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Haddadi K, Zare A, Asadian L. Dandy-Walker Syndrome: A Review of New Diagnosis and Management in Children. J. Pediatr. Rev 2018; 6 (2) :47-52
URL: http://jpr.mazums.ac.ir/article-1-193-en.html

Context: Dandy-Walker syndrome (DWS) or malformation (DWM) characterizes a hereditary abnormality categorized via agenesis or hypoplasia of the cerebellar vermis, cystic dilation of the fourth ventricle, and expansion of the posterior fossa with or without hydrocephalus. This review aimed at describing the basic clinic pathologic features of DWS, its diagnosis by other central nervous system (CNS), systemic genetic relatives, and new treatment modalities.
Evidence Acquisition: Training and publishing in this field is very limited. Among 54 articles in this era, a total of in 31 articles were included in the current evaluation.
Results: The etiology of DWS is uncertain, however, is supposed to be a consequence of the mixture of ecological and genetic causes. The most common postnatal appearance is macro crania. Additional signs and symptoms could include huge posterior fossa, sun- set sign, seizures, spasticity, apnea, respiratory failure, delayed milestones, hydrocephalus, and increased intracranial pressure. Currently, DWS is diagnosed completely with anatomic definitions that can be recognized on ultrasound or Magnetic resonance imaging (MRI) and computed tomography (CT) intrauterine or postnatal.
Conclusions: Dandy walker syndrome occurs as a comparatively rare origin of hydrocephalus and might go along with numerous inherited CNS and systemic anomalies. Even though it is usually treated for related hydrocephalus, the abnormality causes no de- tectable clinical syndrome. It is identified with antenatal or postnatal imaging via ultrasound, CT, or MRI. Hydrocephalus and the cyst in posterior fossa can be treated with surgery, via shunting processes, endoscopy, or both. Prognosis has enhanced meaningfully from the time of its original report; yet, it is typically dependent on the accompanying abnormalities.