Various deformations of the skull are summarized under the term craniostenosis . Premature ossification of the sutures of the skull is responsible for these deformations. The malformations can usually be corrected surgically in infancy.
What is craniostenosis?
In the keeled skull, the frontal suture of the skull ossifies early. A short or towering skull, on the other hand, is due to premature ossification of the coronary suture on both sides. See AbbreviationFinder for abbreviations related to craniostenosis.
Craniostenosis is a malformation of the skull . The physician distinguishes between different forms. In the second year of life, the skull begins to ossify. The previously unossified skull sutures adapt flexibly to the growth processes of the brain. The ossification reaches its end in the sixth to eighth year of life. If the ossification begins too early or ends too quickly, then this results in craniostenosis.
Both the keel skull and the short skull, as well as the longitudinal skull and the tower skull are craniostenoses.
- The keeled skull is also known as the trigonocephalus.
- The short skull is technically referred to as brachycephalus.
- The tower skull is medically called turricephalus.
- Medicine sometimes also refers to the longitudinal skull as scaphocephalus or dolichocephalus.
In Central Asia and later also in Central Europe, people artificially deformed their skulls in the first century in order to form a towered skull.
In the keeled skull, the frontal suture of the skull ossifies early. A short or towering skull, on the other hand, is due to premature ossification of the coronary suture on both sides. A sagittal suture that ossifies too early is responsible for the longitudinal skull, and the medicine refers to premature ossification of all sutures as microcephalus. These ossification disorders can occur in the context of syndromes such as Crouzon’s disease or Apert’s disease.
Disorders of the bone metabolism can also be related to the phenomenon. Some patients with craniostenosis have additional malformations in other skeletal regions. In others, craniostenosis is an entirely isolated phenomenon. The positioning of the infant is relevant for some skull deformities. For example, if an infant is always placed on the same side, this can also deform the skull. However, craniostenosis is not related to this phenomenon. In the narrower sense, only natural deformations are designated as such, independent of external influences.
Symptoms, Ailments & Signs
Symptoms of craniostenosis depend on the shape of the deformity. In the tower skull, for example, the head reaches a considerable length. In the case of the keeled skull, the head takes on the shape of a triangle. The short skull runs upwards and the long skull hardly grows in width, but does increase in height. These four forms of craniostenosis are due to the 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 sutures of the skull ossify prematurely and the skull remains small in all directions.
This form of craniostenosis is often associated with damage to the brain, which usually cannot grow fully due to the limited space available. A frequent accompanying symptom of this form are increased pressure conditions in the mostly underdeveloped brain of the patient. In contrast, poor eyesight is the most common accompanying symptom in the tower skull.
Diagnosis & course of disease
The diagnosis of craniostenosis usually includes the survey of the so-called skull index. Imaging of the skull helps to assess the situation. X- ray procedures can sometimes make the type of malformation visible. Because of their three-dimensionality, CT images are usually more suitable for getting an exact picture of the situation and, if necessary, for planning therapeutic measures.
Neurological examinations can be used to assess whether and to what extent craniostenosis is already affecting brain functions. Ophthalmological examinations are often ordered for the same purpose. The course of the disease depends heavily on the form of craniostenosis. For example, microcephalus has a less favorable prognosis than ossification of a single suture.
In most cases, craniostenosis can be treated and controlled relatively well, so that there are no further complications after the treatment of this disease. Those affected suffer from various malformations of the skull. This leads to ossification and various deformations of the skullcap. The head cannot expand further, which leads to disturbances in the growth and development of the children.
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 also from headaches. It is not uncommon for craniostenosis to have a negative effect on the eyes, so that those affected suffer from poor eyesight.
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 supplied with little oxygen and can be irreversibly damaged. If the treatment is successful, there is no reduction in the life expectancy of the person affected.
When should you go to the doctor?
If the typical symptoms of craniostenosis are noticed, a doctor’s visit is recommended. External signs such as a short or long skull require medical clarification so that further measures can be taken quickly. Accompanying symptoms such as poor eyesight, headaches or neurological disorders should also be examined. If serious symptoms or complications arise, it is best to consult a doctor immediately who can diagnose or rule out the condition. People who suffer from a disorder of the bone metabolism are particularly susceptible to the development of craniostenosis and should see their family doctor with the symptoms mentioned.
