Treatment options for Dandy–Walker malformation

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  • 1 Department of Neurosurgery, National Institute of Mental Health and Neuro Sciences, Bangalore, India; and Department of Neurosurgery, University of Texas Health Sciences Center at Houston, Texas
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Object

The aim of this study was to assess the efficacy of various treatment options available for children with Dandy–Walker malformation (DWM) and to evaluate the role of endoscopic procedures in the treatment of this disorder.

Methods

The authors conducted a retrospective review of 72 children who underwent surgical treatment for DWM during a 16-year period. All patients underwent computed tomography scanning, and 26 underwent magnetic resonance (MR) imaging. The initial surgical treatment included ventriculoperitoneal (VP) shunt placement in 21 patients, cystoperitoneal (CP) shunt placement in 24, and combined VP and CP shunt insertion in three. Twenty-one patients underwent endoscopic procedures (endoscopic third ventriculostomy [ETV] alone in 16 patients, ETV with aqueductal stent placement in three, and ETV with fenestration of the occluding membrane in two). Three patients underwent membrane excision via a posterior fossa craniectomy. In the 26 patients who had undergone preoperative MR imaging, aqueductal patency was noted in 23 and aqueductal obstruction in three. These three patients underwent placement of a stent from the third ventricle to the posterior fossa cyst in addition to the ETV procedure.

During the follow-up period, 12 patients with a CP shunt and four with a VP shunt experienced shunt malfunctions that required revision. Four patients with a CP shunt also required placement of a VP shunt. In addition, five of the 21 ETVs failed, requiring VP shunt insertion. A reduction in ventricle size noted on postoperative images occurred more frequently in patients with a VP shunt, whereas a reduction in cyst size was more appreciable in patients with a CP shunt. Successful ETV resulted in a slight decrease in ventricle size and varying degrees of reduction in cyst size.

Conclusions

Endoscopic procedures may be considered an acceptable alternative in children with DWM. The authors propose a treatment protocol based on preoperative MR imaging findings of associated aqueductal stenosis.

Abbreviations used in this paper:

CP = cystoperitoneal; CSF = cerebrospinal fluid; CT = computed tomography; DWM = Dandy–Walker malformation; ETV = endoscopic third ventriculostomy; MR = magnetic resonance; VP = ventriculoperitoneal.

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Contributor Notes

Address reprint requests to: Aaron Mohanty, M.Ch., Department of Neurosurgery, University of Texas Health Sciences Center at Houston, 6410 Fannin #1020, Houston, Texas 77030. email: aarmohanty@yahoo.com.
  • 1

    Almeida GM, , Matshita H, , Mattosinho-França LC, & Shibata MK: Dandy–Walker syndrome: posterior fossa craniectomy and cyst fenestration after several shunt revisions. Childs Nerv Syst 6:335337, 1990

    • Search Google Scholar
    • Export Citation
  • 2

    Asai A, , Hoffman HJ, , Hendrick EB, & Humphreys RP: Dandy-Walker syndrome: Experience at the Hospital for Sick Children, Toronto. Pediatr Neurosci 15:6673, 1989

    • Search Google Scholar
    • Export Citation
  • 3

    Benda CE: The Dandy-Walker syndrome or the so-called atresia of the foramen of Magendie. J Neuropathol Exp Neurol 13:1439, 1954

  • 4

    Bindal AK, , Storrs BB, & McLone DG: Management of the Dandy-Walker syndrome. Pediatr Neurosurg 16:163169, 1990

  • 5

    Buxton N, , Macarthur D, , Mallucci C, , Punt J, & Vloeberghs M: Neuroendoscopic third ventriculostomy in patients less than 1 year old. Pediatr Neurosurg 29:7376, 1998

    • Search Google Scholar
    • Export Citation
  • 6

    Carmel PW, , Antunes L, , Hilal SK, & Gold AP: Dandy-Walker syndrome. Clinicopathological features and re-evaluation of modes of treatment. Surg Neurol 8:132138, 1977

    • Search Google Scholar
    • Export Citation
  • 7

    Clatterbuck R, & Sipos E: The efficient calculation of neurosurgically relevant volumes from computed tomographic scans using Cavalieri’s Direct Estimator. Neurosurgery 40:339343, 1997

    • Search Google Scholar
    • Export Citation
  • 8

    Dandy WE, & Blackfan KDE: Internal hydrocephalus: an experimental, clinical and pathological study. Am J Dis Child 8:406482, 1914

  • 9

    Domingo Z, & Peter J: Midline developmental abnormalities of the posterior fossa: correlation of classification with outcome. Pediatr Neurosurg 24:111118, 1996

    • Search Google Scholar
    • Export Citation
  • 10

    Edwards MS, & Rafel C, Dandy-Walker syndrome revisited. Discussion of articles by Maria & et al. : Golden et al. : Pediatr Neurosci 13:5051, 1987. (Comment)

    • Search Google Scholar
    • Export Citation
  • 11

    Foltz EL, & Shurtleff DB: Conversion of communicating hydrocephalus to stenosis or occlusion of the aqueduct during ventricular shunt. J Neurosurg 24:520529, 1966

    • Search Google Scholar
    • Export Citation
  • 12

    Fritsch MJ, & Manwaring K, Endoscopic stenting in aqueductal stenosis. in Hellwig D, & Bauer BL: (eds) Minimally Invasive Techniques for Neurosurgery: Current Status and Future Perspectives Berlin, Springer-Verlag, 1998. pp 8792

