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2022, Number 4

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Finlay 2022; 12 (4)

Dandy-Walker Syndrome. Case report and literature review

González ÁWE, Intento GO, Cortizo MNB
Full text How to cite this article

Language: Spanish
References: 10
Page: 475-479
PDF size: 143.60 Kb.


Key words:

genetic predisposition, central nervous system, diagnostic imaging, case reports.

ABSTRACT

Dandy-Walker syndrome is associated with cystic dilatation of the fourth ventricle, varying degrees of abnormality of the cerebellar vermix, and supratentorial hydrocephalus. It is mainly based on an abnormal development of the cerebrospinal fluid communication pathways. Genetic predisposition associated with malformations and chromosomal alterations has been suggested. The diagnosis can be carried out during the prenatal or postnatal stage, clinically and diagnostic imaging studies. We present the case of a newborn who was admitted to the Neonatal Intensive Care Unit due to hypotonic, tongue protrusion with sucking disorders. A transfontanellar ultrasound was performed, with dilation of the lateral ventricles. In addition to the dilation of the lateral ventricles, a cyst in the choroid plexus and signs of brain atrophy were observed. She was diagnosed with Dandy-Walker syndrome, without surgical criteria because she did not present signs of hydrocephalus. The case is presented because it is a rare malformation of the central nervous system.


REFERENCES

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  2. Auqui DA, Espinoza EE, Cahuasqui JS, Espín PA. Diagnóstico prenatal del Síndrome de Dandy Walker reporte de un caso. Dom Cien[Internet]. 2020[citado 15/11/2021];6(4):[aprox. 4p.]. Disponible en: Disponible en: https://www.dominiodelasciencias.com/ojs/index.php/es/article/view/1615 2.

  3. Rahmani R, Lotfian N, Rahmani M, Amiri M, Darafshi R. Dandy-walker syndrome: a rare case report. J Mazandaran Univer Med Scien. 2018;27(156):218-25.

  4. Monteagudo A. Dandy-Walker Malformation. Am J Obstet Gynecol. 2020;223(6):B38-41.

  5. Zamora EA, Ahmad T. Dandy Walker Malformation[Internet]. Bethesda:National Library of Medicine;2019[citado 4/7/2021]. Disponible en: Disponible en: https://www.ncbi.nlm.nih.gov/books/NBK538197/ 5.

  6. Santoro M, Coi A, Barišić I, Garne E, Addor MC, Bergman JE, et al. Epidemiology of Dandy-Walker malformation in Europe: a EUROCAT population-based registry study. Neuroepidemiology. 2019;53(3-4):169-79.

  7. Zacharis K, Kravvaritis S, Papakonstantinou A, Charitos T, Chrysafopoulou E, Fouka A. Dandy-Walker Malformation: Prenatal sonographic diagnosis. HJOG. 2020;19(2):101-4.

  8. Orphanet. Malformación de Dandy-Walker aislada[Internet]. Paris:Orphanet;2022[citado 21/9/2022]. Disponible en: Disponible en: https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=ES&Expert=217 8.

  9. Rodríguez OL, Villafuerte D, Melo ZA, Gil M. Presentación de un caso con síndrome de Dandy Walker. CCM[Internet]. 2017[citado 27/3/2021]. Disponible en: Disponible en: https://scielo.sld.cu/scielo.php?script=sci_arttext&pid=S1560-43812017000400025 9.

  10. Dastoli PA, Nicácio JM, Da Costa MD, Suriano IC, Da Silva MC, Cavalheiro S. Hydrocephalus and Dandy-Walker Malformation: a review. Arch Pediatric Neurosur. 2020;2(3):e442020.




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