agenesia del cuerpo calloso e hidrocefalia

7/28/2019 Agenesia Del Cuerpo Calloso e Hidrocefalia

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Malformaciones congnitas del SNC:

Hidrocefalia, agenesia del cuerpocalloso.

Orellana Cullar, Jota Walter


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DEFINICIN

Hidrocefalia congnita:

Crecimiento progresivo ventricular por acmuloanormal de LCR, que aparece desde el primer da de

vida, por consecuente con aparicin inutero.

J. Volpe. Neurology of the New Borne. 3a edicion, 31-35, 1994


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Produccin de lquidocefalorraqudeo

El lquido cefalorraqudeo es producido por el plexocoroides. A travs de los agujeros de Magendie y deLuschka fluye hasta el cuarto ventrculo. Finalmente

es absorbido en los cuerpos de Pacchioni y en lasvellosidades aracnoideas.

Velocidad de produccin en los adultos 0.33ml/min = 475 ml en 24 h.

Velocidad de produccin en los nios 0.35 ml/min= 504 ml en 24 h.

HJ Lpez. Hidrocefalia congnita asociada a Sndrome de Dandy-Walker. Rev Mex Ped; 67; 77-82; 2000.


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Produccin de liquido cefalorraqudeo

El volumen total de LCR es aproximadamente unos 50 ml en ellactante, la mayor parte se encuentra fuera de los ventrculos.

El lquido una vez absorbido pasa hacia el seno sagital, y deall pasa a la sangre. Una vez en la sangre, es llevado y filtradopor los riones e hgado en la misma manera que cualquierotro fluido orgnico.

Nelson. Tratado de pediatra. Ed McGrawHill.16 Ed. P 1971, 2001


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Causas de hidrocefalia congnita

Estenosis acueductal 33%

Mielomeningocele (Arnodl Chiari) 28%

Hidrocefalia comunicante 22%

Malformacin Dandy Walker 7% Otros 10%

J. Volpe. Neurology of the New Borne. 3a edicion, 31-35, 1994


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Cuadro clnico Permetro ceflico aumentado.

Separacin de suturas craneales.

Somnolencia, rechazo progresivo a la va oral.

Anormalidades oculares (atrofia ptica yestrabismo).

Disfuncin del tronco enceflico (pobre

succin, vmitos, broncoaspiracin). Muerte por herniacin transtentorial o

transforaminal.


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AGENESIA DEL CUERPO CALLOSO


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AGENESIS OF CORPUS CALLOSUM

Agenesis of the corpus callosum (ACC) is a rare birth defect (congenital

disorder) in which there is a complete or partial absence of the corpuscallosum.

The development of the fibers that would otherwise form the corpuscallosum become longitudinally oriented within each hemisphere andform structures called Probstbundles.

In addition to agenesis of the corpus callosum, other congenital callosaldisorders include

1. hypogenesis (partial formation),

2. dysgenesis (malformation) of the corpus callosum,

3. hypoplasia (underdevelopment) of the corpus callosum.

Diagnosis

Callosal disorders can be diagnosed only through a brain scan

They may be diagnosed through an MRI, CT scan, prenatal ultrasound,or prenatal MRI


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Cause Agenesis of the corpus callosum is caused by disruption to

development of the fetal brain between the 3rd and 12th weeksof pregnancy.

In most cases, it is not possible to know what caused anindividual to have ACC or another callosal disorder.

However, research suggests that some possible causes mayinclude

chromosome errors, inherited genetic factors,

prenatal infections or injuries, prenatal toxic exposures,

structural blockage by cysts or other brain abnormalities metabolic disorders.


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Signs and symptoms Vary greatly among individuals

Some characteristics common in individuals with callosal disorders include

Research : shown to have some cognitive disabilities (difficulty in complex problemsolving) and social difficulties (missing subtle social cues), even when theirintelligence quotient is normal.

Other characteristics sometimes associated with callosal disorders includeseizures, spasticity, early feedingdifficulties and/or gastric reflux, hearing

impairments, abnormal head and facial features, and mental retardation.

1. Poor motor coordination,2. Delays in motor milestones such as sitting and walking,3. Delayed toilet training,4. Chewing and swallowing difficulties

5. Vision impairments,6. Hypotonia

7. Low perception ofpain, .


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Associated syndromes ACC can occur as an isolated condition or in combination with other cerebral

abnormalities, including Arnold-chiari malformation, Dandy-walkersyndrome,

Andermannsyndrome(motor and sensory neuropathy) Schizencephaly (clefts or deep divisions in brain tissue) Holoprosencephaly (failure of the forebrain to divide into lobes.)

Girls may have a gender-specific condition called Aicardi'ssyndrome, whichcauses severe mental retardation, seizures(infantile spasms), abnormalities inthe vertebra of the spine, and lesions(lacunae) on the retina of the eye.

ACC can also be associated with malformations in other parts of the body,such as midline facialdefects.

The effects of the disorder range from subtle or mild to severe, depending on

associated brain abnormalities.


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agenesia del cuerpo calloso e hidrocefalia

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