THE ROLE OF DISPOSITION OF CONNECTING TISSUE IN THE RISE OF NONTRAUMATIC SUBARACHNOIDAL HEMORRHAGE IN YOUNG AGE

Purpose. To identify possible effects of systemic dysmorphogenesis of connective tissue on the formation and characteristics of the course of nontraumatic subarachnoid hemorrhage at a young age, with further development of an algorithm for screening physical examination of patients with an increased risk of aneurysmal hemorrhage.

Patients and methods. A group of examined patients who underwent SAH under the age of 45 years were 78 people. Methods: neurological examination according to a conventional scheme using traditional methods with assessment of the main functional systems and determining the depth of their damage, determination of constitutional types, assessment of the degree of phenotypic manifestation of DCT by the sum of scores, general clinical laboratory, neuroimaging, and statistical.

In order to determine the signs of connective tissue pathology in all patients with SAH, the general examination included determining the constitution, height, weight with the definition of the Ketle index. Attention was also paid to the presence of visible small vessels on the face, chest and other parts of the body, telangiectasias, angiomas, thoracic cell deformities, scoliosis, flat feet, arachnodactyly, diastase of the rectus abdominis muscles, hernia of the anterior abdominal wall. Among the many undifferentiated phenotypic signs of DCT, external (changes in the skin and its derivatives, changes in bones, joints, spine, features of the structure of the hands and feet, peculiarities of the structure of the auricle, eyes, oral cavity, craniocephalic features) and internal (the presence of additional chords, heart valve prolapses, nephroptosis and kidney development abnormalities).

Results. When examining 78 patients with subarachnoid hemorrhage at a young age, the risk factors for its development were revealed (non-closed Willisian circle (86.6%), pathological tortuosity of the cerebral vessels (81.6%), dysplastic changes in the heart (68.3%) and kidneys (63.3%), which significantly differed from the comparison group (arterial hypertension – 97.8%, heart disease (IHD, atrial fibrillation) – 53.2%, hypercholesterolemia – 97.8%.) The value of systemic dysplastic change in connective tissue in the formation and flow of SAC at young age is shown. The constitutional-anatomical and functional features, characteristic for external and internal phenotypic signs of connective tissue dysplasia in different age groups, are revealed, and the role of additional vascular risk factors in the course of SAH are found. Conclusion. The complex study of connective tissue dysplasia as a risk factor for the development of aneurysms with a clinical manifestation in the form of SAH at a young age can be used in clinical practice for the timely detection and surgical treatment of aneurysms without signs of SAH. The obtained data can become a basis for developing an algorithm for screening physical examination of patients with an increased risk of aneurysmal hemorrhage.

1. Displaziya soedinitel’noi tkani [Dysplasia of connective tissue]. Edited by T.I.Kadurina, V.N.Gorbunova. St.Petersburg: «Elbi» Publ., 2009, 714 p. (In Russian).

2. Zemtsovskyi EV, Malev EG, Reeva SV, Luneva EB, Parfenova NN, Lobanov MYu, et al. Diagnostics of inherited connective tissue disorders: achievements and future directions. Russian Journal of Cardiology. 2013;4(102):38-44. (In Russian).

3. Evtushenko SK, Lisovskii EV, Evtushenko OS. Displaziya soedinitel’noi tkani v nevrologii i pediatrii [Dysplasia of connective tissue in neurology and pediatrics]. Donetsk: Publishing house «Zaslavskii», 2009, 361 p. (In Russian).

4. Klemenov AV. Hererditary connective tissue disorders: unresolved questions and controversial issues. Medical news of the North Caucasus. 2016;11(2):358-69. DOI: 10.14300/ mnnc.2016.11078 (In Russian).

5. Kadurina TI, Abbakumova LN. Сonnective tissue dysplasia: way to diagnosis. Bulletin of the Ivanovo Medical Academy. 2014;19(3):5-11. (In Russian).

6. Hereditary disorders of connective tissue. Russian recommendations. All-Russian Scientific Society of Cardiology. Russian Journal of Cardiology. Приложение 1. 2012;4 S1(96):32. (In Russian).

