Almanac of Clinical MedicineAlmanac of Clinical MedicineАльманах клинической медицины2072-05052587-9294Moscow Regional Research and Clinical Institute (MONIKI)79510.18786/2072-0505-2018-46-2-146-159REVIEW ARTICLEОБЗОРCavernous malformations in children: a review of the literatureКавернозные мальформации у детей: обзор литературыPopovV. E.ПоповВ. Е.
Russian Federation

Vladimir E. Popov – MD, PhD, Senior Research Fellow, Department of Pediatric Surgery.

61/2–9 Shchepkina ul., Moscow, 129110, tel.: +7 (926) 294 06 79

Попов Владимир Евгеньевич – кандидат медицинских наук, старший научный сотрудник, отделение детской хирургии.

129110, Москва, ул. Щепкина, 61/2–9, тел.: +7 (926) 294 06 79

1popov1@list.ruLivshitsM. I.ЛившицМ. И.
Russian Federation

Matvey I. Livshits – MD, PhD, Associate Professor, Head of the Neurosurgical Department, Center for Treatment of Cerebrovascular Pathology in Children and Adolescents.

1/9 Dobryninskiy pereulok, Moscow, 119049

Лившиц Матвей Игоревич – кандидат медицинских наук, доцент, заведующий нейрохирургическим отделением, Центр по лечению цереброваскулярной патологии у детей и подростков.

119049, Москва, 4-й Добрынинский переулок, 1/9

BashlachevM. G.БашлачевМ. Г.
Russian Federation

Mikhail G. Bashlachev – MD, Neurosurgeon, Clinic of Nervous Diseases named after A.Ya. Kozhevnikov, University Clinical Hospital No 3.

8/2 Trubetskaya ul., Moscow, 119991

Башлачев Михаил Григорьевич – врач-нейрохирург, Клиника нервных болезней им. А.Я. Кожевникова, Университетская клиническая больница № 3.

119991, Москва, ул. Трубецкая, 8/2

NalivkinA. E.НаливкинА. Е.
Russian Federation

Aleksandr E. Nalivkin – MD, PhD, Professor, Course of Pediatric Surgery, Postgraduate Training Faculty.

61/2 Shchepkina ul., Moscow, 129110

Наливкин Александр Евгеньевич – доктор медицинских наук, профессор курса детской хирургии, факультет усовершенствования врачей.

129110, Москва, ул. Щепкина, 61/2

Moscow Regional Research and Clinical Institute (MONIKI)ГБУЗ МО «Московский областной научно-исследовательский клинический институт им. М.Ф. Владимирского»Morozov Children's City Clinical HospitalГБУЗ Морозовская детская городская клиническая больница Департамента здравоохранения города МосквыI.M. Sechenov First Moscow State Medical UniversityФГАОУ ВО Первый Московский государственный медицинский университет имени И.М. Сеченова Минздрава России (Сеченовский университет)060620184621461590506201805062018Copyright ©; 2018, Popov V.E., Livshits M.I., Bashlachev M.G., Nalivkin A.E.Copyright ©; 2018, Попов В.Е., Лившиц М.И., Башлачев М.Г., Наливкин А.Е.2018Popov V.E., Livshits M.I., Bashlachev M.G., Nalivkin A.E.Попов В.Е., Лившиц М.И., Башлачев М.Г., Наливкин А.Е.https://creativecommons.org/licenses/by/4.0https://almclinmed.ru/jour/article/view/795

Cavernous malformation (CM) is a rare vascular disorder of the brain in children. Its prevalence in the general population is in the range of 0.1 to 0.9%, with about one quarter of cases occurring in children. This descriptive review presents an analysis of 17 publications in English and Russian literature on the series of children with cavernomas of the central nervous system and describes their etiology, pathophysiology, clinical particulars, and methods of diagnosis, management, prognosis and outcomes in pediatric practice. In most of the published studies, surgery was performed for 35.3 to 100% of the CM diagnosed during a hemorrhage. The indications for surgical management of CM have been poorly described, and this issue remains unresolved so far. There is also no consensus on the treatment strategy for deeply located cavernomas (brainstem, thalamus and basal ganglia), asymptomatic supratentorial cavernomas and CM located in the functionally important zones of the cortex. Criteria for determination of the intervention volume in patients with CM and symptomatic epilepsy have not been determined, as well. All these issues require further evaluation in multicenter randomized trials.

