Hydranencephaly: symptoms, causes and treatment

The development of our body is a bit complex. From the moment the sperm fertilizes the egg until we are born, many processes occur that generate our organs and body systems. The nervous system is one of the first to appear, With the heart, and will develop both during pregnancy and throughout life.

However, sometimes during pregnancy different problems arise that can cause our brain training to fail. One of the many alterations that can occur is hydranencephaly, which we will talk about in this article.

    What is hydranencephaly?

    It is understood by hydranencephaly a type of birth defect where most of the brain is missing, More precisely of the cerebral cortex, being the space generally occupied by this one 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 generally properly formed and functioning, and there are basic vital functions such as breathing and the heartbeat and respiratory rate. Other subcortical structures can also be preserved.

    The absence of a brain is due to the appearance of some kind of destructive process during pregnancy, starting from the twelfth week. In most cases, this will have serious repercussions, as these children will generally not be able to perform the basic functions that this structure would perform.

    Although this may seem surprising due to the complete or almost total absence of a cerebral cortex, some children born with this problem may initially exhibit appropriate behavior and manner of interacting with the world, being able to feed themselves properly and not to be. diagnosed immediately. But in general, we observe the existence of different alterations such as seizures, paralysis or sensory disturbances such as blindness or deafness. They may present with an absence or slowing of growth, hypotonia or severe irritability. The presence of some degree of mental and physical disability is expected, requiring and dependent on outpatient care.

    In most cases, the prognosis for children with this disorder is very negative, with most of those who have it dying before the age of one. But there are also many cases in which despite their condition, they manage to survive and even pass adolescence and reach adulthood.

      Hydranencephaly and hydrocephalus: differences

      It is important to keep in mind, as these are confusing diagnoses, that hydranencephaly and hydrocephalus are not the same. The two disorders are similar in that there are large pockets of cerebrospinal fluid in the brain that occupy a large part of the skull, but while in hydrocephalus the excess fluid pushes the brain tissue and can be observed the existence of at least part of the brain irrigated tissue, in hydranencephaly directly this tissue does not exist.

      The two disorders can be related, but it should be noted that hydrocephalus cannot be a consequence of hydrocephalus. What is possible is that hydranencephaly ends up causing hydrocephalus: the fluid that occupies the place of the brain can accumulate before more cerebrospinal fluid is produced, causing an increase in intracranial pressure and / or a protrusion of the skull.

      Possible causes

      Hydrocephalus is a congenital malformation that can have multiple causes. Usually it’s like we said earlier in a destructive process that affects brain tissue during pregnancy. This destruction can be caused by different elements, the most common being the existence of heart attacks or strokes caused by the rupture of the internal carotid artery.

      Other causes of stroke can be found in infection with different types of viruses or intoxication resulting from the mother’s use of alcohol or drugs during the developing fetus. To finish, it can be generated by diseases and genetic disorders.


      Hydranencephaly, as a congenital disorder, currently does not benefit from any curative treatment. This is not to say that no therapy can be used, although it tends to be palliative and aims to improve the patient’s quality of life. It is not uncommon for certain types of drainage or bypass of cerebrospinal fluid to prevent possible hydrocephalus and buildup.

      It is also very important to take care of the parents and the environment of the child, requiring psychoeducation and counseling in the difficult situation of this disorder, as well as the risks that the baby will face. The support of a support group can also be very helpful. as well as psychotherapy to fight against beliefs, fears and emotional alterations generated by the diagnosis (may in some cases present disorders such as depression).

      In cases where there is survival, the baby will need different supports and aids. Use of physiotherapy, speech therapy, basic life skills training, special education, and other professional care may be required.

      Bibliographical references:

      • Kinsman, SL; Johnston, MV (2016), Congenital anomalies of the central nervous system. A: Kliegman, RM; Stanton, BF; Saint-Geme, JW; Schor, NF (ed.). Nelson Handbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier ;: chap. 591.
      • Marin, CC and Robayo, G. (sf). Hydranencephaly. Report a case. Catalan Pediatric Society, 40 (4).

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