| A Case of Dandy-Walker Syndrome Associated with Multiple Congenital Anomalies. |
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Gye Weon Shin, Chul Ho Lee, Bo Kyung Cho, Chung Sik Chun, Sung Hun Cho |
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Department of Pediatrics, Catholic University Medical College, Seoul, Korea |
| 다발성 선천성 기형을 동반한 Dandy-Walker 증후군 1례 |
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신계원, 이철호, 조보경, 전정식, 조성훈 |
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가톨릭의대 의학부 소아과학교실 |
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Received: 9 November 1989 • Accepted: 23 February 1990 |
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| Abstract |
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The Dandy-Walker Syndrome is a developmental disorders of the brain characterized by cystic
deformity of fourth ventricle, agenesis or hypoplasia of the cerebellar vermis, and hydrocephalus.
This syndrome is frequently associated with central nervous system malformation and systemic
anomalies.
We experienced a case in a 2-day-old female who has multiple congenital anomalies. On physical
examinations, macrocephaly with prominent occiput, encephalocele in the occipital area, microphth-
almia, opacity of both lenses, downward displaced eyeballs, microglossia, micrognathia, simian crease
on both hands, polydactyly of the right hand, and PDA were present. On transilluminatin over the
posterior skull, increased transluscency of typical triangular shape was demonstrated. Cranial
ultrasonography defines the posterior fossa cyst and hydrocephalus. The Brain CT revealed dilatation
of the fourth ventricle, large posterior fossa cyst, hypoplasia of cerebellar hemisphere, agenesis of
vermis, marked dilatation of third and lateral ventricles, and dysgenesis of Corpus Callosum and
Septum Pallucidum. |
| Key Words:
Dandy-Walker syndrome, Multiple congenital anomalies |
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