Skip to main content
Log in

Hydrocephalus in Dandy–Walker malformation

  • Special Annual Issue
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Introduction

Even if the first description of Dandy–Walker dates back 1887, difficulty in the establishment of correct diagnosis, especially concerning differential diagnosis with other types of posterior fossa CSF collection, still persists. Further confusion is added by the inclusion, in some classification, of different malformations with different prognosis and therapeutic strategy under the same label of “Dandy–Walker”.

Methods

An extensive literature review concerning embryologic, etiologic, pathogenetic, clinical and neuroradiological aspects has been performed. Therapeutic options, prognosis and intellectual outcome are also reviewed.

Conclusion

The correct interpretation of the modern neuroradiologic techniques, including CSF flow MR imaging, may help in identifying a “real” Dandy–Walker malformation. Among therapeutical strategies, single shunting (ventriculo-peritoneal or cyst-peritoneal shunts) appears effective in the control of both ventricle and cyst size. Endoscopic third ventriculostomy may be considered an acceptable alternative, especially in older children, with the aim to reduce the shunt-related problems. Prognosis and intellectual outcome mostly depend on the presence of associated malformations, the degree of vermian malformation and the adequate control of hydrocephalus.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9
Fig. 10
Fig. 11

Similar content being viewed by others

References

  1. Achiron R, Achiron A (1991) Transvaginal ultrasonic assessment of the early fetal brain. Ultrasound Obstet Gynecol 1:336–344

    Article  PubMed  CAS  Google Scholar 

  2. Aldinger KA, Lehmann OJ, Hudgins L, Chizhikov VV, Bassuk AG, Ades LC, Krantz ID, Dobyns WB, Millen KJ (2009) FOXC1 is required for normal cerebellar development and is a major contributor to chromosome 6p25.3 DandyWalker malformation. Nat Genet 41:1037–1042

    Article  PubMed  CAS  Google Scholar 

  3. Alexiou GA, Sfakianos G, Prodromou N (2010) Dandy–Walker malformation: analysis of 19 cases. J Child Neurol 25:188–191

    Article  PubMed  Google Scholar 

  4. Almeida GM, Matushita H, Mattosinho-Franča SMK (1990) Dandy–Walker syndrome: posterior fossa craniectomy and cyst fenestration after several shunt revisions. Childs Nerv Syst 6:335–337

    Article  PubMed  CAS  Google Scholar 

  5. Asai A, Hoffman HJ, Hendrick EB, Humphreys RP (1989) Dandy–Walker syndrome: experience at the Hospital for Sick Children, Toronto. Pediatr Neurosci 15:66–73

    Article  PubMed  CAS  Google Scholar 

  6. Barkovich AJ, Kjos BO, Norman D, Edwards MS (1989) Revised classification of posterior fossa cysts and cyst-like malformations based on the results of multiplanar MR imaging. AJNR 10:977–988

    Google Scholar 

  7. Benda CE (1954) The Dandy–Walker syndrome or the so-called atresia of the foramen of Magendie. J Neuropathol Exp Neurol 13:14–29

    PubMed  CAS  Google Scholar 

  8. Bindal AK, Storrs BB, McLone DG (1990–1991) Management of Dandy–Walker syndrome. Pediatr Neurosurg 16:163–169

    Article  Google Scholar 

  9. Bindal AK, Storrs BB, McLone DG (1991) Occipital meningoceles in patients with the Dandy–Walker syndrome. Neurosurgery 28:844–847

    Article  PubMed  CAS  Google Scholar 

  10. Blake JA (1900) The roof and lateral recesses of the fourth ventricle, considered morphologically and embryologically. J Comp Neurol 10:79–108

    Article  Google Scholar 

  11. Boddaert N, Klein O, Ferguson N, Sonigo P, Parisot D, Hertz-Pannier L, Baraton J, Emond S, Simon I, Chigot V, Schmit P, Pierre-Kahn A, Brunelle F (2003) Intellectual prognosis of the Dandy–Walker malformation in children: the importance of vermian lobulation. Neuroradiology 45:320–324

    PubMed  CAS  Google Scholar 

  12. Boltshauser E (2001) Cerebellar imaging—an important signpost in pediatric neurology. Child’s Nerv Syst 17:211–216

    Article  CAS  Google Scholar 

  13. Brown J (1977) The Dandy–Walker syndrome. In: Vincken PJ, Bruyn GW (eds) Handbook of clinical neurology, vol 30. North Holland, Amsterdam, p 623

