both shunting devices, MRI confirmed craniocerebral disproportion with slit ventricles. A biparietal cranial expansion was therefore performed. However, postoperative CSF drainage still ranged between 1400 mL and 1700 mL per day, requiring once again dual shunting. After 5 weeks a new shunt malfunction required exteriorization of both CSF shunts, confirming daily overproduction of CSF (about 1500 mL/d). A pharmacological approach with terlipressin administration was attempted with poor results. Thus, in spite of normal appearance of the choroid plexuses at MRI, endoscopic choroid plexus coagulation was performed bilaterally. Due to small ventricular size, the procedure was performed through bilateral occipital access and using neuronavigation (Figure 3). Since the first post-operative day, the CSF drainage rate halved to 700-800 mL/d. In spite of this important reduction of the CSF production rate, it was necessary to perform dual shunting. The postoperative course was uneventful and the patient was doing well at 6-month follow-up.


Idiopathic cerebrospinal fluid overproduction: case-based review of the pathophysiological mechanism implied in the cerebrospinal fluid production.

Trevisi G, Frassanito P, Di Rocco C - Croat. Med. J. (2014)

Brain magnetic resonance imaging (MRI) after Gadolinium administration ruled out pathological conditions affecting the choroid plexuses (A),(B). Endoscopic pictures of the choroid plexus before (C) and during (D) the coagulation performed using the Thulium laser.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4157373&req=5

Figure 3: Brain magnetic resonance imaging (MRI) after Gadolinium administration ruled out pathological conditions affecting the choroid plexuses (A),(B). Endoscopic pictures of the choroid plexus before (C) and during (D) the coagulation performed using the Thulium laser.
Mentions: A few months later, the child suffered again from symptoms of increased intracranial pressure. After surgical revision of both shunting devices, MRI confirmed craniocerebral disproportion with slit ventricles. A biparietal cranial expansion was therefore performed. However, postoperative CSF drainage still ranged between 1400 mL and 1700 mL per day, requiring once again dual shunting. After 5 weeks a new shunt malfunction required exteriorization of both CSF shunts, confirming daily overproduction of CSF (about 1500 mL/d). A pharmacological approach with terlipressin administration was attempted with poor results. Thus, in spite of normal appearance of the choroid plexuses at MRI, endoscopic choroid plexus coagulation was performed bilaterally. Due to small ventricular size, the procedure was performed through bilateral occipital access and using neuronavigation (Figure 3). Since the first post-operative day, the CSF drainage rate halved to 700-800 mL/d. In spite of this important reduction of the CSF production rate, it was necessary to perform dual shunting. The postoperative course was uneventful and the patient was doing well at 6-month follow-up.

Bottom Line: We report a unique case of a male infant with Crouzon syndrome who presented with intracranial hypertension, caused by up to 4-fold increase in CSF daily production.Medical therapy failed to reduce CSF production and the patient underwent several shunting procedures, cranial expansion, and endoscopic coagulation of the choroid plexus.This article thoroughly reviews pertinent literature on CSF production mechanisms and possible therapeutic implications.

View Article: PubMed Central - PubMed

Affiliation: Paolo Frassanito, Pediatric Neurosurgery, Catholic University Medical School, Largo Agostino Gemelli 8, 00168 Rome, Italy, paolo.frassanito@gmail.com.

ABSTRACT
Cerebrospinal fluid (CSF) overproduction results from either CSF infection or choroid plexus hypertrophy or tumor, with only a single idiopathic case described so far. We report a unique case of a male infant with Crouzon syndrome who presented with intracranial hypertension, caused by up to 4-fold increase in CSF daily production. Conditions related to CSF overproduction, namely central nervous system infections and choroid plexus hypertrophy or tumor, were ruled out by repeated magnetic resonance imaging and CSF samples. Medical therapy failed to reduce CSF production and the patient underwent several shunting procedures, cranial expansion, and endoscopic coagulation of the choroid plexus. This article thoroughly reviews pertinent literature on CSF production mechanisms and possible therapeutic implications.

Show MeSH
Related in: MedlinePlus