Right in the first pediatric appointments of a baby’s life, the head circumference is measured. Through this measurement, it is possible to monitor development within the statistical norms for each age and stage of the child. When it is noticed during a consultation that these measurements are below or above what is considered normal, tests may be requested for case analysis and confirmation of the problem.

Hydrocephalus1 and macrocephaly2 are among the main reasons for changes in a baby’s head circumference and must be carefully analyzed until a correct diagnosis is reached so that appropriate treatment can be given. Of course, it’s frightening for parents to hear that their child may have a problem, but understanding what it is can bring greater comfort.

Hydrocephalus is a disease that is mainly characterized by the accumulation of fluid inside the skull. This fluid, also known as cerebrospinal fluid (CSF), in excess causes increased brain pressure and swelling of the head. It is the fluid that surrounds the brain and spinal cord and serves to protect these areas, but when it is prevented from doing its job and is blocked for any reason, it will accumulate in the cranial area. Excess fluid can also occur due to poor absorption by the body and result in hydrocephalus. Unfortunately, a cure for the disease has not yet been found, but it is possible to treat and control hydrocephalus by relieving symptoms.

There are 3 types of hydrocephalus, each related to different causes:

  • Fetal Hydrocephalus – When the disease is discovered during pregnancy through ultrasound, it is called fetal or congenital hydrocephalus. The problem can be caused by genetic factors, drug use, or infections that the mother may have contracted during pregnancy, such as toxoplasmosis, syphilis, and rubella. During fetal development, malformation of the central nervous system leads to fluid accumulation in the skull and an increase in head size.

  • Infant Hydrocephalus – Not all people with hydrocephalus are born with the condition. Some develop it in early childhood, which may result from brain tumors, malformation, or even a cyst that is blocking the passage of fluid. In the case of a cyst, hydrocephalus is specifically called obstructive hydrocephalus, where hemorrhages, bleeding, and infections in the nervous system can also occur.

  • Pressure Hydrocephalus – Adults can also develop the disease at any stage of life. Typically, people over 65 have a higher chance of developing the problem, especially if they have had issues such as a stroke, hemorrhage, Alzheimer’s disease, or brain tumor. Pressure hydrocephalus in these cases occurs due to poor absorption of the fluid by the body or increased production of this fluid3.

Another problem marked by head growth, macrocephaly is usually considered to be a family genetic trait. However, it can also result from a variety of other health issues and therefore must be investigated for an accurate diagnosis. The main causes of macrocephaly are:

  • Hydrocephalus
  • Chronic hematomas
  • Intracranial hemorrhage
  • Brain tumor

Some diseases can also result in increased head circumference, such as Morquio syndrome, Hurler syndrome, and Canavan disease. Regular monthly check-ups with the pediatrician are extremely important, as it is through this follow-up that a problem can be detected. Of course, simply measuring the head is not enough to give a correct diagnosis, but by using growth charts for weight and height, it’s possible to see if anything is out of the ordinary and to request further tests to confirm if something is wrong.

The first step when excessive head growth is noted is to analyze the child’s brain condition, so an MRI or computed tomography scan is requested. Based on the results, the pediatrician will assess the situation and recommend the appropriate treatment.

Symptoms and Treatment of Hydrocephalus and Macrocephaly

Both hydrocephalus and macrocephaly have abnormal head growth as their main symptom. However, more specific symptoms may be observed and are even checked by the pediatrician to confirm the diagnosis. In babies, hydrocephalus may also cause poor head control, irritability, downward-looking eyes, vomiting, seizures, and bulging veins on the head and fontanelle.

In older children, symptoms may include severe headache, loss of movement, vomiting, urinary incontinence, learning and memory difficulties, speech changes, strabismus, and slow growth. In adults who develop hydrocephalus, the head size does not change because the cranial bones have already developed, but memory loss and urinary incontinence happen frequently.

Hydrocephalus treatment may involve medication or surgery. The surgical procedure aims to drain the fluid by redirecting it to other parts of the body, thereby relieving brain pressure. This surgery, in which a valve is placed, has yielded good results and improved quality of life for children and patients with hydrocephalus. However, it is necessary to watch for signs and possible problems with the valve, as it can be an entry point for infections and may even malfunction.

Symptoms of macrocephaly are very similar to those of hydrocephalus and may be accompanied by loss of appetite, excessive sleepiness, mood changes, vomiting, and involuntary eye movements. Treatment consists of relieving the symptoms and their complications. If macrocephaly results from hydrocephalus, treatment is aimed at managing that condition. When it is considered to be inherited genetically within the family, there is no specific treatment except for managing certain symptoms.

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Photo: Tania VDB