nnpNeurology, Neuropsychiatry, PsychosomaticsНеврология, нейропсихиатрия, психосоматика2074-27112310-1342"IMA-Press", LLC10.14412/2074-2711-2017-3-62-67nnp-775Research ArticleCLINICAL OBSERVATIONSКЛИНИЧЕСКИЕ НАБЛЮДЕНИЯDandy–Walker malformation is a rare cause of syringomyelia in adultsАномалия Денди–Уокера – редкая причина сирингомиелии у взрослыхЕвзиковГ. Ю.EvzikovG. Yu.

119021, Москва, ул. Россолимо, 11

Кафедра нервных болезней и нейрохирургии

11, Rossolimo St., Moscow 119021Department of Nervous System Diseases and Neurosurgery

mmaevzikov@mail.ruБашлачевМ. Г.BashlachevM. G.

119021, Москва, ул. Россолимо, 11

Кафедра нервных болезней и нейрохирургии

11, Rossolimo St., Moscow 119021Department of Nervous System Diseases and Neurosurgery

БелозерскихК. А.BelozerskikhK. A.

119021, Москва, ул. Россолимо, 11

Кафедра нервных болезней и нейрохирургии

11, Rossolimo St., Moscow 119021Department of Nervous System Diseases and Neurosurgery

ПарфеновВ. А.ParfenovV. A.

119021, Москва, ул. Россолимо, 11

Кафедра нервных болезней и нейрохирургии

11, Rossolimo St., Moscow 119021Department of Nervous System Diseases and Neurosurgery

ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава РоссииРоссияI.M. Sechenov First Moscow State Medical University, Ministry of Health of RussiaRussian Federation201707112017936267Copyright © Evzikov G.Y., Bashlachev M.G., Belozerskikh K.A., Parfenov V.A., 20172017Евзиков Г.Ю., Башлачев М.Г., Белозерских К.А., Парфенов В.А.Evzikov G.Y., Bashlachev M.G., Belozerskikh K.A., Parfenov V.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://nnp.ima-press.net/nnp/article/view/775

Neural tube defects are the most common central nervous system malformation. Dandy–Walker malformation (DWM) is a rare abnormality of the posterior cranial fossa, which is generally diagnosed in the prenatal period or early infancy.The paper describes a case of the late clinical manifestation of DWM, which has caused syringomyelia. All variants of pathological changes within the Dandy–Walker complex very rarely become a cause of syringomyelia. Only four cases of DWM, in which syringomyelia was found in adults, are known.The authors believe that formation of syringomyelia cysts in these patients is associated with the impaired circulation of normal cerebrospinal fluid between the superior cistern and the subarachnoid spaces of the spinal cord due to the caudal dislocation of the cyst formed in the fourth ventricle.Decompression of the craniovertebral junction, by resecting the caudal portion of the cyst in the foramen magnum, and repair of the free communication between the superior cistern and the cerebrospinal fluid spaces of the spinal cord, which is complemented by duroplasty, are pointed out to be an optimal treatment in these patients.

Дефекты нервной трубки являются наиболее частой аномалией развития ЦНС. Аномалия Денди–Уокера (АДУ) – редкая патология задней черепной ямки, которая, как правило, диагностируется в дородовом периоде или раннем младенческом возрасте.Представлено наблюдение поздней клинической манифестации АДУ, которая стала причиной развития сирингомиелии. Все варианты патологических изменений, входящих в «комплекс Денди–Уокера», крайне редко становятся причиной развития сирингомиелии. Известно только четыре случая АДУ, при которой была обнаружена сирингомиелия у взрослых.Авторы полагают, что образование сирингомиелической кисты у таких пациентов связано с нарушением нормальной ликвороциркуляции между большой цистерной и субарахноидальными пространствами спинного мозга вследствие каудальной дислокации кисты, формирующейся в IV желудочке.Указано, что декомпрессия краниовертебрального перехода с резекцией каудальной части кисты, дислоцированной в большое затылочное отверстие, и восстановление свободного сообщения большой цистерны с ликвороносными пространствами спинного мозга, дополненное дуропластикой, – оптимальный метод лечения таких больных.

