Craniostenosis
The content of the article:
- Causes and risk factors
- Forms
- Craniostenosis symptoms
- Diagnostics
- Craniostenosis treatment
- Complications and consequences of craniostenosis
- Forecast
- Prevention
Craniostenosis is a pathological condition that leads to deformation of the skull in newborns, resulting from premature overgrowth of the cranial sutures. Most often, the sagittal and coronary cranial sutures undergo premature infection.
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The volume of a deformed skull does not correspond to the size of an actively growing brain, compression and damage to brain structures occurs, which prevents their development and can lead to irreversible consequences.
The incidence of this disease is 1 case per 2000 newborns; it is observed in boys twice as often as in girls.
Causes and risk factors
Craniostenosis is caused by premature mineralization of the cranial sutures (craniosynostosis). The most common factors of premature infection are:
- genetic abnormalities;
- deficiency of vitamins (B8, D) and trace elements (magnesium, zinc, calcium) in the diet of the expectant mother;
- mechanical impact on the fetal skull during pregnancy and childbirth;
- hormonal disorders, especially those associated with thyroid hormones;
- infectious diseases of the expectant mother - herpes, rubella, flu;
- smoking mother during pregnancy;
- adverse environmental factors.
Craniosynostosis leads to deformation of the skull and an increase in intracranial pressure, which, in turn, leads to compression of the brain during the period of its active growth.
Forms
Depending on whether craniostenosis in a newborn is associated with other developmental anomalies, two forms are distinguished:
- non-syndromic, or isolated - proceeds without extracranial deformities, has a more favorable prognosis;
- syndromic - accompanied by extracranial deformities, abnormalities of the cardiovascular, respiratory, nervous systems, fusion of fingers and toes, clefts of the palate and upper lip and other malformations.
Depending on the number of prematurely mineralized seams, the following forms are distinguished:
- monosynostosis - contamination of one suture;
- polysynostosis - multiple closure of sutures;
- pansynostosis - infection of all cranial sutures.
There is also a classification that takes into account the deformation of the skull and which sutures underwent early mineralization. In accordance with it, the following forms of craniostenosis are distinguished:
- scaphocephaly - affects the sagittal suture; the most common form. It is characterized by an increase in the skull in the anteroposterior direction, narrowing of the head. The temples are depressed, the child's face has a scaphoid shape. Neurological symptoms are uncommon for this form;
- brachycephaly - the coronal and lambdoid sutures are fused. The skull is enlarged laterally. This form is characterized by neurological and ophthalmic symptoms;
- trigonocephaly - affects metopic sutures. It is characterized by a triangular expansion of the skull in the forehead. This form is characterized by hypertelorism (increased distance between the eyes) and visual impairment;
- microcephaly - occurs due to multiple fusion of cranial sutures. It is characterized by a uniform decrease in the size of the skull.
Craniostenosis symptoms
Craniostenosis is characterized by the following symptoms:
- deformed skull, unnatural head shape;
- increased intracranial pressure, which is the cause of persistent headache;
- nausea and vomiting;
- exophthalmos, strabismus, congestion in the retina, which lead to decreased vision;
- sleep disorders;
- Difficulty eating
- meningeal symptoms;
- lag in psychomotor development;
- periodic seizures.
Some symptoms do not appear immediately after the birth of a child and become pronounced only in the second year of life, which complicates the diagnosis of craniostenosis in newborns.
Diagnostics
Diagnosis of craniostenosis in newborns begins with a clinical examination, during which the shape and size of the skull is assessed, the fontanelles are palpated, and the size of the skull is measured with a measuring tape (craniometry).
Examination by a neurologist reveals the presence of pathological reflexes and other symptoms of brain damage. An examination by an ophthalmologist is also required to detect violations of the organ of vision - congestion of the fundus, exophthalmos, atrophy of the optic nerve.
An important role in the diagnosis is the collection of life and family history. The nature of the course of pregnancy (complications, infectious diseases, bad habits of the mother), the presence of such deformities in the family, especially childbirth (trauma, complications) are taken into account.
The main instrumental method for diagnosing craniostenosis is X-ray examination. The main diagnostic feature is the absence of one or more sutures in the image; the condition of the bone tissue is also assessed: with craniostenosis, it is thinned in the region of the cranial vault, and characteristic digital impressions are also revealed there.
Ultrasound examination of the main veins of the head and neck reveals circulatory disorders in the cranial cavity, which may be evidence of intracranial hypertension.
Computed tomography of the brain is used to confirm the adhesion of the cranial sutures, as well as to identify concomitant brain pathologies.
Other instrumental methods are also used:
- angiography of cerebral vessels;
- electroencephalography;
- neurosonography;
- magnetic resonance imaging;
- Ultrasound of internal organs to detect associated extracranial anomalies.
Craniostenosis treatment
Treatment of craniostenosis is mainly surgical. The primary task of surgical intervention is to eliminate increased intracranial pressure and create conditions for further growth and development of the brain. In some cases, operations can eliminate a cosmetic defect in the skull and correct the shape of the head.
The optimal age for the intervention is the early period - 3–9 months. After the third year of life, when the period of the most active brain growth is over, operations may be ineffective.
In the treatment of craniostenosis, the following types of surgical interventions are used:
- linear craniotomy - indicated at an early age;
- circular craniotomy - more often used at an older age;
- fragmentation of the cranial vault - indicated with multiple overgrowth of cranial sutures and only at an older age;
- flap bilateral craniotomy - indicated in severe cases of decompensated craniostenosis.
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After surgical treatment, control radiography or computed tomography is performed to assess the degree of cranial deformity correction.
Complications and consequences of craniostenosis
With an untimely diagnosis and in the absence of treatment, the following complications may develop:
- persistent deformation of the skull, leading to a significant cosmetic defect;
- persistent headaches;
- recurrent seizures (involuntary contractions of the muscles of the arms and legs, sometimes with loss of consciousness);
- development of visual impairment, up to its complete loss;
- mental retardation.
Forecast
In the case of promptly undertaken surgical treatment and with an isolated form of craniostenosis, the symptoms of the disease may regress.
If untreated, craniostenosis is usually disabling due to vision loss, mental retardation, and other complications. The unnatural shape of the skull and the violation of the proportions of the face leads to a serious cosmetic defect that affects the later life of the child.
Prevention
Prevention of craniostenosis during pregnancy includes:
- medical genetic consultations;
- complete elimination of bad habits (smoking, alcohol abuse);
- avoiding beverages and foods high in phosphates;
- nutritional control - it must include a sufficient amount of vitamins and minerals in the right combination;
- regular monitoring of the condition of the fetus using ultrasound and other examination methods;
- prevention of infectious diseases of the expectant mother.
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Anna Kozlova Medical journalist About the author
Education: Rostov State Medical University, specialty "General Medicine".
The information is generalized and provided for informational purposes only. At the first sign of illness, see your doctor. Self-medication is hazardous to health!
