Cerebrovascular Diseases

Original Paper

Cerebral Cavernous Malformations: Patient-Reported Outcome Validates Conservative Management

Bicalho V.C.a · Bergmann A.b · Domingues F.c · Frossard J.T.c · de Souza J.P.B.M.a

Author affiliations

aDepartment of Surgery, Division of Neurosurgery, Hospital Universitário Clementino Fraga Filho and Program of Post-Graduation in Surgical Sciences of the Universidade Federal do Rio de Janeiro, bDepartment of Research, Instituto Nacional de Câncer, and cDepartment of Surgery, Division of Neurosurgery, Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil

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Cerebrovasc Dis 2017;44:313–319

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Article / Publication Details

First-Page Preview
Abstract of Original Paper

Received: June 14, 2017
Accepted: August 07, 2017
Published online: October 03, 2017
Issue release date: December 2017

Number of Print Pages: 7
Number of Figures: 3
Number of Tables: 4

ISSN: 1015-9770 (Print)
eISSN: 1421-9786 (Online)

For additional information: https://www.karger.com/CED

Abstract

Background: Cerebral cavernous malformations (CCM) are clusters of dilated sinusoidal channels lined by a single layer of endothelium. In contradistinction to arteriovenous malformations, these lesions do not have smooth muscle or elastin in their lining and they are angiographically occult, and the MRI is the most sensitive test for CCM detection. CCM are one of the most prevalent vascular malformations of the central nervous system, affecting about 0.4-0.6% of the general population. The main complication of this malformation is the risk of bleeding, which may cause neurological deficits that affect the quality of life (QoL) in patients. When symtomatic, they may be surgically treated for relieving the mass effect and seizures refractory to drug uses, hemorrhage and drug-refractory epilepsy. Patient-reported outcome (PRO) may be a strategy that can be used to evaluate QoL of CCM population and was used in a sample of non-operated patients. Methods: An observational, cross-sectional analysis to evaluate the PRO using the SF-36 and EuroQol 5 dimensions (EQ-5D) questionnaires of QoL added to functional metrics using the Karnofsky Performance Status (KPS) in 49 patients not submitted to intervention and with long-term follow-up. Results: During the 364 person-years of follow-up, there was an average of individual follow-up of 7.42 years. The mean age was 46.8 years (18-84) - 57% of them were female, 71% had superficial lesions, and 65% had the familial form. Comparisons of SF-36 dimensions with KPS graded <100 had a worse score only in terms of the pain (p = 0.04), vitality (p = 0.001), and general state of health (p = 0.03) domains. The domain mental health was worse in patients without surgical indication (p = 0.032). The functional capacity domain had the highest overall grading in the group. The EQ-5D dimensions of mobility (p = 0.03) and pain/discomfort (p = 0.001) were the ones with lower score compared to KPS <100. Conclusion: The study is the first to evaluate, with validated tools, the PRO of non-operated CCM patients and has demonstrated in a selected group of patients that it was possible to achieve long-term clinical stability, thereby maintaining QoL and functional neurological outcome.