    Google Scholar 

  14. Carmel PW, Antunes JL, Hilal SK, Gold AP (1977) Dandy–Walker syndrome: clinico-pathological features and re-evaluation of modes of treatment. Surg Neurol 8:132–138

    PubMed  CAS  Google Scholar 

  15. Chen FP, Chu KK (1994) Prenatal diagnosis of Dandy–Walker malformation: report of a case. J Formos Med Assoc 93:967–970

    PubMed  CAS  Google Scholar 

  16. Cinalli G (1999) Alternatives to shunting. Childs Nerv Syst 15:718–731

    Article  PubMed  CAS  Google Scholar 

  17. Cinalli G, Sainte-Rose C, Zerah M (1997) La malformation de Dandy–Walker. Proceedings of the French Society of Neurosurgery. Fort-de-France, Martinique, 19–24 January 1997

  18. Cinalli G, Spennato P, Del Basso De Caro ML, Buonocore MC (2004) Hydrocephalus and Dandy Walker malformation. In: Cinalli C, Maixner WJ, Sainte-Rose C (eds) Pediatric hydrocephalus. Springer, Milan, pp 259–277

    Chapter  Google Scholar 

  19. Cinalli G, Vinikoff L, Zerah M, Renier D, Pierre-Kahn A (1997) Dandy–Walker malformation associated with syringomyelia. J Neurosurg 86:571

    Article  PubMed  CAS  Google Scholar 

  20. Costa C, Hauw JJ (1995) Pathology of the cerebellum, brain stem, and spinal cord. In: Duckett S (ed) Pediatric neuropathology. Williams & Wilkins, Baltimore, pp 217–238

  21. Dandy WE, Blackfan KD (1914) Internal hydrocephalus. An experimental, clinical and pathological study. Am J Dis Child 8:406–482

    Google Scholar 

  22. Domingo Z, Peter J (1996) Midline developmental abnormalities of the posterior fossa: correlation of classification with outcome. Pediatr Neurosurg 24:111–118

    Article  PubMed  CAS  Google Scholar 

  23. Elgamal EA, El-Dawlatly AA, Murshid WR, El-Watidy SM, Jamjoom ZA (2011) Endoscopic third ventriculostomy for hydrocephalus in children younger than 1 year of age. Childs Nerv Syst 27:111–116

    Article  PubMed  Google Scholar 

  24. Elsen GE, Choi LY, Millen KJ, Grinblat Y, Prince VE (2008) Zic1 and Zic4 regulate zebrafish roof plate specification and hindbrain ventricle morphogenesis. Dev Biol 314(2):376–392

    Article  PubMed  CAS  Google Scholar 

  25. Fischer EG (1973) Dandy–Walker syndrome: an evaluation of surgical treatment. J Neurosurg 39:615–621

    Article  PubMed  CAS  Google Scholar 

  26. Frieden IJ, Reese V, Cohen D (1996) PHACE syndrome. The association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Arch Dermatol 132:307–311

    Article  PubMed  CAS  Google Scholar 

  27. Gardner WJ (1977) Hydrodynamic factors in Dandy–Walker and Arnold–Chiari malformations. Child’s Brain 3:200–212

    PubMed  CAS  Google Scholar 

  28. Gerszten PC, Albright AL (1995) Relationship between cerebellar appearance and function in children with Dandy–Walker syndrome. Pediatr Neurosurg 23:86–92

    Article  PubMed  CAS  Google Scholar 

  29. Gibson JB (1955) Congenital hydrocephalus due to atresia of the foramen of Magendie. J Neuropathol Exp Neurol 14:244–262

    Article  PubMed  CAS  Google Scholar 

  30. Gilbert-Barness E, Debich-Spicer D, Cohen MM Jr, Opitz JM (2001) Evidence for the “midline” hypothesis in associated defects of laterality formation and multiple midline anomalies. Am J Med Genet 101:382–387

    Article  PubMed  CAS  Google Scholar 

  31. Glasauer FE (1975) Isotope cisternography and ventriculography in congenital anomalies of the central nervous system. J Neurosurg 43:18–26

