eMedicine Specialties > Radiology > Pediatrics

Dandy-Walker Malformation: Follow-up

Author: Lutfi Incesu, MD, Professor, Department of Radiology, Ondokuz Mayis University School of Medicine; Chief, Neuroradiology and MR Unit, Department of Radiology, Ondokuz Mayis University Hospital, Turkey
Coauthor(s): Anil Khosla, MBBS, Assistant Professor, Department of Radiology, Section of Neuroradiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine, Veterans Affairs Medical Center of St Louis
Contributor Information and Disclosures

Updated: Sep 11, 2009

Intervention

Treatment usually consists of a shunt to treat associated hydrocephalus. The neurosurgeon can shunt the cyst (cystoperitoneal), the ventricles (ventriculoperitoneal), or both.20,34

Medicolegal Pitfalls

  • Failure to diagnose Dandy-Walker malformation with hydrocephalus in a neonate or a child can cause serious neurologic complications.
  • A faulty diagnosis, such as mega cisterna magna or arachnoid cyst instead of Dandy-Walker malformation, can lead to morbidity and mortality.


Special Concerns

  • US can be performed during pregnancy, and the diagnosis of Dandy-Walker malformation can be made antenatally. To confirm the diagnosis and gain detailed information, MRI evaluation can be used antenatally.28
  • Genetic counseling may be needed because a familial occurrence has been reported. Fetal karyotyping is also recommended for chromosomal analysis.1,16,18
 


More on Dandy-Walker Malformation

Overview: Dandy-Walker Malformation
Imaging: Dandy-Walker Malformation
Follow-up: Dandy-Walker Malformation
Multimedia: Dandy-Walker Malformation
References
Further Reading
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References

  1. Murray JC, Johnson JA, Bird TD. Dandy-Walker malformation: etiologic heterogeneity and empiric recurrence risks. Clin Genet. Oct 1985;28(4):272-83. [Medline].

  2. Richter EO, Pincus DW. Development of syringohydromyelia associated with Dandy-Walker malformation: treatment with cystoperitoneal shunt placement. Case report. J Neurosurg. Mar 2006;104(3 Suppl):206-9. [Medline].

  3. Cakmak A, Zeyrek D, Cekin A, Karazeybek H. Dandy-Walker syndrome together with occipital encephalocele. Minerva Pediatr. Aug 2008;60(4):465-8. [Medline].

  4. Dandy WE, Blackfan KD. Internal hydrocephalus: an experimental, clinical and pathological study. Am J Dis Child. 1914;8:406-82.

  5. BENDA CE. The Dandy-Walker syndrome or the so-called atresia of the foramen Magendie. J Neuropathol Exp Neurol. Jan 1954;13(1):14-29. [Medline].

  6. D'AGOSTINO AN, KERNOHAN JW, BROWN JR. THE DANDY-WALKER SYNDROME. J Neuropathol Exp Neurol. Jul 1963;22:450-70. [Medline].

  7. Hart MN, Malamud N, Ellis WG. The Dandy-Walker syndrome. A clinicopathological study based on 28 cases. Neurology. Aug 1972;22(8):771-80. [Medline].

  8. Altman NR, Naidich TP, Braffman BH. Posterior fossa malformations. AJNR Am J Neuroradiol. Mar-Apr 1992;13(2):691-724. [Medline].

  9. 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. Dec 1989;153(6):1289-300. [Medline].

  10. Sasaki-Adams D, Elbabaa SK, Jewells V, Carter L, Campbell JW, Ritter AM. 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. Sep 2008;2(3):194-9. [Medline].

  11. Strand RD, Barnes PD, Poussaint TY, Estroff JA, Burrows PE. Cystic retrocerebellar malformations: unification of the Dandy-Walker complex and the Blake's pouch cyst. Pediatr Radiol. 1993;23(4):258-60. [Medline].

  12. Salihu HM, Kornosky JL, Alio AP, Druschel CM. Racial disparities in mortality among infants with Dandy-Walker syndrome. J Natl Med Assoc. May 2009;101(5):456-61. [Medline].

