Abstract
A case of holoprosencephaly associated with Dandy-Walker cyst is reported. The patient was a male baby whose mother had normal serum titers for toxoplasma, syphilis, rubella and hepatitis B. She had no history of dibbetes mellitus, administration of drugs or irradiation during the pregnancy. At the 8th month of gestation, fetal hydrocephalus was diagnosed by ultrasonic imaging. He was delivered by caesarean section at 34 weeks and 4 days, weighing 2,644 g. His head circumference was 42 cm; the anterior fontanel was not distended and its tension was normal. The chromosomal karyotype was a normal 46 XY. X-ray CT showed a large dorsal sac cyst in the supratentorial space and a hypoplastic cerebellum with a large cyst in the posterior fossa. He received a cyst-peritoneal shunt 24 days after birth. After this procedure, his head enlargement was arrested. We discuss the etiology of this rare coexistence of two major malformations.
Similar content being viewed by others
References
Brodal A, Hauglie-Hanssen E (1959) Congenital hydrocephalus with defective development of the cerebellar vermis (Dandy-Walker syndrome). J Neurol Neurosurg Psychiatry 22:99–108
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
Dekaban A (1959) Arhinencephaly in an infant born to a diabetic mother. J Neuropathol Exp Neurol 18:620–626
DeMyer W (1964) Vinblastine-induced malformations of face and nervous system in two rat strains. Neurology (Minneap) 14:806
DeMyer W, Zeman W (1963) Alobar holoprosencephaly (arhinencephaly) with median cleft lip and palate: clinical, electroencephalographic and nosologic considerations. Confin Neurol 23:1–36
Gardner E, O'Rahilly R, Prolo D (1975) The Dandy-Walker and Arnold-Chiari malformations. Arch Neurol 32:393–407
Hayashi T, Kuramoto S, Takagi S, Kojyo N (1980) Two cases of holoprosencephaly (in Japanese). Shoni No Noshinkei 5:153–160
Keeler RF, Binns W (1968) Teratogenic compounds of veratrum californicum (Durand). Teratology 1:5–10
Maeda T, Yokota H, Ogashiwa M, Takeuchi K (1984) Holoprosencephaly with Dandy-Walker cyst and aqueductal atresia. Case report (in Japanese). Shoni No Noshinkei 9:83–89
Matsunaga E, Shiota K (1977) Holoprosencephaly in human embryos: epidemiologic studies of 150 cases. Teratology 16:261–272
Matsushima T, Miyazaki M, Ohye C, Tajima S, Handa I (1984) Two cases of dysgenetic hydrocephaly (in Japanese). Shoni No Noshinkei 9:281–286
McDermott A, Insley J, Barton ME, Rowe P, Edwards JH, Cameron AH (1968) Arrhinencephaly associated with a deficiency involving chromosome 18. J Med Genet 5:60–67
Murakami U (1967) Comparative developmental pathology on congenital malformations of the central nervous system (in Japanese). Clin Neurol (Jpn) 7:443–453
Nakagawa T, Takamatsu H, Suematsu K, Tsuchita H, Mochizuki Y, Goto M (1980) Holoprosencephaly with presence of olfactory nerve. A case report and a review of literature (in Japanese). No To Shinkei 32:675–681
Osaka K, Matsumoto S (1978) Holoprosencephaly in neurosurgical practice. J Neurosurg 48:787–803
Osaka K, Shiota K (1981) Clinical problems and teratological mechanisms of the developmental anomaly in the brain — special reference to holoprosencephaly (in Japanese). Igaku No Ayumi 116:206–220
Tanimura T, Uwabe C (1971) Eighty-one cases of holoprosencephaly in Japanese embryos (in Japanese). Senten-Ijo 11:130
Tokimura H, Kadota K, Uetsuhara K, Oda H, Todoroki K, Tamura M, Asakura T (1987) A case of holoprosencephaly accompanied with dysgenesis of the cerebellum (in Japanese). Shoni No Noshinkei 12:423–428
Ueda S, Ueguchi T, Sekimoto T, Hirakawa K (1986) On the concept of Dandy-Walker syndrome and Chiari malformation type IV with report of our case (in Japanese). Shoni No Noshinkei 11:1–7
Yamasaki M, Akagi K, Horibe K, Go J, Kohmura E, Yamada M, Takimoto M, Masaki S, Kurata A (1984) Two autopsied cases of Dandy-Walker cyst (in Japanese). Shoni No Noshinkei 9:223–229
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Kurokawa, Y., Tsuchita, H., Sohma, T. et al. Holoprosencephaly with Dandy-Walker cyst. Child's Nerv Syst 6, 51–53 (1990). https://doi.org/10.1007/BF00262269
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00262269