    Article  PubMed  CAS  Google Scholar 

  32. Golden JA, Rorke LB, Bruce DA (1987) Dandy–Walker syndrome and associated anomalies. Pediat Neurosci 13:38–44

    Article  CAS  Google Scholar 

  33. Hammond CJ, Chitnavis B, Penny CC, Strong AJ (2002) Dandy–Walker complex and syringomyelia in an adult: case report and discussion. Neurosurgery 50:191–194

    PubMed  Google Scholar 

  34. Hanigan WC, Wright R, Wright S (1986) Magnetic resonance imaging of the Dandy–Walker malformation. Pediatr Neurosci 12:151–156

    Article  CAS  Google Scholar 

  35. Hart MN, Malamud N, Ellis WG (1972) The Dandy–Walker syndrome. A clinico-pathological study based on 28 cases. Neurology 22:771–780

    PubMed  CAS  Google Scholar 

  36. Harwood-Nash DC, Fitz CR (1976) Neuroradiology in infants and children, vol 3. Mosby, St Louis, pp 1014–1019

    Google Scholar 

  37. Hirsch JF (1982) Percutaneous ventriculocisternostomies in non-communicating hydrocephalus. Monogr Neurol Sci 8:170–178

    Google Scholar 

  38. Hirsch JF, Pierre-Kahn A, Renier D, Sainte-Rose C, Hoppe-Hirsch E (1984) The Dandy–Walker malformation. A review of 40 cases. J Neurosurg 61:515–522

    Article  PubMed  CAS  Google Scholar 

  39. Hoffman HJ, Harwood-Nash D, Gilday DL, Craven MA (1980) Percutaneous ventriculostomy in the management of non-communicating hydrocephalus. Neurosurgery 7:1330–1337

    Google Scholar 

  40. Imataka G, Yamanouchi H, Arisaka O (2007) Dandy–Walker syndrome and chromosomal abnormalities. Congenit Anom (Kyoto) 47:113–118

    Article  Google Scholar 

  41. Jalali A, Aldinger KA, Chary A, McLone DG, Bowman RM, Le LC, Jardine P, Newbury-Ecob R, Mallick A, Jafari N, Russell EJ, Curran J, Nguyen P, Ouahchi K, Lee C, Dobyns WB, Millen KJ, Pina-Neto JM, Kessler JA, Bassuk AG (2008) Linkage to chromosome 2q36.1 in autosomal dominant Dandy–Walker malformation with occipital cephalocele and evidence for genetic heterogeneity. Hum Genet 8(123):237–245

    Article  Google Scholar 

  42. James HE, Kaiser G, Schut L, Bruce DA (1979) Problems of diagnosis and treatment in the Dandy–Walker syndrome. Child’s Brain 5:24–30

    PubMed  CAS  Google Scholar 

  43. Jaspan T (2008) New concepts on posterior fossa malformations. Pediatr Radiol 38(Suppl 3):S409–S414

    Article  PubMed  Google Scholar 

  44. Kawaguchi T, Jokura H, Kusaka Y, Shirane R, Yoshimoto T (2003) Intraoperative direct neuroendoscopic observation of the aqueduct in Dandy–Walker malformation. Acta Neurochir 75:63–67

    Article  Google Scholar 

  45. Klein O, Pierre-Kahn A, Boddaert N, Parisot D, Brunelle F (2001) Dandy–Walker malformation: prenatal diagnosis and prognosis. Child’s Nervous System 19:484–489, 2003

    Article  Google Scholar 

  46. Kollias SS, Ball WS Jr, Prenger EC (1993) Cystic malformations of the posterior fossa: differential diagnosis clarified through embryologic analysis. Radiographics 13:1211–1231

    PubMed  CAS  Google Scholar 

  47. Kumar R, Jain MK, Chabra DK (2001) Dandy–Walker syndrome: different modalities of treatment and outcome in 42 cases. Childs Nerv Syst 17:348–352

    Article  PubMed  CAS  Google Scholar 

  48. Lee M, Leahu D, Weiner HL, Abbott R, Wisoff JH, Epstein FJ (1995) Complications of fourth-ventricle shunts. Pediatr Neurosurg 22:309–314

    Article  PubMed  CAS  Google Scholar 

  49. Lipton HL, Preziosi TJ, Moses H (1978) Adult onset in Dandy–Walker syndrome. Arch Neurol 35:672–675

    PubMed  CAS  Google Scholar 

  50. Liu A, Joyner AL (2001) Early anteroposterior patterning of the midbrain and cerebellum. Annu Rev Neurosci 24:869–896