  13. Salihu HM, Kornosky JL, Druschel CM. Dandy-Walker syndrome, associated anomalies and survival through infancy: a population-based study. Fetal Diagn Ther. 2008;24(2):155-60. [Medline].

  14. Raybaud C. Cystic malformations of the posterior fossa. Abnormalities associated with the development of the roof of the fourth ventricle and adjacent meningeal structures. J Neuroradiol. 1982;9(2):103-33. [Medline].

  15. Taggart JK, Walker AE. Congenital atresia of the foramens of Luschka and Magendie. Arch Neurol Psychiat. 1942;48:583-612.

  16. Bordarier C, Aicardi J. Dandy-Walker syndrome and agenesis of the cerebellar vermis: diagnostic problems and genetic counselling. Dev Med Child Neurol. Apr 1990;32(4):285-94. [Medline].

  17. Cavalcanti DP, Salomao MA. Dandy-Walker malformation with postaxial polydactyly: further evidence for autosomal recessive inheritance. Am J Med Genet. Jul 16 1999;85(2):183-4. [Medline].

  18. Ulm B, Ulm MR, Deutinger J, Bernaschek G. Isolated Dandy-Walker malformation: prenatal diagnosis in two consecutive pregnancies. Am J Perinatol. 1999;16(2):61-3. [Medline].

  19. Lavanya T, Cohen M, Gandhi SV, Farrell T, Whitby EH. A case of a Dandy-Walker variant: the importance of a multidisciplinary team approach using complementary techniques to obtain accurate diagnostic information. Br J Radiol. Oct 2008;81(970):e242-5. [Medline].

  20. Kalidasan V, Carroll T, Allcutt D, Fitzgerald RJ. The Dandy-Walker syndrome--a 10-year experience of its management and outcome. Eur J Pediatr Surg. Dec 1995;5 Suppl 1:16-8. [Medline].

  21. Phillips JJ, Mahony BS, Siebert JR, Lalani T, Fligner CL, Kapur RP. Dandy-Walker malformation complex: correlation between ultrasonographic diagnosis and postmortem neuropathology. Obstet Gynecol. Mar 2006;107(3):685-93. [Medline].

  22. Menon RK, Nadkarni TD, Desai KI, Goel A. Dandy-Walker malformation associated with polycystic kidneys: Goldston syndrome revisited. J Clin Neurosci. Oct 2006;13(8):875-7. [Medline].

  23. Sato K, Kubota T, Nakamura Y. Adult onset of the Dandy-Walker syndrome. Br J Neurosurg. Feb 1996;10(1):109-12. [Medline].

  24. McClelland S 3rd, Charnas LR, SantaCruz KS, Garner HP, Lam CH. Progressive brainstem compression in an infant with neurocutaneous melanosis and Dandy-Walker complex following ventriculoperitoneal shunt placement for hydrocephalus. Case report. J Neurosurg. Dec 2007;107(6 Suppl):500-3. [Medline].

  25. Ecker JL, Shipp TD, Bromley B, Benacerraf B. The sonographic diagnosis of Dandy-Walker and Dandy-Walker variant: associated findings and outcomes. Prenat Diagn. Apr 2000;20(4):328-32. [Medline].

  26. Marnet D, Vinchon M, Mostofi K, Catteau B, Kerdraon O, Dhellemmes P. Neurocutaneous melanosis and the Dandy-Walker complex: an uncommon but not so insignificant association. Childs Nerv Syst. Aug 27 2009;[Medline].

  27. Hadzagic-Catibusic F, Maksic H, Uzicanin S, Heljic S, Zubcevic S, Merhemic Z, et al. Congenital malformations of the central nervous system: clinical approach. Bosn J Basic Med Sci. Nov 2008;8(4):356-60. [Medline].

