Systematic dysplasia of connective tissue and clinical manifestation of intracranial aneurysms

Objective: to estimate the frequency of systematic connective tissue dysplasia at patients with intracranial aneurysms (IA) and its influence on clinical manifestation of intracranial aneurysms. Material and methods: The conducted examination of 199 patients with confirmed intracranial aneurysms (IA) comparing with 194 patients in control group (healthy donors) revealed 12 following external markers of deficiency (dysplasia) of connective tissue with the frequency of these markers statistically much more in examined group than in control group: visible vessels of face and chest (59,8%), scoliosis (44,7%), varicose veins on legs (39,7%), flatfoot (34,6%), skin hyperextensible in supraclavicular region - more than 4,0 cm (33,6%), spontaneous nasal hemorrhage (25,6%), easy formation of bruises (20,6%), abdominal hernia (13,6%), paradontosis (10,5%), chest distortion (7,5%), abdominal striae (3,5%), joints hypermobility (2,5%). Results: The mean number of markers at patients with IA was 3,07 comparing with 1,17 at patients in control group. The systematic connective tissue disorders at patients with IA were confirmed by morphological examinations of intraoperative biopsy samples of superficial temporal artery (STA) and skin of temporal region, which were conducted at 50 patients with IA comparing with 18 persons in control group (patients with head injury). The signs of mesenchymal dystrophia were revealed at 98,0% patients with IA while investigating the STA biopsy samples and at 82,0% patients - in biopsy samples of temporal region skin. Conclusion: The most expressed changes were seen at patients with presence of 3 and more external markers. The following clinical features were seen at patients with IA with number of external dysplasia markers 3 and more: multiple aneurysms were seen 5 times more among such patients comparing with patients with the number of markers less than 3 (р=0,005) as well as ischemic complications during intracranial hemorrhage (р=0,04).

интракраниальные аневризмы, факторы риска, соединительно-тканные нарушения, мезенхимальная дистрофия, множественные аневризмы, intracranial aneurysms, risk factors, connective tissue disorders, mesenchymal dystrophia, multiple aneurysms

1. Медведев Ю.А., Берснев В.П., Забродская Ю.М. О сегментарном строении артериального круга большого мозга, сочленениях в нем и ранее неизвестной болезни этих сочленений // Нейрохирургия. - 1998. - № 2. - С. 9-17.

2. Серов В.В., Шехтер А.Б. Соединительная ткань. - М.: Медицина, 1981. - 312 с.

3. Шишкина Л.В., Лазарев В.А., Мещерякова А.В., Принцева О.Ю. Патология соединительной ткани у больных с аневризмами головного мозга (синдром Элерса-Данло) // Арх. патол. - 1993. - № 4. - С. 16-20.

4. Arn P.H., Scherer L.R., Haller J.A. Jr., Pyeritz R.E. Outcome of pectus excavatum in patients with Marfan syndrome and in the general population // J. Pediatr. - 1989. - № 115. - P. 954-958.

5. Berg H.W.M., Bijlsma J.B., Pires J.A.V. et al. Familial association of intracranial aneurysms and multiple congenital anomalies // Arch. Neurol. - 1986. - Vol. 43. - P. 30-33.

6. Campbell B. Varicose veins and their management // B.M.J. - 2006. - Vol. 333. - № 5. - P. 287-92.

7. Dittrich R., Heidbreder A., Rohsbach D. et al. Connective tissue and vascular phenotype in patients with cervical artery dissection // Neurology. - 2007. - № 12. - P. 2120-2124.

8. Gonzales T.S., Coleman G.C. Periodontal manifestations of collagen vascular disorders // Periodontol. - 2000. - Vol. 21. P. - 94-105.

9. Grahame R., Bird H.A., Child A. The revised (Brighton 1998) criteria for the diagnosis of benign joint hypermobility syndrome (B.J.H.S.) // J. Rheumatol. - 2000. - Vol. 27. - P. 1777-1786.

10. Grahame R. Joint hypermobility and genetic collagen disorders: are they related? // Arch. Dis. Child. - 1999. - Vol. 80. - P. 188-191.

11. Grond-Ginsbach C., Schnippering H., Hausser I. et al. Ultrastructural connective tissue aberrations in patients with intracranial aneurysms // Stroke. - 2002. - Vol. 33. P - 2192-2198.

12. Khan A.A., Eid R.A., Hamdi A. Structural changes in the tunica intima of varicose veins: a histopathological and ultrastructural study // Pathology. - 2000. - Vol. 32. P. - 253-260.

13. Lea R.D., Gerhardt J.J. Range-of-motion measurements // J. Bone Joint Surg. Am. - 1995. - Vol. 77. P. - 784-798.

14. Pope F.M., Smith R. Inherited defects of connective tissue. In: Pope F.M., Smith R. Color atlas of inherited connective tissue disorders. - London: Mosby-Wolfe., 1995. - P. 1-45.

15. Pottkotter L. Striae and systemic abnormalities of connective tissue // J.A.M.A. - 1989. 262. - P. 3132.

16. Richards B.S., Bernstein R.M., D’Amato C.R., Thompson G.H. Standardization of criteria for adolescent idiopathic scoliosis brace studies: SRS Committee on Bracing and Nonoperative Management // Spine. - 2005. - Vol. 15. P. - 2068 2075.

17. Ruigrok Y.M., Rinkel G.J.E., Wijmenga C. Genetics of intracranial aneurysms // Lancet Neurology. - 2005. - № 4. - P. 179-189.

18. Schievink W.I., Michels V.V., Piepgras D.G. Neurovascular manifestation of heritable connective tissue disorders // Stroke. - 1994. - Vol. 25. - P. 889 - 903.

19. Shievink W.I. Genetics and aneurysm formation // Neurosurg. Clin. N. Am. - 1998. - Vol. 9. - P. 485-495.

20. Stehbens W.E. Pathogenesis of idiopathic scoliosis revisited // Exp. Mol. Pathol. - 2003. Vol. 74. P. - 49-60.

21. Tareco J.M., Miller N.H., MacWilliams B.A., Michelson J.D. Defining flatfoot // Foot Ankle Int. - 1999. - Vol. 20. P. - 456-460.

22. van Gijn J., Kerr R.S., Rinkel G.J. Subarachnoid haemorrhage // Lancet. - 2007.- Vol. 27. - P. 306-318.

23. Wolswijk R.H., Ruigrok Y.M., Rinkel G.J., Brilstra E.H., Engelbert R.H. Searching for subtle features of laxity of connective tissue in patients with ruptured intracranial aneurysms: a pilot study // Cerebrovasc. Dis. - 2007. - Vol. 24. P. - 51-56.