    Article  PubMed  CAS  Google Scholar 

  51. Liu JC, Ciacci JD, George TM (1995) Brainstem tethering in Dandy–Walker syndrome: a complication of cystoperitoneal shunting. J Neurosurg 83:1072–1074

    Article  PubMed  CAS  Google Scholar 

  52. Mademont-Soler I, Morales C, Armengol L, Soler A, Sánchez A (2010) Description of the smallest critical region for Dandy–Walker malformation in chromosome 13 in a girl with a cryptic deletion related to t(6;13)(q23;q32). Am J Med Genet A 152A(9):2308–2312

    Article  PubMed  Google Scholar 

  53. Maria BL, Zinreich SJ, Carson BC, Rosenbaum AE, Freeman JM (1978) Dandy–Walker syndrome revisited. Pediatr Neurosci 13:45–51

    Article  Google Scholar 

  54. Marinov M, Gabrovski S, Undjian S (1991) The Dandy–Walker syndrome: diagnostic and surgical consideration. Br J Neurosurg 5:475–483

    Article  PubMed  CAS  Google Scholar 

  55. Matson DD (1956) Prenatal obstruction of the fourth ventricle. Am J Roentg 76:499–506

    PubMed  CAS  Google Scholar 

  56. Milhorat TH, Capocelli AL, Anzil AP, Kotzen RM, Milhorat RH (1995) Pathological basis of spinal cord cavitation in syringomyelia: analysis of 105 autopsy cases. J Neurosurg 82:802–812

    Article  PubMed  CAS  Google Scholar 

  57. Mohanty A (2003) Endoscopic third ventriculostomy with cystoventricular stent placement in the management of Dandy–Walker malformation: technical case report of three patients. Neurosurgery 53:1223–1228

    Article  PubMed  Google Scholar 

  58. Mohanty A, Biswas A, Satish S, Praharaj SS, Sastry KV (2006) Treatment options for Dandy–Walker malformation. J Neurosurg 105(5 Suppl):348–356

    PubMed  Google Scholar 

  59. Montes JL, Clarke DB, Farmer JP (1994) Stereotactic transtentorial hiatus ventriculoperitoneal shunt for the sequestered fourth ventricle. Technical note. J Neurosurg 80:759–761

    Article  PubMed  CAS  Google Scholar 

  60. Murray JC, Johnson JA, Bird TD (1985) Dandy–Walker malformation: etiologic heterogeneity and empiric recurrence risks. Clin Genet 28:272–283

    Article  PubMed  CAS  Google Scholar 

  61. Nagdeve NG, Mudkhedkar KP (2009) Large destructive facial hemangioma in PHACE syndrome. J Indian Assoc Pediatr Surg 14:113–114

    Article  PubMed  CAS  Google Scholar 

  62. Naidich TP, Altman NR, Gonzales-Arias SM (1993) Phase contrast cine magnetic resonance imaging: normal cerebrospinal fluid oscillation and applications to hydrocephalus. Neurosurg Clin N Am 4:677–705

    PubMed  CAS  Google Scholar 

  63. Naidich TP, Radkowski MA, McLone DG, Leestma J (1986) Chronic cerebral herniation in shunted Dandy–Walker malformation. Radiology 158:431–434

    PubMed  CAS  Google Scholar 

  64. Nelson MD Jr, Maher K, Gilles FH (2004) A different approach to cysts of the posterior fossa. Pediatr Radiol 34:720–732

    Article  PubMed  Google Scholar 

  65. O’Rahilly R, Muller F (1986) The meninges in human development. J Neuropath Exp Neurol 45:588–608

    Article  PubMed  Google Scholar 

  66. Ogiwara H, Dipatri AJ Jr, Alden TD, Bowman RM, Tomita T (2010) Endoscopic third ventriculostomy for obstructive hydrocephalus in children younger than 6 months of age. Childs Nerv Syst 26:343–347

    Article  PubMed  Google Scholar 

  67. Opitz JM, Gilbert EF (1982) CNS anomalies and the midline as a “developmental field”. Am J Med Genet 12:443–455

    Article  PubMed  CAS  Google Scholar 

  68. Osenbach RK, Menezes AH (1992) Diagnosis and management of the Dandy–Walker malformation: 30 years experience. Pediatr Neurosurg 18:179–189

