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The Dandy-Walker syndrome.

C Hutterer1, P Baierl, E Ring-Mrozik

  • 1Dr. v. Haunersches Kinderspital, Dept. Pediatric Surgery, Univ. Munich, W. Germany.

Zeitschrift Fur Kinderchirurgie : Organ Der Deutschen, Der Schweizerischen Und Der Osterreichischen Gesellschaft Fur Kinderchirurgie = Surgery in Infancy and Childhood
|December 1, 1990
PubMed
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Dandy-Walker syndrome, a congenital brain malformation, often presents with associated anomalies in children. Surgical shunting of the lateral ventricular system provided satisfactory treatment but carried a high complication rate.

Area of Science:

  • Pediatric Neurology
  • Developmental Neuroscience
  • Congenital Malformations

Background:

  • Dandy-Walker syndrome is a complex congenital brain malformation.
  • It is characterized by cerebellar and fourth ventricle abnormalities.
  • Associated anomalies are frequently observed in affected individuals.

Purpose of the Study:

  • To present ten cases of Dandy-Walker syndrome.
  • To analyze the clinical manifestations and associated anomalies.
  • To evaluate treatment outcomes and complications.

Main Methods:

  • Retrospective case series analysis.
  • Review of clinical data and patient histories.
  • Magnetic resonance imaging (MRI) for characteristic findings.

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Main Results:

  • Ten cases of Dandy-Walker syndrome were analyzed.
  • Approximately 80% of patients exhibited associated anomalies.
  • Characteristic findings were identified via MRI scans.
  • Lateral ventricular shunting to the peritoneum was the primary treatment.
  • A high incidence of complications was noted post-treatment.

Conclusions:

  • Dandy-Walker syndrome frequently involves multiple congenital anomalies.
  • MRI is crucial for diagnosing characteristic Dandy-Walker findings.
  • Surgical management via shunting can be effective but has significant complication risks.