The same applies to people with Crouzon’s or Apert’s disease. Parents who notice growth disorders or ossification in the area of the skull in their child should consult the pediatrician. Most cases of craniostenosis are diagnosed immediately after birth, but occasionally the symptoms are so mild that they take months or years to become apparent. Craniostenosis always requires medical examination and treatment.
Treatment & Therapy
Craniostenosis can only be treated surgically. The affected skull region is remodeled during this procedure. A CT, together with a modeling plan, dictates the course of the operation. With all head remodeling, the doctor opens the bony skull. The corrective measures he takes afterwards depend on the type of deformation. To correct a brachycephalus, he removes the skull cap, for example, via a craniectomy.
He usually uses plastic plates and plastic screws to remodel the opened skull regions. In the future, these materials will keep the skull in shape and do not need to be replaced. Follow-up operations are therefore usually not necessary. As a rule, the so-called craniotomy works so well that those affected will no longer be conspicuous in their future lives because of their deformities. However, doctors recommend an early correction of the skull shape, as this may prevent brain damage.
The ideal age for correction is between seven and twelve months. Craniotomies are particularly necessary when the intracranial pressure increases to alarming levels due to the deformation. The opening of the bony skull regulates intracranial pressure and thus protects the patient from permanent brain damage. Increased intracranial pressure impairs the oxygen supply and, in the worst case, can cause tissue in the brain to die off.
Outlook & Forecast
The prognosis of craniostenosis is favorable in most patients. The disruption of the ossifications can already be adequately treated in the first years of life with the use of medical care. Without treatment, the irregularities remain for life and can lead to impairments or subsequent disorders. The deformation of the skullcap then becomes an irreversible disorder. Medical care is therefore essential for a good prognosis.
If the chosen treatment method proceeds without further complications, the patient can be discharged as recovered after the therapy has been completed. Normally, further check-ups are recommended so that irregularities in the patient’s future development and growth process can be identified as quickly as possible.
In particularly severe cases, the malformations on the skull cannot be completely corrected despite all efforts. In these cases, the deformation of the skullcap is treated as best as possible. Nevertheless, visual abnormalities or the occurrence of complaints such as headaches can occur throughout life. In these patients, the prognosis is correspondingly worse. In addition, the risk of emotional and psychological disorders is significantly increased for those affected. It must be taken into account that complications such as scarring or lesions on the inside of the head can occur during the operations performed. In most cases, these can be dealt with as lifelong impairments and therefore lead to an unfavorable course of the disease.
Some skull deformities can be prevented by not always placing your child in the same position. However, these preventive measures do not apply to craniostenosis. Its etiology has not yet been sufficiently clarified to enable strategies to avoid this phenomenon to be developed.
In the case of craniostenosis, there are usually no special aftercare measures available to those affected. In the case of this disease, the quick and, above all, 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-healing cannot occur in this case.
In the case of craniostenosis, those affected are dependent on surgical interventions that can relieve the symptoms permanently. However, these interventions depend very much on the severity of the complaints and the deformations, so that a general prediction is usually not possible. Furthermore, some of those affected are dependent on the help and support of their own family and friends because of the disease in their lives.
Loving conversations can also be necessary, as this can prevent depressive moods. The craniostenosis may also reduce the life expectancy of the affected person, although no general statement can usually be made about this.
You can do that yourself
If craniostenosis has been diagnosed in a newborn, an operation is usually prepared immediately. After this procedure, care must be taken that the child does not touch the seams or even open them. Parents should keep a close eye on the child and make sure that a doctor is available quickly if there are any unusual symptoms. In addition, the child must rest and sleep a lot.
Since a surgical procedure always places a great deal of strain on a child’s body, medication may also have to be used. Here, too, parents are asked to pay attention to any side effects and interactions and to inform the responsible doctor immediately. In addition, strict hygiene measures must be observed so that the wound does not become inflamed or scars remain.
After the wound has healed, the doctor should carry out another comprehensive examination. In most cases, there are no late effects, but in individual cases further deformations can occur, which usually only become apparent during growth. Therefore, the affected child must be examined regularly in the first 15 to 20 years of life and, if necessary, operated on again.