Hydrocephalus - Real clinical case

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The word hydrocephaly derives from thousands of years ago and comes from the Greek where Hydro refers to water, and Cephalus to the head, hence the term, is described as the abnormal accumulation of cerebrospinal fluid (CSF) in the brain, which if not treated or detected in time, has the ability to cause one of the dreaded complications called Endocranial Hypertension.

This pathology can occur at any stage of life, although it has been observed with greater incidence in newborns, infants and adults over 60 years, has no predilection for sex or race.

The cerebrospinal fluid (CSF) constitutes approximately 10% of the intracranial volume and through it protects and nourishes the brain and the entire spinal cord, in addition to this important quality, this liquid has the ability to allow the transport of nutrients and metabolites and even promote the distribution of drugs whose therapeutic target organ is the Central Nervous System.


Human Brain. Public domain image. Sourec Flickr

Anatomy of the nervous system. Brain Ventricles

Although our Nervous System is composed of two hemispheres and these in turn are subdivided into lobes; frontal, occipital, temporal, and parietal where each exercises its functions.

There are other vital and important structures for the brain called Cerebral Ventricles, which are cavities that are interconnected with each other, constituting an entire system and allowing the passage of the CSF.

Next we have the lateral ventricles in number of two, to each cerebral hemisphere constituted by an anterior horn that goes towards the frontal lobe, another posterior horn towards the occipital lobe and a inferior horn that goes down and in front of the temporal lobe, these ventricles communicate with the Third ventricle by means of the Monro Hole.

The Third Ventricle is a much smaller and more delicate cavity located in the thalamus that communicates with the fourth ventricle through Silvio's aqueduct.

Finally we have the Fourth Ventricle, located between the cerebellum and the brain stem, this structure is what allows the exit of the CSF to the Subarachnoid space. It is related to the spinal cord through the Ependymal canal.


Ventricles of the Brain. Licensed CC BY-SA 3.0. Author BruceBlaus

Circulation of cerebrospinal fluid

Cerebrospinal fluid (CSF) or also called cerebrospinal fluid (CSF) has the properties of being an odorless, colorless fluid that coats and protects the brain and spinal cord. Under normal conditions it has a volume of approximately 100 to 150 ml.

It consists mainly of water and other elements such as sodium, potassium, chlorine, calcium, amino acids, glucose, and protein.

This liquid marvel that nourishes us and protects our Nervous System in its totality, is elaborated from the choroidal plexuses, in almost 70% and the other remaining 30% comes from its elaboration in the Epéndimo (Aracnoid membrane), where they go towards the lateral ventricles, from there it goes to the Third Ventricle by means of the hole of Monro.

It then passes through Silvio's Hole to the Fourth Ventricle, and from here where two important orifices called Luschka and Magendie meet allows the CSF to go into the subarachnoid space and finally surround the entire Central Nervous System.

It is absorbed by the arachnoid villi, which are in close relationship with the venous sinuses, which contains an important membrane that covers the brain and from here this fluid is directed to the bloodstream.

This replacement or renewal of CSF takes place under normal conditions every 3 to 4 hours, generating a daily production of approximately 500ml.


CFS Circulation. Licensed CC BY 4.0. Author OpenStax

Why is Hydrocephalus present?

First of all, we must all be clear that hydrocephalus is nothing more than the exaggerated increase of the CSF inside the cranial cavity, which results in an increase in the size of the cavities through which this fluid circulates called ventricles, subarachnoid spaces and cisterns.

On the basis of what has been defined, we can therefore conclude that hydrocephalus is no more than the product of the loss of balance between the amount of processing and the amount of absorption of the CSF into the bloodstream.

Thus determining that the presence of this pathology is secondary:

  • Excesses in the production of Cerebrospinal Fluid that can be originated by the presence of Papilloma of Coroid Plexuses.
  • Deficit or failure in the absorption of the CSF, can be secondary to thrombosis of venous sinuses.
  • Obstruction of some of the cavities through which the CSF circulates and that does not allow the passage of the same one of correct form.
  • Classification

    Depending on its presentation, hydrocephalus may be:

    Congenital: which develop or present during intrauterine life or after birth and which are usually the result of untreated infections during pregnancy, nutritional deficiencies (such as vitamin A deficiency, B12, folic acid among others), teratogenesis secondary to drugs or viruses, inherited.

    Acquired: later presentation, in young or older adults and may be caused by the presence of space-occupying lesions such as tumors, abscesses or infectious processes at the level of the central nervous system.

    According to your clinical presentation:

    Communicating or non Obstructive Hydrocephalus, which will be product of an exaggerated elaboration or failure in the absorption of the Cerebrospinal Liquid, generally are observed in pathologies such as Papinoloma of Plexus Choroid Vitamin or nutritional deficiency in the child, some toxins, infections of the CSF and hereditary.

    Non-communicating hydrocephalus or Obstructive type, there is the presence of a lesion that obstructs the passage of fluid through cavities of the brain called ventricle, subarachnoid space, is often observed ventricular tumors.


    Hydrocephalus in Children. Licensed CC BY-SA 3.0. Author CDC

    Clinical Manifestations

    They are different according to the age group that is affected, in the newborn or infant congenital malformations are observed, increase of the circumference of the skull, headache, deviation of the look, vomiting and marked drowsiness.

    In adults it manifests with progressive headache, persistent with alteration of visual acuity, vomiting, cognitive deterioration and even if accompanied by endocranial hypertension we can observe at systemic level arterial hypertension, bradycardia, alteration of the respiratory pattern and neurological compromise. In severe cases, convulsions, cranial peer involvement and coma may be observed.