Аномалия Денди–Уокерапоздняя клиническая манифестациясирингомиелияDandy–Walker malformationlate clinical manifestationsyringomyeliaReferencesTutus S, Ozyurt S, Yilmaz E, et al. Evaluation and prevalence of major central nervous system malformations: a retrospective study. North Clin Istanb. 2014 Dec 8;1(2):78-83. doi: 10.14744/nci.2014.84803. eCollection 2014.Tutus S, Ozyurt S, Yilmaz E, et al. Evaluation and prevalence of major central nervous system malformations: a retrospective study. North Clin Istanb. 2014 Dec 8;1(2):78-83. doi: 10.14744/nci.2014.84803. eCollection 2014.Agrawal S, Dave M, Mahur H, et al. Dandy Walker Syndrome: A Rare Presentation in Adult. J Assoc Physicians India. 2016 Oct; 64(10):112.Agrawal S, Dave M, Mahur H, et al. Dandy Walker Syndrome: A Rare Presentation in Adult. J Assoc Physicians India. 2016 Oct; 64(10):112.Alaywan M, Chahine NA, Hage P, Nachanakian AK. Dandy–Walker malformation: Surgical treatment of 17 cases. Pan arab journal of neurosurgery. 2008;12:25-30.Alaywan M, Chahine NA, Hage P, Nachanakian AK. Dandy–Walker malformation: Surgical treatment of 17 cases. Pan arab journal of neurosurgery. 2008;12:25-30.Cardoso J, Lange MC, Lorenzoni PJ, Scola RH, Werneck LC. Dandy–Walker syndrome in adult mimicking myasthenia gravis. Arq Neuropsiquiatr 2007 Mar;65(1): 173-5.Cardoso J, Lange MC, Lorenzoni PJ, Scola RH, Werneck LC. Dandy–Walker syndrome in adult mimicking myasthenia gravis. Arq Neuropsiquiatr 2007 Mar;65(1): 173-5.Hamid HA. Dandy–Walker Malformation. Egypt. J Hum Genet. 2007 nov; 8(2):115-120.Hamid HA. Dandy–Walker Malformation. Egypt. J Hum Genet. 2007 nov; 8(2):115-120.Singh RK, Shahi M, Mhaske AN. Dandy Walker Syndrome in 5th Decade of Life Case Report. Journal of Dental and Medical Sciences. 2013 nov-dec; 11(1):05-08.Singh RK, Shahi M, Mhaske AN. Dandy Walker Syndrome in 5th Decade of Life Case Report. Journal of Dental and Medical Sciences. 2013 nov-dec; 11(1):05-08.Dandy WE, Blackfan KD. Internal hydrocephalus. An experimental, clinical and pathological study. Am J Dis Child. 1914;8:406-482.Dandy WE, Blackfan KD. Internal hydrocephalus. An experimental, clinical and pathological study. Am J Dis Child. 1914;8:406-482.Taggart TK, Walker AE. Congenital atresia of the foramina of Luschka and Magendie. Arch Neurol Psychiatry. 1942;48:583-612.Taggart TK, Walker AE. Congenital atresia of the foramina of Luschka and Magendie. Arch Neurol Psychiatry. 1942;48:583-612.Benda CE. The Dandy–Walker syndrome or the so-called atresia of the foramen of Magendie. J Neuropathol Exp Neurol. 1954 Jan;13(1):14-29.Benda CE. The Dandy–Walker syndrome or the so-called atresia of the foramen of Magendie. J Neuropathol Exp Neurol. 1954 Jan;13(1):14-29.Romero R, Pilu G, Jeanty P, Ghidini A, Hobbins JC. Prenatal diagnosis of congenital anomalies. Publishing Division of Prentice Hall, 2002. 79 P.Romero R, Pilu G, Jeanty P, Ghidini A, Hobbins JC. Prenatal diagnosis of congenital anomalies. Publishing Division of Prentice Hall, 2002. 79 P.Hart MN, Malamud N, Ellis WG. The Dandy–Walker syndrome. A clinicopathological study based on 28 cases. Neurology. 1972 Aug;22(8):771-80.Hart MN, Malamud N, Ellis WG. The Dandy–Walker syndrome. A clinicopathological study based on 28 cases. Neurology. 1972 Aug;22(8):771-80.Hirsch JF, Pierre-Kahn A, Renier D, et al. The Dandy–Walker malformation. A review of 40 cases. J Neurosurg. 1984 Sep;61(3):515-22.Hirsch JF, Pierre-Kahn A, Renier D, et al. The Dandy–Walker malformation. A review of 40 cases. J Neurosurg. 1984 Sep;61(3):515-22.Gardner WJ. Hydrodynamic factors in Dandy–Walker and Arnold-Chiari malformations. Childs Brain. 