Dolichocephaly: Causes, Clinical Manifestation, Diagnosis, and Treatment

dolichocephaly causes

Dolichocephaly is a condition that consists of a noticeable malformation, a tall, narrow head.

This term refers to an inherited malformation of the skull, which primarily affects infants.

Dolichocephaly is not a life-threatening condition, but it’s usually related to dozens of developmental disabilities that could potentially lead to mental retardation.

The abnormal shape of the skull can also result in difficulties to chew and swallow, as well as issues with vision and breathing. The longer the head gets, the higher the risk for the child to develop most of these problems.

In severe cases, the malformation can put remarkably high pressure on the growing brain, and that could result in brain impairment as well as retardation.

Dolichocephaly Causes

Usually, when a baby ‘s brain grows, open borders (sutures) between the bones of the skull gradually expand so the child’s head gets to develop.

The main reason why this condition occurs is due to premature closure of such sutures. That causes the skull to expand in the direction of the borders that remain open.

The specific points affected by this condition are the sagittal sutures, the ones that run from the front to the back of the head.

Dolichocephaly prevents the skull from expanding in width, which is why the head begins to grow towards open sutures, such as the coronal sutures that run down the side of the head.

This abnormal development can occur because of the positioning of the growing baby during pregnancy, a sleeping position detrimental to the natural growth of the child, or neck tightness.

In other cases, the cause of this cranial malformation might be a genetic factor.

It could occur due to an underlying inherited condition, or a genetic mutation that negatively impacts the osteogenic development of the baby.

Clinical Manifestation

The primary symptom of Dolichocephaly is the remarkably long skull.

In mild cases, this malformation is only an aesthetic issue. So it’s pretty unlikely that the patient displays any other notable symptoms.

But in more severe cases, this condition can cause delayed growth or development of the brain cortex. The abnormal shape of the head will lead to trouble breathing and chewing food.

Such patients are likely to develop issues with their auditory system, and even vision disorders.

Dolichocephaly also features an inverted curvature of the parietal and temporal bones, with their convexity orientated to the surface of the cortex.

This malformation can cause horizontal strabismus, an anomaly of ocular alignment in which the visual axis gets deviated to the horizontal plane.

When a child becomes aware of their condition, they may suffer from psychological issues such as depression or low self-esteem.


The first sign a medical expert will notice is the abnormally long head. The front and back parts of the skull are too noticeable, and the sides of the back of the head are not as prominent as they should be.

A healthcare provider may take a history of the child from the parents.

The history will likely focus on the mother’s pregnancy, the position of the growing baby during pregnancy, and the circumstances of the birth (such as if the infant was born prematurely).

When it comes to the baby, doctors will look for signs of any possible birth trauma, and evaluate the kid’s sleeping position.

Medical experts can also measure the skull to make an exact diagnosis, as Dolichocephaly features a cephalic index of 75 or less (the standard cephalic index is between 76 and 80).

Several doctors stated that most severe cases of Dolichocephaly have patients with a cephalic index way below 75.

Dolichocephaly Treatment

As explained before, the cause of this malformation is the sagittal sutures closing prematurely. That is why medical experts focus on removing bone from the middle of this suture to fix the issue.

Some people say that the best moment to treat the kid and proceed with the surgery is before the child reaches the age of one year.

However, a study carried out in France shows that over 90% of the cases where children underwent the treatment before the nine months of age developed an IQ below 90.

But in the cases where the infants got treated after reaching the age of one year, less than 80% of the children developed an IQ below 90.