Abstract
Introduction
We report an isolated pedigree in which a consanguineous couple had twin sons with Dandy–Walker malformation (DWM). The mother is similarly affected with the disorder.
Discussion
DWM is an abnormality of the central nervous system, which leads to hydrocephalus and is associated with other abnormalities.
Conclusion
Inheritance of the disorder remains controversial, with the majority perceived to be sporadic cases. This report suggests an autosomal inheritance.
Similar content being viewed by others
References
Barkovich AJ, Kjos BO, Norman D, Edwards MS (1989) Revised classification of posterior fossa cysts and cystlike malformations based on the results of multiplanar MR imaging. Am J Neuroradiol 10:977–988
Benda CE (1954) The Dandy–Walker syndrome or the so-called atresia of the foramen Magendie. J Neuropathol Exp Neurol 13:14–29
Bonnevie K (1943) Hereditary hydrocephalus in the house mouse I. Manifestation of the hymutation after birth and in embryos 12 days old or more. Skr Nor Vidensk Akad I Math Nat K1 4:1–32
Brodal A, Bonnevie K, Harkmark W (1944) Hereditary hydrocephalus in the house mouse II. The anomalies of the cerebellum: partial defective development of the vermis. Skr Nor Vidensk Akad I Math Nat K1 8:1–42
Chemke J, Czernobilsky B, Mundel G et al (1975) A familial syndrome of central nervous system and ocular malformations. Clin Genet 7:1–7
Chitayat D, Moore L, Del Bigio MR, MacGregor D, Ben-Zeev B, Hodgkinson K, Deck J, Stothers T, Ritchie S, Toi A (1994) familial Dandy–Walker malformation associated with macrocephaly, facial anomalies, developmental delay, and brain stem dysgenesis: prenatal diagnosis and postnatal outcome in brothers. A new syndrome? Am J Med Genet 52:406–415
Cowles T, Furman P, Wilkins I (1993) Prenatal diagnosis of Dandy Walker malformation in a family displaying X-linked inheritance. Prenat Diagn 13:87–91
Dandy WE, Blackfan KD (1914) Internal hydrocephalus. An experimental, clinical and pathological study. Am J Dis Child 8:406–482
Hart MN, Malamud N, Ellis WG (1972) The Dandy–Walker syndrome. A clinicopathological study based on 28 cases. Neurology 22:771–780
Harwood-Nash DC, Fitz CR (1977) Neuroradiology in infants and children, vol 3. Mosby, St. Louis, pp 1014–1019
Hirsch JF, Pierre-Khan A, Renier D, Sainte-Rose C, Hoppe-Hirsch E (1984) The Dandy–Walker malformation: a review of 40 cases. J Neurosurg 61:515–522
Jenkyn LR, Roberts DW, Merlis AL et al (1981) Dandy–Walker malformations in identical twins. Neurology 31:337–341
Kalter H, Warkany J (1957) Congenital malformations in inbred strains of mice induced by riboflavin-deficient, galactoflavin-containing diets. J Exp Zool 136:531–565
Lehman RM (1981) Dandy–Walker syndrome in consecutive siblings: familial hindbrain malformation. Neurosurgery 8:717–719
Murray JC, Johnson JA, Bird TD (1985) Dandy–Walker malformation: etiologic heterogeneity and empiric recurrence risks. Clin Genet 28:272–283
Planas S, Peiro R, Rubio R, Villanueva R, Sere A, Carreras R (2003) A new report of mesomelic camtomelia. Polydactyly and Dandy–Wlaker complex in siblings. Prenat Diagn 23:372–374
Saitoh K, Kawamoto T, Ebina T et al (1978) Six cases of Dandy–Walker syndrome. No To Shinkei 30:875–879
Sawaya R, McLaurin RL (1981) Dandy–Walker syndrome. Clinical analysis of 23 cases. J Neurosurg 55:89–98
Sutton JB (1887) The lateral recesses of the fourth ventricle; their relation to certain cysts and tumours of the cerebellum, and to occipital meningocele. Brain 9:352–361
Wakeling EL, Jolly M, Fisk NM, Gannon C, Holder SE (2002) X-linked inheritance of Dandy–Walker variant. Clin Dysmorphol 11(1):15–18
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Bragg, T.W.H., St. George, E.J., Wynne-Jones, G.A. et al. Familial Dandy–Walker syndrome: a case report supporting an autosomal inheritance. Childs Nerv Syst 22, 539–541 (2006). https://doi.org/10.1007/s00381-005-0007-1
Received:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-005-0007-1