1977;3(4):200-12.Gardner WJ. Hydrodynamic factors in Dandy–Walker and Arnold-Chiari malformations. Childs Brain. 1977;3(4):200-12.Gardner WJ, Abdullah AF, McCormack LJ. The varying expression of the embryonal atresia of fourth ventricle in adult. Arnold-Chiari malformation, Dandy–Walker syndrome, «arachnoid cyst» of cerebellum, and syringomyelia. J Neurosurg. 1957 Nov;14(6):591-605.Gardner WJ, Abdullah AF, McCormack LJ. The varying expression of the embryonal atresia of fourth ventricle in adult. Arnold-Chiari malformation, Dandy–Walker syndrome, «arachnoid cyst» of cerebellum, and syringomyelia. J Neurosurg. 1957 Nov;14(6):591-605.Sasaki-Adams D, Elbabaa SK, Jewells V, et al. The Dandy–Walker variant: a case series of 24 pediatric patients and evaluation of associated anomalies, incidence of hydrocephalus, and developmental outcomes. J Neurosurg Pediatr. 2008 Sep;2(3):194-9. doi: 10.3171/PED/2008/2/9/194.Sasaki-Adams D, Elbabaa SK, Jewells V, et al. The Dandy–Walker variant: a case series of 24 pediatric patients and evaluation of associated anomalies, incidence of hydrocephalus, and developmental outcomes. J Neurosurg Pediatr. 2008 Sep;2(3):194-9. doi: 10.3171/PED/2008/2/9/194.Barkovich AJ, Kjos BO, Norman D, Edwards MS. Revised classification of posterior fossa cysts and cystlike malformations based on the results of multiplanar MR imaging. AJR Am J Roentgenol. 1989 Dec;153(6):1289-300.Barkovich AJ, Kjos BO, Norman D, Edwards MS. Revised classification of posterior fossa cysts and cystlike malformations based on the results of multiplanar MR imaging. AJR Am J Roentgenol. 1989 Dec;153(6):1289-300.Tortori-Donati P, Fondelli MP, Rossi A. Cystic malformations of the posterior cranial fossa originating from a defect of the posterior membranous area. Mega cisterna magna and persisting Blake's pouch: two separate entities. Childs Nerv Syst. 1996 Jun;12(6):303-8.Tortori-Donati P, Fondelli MP, Rossi A. Cystic malformations of the posterior cranial fossa originating from a defect of the posterior membranous area. Mega cisterna magna and persisting Blake's pouch: two separate entities. Childs Nerv Syst. 1996 Jun;12(6):303-8.Azab WA, Shohoud SA, Elmansoury TM, et al. Blake's pouch cyst. Surg Neurol Int. 2014 Jul 24;5:112. doi: 10.4103/2152-7806.137533. eCollection 2014.Azab WA, Shohoud SA, Elmansoury TM, et al. Blake's pouch cyst. Surg Neurol Int. 2014 Jul 24;5:112. doi: 10.4103/2152-7806.137533. eCollection 2014.Bano S, Chaudhary V, Yadav S. Congenital Malformation of the Brain. Neuroimaging – Clinical Applications, 2012. http://www.intechopen.com/books/neuroimaging-clinicalapplications/congenital-malformations-of-thebrainBano S, Chaudhary V, Yadav S. Congenital Malformation of the Brain. Neuroimaging – Clinical Applications, 2012. http://www.intechopen.com/books/neuroimaging-clinicalapplications/congenital-malformations-of-thebrainErdal M, Plikcioglu AC, Bikmaz K, Cosar M. Dandy–Walker Complex and Syringomyelia in an Adult: Case Report and Discussion. Neurosurgery. 2003 Jun;52(6):1504-5.Erdal M, Plikcioglu AC, Bikmaz K, Cosar M. Dandy–Walker Complex and Syringomyelia in an Adult: Case Report and Discussion. Neurosurgery. 2003 Jun;52(6):1504-5.Hammond CJ, Chitnavis B, Penny CC, Strong AJ. Dandy–Walker Complex and Syringomyelia in an Adult: Case Report and Discussion. Neurosurgery. 2002 Jan;50(1):191-4.Hammond CJ, Chitnavis B, Penny CC, Strong AJ. Dandy–Walker Complex and Syringomyelia in an Adult: Case Report and Discussion. Neurosurgery. 2002 Jan;50(1):191-4.Milhorat TH. Classification of syringomyelia. Neurosurg. Focus. 2000;8(3):1-6.Milhorat TH. Classification of syringomyelia. Neurosurg. Focus. 2000;8(3):1-6.

The authors declare that there are no conflicts of interest present.