    Article  PubMed  CAS  Google Scholar 

  69. Pascual-Castroviejo I, Velez A, Pascual-Pascual SI, Roche MC, Villarejo F (1991) Dandy–Walker malformation: analysis of 38 cases. Childs Nerv Syst 7:88–97

    PubMed  CAS  Google Scholar 

  70. Raimondi AJ, Samuelson G, Yarzagaray L, Norton T (1969) Atresia of the foramen of Luschka and Magendie: the Dandy–Walker cyst. J Neurosurg 31:202–216

    Article  PubMed  CAS  Google Scholar 

  71. Raybaud C (1982) Cystic malformations of the posterior fossa. Abnormalities associated with the development of the roof of the fourth ventricle and adjacent meningeal structures. J Neuroradiol 9:103–133

    PubMed  CAS  Google Scholar 

  72. Sasaki-Adams D, Elbabaa SK, Jewells V, Carter L, Campbell JW, Ritter AM (2008) The Dandy–Walker variant: a case series of 24 pediatric patients and evaluation of associated anomalies, incidence of hydrocephalus, and developmental outcomes. J Neurosurg Pediatrics 2:194–199

    Article  Google Scholar 

  73. Sawaya R, McLaurin RL (1981) Dandy–Walker syndrome. Clinical analysis of 23 cases. J Neurosurg 55:89–98

    Article  PubMed  CAS  Google Scholar 

  74. Shaw CM, Alvord EC (1995) Hydrocephalus. In: Duckett S (ed) Pediatric neuropathology. Williams & Wilkins, Baltimore, pp 149–211

  75. Taggart JK, Walker AE (1942) Congenital atresia of the foramen of Luschka and Magendie. Arch Neurol Psychiatry 48:583–612

    Google Scholar 

  76. Takami H, Shin M, Kuroiwa M, Isoo A, Takahashi K, Saito (2010) Hydrocephalus associated with cystic dilation of the foramina of Magendie and Luschka. J Neurosurg Pediatr 5:415–418

    Article  PubMed  Google Scholar 

  77. Tal Y, Freigang B, Dunn HG, Durity FA, Moyes PD (1980) Dandy–Walker syndrome: analysis of 21 cases. Dev Med Child Neurol 22:189–201

    Article  PubMed  CAS  Google Scholar 

  78. Tortori-Donati P, Fondelli MP, Rossi A, Carini S (1996) 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. Child’s Nerv Syst 12:303–308

    Article  CAS  Google Scholar 

  79. Udvarhelyi GB, Epstein MH (1975) The so-called Dandy–Walker syndrome: analysis of 12 operated cases. Child’s Brain 1:158–182

    PubMed  CAS  Google Scholar 

  80. Villavicencio AT, Wellos JC, George TM (1998) Avoiding complicated shunt systems by open fenestration of symptomatic fourth ventricular cysts associated with hydrocephalus. Pediatr Neurosurg 29:314–319

    Article  PubMed  CAS  Google Scholar 

  81. Vuia O, Pascu G (1971) The Dandy–Walker syndrome associated with syringomyelia in an infant. Confin Neurol 33:33–40

    Article  PubMed  CAS  Google Scholar 

  82. Weinzierl MR, Coenen VA, Korinth MC, Gilsbach JM, Rohde V (2005) Endoscopic transtentorial ventriculocystostomy and cystoventriculoperitoneal shunt in a neonate with Dandy–Walker malformation and associated aqueductal obstruction. Pediatr Neurosurg 41:272–277

    Article  PubMed  Google Scholar 

  83. Wolpert SM, Scott RM, Runge VM, Kwan ES (1986) Difficulties of diagnosing congenital posterior fossa fluid collection after shunting procedures. AJNR 8:653–656

    Google Scholar 

  84. Yildiz H, Yazici Z, Hakyemez B, Erdogan C, Parlak M (2006) Evaluation of CSF flow patterns of posterior fossa cystic malformations using CSF flow MR imaging. Neuroradiology 48:595–605

    Article  PubMed  Google Scholar 

  85. Cinalli C, Maixner WJ, Sainte-Rose C (eds) (2004) Pediatric hydrocephalus. Springer, Milan

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Giuseppe Cinalli.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Spennato, P., Mirone, G., Nastro, A. et al. Hydrocephalus in Dandy–Walker malformation. Childs Nerv Syst 27, 1665–1681 (2011). https://doi.org/10.1007/s00381-011-1544-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-011-1544-4

Keywords

Navigation