The term craniostenosis is used to describe various deformities of the skull. Premature ossification of the cranial sutures is responsible for these deformities. The deformities can usually be corrected surgically in infancy.
What is craniostenosis?
Craniostenosis is a malformation of the skull. The medical profession distinguishes between different forms. In the second year of life, the skull begins to ossify. The previously ossified cranial sutures adapt flexibly to the growth processes of the brain. Ossification reaches its end in the sixth to eighth year of life. If ossification starts too early or comes to an end too quickly, then craniostenoses result. Both the keel skull and the short skull, as well as the longitudinal skull and the tower skull are craniostenoses.
- Keel skull is also known as trigonocephalus.
- The short skull is technically known as brachycephalus.
- The tower skull is medically called turricephalus.
- The longitudinal skull is sometimes referred to in medicine as scaphocephalus or dolichocephalus.
In Central Asia and later in Central Europe, people in the first century artificially deformed their skulls to form a tower skull.
Causes
In a keel skull, the frontal suture of the skull ossifies early. In contrast, a short skull or tower skull results from premature ossification of the coronal suture on both sides. A longitudinal skull is caused by premature ossification of the sagittal suture, and microcephaly is the medical term for premature ossification of all cranial sutures. These ossification disorders can occur in the context of syndromes such as Crouzon’s disease or Apert’s disease. Bone metabolism disorders may also be associated with the phenomenon. Some patients with craniostenosis have additional malformations in other skeletal regions. In others, craniostenosis is a completely isolated phenomenon. For some cranial deformities, the positioning of the infant is relevant. For example, if an infant is always placed on the same side, then this can also deform the skull. However, craniostenoses are not related to this phenomenon. In a narrower sense, only natural deformations independent of external influence are referred to as such.
Symptoms, complaints and signs
The symptoms of craniostenosis depend on the form of the deformity. For example, in the tower skull, the head reaches a considerable length. In the keel skull, the head takes the shape of a triangle. The short skull runs upwards and the longitudinal skull hardly grows in width, but it grows in height. These four forms of craniostenosis are due to premature ossification of a single cranial suture. The head can no longer expand in the direction of this cranial suture and compensates for this lack of space by expanding in one of the previously unossified directions. In microcephalus, all cranial sutures ossify prematurely and the skull thus remains small in all directions. This form of craniostenosis is often accompanied by damage to the brain, which usually cannot fully grow out because of the minimal space available. A frequent accompanying symptom of this form is increased pressure in the usually underdeveloped brain of the patient. In tower skull, on the other hand, visual impairment is one of the most common accompanying symptoms.
Diagnosis and course of the disease
Diagnosis of craniostenosis usually involves taking the so-called cranial index. Imaging of the skull helps assess the location. X-ray procedures may be able to visualize the nature of the deformity. However, because of their three-dimensionality, CT images are usually more suitable for obtaining an exact picture of the situation and, if necessary, planning therapeutic measures. Neurological examinations can be used to assess whether and to what extent the craniostenosis is already affecting brain functions. Ophthalmological examinations are often ordered for the same purpose. The course of the disease strongly depends on the form of the craniostenosis. Microcephalus, for example, is prognostically less favorable than ossifications of a single suture.
Complications
In most cases, craniostenosis can be treated and limited relatively well, so there are no further complications after treatment of this condition.Those affected suffer from various deformities of the skull. This leads to ossification and further to various deformations of the skull. The head cannot expand any further, so that the growth and development of the children is disturbed. Likewise, the brain cannot develop further due to the craniostenosis, so that without treatment there are significant mental limitations and also retardation. The patients also suffer from a very unpleasant feeling of pressure in the head and furthermore also from headaches. Not infrequently, craniostenosis also has a negative effect on the eyes, so that those affected suffer from impaired vision. In most cases, craniostenosis is treated immediately after birth. There are no particular complications. The earlier the correction is made, the lower the probability of possible consequential damage or complications. Without treatment, the brain is also deprived of oxygen and can be irreversibly damaged. If treatment is successful, there is no reduction in the life expectancy of the affected person.
When should you see a doctor?
