Craniosynostosis
What is it?
There are many conditions that lead to cranial and facial deformities.
Craniostenosis can sometimes be associated with very complex polymalformative syndromes (Aper syndrome or Crouzon syndrome), but more often it occurs sporadically and is not associated with neurological or systemic problems.
The most common variants are:
- scaphocephaly;
- plagiocephaly;
- trigonocephaly;
- brachycephaly.
Causes and risk factors
At birth, the multiple bones of a child’s skull are not joined together as in other parts of the body, but are connected to each other by a special kind of cartilage known as sutures: under the influence of brain growth, these sutures allow the skull to expand easily and symmetrically.
If for any reason one of these sutures ossifies too early, the skull is forced to grow in a direction in which it meets no resistance, resulting in a marked deformity.
Which are the symptoms?
Some types of craniostenosis manifest from birth, others take several months to become noticeable; however, all forms of craniostenosis manifest within the first year of life.
In addition to cranial asymmetry, there is often early excessive closure of the anterior fontanelle, which usually occurs by the age of one year, and a palpable bone spur along the corresponding suture.
How is it diagnosed?
Diagnosis, in addition to a thorough clinical examination, includes a special type of computed tomography (CT) scan of the skull with three-dimensional reconstruction.
Suggested exams
How is it treated?
Surgical intervention is performed; the optimal time for surgery and type of intervention depends on the craniostenosis variant.
The interaction of a team of neurosurgeons and maxillofacial surgeons is often required, and surgery is preferably performed during the first year of life to obtain the best aesthetic results and minimize the damage associated with compression of the brain and orbital structures.
Surgeries involves removing some parts of the skull bones, remodeling them, and then reconnecting them and fixing them with microscrews and titanium microplates.
These surgeries often require a blood transfusion, but since they are not urgent surgeries, a parent or other compatible family member can be scheduled to donate blood.
Postoperative hospitalization lasts about one week.
After surgery, there is an outpatient checkup one month after discharge and then three months later. Approximately six months after surgery, a repeat cranial CT scan with three-dimensional reconstruction is performed to confirm proper cranial remodeling.
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