  28. Kölble N, Wisser J, Kurmanavicius J, Bolthauser E, Stallmach T, Huch A, et al. Dandy-walker malformation: prenatal diagnosis and outcome. Prenat Diagn. Apr 2000;20(4):318-27. [Medline].

  29. Yildiz H, Yazici Z, Hakyemez B, Erdogan C, Parlak M. Evaluation of CSF flow patterns of posterior fossa cystic malformations using CSF flow MR imaging. Neuroradiology. Sep 2006;48(9):595-605. [Medline].

  30. Bromley B, Nadel AS, Pauker S, Estroff JA, Benacerraf BR. Closure of the cerebellar vermis: evaluation with second trimester US. Radiology. Dec 1994;193(3):761-3. [Medline].

  31. Hata T, Yanagihara T, Matsumoto M, Hanaoka U, Ueta M, Tanaka Y, et al. Three-dimensional sonographic features of fetal central nervous system anomaly. Acta Obstet Gynecol Scand. Aug 2000;79(8):635-9. [Medline].

  32. Russ PD, Pretorius DH, Johnson MJ. Dandy-Walker syndrome: a review of fifteen cases evaluated by prenatal sonography. Am J Obstet Gynecol. Aug 1989;161(2):401-6. [Medline].

  33. Wolpert SM, Haller JS, Rabe EF. The value of angiography in the Dandy-Walker syndrome and posterior fossa extra-axial cysts. Am J Roentgenol Radium Ther Nucl Med. Jun 1970;109(2):261-72. [Medline].

  34. Cedzich C, Lunkenheimer A, Baier G, Müller J, Kühner A. Ultrasound-guided puncture of a Dandy-Walker cyst via the lateral and III ventricles. Childs Nerv Syst. Sep 1999;15(9):472-6. [Medline].

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Keywords

Dandy-Walker malformation, Dandy-Walker, Dandy-Walker syndrome, Dandy-Walker cyst, Dandy-Walker deformity, Dandy Walker, cerebellum, cerebellar disease, brain disease, central nervous system disease, Luschka-Magendie foramina atresia

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Contributor Information and Disclosures

Author

Lutfi Incesu, MD, Professor, Department of Radiology, Ondokuz Mayis University School of Medicine; Chief, Neuroradiology and MR Unit, Department of Radiology, Ondokuz Mayis University Hospital, Turkey
Lutfi Incesu, MD is a member of the following medical societies: American Society of Neuroradiology and Radiological Society of North America
Disclosure: Nothing to disclose.

Coauthor(s)

Anil Khosla, MBBS, Assistant Professor, Department of Radiology, Section of Neuroradiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine, Veterans Affairs Medical Center of St Louis
Anil Khosla, MBBS is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, American Society of Neuroradiology, North American Spine Society, and Radiological Society of North America
Disclosure: Nothing to disclose.

Medical Editor

Charles M Glasier, MD, Professor, Departments of Radiology and Pediatrics, University of Arkansas for Medical Sciences; Chief, Magnetic Resonance Imaging, Vice-Chief, Pediatric Radiology, Arkansas Children's Hospital
Charles M Glasier, MD is a member of the following medical societies: American College of Radiology, American Society of Neuroradiology, Radiological Society of North America, and Society for Pediatric Radiology
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

Marta Hernanz-Schulman, MD, FAAP, Professor, Radiology, Radiological Sciences, and Pediatrics, Director, Department of Pediatric Radiology, Radiologist-in-Chief, Director, Department of Diagnostic Imaging, Vanderbilt University Medical Center, Vanderbilt Children's Hospital
Marta Hernanz-Schulman, MD, FAAP is a member of the following medical societies: American Institute of Ultrasound in Medicine and American Roentgen Ray Society
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Resolution Imaging Medical Corporation
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

James G Smirniotopoulos, MD, Professor of Radiology, Neurology, and Biomedical Informatics, Chairman, Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences
James G Smirniotopoulos, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, American Society of Head and Neck Radiology, American Society of Neuroradiology, American Society of Pediatric Neuroradiology, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.

 
 
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