Кавернозная мальформация (КМ) относится к  редким сосудистым патологиям головного мозга у детей. В общей популяции частота встречаемости КМ составляет от 0,1 до 0,9%, четверть из этих случаев приходится на детский возраст. В обзорной статье проанализированы данные 17 публикаций серий наблюдений детей с каверномами центральной нервной системы. Описаны этиология, патогенез, особенности клинической картины, методы диагностики и лечения, прогноз и исходы лечения данной патологии в педиатрической практике. В большинстве опубликованных исследований хирургическому лечению подверглись от 35,3 до 100% КМ, диагностированных в  период кровоизлияния. Мало описаны показания к хирургическому лечению КМ, и этот вопрос остается открытым по сей день. Не определена и однозначная тактика лечения каверном глубинной локализации (ствол мозга, таламус и базальные ядра), бессимптомных супратенториальных каверном и КМ, расположенных в области функционально значимых зон коры больших полушарий. Не сформированы показания к объему операции у пациентов с КМ и симптоматической эпилепсией. Все эти вопросы требуют дальнейшего изучения в многоцентровых и рандомизированных исследованиях.

cavernous malformationbrain cavernous hemangiomacentral nervous systembrain vascular malformationsepilepsychildrendiagnosissurgerytreatmentoutcomeкавернозная мальформациякавернозная гемангиома головного мозгацентральная нервная системасосудистая патология головного мозгаэпилепсиядетидиагностикахирургическое лечениеисход1. Lynch JK, Han CJ. Pediatric stroke: what do we know and what do we need to know? Semin Neurol. 2005;25(4): 410–23. doi: 10.1055/s2005-923535.2. Jordan LC, Hillis AE. Hemorrhagic stroke in children. Pediatr Neurol. 2007;36(2): 73–80. doi: 10.1016/j.pediatrneurol.2006.09.017.3. Lee JW, Kim DS, Shim KW, Chang JH, Huh SK, Park YG, Choi JU. Management of intracranial cavernous malformation in pediatric patients. Childs Nerv Syst. 2008;24(3): 321–7. doi: 10.1007/s00381-007-0465-8.4. Avci E, Oztürk A, Baba F, Karabağ H, Cakir A. Huge cavernoma with massive intracerebral hemorrhage in a child. Turk Neurosurg. 2007;17(1): 23–6.5. Acciarri N, Galassi E, Giulioni M, Pozzati E, Grasso V, Palandri G, Badaloni F, Zucchelli M, Calbucci F. Cavernous malformations of the central nervous system in the pediatric age group. Pediatr Neurosurg. 2009;45(2): 81–104. doi: 10.1159/000209283.6. Орлов ЮА, Орлов МЮ. Кавернозные мальформации головного мозга у детей (обзор литературы и анализ собственных наблюдений). Український нейрохірургічний журнал. 2004;(4): 38–45.7. Agrawal A, Banode P, Shukla S. Giant cavernous hemangiomas of the brain. Asian J Neurosurg. 2012;7(4): 220–2. doi: 10.4103/17935482.106660.8. Amato MC, Madureira JF, Oliveira RS. Intracranial cavernous malformation in children: a single-centered experience with 30 consecutive cases. Arq Neuropsiquiatr. 2013;71(4): 220–8. doi: http://dx.doi.org/10.1590/0004282X20130006.9. Hugelshofer M, Acciarri N, Sure U, Georgiadis D, Baumgartner RW, Bertalanffy H, Siegel AM. Effective surgical treatment of cerebral cavernous malformations: a multicenter study of 79 pediatric patients. J Neurosurg Pediatr. 2011;8(5): 522–5. doi: 10.3171/2011.8.PEDS09164.10. Corapçioğlu F, Akansel G, Gönüllü E, Yildiz K, Etuş V. Fatal giant pediatric intracranial cavernous angioma. Turk J Pediatr. 2006;48(1): 89–92.11. Gross BA, Smith ER, Goumnerova L, Proctor MR, Madsen JR, Scott RM. Resection of supratentorial lobar cavernous malformations in children: clinical article. J Neurosurg Pediatr. 