The spectrum of clinical manifestations of the pediatric stroke is wide enough – from mild focal and isolated general cerebral symptoms to the formation of a significant neurologic deficiency with a predisposition to recurrence with the subsequent sustained disability and a high risk of fatality. Thanks to a widespread distribution and technical improvement of neuroimaging methods, the pediatric stroke is diagnosed with the ever increasing frequency. Nevertheless, the low awareness of neurologists about the CVD problem in childhood, including the transitory ischemic attacks (TIAs), frequently leads to difficulties in diagnostics and, consequently, to delayed and insufficient medical aid. Due to the variety of reasons, clinical manifestations and the course of the pediatric stroke, selecting the patient management approach becomes difficult, especially, in neurology and brain surgery departments in small city hospitals, where medical specialists lack sufficient experience in treatment of this disease.
This book presents basic literature data on etiology, pathogenesis, clinical manifestations of a pediatric stroke, examination methods and approach to the conservative and surgical treatment of acute and chronic cerebral ischemia in children as well as our own studies on diagnostics and results of conservative and surgical treatment of children with the disease onset at the age of the 1-st day of life up to 18 years old, all obtained on the basis of Burdenko Neurosurgery Institute and FSBEI of Higher Professional Education «Urals State Medical University» of the Ministry of Healthcare of the Russian Federation.
This book will enable a wide range of pediatric specialists to get an idea about the specific features of a pediatric ischemic stroke, its diagnostics, conservative treatment principles and options for surgical treatment of this disease.
Chapter I.
Pediatric stroke. General information
Nowadays, the mortality rate of cerebrovascular diseases in Russia is one of the world’s highest. A cerebrovascular disease holds one of the first places among the most frequent mortality and disability causes, just as in economically developed countries too. The World Health Organization’s Multinational Monitoring of Trends and Determinants in Cardiovascular Disease (MONICA) determines the course of a stroke as «a sudden neurologic deficiency sustaining for over 24 hours, or a sudden death». This definition includes both ischemic and hemorrhagic strokes [262].
In a pediatric population, an ischemic stroke is a less frequent pathology as compared to the adult population. Strokes occur in children of any age [33]. Delayed or erroneous diagnosis of a stroke in children still remains a common enough event [80; 104].
The descriptions of individual clinical cases of cerebrovascular diseases in children can be found in literature since the 17-th century. The first description of a stroke in a child is considered to be made by T. Willis in 1667. J. Wepfer (1658) mentioned sick children, who had a hemiplegia, which was emerging and regressing within a day or faster [278]. The disease termed as «an infantile hemiplegia» was presented in the works by W. Osler (1889), B. Sachs and F. Peterson (1890) as well as S. Freud (1893) in a series of pediatric patients, who had suffered a stroke. It was only in 1927 that F. Ford and A. Schaffer published the first ever systematized description of methods for assessment and treatment of children with ischemic strokes. The authors analyzed the etiology of a pediatric stroke as well as the methods and the results of treatment, which had a subsequent effect on the quality of life [98]. V. Hachinski (1982) described non-specific symptoms, such as a headache and syncopes [118]. It is important to note that many problems outlined by them still remain pertinent even today.
The works by M. Norman (1957), C. Fischer (1959), E. Frantzen (1961), E. Bickertaff (1964), J. Jackson (1970), J. Abraham (1971), W. Kannel (1972) about pediatric strokes are, doubtless, interesting, although these publications did not contain any mentioning of the transient cerebrovascular diseases in childhood [131]. In later studies, the transient cerebrovascular diseases, or, in other terms, the transitory ischemic attacks (TIAs), were noted to occur in children much more frequently than strokes [209]. In 2006 G. Ganesan et al. published an article on the results of a retrospective (from 1978 to 1990) and prospective (from 1990 to 2000) survey of children, who had suffered a stroke, with the use of a neuroimaging. They described 212 patients, including 97 ones with an erroneous initial diagnosis. 79 children were noted to have a growing neurologic deficiency (29 strokes, 46 TIAs, 4 fatal cases due to a recurrent stroke), while during the analysis of the subsequent 5 years 51 children (67%) were noted to have recurrent episodes of cerebrovascular diseases [104].
The number of publications on the subject of a pediatric stroke is growing worldwide with every year. In recent years, educational seminars and topical sessions on this problem appeared in the European Stroke Organization Congress program (Nice, 2014; Glasgow, 2015; Barcelona, 2016). Nowadays, practitioners working abroad can be guided by two manuals: an American one – «Management of Stroke in Infants and Children» released in 2008 and a European one – «Stroke and cerebrovascular disease in childhood» published in 2011 in London [105; 267]. Thus, practical manuals accumulating the results of scientific research and permitting to make clinical decisions are solitary and rarely updated.
The lack of universally accepted international recommendations or guidelines hampers the choice of approaches to treatment and prevention of pediatric strokes. A low awareness of pediatric neurologists on the problem of pediatric strokes and TIAs often leads to difficulties in diagnostics and inadequate therapy of pediatric patients and, therefore, to a delayed and inadequate care, which was noted by V.P. Zykov (2008), F. Kirkham (2011) and A. Mallick (2014) in their works. The main drawback of the research studies presented in literature consists in the fact that only some individual states were specified as those relevant to risks, which, as a rule, was determined by the specialty of a research team (infectologists, rheumatologists, geneticists, hematologists, etc.). The paucity of assessed sampling children in these studies and the restriction of data acquisition to a specific age group (infants, teenagers, etc.) hampered the potential generalization of results obtained within the boundaries of all age groups.
1. Epidemiology
The rate of strokes in the adult population of the Russian Federation is about 500,000 cases per annum, the average stroke morbidity rate is 4.6 incidents per 1,000 of the population annually (the NSA data, 2003).
According to literature data, the average global pediatric stroke morbidity rate varies within a range of 0.93 – 13 incidents per 100,000 of the population annually [33]. According to data of the American Heart Association & American Stroke Association, 2012, the highest stroke incidence rate is noted during the first year of life – approximately 1 incident per 4,000 liveborns [219]. The stroke morbidity rate in children aged from 0 to 15 years in the USA is 6.4 incidents per 100,000 children [222]. During the last 10 years, this number remained stable, but the recent research showed that the incidence rate was 3—4 times higher, than it had been stated earlier [39].
M. Giroud et al. (France, 1995) report that in children below 16 years old the CVD incidence rate reaches 13 incidents per 100,000 children annually. This number includes 7.9 cases of ischemic CVD per 100,000 children and 5.1 cases of hemorrhagic CVD [110].
According to data of the Canadian Pediatric Ischemic Stroke Registry (CPISR), in 2000 this parameter was recorded at the level of 2.7 incidents per 100,000 annually [160]. F. Kirkham et al. reported that in 2004 the incidence rate of CVD in British children was 13 incidents per 100,000 of pediatric population [143].
Thus,