Klein et al identified prognostic factors for patients with the Dandy-Walker malformation. These can help distinguish a patient with a relatively normal outcome from a patient with a poor prognosis. The authors are from Hopital Necker Enfants-Malades in Paris.
Patient selection: One who meets all of the criteria for the Dandy-Walker malformation (see previous section)
A patient with all of the following should have a normal outcome:
(1) normal karyotype
(2) minimal abnormality of the vermis, with partial agenesis
(3) rest of brain normal, without malformation
A patient with any of the following may have a poor outcome, with mental retardation and neurological impairment:
(1 abnormal karyotype
(2) severely dysplastic and malformed vermis
(3) severe brain malformations in the supratentorium, typically in the midline
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