2013;12(4): 367–73. doi: 10.3171/2013.7.PEDS13126.12. Kim YS, Lee JI, Choi CH, Ko JK. Massive intracerebral hemorrhage caused by a cavernous malformation. J Korean Neurosurg Soc. 2012;51(1): 37–9. doi: 10.3340/jkns.2012.51.1.37.13. Udayakumaran S, Paraskevopoulos D, Cagnano E, Roth J, Constantini S. Spontaneous bleeding into a suprasellar cavernous angioma of a neonate: case report and literature review. Childs Nerv Syst. 2011;27(2): 303–11. doi: 10.1007/s00381-010-1161-7.14. Domingues F, Gasparetto EL, Andrade R, Noro F, Eiras A, Gault J, Correia CE, de Souza JM. Familial cerebral cavernous malformations: Rio de Janeiro study and review of the recommendations for management. Arq Neuropsiquiatr. 2008;66(4): 795–9. doi: http://dx.doi.org/10.1590/S0004-282X2008000600003.15. Yang WC, Chen CY, Wu KH, Wu HP. Acute onset of dizziness caused by a cavernous malformation lateral to the fourth ventricle: a case report. Pediatr Neonatol. 2011;52(2): 113–6. doi: 10.1016/j.pedneo.2011.02.011.16. Al-Holou WN, O'Lynnger TM, Pandey AS, Gemmete JJ, Thompson BG, Muraszko KM, Garton HJ, Maher CO. Natural history and imaging prevalence of cavernous malformations in children and young adults. J Neurosurg Pediatr. 2012;9(2): 198–205. doi: 10.3171/2011.11.PEDS11390.17. Vanaman MJ, Hervey-Jumper SL, Maher CO. Pediatric and inherited neurovascular diseases. Neurosurg Clin N Am. 2010;21(3): 427–41. doi: 10.1016/j.nec.2010.03.001.18. Белоусова ОБ, Коновалов АН, Гаврюшин АВ, Голанов АВ, Окишев ДН, Сазонова ОБ, Хухлаева ЕА, Шишкина ЛВ. Кавернозные мальформации центральной нервной системы. М.: Антидор; 2014. 256 с.19. Кивелев ЮВ. Каверномы мозга. СПб.: Человек и здоровье; 2013. 210 с. 20. Mazza C, Scienza R, Beltramello A, Da Pian R. Cerebral cavernous malformations (cavernomas) in the pediatric age-group. Childs Nerv Syst. 1991;7(3): 139–46. doi: https://doi.org/10.1007/BF00776709.21. Scott RM. Brain stem cavernous angiomas in children. Pediatr Neurosurg. 1990–1991;16(6): 281–6. doi: 10.1159/000120543.22. Giulioni M, Acciarri N, Padovani R, Frank F, Galassi E, Gaist G. Surgical management of cavernous angiomas in children. Surg Neurol. 1994;42(3): 194–9. doi: https://doi.org/10.1016/0090-3019(94)90262-3.23. Di Rocco C, Iannelli A, Tamburrini G. Surgical management of paediatric cerebral cavernomas. J Neurosurg Sci. 1997;41(4): 343–7.24. Mottolese C, Hermier M, Stan H, Jouvet A, Saint-Pierre G, Froment JC, Bret P, Lapras C. Central nervous system cavernomas in the pediatric age group. Neurosurg Rev. 2001;24(2– 3): 55–71.25. Хачатрян ВА, Самочерных КА, Трофимова ТН. Цереброваскулярная патология у детей. СПб.: Десятка; 2006. 278 c.26. Mottolese C, Hermier M, Szathmari A, Bruno C. Cavernous malformations in children and adolescents. In: Lanzino G, Spetzler RF, editors. Cavernous malformations of the brain and spinal cord. New York: Thieme; 2007. p. 114–24.27. Xia C, Zhang R, Mao Y, Zhou L. Pediatric cavernous malformation in the central nervous system: report of 66 cases. Pediatr Neurosurg. 2009;45(2): 105–13. doi: 10.1159/000209284.28. Consales A, Piatelli G, Ravegnani M, Pavanello M, Striano P, Zoli ML, Capra V, Rossi A, Garrè ML, Calevo MG, Cama A. Treatment and outcome of children with cerebral cavernomas: a survey on 32 patients. Neurol Sci. 2010;31(2): 117–23. doi: 10.1007/s10072-009-0157-0.29. Bigi S, Capone Mori A, Steinlin M, Remonda L, Landolt H, Boltshauser E. Cavernous malformations of the central nervous system in children: presentation, treatment and outcome of 20 cases. Eur J Paediatr Neurol. 2011;15(2): 109–16. doi: 10.1016/j.ejpn.2010.09.004.30. Song J, Qiao N, Xie L, Qiu T, Li P, Wu J, Zhu W, Chen L, He KM, Zhang N, Mao Y, Zhao Y. Clinical features and microsurgical treatment of pediatric patients with cerebral cavernous malformation. J Clin Neurosci. 2011;18(10): 1303–7. doi: 10.1016/j.jocn.2011.01.033.31. Gross BA, Du R, Orbach DB, Scott RM, Smith ER. The natural history of cerebral cavernous malformations in children. J Neurosurg Pediatr. 