O. Belousova - Pediatric stroke. Revascularization and reconstructive surgery in children with cerebrovascular disease

Pediatric patients find themselves in a hospital, on the average, within the first three hours from the onset of the ACVD symptoms, but they get to the neuroradiologists within 8 hours, whereas for adult patients these deadlines are 8 and 2 hours respectively [63; 103; 179; 247]. Transient motor and/or sensorial disturbances in the structure of partial attacks lead to the initial diagnoses, which are most common in children (epilepsy, neuroinfection, crainio-cerebral injury, etc.) and which, therefore, occupy top positions in the immediate memory of emergency phase doctors [103; 247].

During the analysis of the clinical picture in children with IS, examined by specialists of the FSBEI of Higher Professional Education «Urals State Medical University», the following data on the varying occurrence of clinical symptoms were obtained in 162 children (Table 4). Within the first 24 hours of the disease the comparability was noted between the registration rates of general cerebral and focal neurological symptoms. During the acute period of the IS, the most typical combination of symptoms in children were the degradation of consciousness level and the central pareses of limbs and mimic muscles.

Practically, every fourth child at an age enabling the adequate assessment of these symptoms had signs of ataxia and speech disturbances. Thus, the most prominent combinations of symptoms, which form the diagnostic rules such as «Give me five» 1 1 It includes the first 5 signs of a stroke: a sudden inexplicable headache; weakness in the muscles and/or numbness in the half or part of the body (face, leg, arm); vision disorder with possible diplopia; slurred speech; a sudden loss of balance often combined with nausea and faintness. and FAST 2 2 (Face, Arm, Speech, Time Scale). in stroke diagnostics at the age typical for IS and TIA, can also be successfully applied in pediatric practice.

The spectrum of focal neurologic symptoms in patients was consistent with the blood supply systems and the infarction location. In the hospital stay period, the neurologic deficiency persistence had a direct positive connection with the size of the stroke zone on CT or MRI ( r =0.56, p <0.05).

The rate of admitting children with verified IS ( n =158) and TIA ( n =62) to the specialized care delivery stage was analyzed (Table 2).

Thus, only a little more than half of the children with IS get to the specialized care delivery stage within the reported deadlines accepted for hospitalization of adults – within the boundaries of the so called «time slot» (up to three hours) – 59% ( n =93), when thrombolysis is possible. For children with TIA the onset of focal or general cerebral symptoms did not remain unnoticed also in more than a half of cases – 60% ( n =37). On the other hand, a short-term and transient nature of symptoms in TIA have led to the lack of the emergency hospitalization and to scheduled health-seeking in 40% of children in this group ( n =25).

Blood circulation diseases of the VBS have such typical symptoms as vertigo with nausea and vomit, disorder of static equilibrium and gait, ataxia in limbs and nystagmus, and such less typical symptoms as pareses in the limbs and various sensibility variations, lesions of cerebral nerves (CN) caused by a cerebrovascular disease in the brain stem. In ischemia of occipital lobes there may be some disturbances of visual functions [17].

Table 2:Time passed from the onset of the first ACVD symptoms till the admission to an emergency / neurology specialized healthcare facility

A chronic cerebrovascular ischemia most often manifests itself in signs of a dyscirculatory encephalopathy (DEP) characterized by diffuse-type headaches, vertigo, tinnitus, memory derangements, emotional lability, increased fatigability and performance impairment, sleep disorder. In DEP, general symptoms prevail without focal neurologic symptoms: severe headaches (77%), increased fatigability (68%), hypomnesia (44%) [17]. Decreased learning capacity is typical for such children.

Regarding the specific features of the clinical course of some types of the pathology, it should be mentioned that clinical manifestations of stenosis and occlusion of major cerebral vessels do not have any specific features and can manifest themselves as various ischemic cerebrovascular diseases. A moderate decrease of the cerebral blood flow usually does not manifest itself clinically (asymptomatic disease course), or it may be accompanied by some unspecific complaints.

At the onset of the moyamoya disease and syndrome, their clinical manifestations are rather diverse, and they may resemble the clinical manifestations of cerebrovascular disturbances in pathologic deformations of major cerebral vessels, thrombosis and atherosclerosis of intracranial arteries as well as the manifestations of other diseases (epilepsy, malformations of cerebral vessels, subarachnoid and intra-cerebral hemorrhages of various genesis [192; 196; 270].

Headache is mentioned as a manifesting symptom in a variety of studies on the moyamoya disease occurring in children too. In literature, there is even an individual notion – a headache associated with the moyamoya disease (HAMD) [141; 233; 238; 288]. Headache is often the only symptom at the onset of this disease. The headache is supposed to be caused by a compensatory dilatation of meningeal and leptomeningeal arteries, which can stimulate nociceptive receptors of dura mater of brain (DMB). The headache may have a migraine-like nature and be resistant to a drug therapy. However, this symptom usually is not considered as a fatal sign. In most patients, the headache regresses after the surgery [71; 136; 238].

The subsequent joining of transient focal neurologic symptoms is often considered by neurologists as a manifestation of sub- or de-compensation of residual organic background under the effect of school loads, intense sports activities, viral infections, vaccinations, etc. They are acknowledged as TIAs most often retrospectively, after the verification of the moyamoya disease. A short-term and transient nature of symptoms in children, often combined with the inability to describe their «unusual» complaints verbally, result in delayed help-seeking and late hospitalization. According to literature data, the delayed diagnosing is noted in all patients, and it may exceed two years. The moyamoya disease is usually identified only after the child has suffered a typical IS, which is followed by various neuroimaging examinations. Clinical manifestations in children with the moyamoya disease are distributed in the occurrence rate as follows: ischemic symptoms – 80% of cases (including strokes – 40% and transitory ischemic attacks – 41%) [21; 80; 224; 242; 254]; epilepsy – 5%, intracranial hemorrhages – 2.5%; other symptoms – 12.5% of cases (headache, motor disturbances, or combined symptoms) [72; 80; 224; 242; 254].

A nation-wide Russian recommended list of diagnostic procedures aimed at differential diagnostics of a CVD at an early age does not exist. It is actively discussed and formed in some individual centers. The variability of the CVD causes considerably hinders the diagnostic search. During the most acute and acute disease periods, the efforts are focused on identifying the pathogenic variant of the CVD, primarily, on identification of the most frequent diseases, whose therapy can be started immediately (cardiac pathology, congenital clotting disorders, congenital and acquired pathology of cerebral vessels) with due regard for the age [22; 34]. If the cause has not been determined, it is recommended afterwards to rule out consecutively other, less common, causes of the CVD at an early age [7; 9; 22; 24; 134].