7. Nesterenko ZV. Fenomen displazii soedinitel’noi tkani. Ukraїns’kii medichnii al’manakh. 2008;4:105-9. (In Russian).

8. Nechaeva GI, Viktorova IA. Displaziya soedinitel’noi tkani: terminologiya, diagnostika, taktika vedeniya patsientov [Dysplasia of connective tissue: terminology, diagnosis, tactics of patient management]. Omsk: «BLANKOM» Publ., 2007, 188 p. (In Russian).

9. Nechaeva GI, Viktorova IA, Demina GI, Glotov AV. Displastikozavisimaya i assotsiirovannaya patologiya pochek – odin iz mekhanizmov formirovaniya arterial’noi gipertenzii pri displazii soedinitel’noi tkani. Konsilium. 2002;1:15-18. (In Russian).

10. Lebedeva ER, Kolotvinov VS, Sakovich VP, Medvedeva SJu. Systematic dysplasia of connective tissue and clinical manifestation of intracranial aneurysms. The Russian Journal of Neurosurgery (Neirokhirurgiya). 2013;2:42-8. (In Russian).

11. Jaja BNR, Saposnik G, Lingsma HF, Macdonald E, Thorpe KE, Mamdani M, et al. Development and validation of outcome prediction models for aneurysmal subarachnoid haemorrhage: the SAHIT multinational cohort study. BMJ. 2018 Jan 18;360:j5745. DOI: 10.1136/bmj.j5745.

12. Al-Khindi T, Macdonald RL, Schweizer TA. Cognitive and functional outcome after aneurysmal subarachnoid hemorrhage. Stroke. 2010 Aug;41(8):e519-36. DOI: 10.1161/ STROKEAHA.110.581975.

13. Lutsik AA, Ratkin IK, Nikitin MN. Kraniovertebral’nye povrezhdeniya i zabolevaniya [Craniovertebral injury and disease]. Novosibirsk, 1998, 235 p. (In Russian).

14. Yakovlev VM, Karpov RS, Shvetsova EV. Soedinitel’notkannaya displaziya mitral’nogo klapana [Connective tissue dysplasia of the mitral valve]. Tomsk, 2003, 144 p. (In Russian).

15. The 2017 International Classification of the Ehlers-Danlos Syndroms. American Journal of Medical Jenetics Part C 2017.-№8 (26).175 р.

16. Kirk JH, Ansell BM, Bywaters EGL. The hypermobility syndrome. Ann Rheum Dis. 1967 Sep;26(5):419-25.

17. Mitral Valve: Floppy Mitral Valve, Mitral Valve Prolapse, Mitral Valvular Regurgitation. Еd by H.Boudoulas, Ch.F.Wooley. NY: Futura Publlishing Company, 2000, 754 p. DOI: doi.org/10.1016/S0300-8932(00)75299-7

18. Hunt W, Hess R. Surgical risk as related to time of intervention in the repair of inracranial aneurisms. Neurosurgery 1968;28:1420. DOI: doi.org/10.3171/jns.1968.28.1.0014

19. Fisher CM, Kistler JP, Davis JM. The correlation of cerebral vasospasm and the amount of subarahnoid blood detected by computerized cranial tomogrphy after aneurism rupture. Cerebral Arterial Spasm. Wilkins RH ed. Baltimore 1980;397-408.

20. Klemenov AV. Hereditary connective tissue disorders: nomenclature and diagnostic algorithm. Klinitsist (The Clinician). 2015;1:43-9. DOI: 10.17650/1818-8338-2015-1-42-49 (In Russian).

21. Hereditary disorders of connective tissue. Clinical recommendations. All-Russian Scientific medical Society of therapeutist, 2017, 180 p. Available at: https://www.rnmot.ru/public/uploads/2018/RNMOT/Клинические%20рекомендации_ДСТ%20сайт%2016.02.18г_.pdf (In Russian).

22. Lubyagina AV. Znachenie faktorov riska khronicheskoi tserebral’noi ishemii v molodom vozraste [The significance of risk factors for chronic cerebral ischemia at a young age]. Diss. Novosibirsk, 2005, 23 p. (In Russian).

23. Popova TF, Gribacheva IA. Implication of the dysplasia of connective tissue in elaboration of chronicle cerebral ischemia. Siberian Medical Journal. 2011;26(3-2):42-6. (In Russian).

24. Lawton MT, Vates GE. Subarachnoid Hemorrhage. N Engl J Med. 2017 Jul 20;377(3):257-266. DOI: 10.1056/NEJMcp1605827