    • Search Google Scholar
    • Export Citation
  • 13

    Gerszten PC, & Albright AL: Relationship between cerebellar appearance and function in children with Dandy-Walker syndrome. Pediatr Neurosurg 23:8692, 1995

    • Search Google Scholar
    • Export Citation
  • 14

    Golden JA, , Rorke LB, & Bruce DA: Dandy-Walker syndrome and associated anomalies. Pediatr Neurosci 13:3844, 1987

  • 15

    Hirsch JF, , Pierre-Kahn A, , Reiner D, , Sainte Rose C, & Hoppe-Hirsch E: The Dandy–Walker malformation. A review of 40 cases. J Neurosurg 61:515522, 1984

    • Search Google Scholar
    • Export Citation
  • 16

    Hoffman HJ, , Harwood-Nash D, & Gilday DL: Percutaneous third ventriculostomy in the management of noncommunicating hydrocephalus. Neurosurgery 7:313321, 1980

    • Search Google Scholar
    • Export Citation
  • 17

    Hopf NJ, , Grunert P, , Fries G, , Resch KDM, & Perneczky A: Endoscopic third ventriculostomy: outcome analysis of 100 consecutive procedures. Neurosurgery 44:795804, 1999

    • Search Google Scholar
    • Export Citation
  • 18

    Jones RF, , Stening WA, & Brydan M: Endoscopic third ventriculostomy. Neurosurgery 26:8692, 1990

  • 19

    Kulkarni AV, , Drake JM, , Armstrong DC, & Dirks PB: Imaging correlates of successful endoscopic third ventriculostomy. J Neurosurg 92:915919, 2000

    • Search Google Scholar
    • Export Citation
  • 20

    Kumar R, , Jain MK, & Chhabra DK: Dandy-Walker syndrome: different modalities of treatment and outcome in 42 cases. Childs Nerv Syst 17:348352, 2001

    • Search Google Scholar
    • Export Citation
  • 21

    Liu JC, , Ciacci JD, & George TM: Brain stem tethering in Dandy–Walker syndrome: a complication of cystoperitoneal shunting. J Neurosurg 83:10721074, 1995

    • Search Google Scholar
    • Export Citation
  • 22

    Maria BL, , Zinreich SJ, , Carson BC, , Rosenbaum AE, & Freeman JM: Dandy Walker syndrome revisited. Pediatr Neurosci 13:4551, 1987

  • 23

    Marinov M, , Gabrovsky S, & Undjian S: The Dandy–Walker syndrome: diagnostic and surgical considerations. Br J Neurosurg 5:475483, 1991

    • Search Google Scholar
    • Export Citation
  • 24

    McLaurin RL, & Crone KR, Dandy-Walker malformation. in Rengachary SS, & Wilkins RR: (eds) Neurosurgery, ed 2 New York, McGraw-Hill, 1996. III:pp 36693972

    • Search Google Scholar
    • Export Citation
  • 25

    McLone DG, , Naidich TP, & Cunningham T: Posterior fossa cysts: management and outcome. Concepts Pediatr Neurosurg 7:134141, 1987

  • 26

    Mohanty A: Endoscopic third ventriculostomy with cystoventricular stent placement in the management of Dandy-Walker malformation. Neurosurgery 53:12231229, 2003

    • Search Google Scholar
    • Export Citation
  • 27

    Mohanty A, , Anandh B, , Kolluri VR, & Praharaj SS: Neuroendoscopic third ventriculostomy in the management of fourth ventricular outlet obstruction. Minim Invasive Neurosurg 42:1821, 1999

    • Search Google Scholar
    • Export Citation
  • 28

    Osenbach RK, & Menezes AH: Diagnostic management of the Dandy-Walker malformation: 30 years of experience. Pediatr Neurosurg 18:179189, 1992

    • Search Google Scholar
    • Export Citation
  • 29

    Pillay PK, , Barnett GH, , Lanzeiri C, & Cruse R: Dandy-Walker cyst upward herniation: the role of magnetic resonance imaging and double shunts. Pediatr Neurosci 15:7479, 1989

    • Search Google Scholar
    • Export Citation
  • 30

    Raimondi AJ, , Samuelson G, , Yarzagaray L, & Norton T: Atresia of the foramina of Luschka and Magendie: the Dandy–Walker cyst. J Neurosurg 31:202216, 1969

    • Search Google Scholar
    • Export Citation
  • 31

    Rekate HL, Treatment of hydrocephalus. in McLaurin RL, , Venes JL, , Schut L, & Epstein F: (eds) Pediatric Neurosurgery: Surgery of the Developing Nervous System, ed 2 Philadelphia, WB Saunders, 1989. pp 211212

    • Search Google Scholar
    • Export Citation
  • 32

    Sawaya R, & McLaurin RL: Dandy–Walker syndrome. Clinical analysis of 23 cases. J Neurosurg 55:8998, 1981

  • 33

    Schwartz TH, , Ho B, , Prestiagiacomo CJ, , Bruce JN, , Feldstein NA, & Goodman RR: Ventricular volume following third ventriculostomy. J Neurosurg 91:2025, 1999

    • Search Google Scholar
    • Export Citation
  • 34

    Udvarhelyi GB, & Epstein MH: The so-called Dandy–Walker syndrome. Clinical analysis of 12 operated cases. Childs Brain 1:158182, 1975

    • Search Google Scholar
    • Export Citation

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