© 2017 S. Karger AG, Basel




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References

  1. Robinson JR, Awad IA, Little JR: Natural history of the cavernous angioma. J Neurosurg 1991;75:709-714.
  2. Otten P, Pizzolato GP, Rilliet B, Berney J: [131 cases of cavernous angioma (cavernomas) of the CNS, discovered by retrospective analysis of 24,535 autopsies]. Neurochirurgie 1989;35:82-83, 128-131.
    External Resources
  3. Denier C, Goutagny S, Labauge P, Krivosic V, Arnoult M, Cousin A, et al: Mutations within the MGC4607 gene cause cerebral cavernous malformations. Am J Hum Genet 2004;74:326-337.
  4. Al-Shahi Salman R, Hall JM, Horne MA, Moultrie F, Josephson CB, Bhattacharya JJ, et al: Untreated clinical course of cerebral cavernous malformations: a prospective, population-based cohort study. Lancet Neurol 2012;11:217-224.
  5. Gross BA, Lin N, Du R, Day AL: The natural history of intracranial cavernous malformations. Neurosurgical Focus 2011;30:E24.
  6. Vaquero J, Leunda G, Martínez R, Bravo G: Cavernomas of the brain. Neurosurgery 1983;12:208-210.
  7. Kupersmith MJ, Kalish H, Epstein F, Yu G, Berenstein A, Woo H, et al: Natural history of brainstem cavernous malformations. Neurosurgery 2001;48:47-53; discussion 53-54.
  8. Taslimi S, Modabbernia A, Amin-Hanjani S, Barker FG 2nd, Macdonald RL: Natural history of cavernous malformation: systematic review and meta-analysis of 25 studies. Neurology 2016;86:1984-1991.
  9. Barker FG 2nd, Amin-Hanjani S, Butler WE, Lyons S, Ojemann RG, Chapman PH, et al: Temporal clustering of hemorrhages from untreated cavernous malformations of the central nervous system. Neurosurgery 2001;49:15-24; discussion 24-25.
  10. Cella D, Yount S, Rothrock N, Gershon R, Cook K, Reeve B, Ader, D Fires JF, Bruce B, Rose,M; PROMIS Cooperative Group: The patient-reported outcomes measurement information system: progress of an NIH roadmap cooperative group in the first two years. Med Care 2007;45 (suppl 1):S3-S11.
  11. Basch E: Patient-reported outcomes harnessing patientʼs voices to improve clinical care. N Engl J Med 2017;276:105-107.
  12. Revicki DA, Kawata AK, Harnan N, Chen WH, Hays RD, Cella D: Predicting EuroQoL (EQ-5D) scores from the patient-reported outcomes measurement information system (PROMIS) global items and domain item banks in a United States sample. Qual Life Res 2009;18:783-791.
  13. Brazier JE, Harper R, Jones NM, O'Cathain A, Thomas KJ, Usherwood T, et al: Validating the SF-36 health survey questionnaire: new outcome measure for primary care. BMJ 1992;305:160-164.
  14. Ciconelli RM, Ferraz MB, Santos W, Meinão I, Quaresma MR: Tradução para língua portuguesa e validação do questionário genérico de avaliação de qualidade de vida SF-36 (Brasil SF-36). Rev Bras Reumatol 1999;39:143-150.
  15. Wolfs CA, Dirksen CD, Kessels A, Willems DC, Verhey FR, Severens JL: Performance of the EQ-5D and the EQ-5D+C in elderly patients with cognitive impairments. Health Qual Life Outcomes 2007;5:33.
  16. EuqoQol Group: EQ-5D: a standardised instrument for use a measure of health outcome EQ-5D translations. August 7, 2016.
  17. Yates JW, Chalmer B, McKegney FP: Evaluation of patients with advanced cancer using the Karnofsky performance status. Cancer 1980;45:2220-2224.
  18. Schag CC, Heinrich RL, Ganz PA: Karnofsky performance status revisited: reliability, validity, and guidelines. JClin Oncol 1984;2:187-193.
  19. O'Dell MW, Lubeck DP, O'Driscoll P, Matsuno S: Validity of the Karnofsky Performance Status in an HIV-infected sample. JAcquir Immune Defic Syndr Hum Retrovirol 1995;10:350-357.
    External Resources
  20. Abla AA, Lekovic GP, Turner JD, de Oliveira JG, Porter R, Spetzler RF: Advances in the treatment and outcome of brainstem cavernous malformation surgery: a single-center case series of 300 surgically treated patients. Neurosurgery 2011;68:403-414; discussion 14-15.
  21. Batra S, Lin D, Recinos PF, Zhang J, Rigamonti D: Cavernous malformations: natural history, diagnosis and treatment. Nat Rev Neurol 2009;5:659-670.
  22. Del Curling O Jr, Kelly DL Jr, Elster AD, Craven TE: An analysis of the natural history of cavernous angiomas. J Neurosurgery 1991;75:702-708.
  23. Moriarity JL, Wetzel M, Clatterbuck RE, Javedan S, Sheppard JM, Hoenig-Rigamonti K, et al: The natural history of cavernous malformations: a prospective study of 68 patients. Neurosurgery 1999;44:1166-1171; discussion 72-73.
    External Resources
  24. Al-Shahi Salman R, Berg MJ, Morrison L, Awad IA; Angioma Alliance Scientific Advisory Board: Hemorrhage from cavernous malformations of the brain: definition and reporting standards. Angioma Alliance Scientific Advisory Board. Stroke 2008;39:3222-3230.
  25. Moriarity JL, Clatterbuck RE, Rigamonti D: The natural history of cavernous malformations. Neurosurg Clin N Am 1999;10:411-417.
    External Resources
  26. Kupersmith MJ, Kalish H, Epstein F, Yu G, Berenstein A, Woo H, et al: Natural history of brainstem cavernous malformations. Neurosurgery 2001;48:47-53; discussion 53-54.
  27. Hasegawa T, McInerney J, Kondziolka D, Lee JY, Flickinger JC, Lunsford LD: Long-term results after stereotactic radiosurgery for patients with cavernous malformations. Neurosurgery 2002;50:1190-1197; discussion 7-8.
  28. Tanriover G, Boylan AJ, Diluna ML, Pricola KL, Louvi A, Gunel M: PDCD10, the gene mutated in cerebral cavernous malformation 3, is expressed in the neurovascular unit. Neurosurgery 2008;62:930-938; discussion 8.
  29. Riant F, Bergametti F, Ayrignac X, Boulday G, Tournier-Lasserve E: Recent insights into cerebral cavernous malformations: the molecular genetics of CCM. FEBS J 2010;277:1070-1075.
  30. Washington CW, McCoy KE, Zipfel GJ: Update on the natural history of cavernous malformations and factors predicting aggressive clinical presentation. Neurosurg Focus 2010;29:E7.
  31. Girard R, Khanna O, Shenkar R, Zhang L, Wu M, Jesselson M, et al: Peripheral plasma vitamin D and non-HDL cholesterol reflect the severity of cerebral cavernous malformation disease. Biomark Med 2016;10:255-264.
  32. Gross BA, Lin N, Du R, Day AL: The natural history of intracranial cavernous malformations. Neurosurg Focus 2011;30:E24.

Article / Publication Details

First-Page Preview
Abstract of Original Paper

Received: June 14, 2017
Accepted: August 07, 2017
Published online: October 03, 2017
Issue release date: December 2017

Number of Print Pages: 7
Number of Figures: 3
Number of Tables: 4

ISSN: 1015-9770 (Print)
eISSN: 1421-9786 (Online)

For additional information: https://www.karger.com/CED


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