When the typical symptoms of craniostenosis are noticed, a visit to the doctor is recommended. For example, external signs such as a short or longitudinal skull require medical clarification so that further measures can be initiated quickly. Accompanying symptoms such as impaired vision, headaches or neurological disorders should also be examined. If serious symptoms or complications occur, it is best to consult a physician immediately who can diagnose or rule out the condition. Individuals suffering from a disorder of bone metabolism are particularly susceptible to developing craniostenosis and should see their primary care physician with symptoms mentioned. The same applies to people with Crouzon’s disease or Apert’s disease. Parents who notice growth disorders or ossification in the area of the skull in their child should consult the pediatrician. In most cases, craniostenosis is diagnosed immediately after birth, but occasionally the symptoms are so mild that they do not become apparent for months or years. In any case, craniostenosis requires medical examination and treatment.
Treatment and therapy
Craniostenosis can only be treated surgically. The affected region of the skull is remodeled during this procedure. A CT scan, along with a modeling plan, dictates the surgical procedure. In all remodeling of the head, the doctor opens the bony skull. The corrective measures he takes afterwards depend on the type of deformity. To correct brachycephalus, for example, he removes the skull via a craniectomy. To remodel the opened skull regions, he usually uses plastic plates and plastic screws. These materials keep the skull in shape permanently in the future and do not need to be replaced. Subsequent operations are therefore usually not necessary. As a rule, the so-called craniotomy succeeds so well that the affected person will no longer be noticed for their deformities in their future life. However, doctors recommend an early correction of the skull shape, as this may prevent brain damage. The ideal age for correction is considered to be between seven and twelve months. Craniotomies are mandatory especially when the intracranial pressure rises to alarming levels due to the deformity. Opening the bony skull regulates the intracranial pressure and thus protects the patient from permanent brain damage. Elevated intracranial pressure impairs oxygenation and, in the worst cases, can cause tissue in the brain to die.
Outlook and prognosis
The prognosis of craniostenosis is favorable in most patients. The disorder of ossification can be adequately treated in the early years of life when medical care is sought. Without treatment, the irregularities persist throughout life and can lead to impairment or secondary disorders. The deformity of the cranium then becomes an irreversible disorder. Therefore, medical care is essential for a good prognosis. If the selected treatment method proceeds without further complications, the patient can be discharged as recovered after completion of the therapy.Normally, further control examinations are recommended so that irregularities can also be noticed as quickly as possible in the future development and growth process of the patient. In particularly severe cases, the deformities of the skull cannot be completely corrected despite all efforts. In these cases, the deformities of the skull are treated in the best possible way. Nevertheless, visual abnormalities or the occurrence of complaints such as headaches may persist throughout the patient’s life. In these patients, the prognosis is correspondingly worse. In addition, the risk of emotional and psychological disorders is significantly increased in these patients. It should be taken into account that complications such as scarring or lesions of the interior of the head may occur during the procedures performed. In most cases, these must be managed as lifelong impairments and therefore lead to an unfavorable course of the disease.
Prevention
Some cranial deformities can be prevented by not always placing one’s child in the same position. However, these preventive measures do not apply to craniostenoses. Their etiology has not yet been sufficiently elucidated for avoidance strategies to be developed for this phenomenon.
Follow-up
There are usually no special aftercare measures available to the affected person in craniostenosis. In this disease, first and foremost, the rapid and, above all, the early detection of the disease is very important so that no further complications can occur. The affected person should therefore contact a doctor at the first signs and symptoms. Self-cure cannot occur in this case. Sufferers of craniostenosis are dependent on surgical interventions that can permanently alleviate the symptoms. However, these interventions depend very much on the severity of the symptoms and the deformities, so that a general prediction is usually not possible. Furthermore, some affected persons are dependent on the help and support of their own family and friends due to the disease in their lives. Loving conversations may also be necessary, as depressive moods can be prevented in this way. Possibly, the craniostenosis thereby also reduces the life expectancy of the affected person, although no general statement can usually be made about this.
What you can do yourself
If a newborn has been diagnosed with craniostenosis, surgery is usually prepared immediately. After this procedure, care must be taken to ensure that the child does not touch the sutures or even open them. Parents should constantly monitor the child and make sure that a doctor is quickly available in case of unusual symptoms. In addition, the child must take it easy and sleep a lot. Since a surgical procedure always puts a lot of stress on a child’s body, medications may also need to be used. Here, too, parents are asked to pay attention to any side effects and interactions and to report them immediately to the doctor in charge. In addition, strict hygiene measures must be observed so that the wound does not become infected or leave scars. After the wound has healed, the doctor should carry out another comprehensive examination. In most cases, there are no late sequelae, but in individual cases there may be further deformities, which usually only become apparent during growth. That is why the affected child must be examined regularly during the first 15 to 20 years of life and, if necessary, be operated on again.
