| A Cases of Dandy-Walker Syndrome. |
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Jong Lin Rhi, Yoon Dok Kim, Hyung Ro Moon |
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Dept, of Pediatrics, Seoul National Umiversity Hospital, Seoul, Korea |
| Dandy-Walker 증후군 1례 |
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이종린, 김윤덕, 문형로 |
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서울대학교 의과대학 소아과학교실 |
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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 the fourth ventricle and agenesis or hypoplasia of the cerebellar vermis. Other systemic
anomalies are associated with the syndrome.
We experienced a case in a 15 day old female infant who presented initially with frequent
aspirations and respiratory difficulty. On physical examination an encephalocele on the occipital
area, a rudimentary nipple below the left nipple, and polydactyly of the feet were present. On barium
esophagogram the swallowed barium drained from the oropharynx into both the laryngotracheal and
the esophagus due to malfunctioning of the epiglottis. On transillumination of the skull the halo was
increased on the occipital area but the characteristic triangular area was not demonstrated. The brain
CT revealed dilatation of the lateral and fourth ventricles, large posterior fossa cyst and hypoplastic
cerebellum with no vermis.
The case is reported with the brief review of the literatures.
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| Key Words:
Dandy-Walker Syndrome.
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