    Diagnosis

    By means of neuroimaging studies, either computerized axial Tomography or Magnetic Resonance which allows us to observe the accumulation of cerebrospinal fluid in the different structures of the brain, such as the cerebral ventricles.

    Clinical case

    Male of 30 years of age, without known pathological antecedents, who initiates current disease one month prior to admission presenting intermittent decrease in muscle strength in the left lower limb that made wandering difficult, which is accompanied by headache to occipital predominance of moderate intensity, which relieves with NSAIDs, concomitant escotomas and decrease in visual acuity, Subsequently, vomiting is associated with projectile of bilious content on more than 4 occasions. On the day of admission, there is evidence of generalized tonic clonic movements, with retroversion of the eye, sialorrhea and relaxation of the bladder sphincter, for approximately 1 minute with a subsequent post-ictal period, an episode that was repeated on 2 occasions.

    He denies important personal pathological antecedents with the case, and family antecedents, mother, father and apparently healthy siblings.

    Denies tobacco habits, uses of illicit substances. Occasional alcoholic habits at parties.

    Denies weight loss, previous head trauma, and fever.

    Physical exam:

  • Blood Pressure: 110/70 mmHg.
  • Heart rate: 80 pxm.
  • Breathing rate: 18 rxm.
  • Patient in stable clinical conditions, afebrile to the touch, hydrated, eupneic. Skin: brown, thermal norm to the touch, without apparent lesions, no cervical, axillary, epitrochlear or inguinal lymph nodes are palpated. Chest: symmetrical, elastic norm, expandable norm, apex in 5th intercostal space, mid-clavicular line. Cardiopulmonary: RsCsRs without murmur, audible RsRs in both pulmonary fields without aggregates. Abdomen: flat, RsHsAs (+), soft, depressible, not painful on superficial or deep palpation, no megalias are palpated. Genitals: Male, retractable foreskin, penis without lesions, testicles in their scrotal bags without tumors, Anus permeable, no lesions visible. Extremities: symmetrical, eutrophic, no edema. Neurological: vigilant, conscious, oriented, in time, space and person, without signs of focus, normal pupillary reflex with bilateral horizontal nystagmus, ROT (++), bilateral FM V/V, without signs of meningeal irritation.

    Simple Brain Magnetic Resonance


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    Finding in RNM Cerebral

    At the level of the anterior arm of the right internal capsule there is a small area with changes in the intensity of the signal of isointense magnetic behaviour in T1 with respect to the grey substance, and hyperintense in T2 with hydric restriction in diffusion, measuring 6.0 mm x 5.0 mm in its major axes without repercussion on adjacent structures.

    Symmetrical dilatation is observed in lateral ventricles as well as in the third and fourth ventricles, the latter causing displacement of the midbrain and cerebellar parenchyma in the anterior and posterior sense respectively, associated with moderate hyperintensity of the periventricular white matter in FLAIR sequence in relation to interstitial edema.

    Conclusions

    When there is an alteration of the equilibrium in the CSF circulation and it is not treated or diagnosed in time, a complication known as ENDOCRANEAN HYPERTESION can originate. This complication is no more than the increase in intracranial pressure that depends on its severity and evolution time, it is capable of displacing underlying structures, originating a specific clinic of this pathological entity that consists of a characteristic triad:

    Headache, Vomiting and Pupil Edema.

    If the clinic progresses, it is capable of triggering symptoms expressed in: Bradipsychia, Bradilalia, Temporo-Spatial Disorientation, Drowsiness or Psychomotor Agitation.

    Hydrocephalus and endocranial hypertension are usually diagnosed once the disease is established, the ideal is to determine early the origin of this pathology for its resolution, as is the case presented, where our patient developed these manifestations after his simple brain MRI showed LOE in 4th ventricle that due to CSF obstruction caused an increase in CSF pressure installing his current disease, the current treatment in these patients first of all is to decrease CSF pressure by means of a peritoneal bypass valve and later surgical resolution of space-occupying injury.

    Our patient is currently receiving medical treatment based on: Dexamethasone steroids type 8mg EV OD, Sodium Phenytoin 100 mg VO every 8 hours. Valproic acid 10cc VO every 12 hours. Acetazolamide 250 mg VO every 12 hours. Pending surgical resolution.

    Sources:

  • What is Hydrocephalus?
  • Hydrocephalus by: Joseph H. Piatt Jr., MD
  • About Hydrocephalus
  • Regulation of cerebrospinal fluid (CSF) flow in neurodegenerative, neurovascular and neuroinflammatory disease. Published online 2015 Oct 22. doi: 10.1016/j.bbadis.2015.10.014. Authors Matthew J. Simon and Jeffrey J. Iliff
  • The Ventricles of the Brain
  • Ventricular system/ wikipedia
  • The Central Nervous System
  • Ventricles and Coverings of the Brain
  • Cerebrospinal fluid
  • Cerebrospinal fluid circulation: What do we know and how do we know it?. Authors: Ahmad H Khasawneh, Richard J Garling and Carolyn A Harris
  • Physiology, Cerebral Spinal Fluid (CSF) Lauren N. Telano; Stephen Baker
  • Hydrocephalus
  • What is Hydrocephalus? An Overview
  • What is hydrocephalus, or water on the brain?
  • The definition and classification of hydrocephalus: a personal recommendation to stimulate debate Article (PDF Available) in Cerebrospinal Fluid Research 2008 · January 2008
  • The definition and classification of hydrocephalus: a personal recommendation to stimulate debate. Harold L Rekatecorresponding author
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