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Hydranencephaly: symptoms, causes and treatment

Hydranencephaly: symptoms, causes and treatment

July 18, 2024

The development of our organism is somewhat complex. From the moment in which the sperm fertilizes the ovum until we are born, there are multiple processes that are produced and that are generated by our organs and body systems. The nervous system is one of the first to appear , along with the heart, and it will develop both during pregnancy and throughout life.

However, sometimes during pregnancy different problems occur that can cause the formation of our brain does not come to fruition. One of the multiple alterations that can occur is hydranencephaly, which we will discuss in this article .

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What is hydranencephaly?

It is understood by hidranencefalia a type of congenital malformation consisting of the absence of practically the entire brain , specifically of the cerebral cortex, being the space generally occupied by this replaced by cerebrospinal fluid. Despite this, the shape of the subject's head may be normal, with the cavities and meninges surrounding the skull preserved. The cerebellum and brainstem are usually correctly formed and functional, with basic life functions such as breathing and heart and respiratory rhythm. Also other subcortical structures may be preserved.

The absence of brain is due to the appearance of some type of destructive process during pregnancy, from the twelfth week. This will have in most cases severe repercussions, these children usually not being able to carry out the basic functions that such structure would perform.

Although it may seem surprising due to the total or almost total absence of cerebral cortex, initially some of the children who come to be born with this problem can present a behavior and way of interacting with the appropriate world, being able to feed correctly and not being diagnosed immediately. . But in general, the existence of different alterations such as seizures, paralysis or sensory disturbances such as blindness or deafness . They may present growth absence or slowness, hypotonia or high irritability. The presence of some degree of psychic and physical disability is expected, specifying and depending on external care.

In most cases, the prognosis of children suffering from this disorder is very negative, most of whom die before one year of age die. But there are also numerous cases in which, despite their condition, they manage to survive and even overcome adolescence and reach adulthood.

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Hydranencephaly and hydrocephalus: differences

It is important to keep in mind, since they are diagnoses that can be confused, that hydranencephaly and hydrocephalus are not the same . Both disorders are similar in that there are large pockets of cerebrospinal fluid in the brain that occupy much of the skull, but while in hydrocephalus the excess fluid pushes the brain tissue and the existence of at least part of tissue can be observed cerebral irrigated, in the hydranencephaly directly said tissue does not exist.

Both disorders can be related, but it must be borne in mind that hydranencephaly can not be a consequence of hydrocephalus. What is possible is that a hydranencephaly ends up causing a hydrocephalus: the fluid that occupies the place of the brain can accumulate before the production of more cerebrospinal fluid, causing an increase in intracranial pressure and / or protrusion of the skull.

Possible causes

Hydrocephalus is a congenital malformation whose causes can be multiple. Generally, it is due as we have said before to a destructive process that affects brain tissue during pregnancy . This destruction can be caused by different elements, being the most frequent the existence of infarcts or strokes caused by the rupture of the internal carotid.

Other causes besides the cerebrovascular accidents can be found in the infection by different types of virus or the intoxication derived from the consumption of alcohol or drugs by the mother during the fetal development. By last, it can be generated by genetic diseases and disorders .


The hydranencephaly, as a congenital condition that is, does not currently have any curative treatment. This does not mean that no type of therapy can be used, although will tend to be palliative and aimed at improving the quality of life of the patient . It is not uncommon for some type of drainage or derivation of cerebrospinal fluid to avoid possible hydrocephalus and acomulations.

Also of great importance is the treatment with the parents and the child's environment, requiring psychoeducation and counseling in the face of the difficult situation posed by this disorder, as well as the risks that the baby will face. Assistance to support groups can also be very useful, as well as psychotherapy in order to combat beliefs, fears and emotional disturbances generated by the diagnosis (being able in some cases to experience disorders such as depression).

In cases where there is survival, the baby will need different supports and aids. The use of physiotherapy, logotherapy, training in basic daily life skills, special education and other professional care may be required.

Bibliographic references:

  • Kinsman, S.L .; Johnston, M.V. (2016), Congenital anomalies of the central nervous system. In: Kliegman, R.M .; Stanton, B.F .; St.Geme, J.W .; Schor, N.F. (eds) Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier;: chap 591.
  • Marín, C.C. & Robayo, G. (s.f.). Hydranencephaly Report of a case. Colombian Journal of Pediatrics, 40 (4).

Microhydranencephaly (July 2024).

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