2016;17(2): 123–8. doi: 10.3171/2015.2.PEDS14541.32. Knerlich-Lukoschus F, Steinbok P, Dunham C, Cochrane DD. Cerebellar cavernous malformation in pediatric patients: defining clinical, neuroimaging, and therapeutic characteristics. J Neurosurg Pediatr. 2015;16(3): 256–66. doi: 10.3171/2015.1.PEDS14366.33. Noh JH, Cho KR, Yeon JY, Seol HJ, Shin HJ. Microsurgical treatment and outcome of pediatric supratentorial cerebral cavernous malformation. J Korean Neurosurg Soc. 2014;56(3): 237–42. doi: 10.3340/jkns.2014.56.3.237.34. Bertalanffy H, Kühn G, Scheremet R, Seeger W. Indications for surgery and prognosis in patients with cerebral cavernous angiomas. Neurol Med Chir (Tokyo). 1992;32(9): 659–66. doi: https://doi.org/10.2176/nmc.32.659.35. von der Brelie C, Kuczaty S, von Lehe M. Surgical management and long-term outcome of pediatric patients with different subtypes of epilepsy associated with cerebral cavernous malformations. J Neurosurg Pediatr. 2014;13(6): 699–705. doi: 10.3171/2014.2.PEDS13361.36. Ciricillo SF, Cogen PH, Edwards MS. Pediatric cryptic vascular malformations: presentation, diagnosis and treatment. Pediatr Neurosurg. 1994;20(2): 137–47. doi: 10.1159/000120776.37. Koike T, Yanagimachi N, Ishiguro H, Yabe H, Yabe M, Morimoto T, Shimizu T, Takakura H, Kato S. High incidence of radiation-induced cavernous hemangioma in long-term survivors who underwent hematopoietic stem cell transplantation with radiation therapy during childhood or adolescence. Biol Blood Marrow Transplant. 2012;18(7): 1090–8. doi: 10.1016/j.bbmt.2011.12.582.ъ38. Lew SM, Morgan JN, Psaty E, Lefton DR, Allen JC, Abbott R. Cumulative incidence of radiation-induced cavernomas in long-term survivors of medulloblastoma. J Neurosurg. 2006;104(2 Suppl):103–7. doi: 10.3171/ped.2006.104.2.103.39. Gross BA, Smith ER, Scott RM. Cavernous malformations of the basal ganglia in children. J Neurosurg Pediatr. 2013;12(2): 171–4. doi: 10.3171/2013.5.PEDS1335.40. Цимейко ОА, Гончаров АИ, Орлов МЮ, Скорохода ИИ, Черненко ОГ. Особенности диагностики и лечения кавернозных мальформаций головного мозга. Український нейрохірургічний журнал. 2006;(2)49–56.41. Li D, Hao SY, Tang J, Xiao XR, Jia GJ, Wu Z, Zhang LW, Zhang JT. Surgical management of pediatric brainstem cavernous malformations. J Neurosurg Pediatr. 2014;13(5): 484–502. doi: 10.3171/2014.2.PEDS13536.42. Zabramski JM, Wascher TM, Spetzler RF, Johnson B, Golfinos J, Drayer BP, Brown B, Rigamonti D, Brown G. The natural history of familial cavernous malformations: results of an ongoing study. J Neurosurg. 1994;80(3): 422–32. doi: 10.3171/jns.1994.80.3.0422.43. Kim DS, Park YG, Choi JU, Chung SS, Lee KC. An analysis of the natural history of cavernous malformations. Surg Neurol. 1997;48(1): 9–17. doi: https://doi.org/10.1016/S00903019(96)00425-9.44. Abla AA, Turner JD, Mitha AP, Lekovic G, Spetzler RF. Surgical approaches to brainstem cavernous malformations. Neurosurg Focus. 2010;29(3):E8. doi: 10.3171/2010.6.FOCUS10128.45. Wieser HG, Blume WT, Fish D, Goldensohn E, Hufnagel A, King D, Sperling MR, Lüders H, Pedley TA; Commission on Neurosurgery of the International League Against Epilepsy (ILAE). ILAE Commission Report. Proposal for a new classification of outcome with respect to epileptic seizures following epilepsy surgery. Epilepsia. 2001;42(2): 282–6. doi: https:// doi.org/10.1046/j.1528-1157.2001.35100.x.46. Engel J Jr. Update on surgical treatment of the epilepsies. Summary of the Second International Palm Desert Conference on the Surgical Treatment of the Epilepsies (1992). Neurology. 1993;43(8): 1612–7. doi: https://doi.org/10.1212/WNL.43.8.1612.