CRANIOSTENOSIS (craniostenosis; Greek kranion a skull + stenosis narrowing) — the special type of pathological morphogenesis of a skull which is characterized by a premature synostosis of separate or all cranial seams, deformation of a skull, disturbances of structure oho of bones, shown visual disturbances and various symptoms of insufficiency of functions of a brain. To. it can be combined with other malformations (a syndactylia, brain hernia, a labium leporium, a wolf mouth, a patent ductus arteriosus, anomalies of development of a trachea and bronchial tubes etc.). To. occurs at 0,005 — 0,02% of newborns. Hereditary forms K are described.
The etiology and a pathogeny
the Reasons of premature fusion of seams of a skull are not established. They are connected with various hereditary and pre-natal diseases. A pathogeny of premature fusion of seams and all observed at To. nevrol, many authors explain symptomatology primary patol, changes in the skull and arising at this discrepancy between the volume of an intracranial cavity and volume increasing at a rate of a brain. At the same time the reasonable opinion is expressed that early fusion of cranial seams is only one of symptoms of difficult inborn pathology, in particular primary pathology in development of vascular system of the head. Studying of features of an angioarchitecture and hemodynamics of the head of patients To. revealed anomalies out of - and intracranial arteries and veins, and also the disturbances of cerebral circulation expressed in various degree. These disturbances come to light in two forms — in development of venous stagnation with the subsequent increase of increase in intracranial pressure and in development of ischemic damage of a brain owing to inborn patol, changes in the main arteries of the head (see. Intracranial pressure ). In favor of the vascular theory of a pathogeny To. tell experimental data, according to the Crimea at 2 — lead 4-day rabbits of change of blood circulation of the head to premature fusion of cranial seams.
There is a set of classifications of K. Odnako most of them does not satisfy clinical physicians with either incompleteness, or bulkiness, or inaccuracy of classification criteria. Kliniko-morfologicheski distinguish the following types To. (fig. 1):
1) an oxycephalia (see. Tower skull );
2) scaphocephaly (carinate skull);
3) plagiotsefaliya (slanting skull);
4) bench-formed skull (batrotsefaliya);
5) flat skull (platycephaly);
6) triangular skull (trigonotsefaliya);
7) narrow skull (leptotsefaliya);
8) craniofacial dysostosis (see);
9) an akrotsefalosindaktiliya (see. Apera syndrome ).
Each of these forms K. can be as compensated, and dekompensirovanny. The long time was considered that mechanisms of compensation turn on only in connection with increase in intracranial pressure. Supporters of the vascular theory of a pathogeny To. consider that the organism compensates the available vascular changes and the decompensation can develop or as «venous encephalopathy» or ischemia of a brain.
Clinically compensated form K. it is shown only by change of a shape of the head. The insignificant phenomena of a decompensation can be shown by periodic headaches. At the expressed decompensation, in addition to the pristupoobrazny nature of headaches, nausea, vomiting, congestive changes on an eyeground with transition to an atrophy of optic nerves are observed or their primary atrophy develops, sight decreases. There are signs of defeat and other cranial nerves, change fiziol, reflexes, quite often arise epileptic seizures. Changes of intelligence meet rather seldom.
The diagnosis, as a rule, does not represent special difficulties. Characteristic outward of the patient with an unusual shape of a skull (fig. 2) and data rentgenol, inspections allow to establish the diagnosis without additional researches. Rentgenol. a research of a skull at To. it is directed first of all to identification of signs of premature fusion of seams and deformations of a skull.
At early stages of development To. find only narrowing of one or several seams, most often sagittal and coronal, and a moderate osteosclerosis of bone edges of a seam, sometimes on rather small extent.
In the expressed stage To. deformation of a skull gains more rough character, there is a full ossification of one or several seams to consolidation and a thickening of a bone on the course of a seam up to formation of bone crests. Bones of a base of skull with shortening and deepening of cranial poles, lowering of a roof of orbits and reduction of their volume are deformed that sometimes leads to development of an exophthalmos.
Increase in intracranial pressure is shown on kraniogramm (fig. 3) by thinning of bones of the arch, strengthening of the drawing of finger-shaped impressions (a symptom of «shod silver»), deepening of furrows of sine, education of additional venous graduates, change of the Turkish saddle and discrepancy of not obliterated seams.
Except a kraniografiya, at To. sometimes according to special indications apply contrast methods of a research, in particular pneumoencephalography (see), allowing to establish narrowing of side cerebral cavities and internal hydrocephaly (see). For detection of various accompanying anomalies of development of the main vessels of the head and neck, carotid and vertebrobazilyarny systems apply a vertebralny and carotid angiography (see. Vertebralnaya angiography , Carotid angiography ).
To. it is necessary to differentiate with cephalhematoma (see), nanocephalia (see), a Down syndrome (see. Down disease ), fenilketonuriya (see), deformations of a skull as a result of transferred rickets (see).
Treatment of patients To. can be both conservative, and operational. Patients To. with well compensated intracranial blood circulation do not need treatment. If there are passing signs of disturbance of intracranial blood circulation, then it is necessary to apply the corresponding pharmaceuticals with introduction of certain restrictions of a duty, food etc. In cases of the long decompensation of intracranial blood circulation which is followed by threat of loss of sight there is a need of an operative measure. If at the patient To. there is a decompensation like «venous encephalopathy», operative measures on bones of a calvaria — fragmentation according to King (a section of bones of a calvaria on separate fragments), Bagdasar's operation — Arseni (a resection of bones of a calvaria within 1 — 2 cm on the course of sagittal, coronary, parietal, lambdoid seams), Arendt's operation (use of a section of a frontal bone for increase in capacity of a skull), etc. are admissible. At a decompensation like ischemia of a brain make various operative measures on the main vessels of the head depending on the nature of the pathology found at an angiography in them.
the Forecast for life at To., as a rule, favorable. In cases of untimely treatment because of considerable decrease in visual acuity or a blindness working ability of patients sharply decreases. Considering that a decompensation of intracranial blood circulation at To. can come at any age under the influence of adverse environmental factors, the patient To. work in the conditions of harmful production is contraindicated.
Bibliography: Bavli Ya. G. To a question of a skafokefaliya, a yew., SPb., 1908, bibliogr.; Kozyrev V. A. Craniostenosis, L., 1962, bibliogr.; Romodanov A. P. and L I shch e of N to about D. S. Rol of pathology of vessels in development of a craniostenosis, Vopr, neyrokhir., No. 2, page 28, 1969, bibliogr.; Surgery of the central nervous system, under the editorship of V. M. Ugryumov, p.1, page 842, L., 1969; M ii k e R. Neue Gesicht-spunkte zur Pathogenese und Therapie der Kraniosynostose, Acta neurochir. (Wien), Bd 26, S. 293, 1972; Pediatric neurosurgery, ed. by I. J. Jackson a. R. K. Thompson, p. 134, Springfield, 1959; Sehrbundt Y i a 1 e E. T o n d i M. Remarks on the surgical treatment of the craniosynosto-ses, Neurochirurgia (Stuttg.), Bd 18, S. 204, 1975; Tod P. A. a. Y e 1 1 a n d J. D. H. Craniostenosis, Clin. Radiol., v. 22, p. 472, 1971.
A. P. Romodanov; M of X. Fayzulin (rents.).