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Case 355

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Newborn female patient, term gestation with induced vaginal labour, APGAR 6/5, kept in 5 until 20 minutes of life. High risk prenatal due to neurological malformations, IUGR and hydrocephaly, observed in the 31st week ultrasound (US) and severe bilateral ventriculomegaly, enlarged posterior fossa and III ventricle and hypoplasia of the vermis observed in the 36th week US. After stabilization, patient was submitted to a brain MRI for neurological evaluation.

Considering the clinical data and the images, the most likely diagnosis is:

a) Type II Arnold Chiari.

25%

b) Blake’s pouch cyst.

25%

c) Dandy-Walker malformation

25%

d) PHACE syndrome.

25%
   

Image analysis

 

Image 1 - Analysis: Magnetic resonance imaging (MRI) of the brain, axial view at the pons level, T1-weighted, showing hypoplasia of the vermis (green arrow) and enlarged posterior fossa.

 

Image 2 - Analysis: Magnetic resonance imaging (MRI) of the brain, axial view at the lateral ventricles level, T2-weighted, lateral ventricles level, showing supratentorial ventricle dilatation (green arrows).

 

Image 3 - Analysis: Magnetic resonance imaging (MRI) of the brain, sagittal view, T1 BRAVO-weighted, midline level, showing partial absence of the corpus callosum (blue arrow) and cerebellar vermis hypoplasia (green arrow), with enlarged posterior fossa.

 

Highlights

        - Dandy Walker malformation consists in the cystic dilatation of the IV ventricle, cerebellar vermis hypoplasia, enlarged posterior fossa, hydrocephaly and median and lateral aperture atresia;

       - Etiopathogenesis is not well defined, although it may be associated with genetic alterations, infectious diseases and prenatal exposure;

       - Intrauterine diagnosis may be performed from the 18th week with US;

       - Supportive and symptomatic treatment must be established to control complications;

       - Worst prognosis is associated with precocious manifestation of the disease.

References

       - Wilson M, Lindsay D, Levi C, Ackerman T, Gordon W. US case of the day. Dandy-Walker variant with agenesis of the corpus callosum. RadioGraphics. 1994;14(3):678-681.

       - Fonseca M, Vieira M, Nascimento S, Esposito S. Malformação de Dandy-Walker. Revista da Faculdade de Ciências Médicas de Sorocaba. 2017;19(1):4-5.

       - Khan R, De Leon C, Romagna E, Ballardin P. Variante de Dandy Walker – relato de caso. Scientia Medica. 2009;19(4):186-191.

       - Cueva-Núñez J, Lozano-Bustillo A, Irias-Álvarez M, Vásquez-Montes R, Varela-González D. Variante de Dandy Walker: reporte de un caso. Revista Chilena de Pediatría. 2016;87(5):406-410.

       - Monteagudo A, Timor-Tritsch I. Prenatal diagnosis of CNS anomalies other than neural tube defects and ventriculomegaly [Internet]. Uptodate.com. 2018 [cited 22 September 2018]. Available from: http://bit.ly/2IauISU.

       - Bosemani T, Orman G, Boltshauser E, Tekes A, Huisman T, Poretti A. Congenital Abnormalities of the Posterior Fossa. RadioGraphics. 2015;35(1):200-220.

Author

Bruno Campos Santos, 6th year medical student at Universidade Federal de Minas Gerais.

Email: bruno_campos[at]outlook.com

 

Gessica Roberta Olímpia Piedade Carneiro, 6th year medical student at Universidade Federal de Minas Gerais.

Email: gessica.olimpia4[at]gmail.com

 

Supervisor

Karla Emília de Sá Rodrigues, MD, Ph.D., Associated Professor and Doctor of the Department of Pediatrics at the Medical School of Universidade Federal de Minas Gerais.

Email: kesrodrigues[at]gmail.com

 

Carlos Magno da Silva, MD, Ph.D., Voluntary Professor and Doctor of the Radiology Service at the Hospital das Clínicas da UFMG.

Email: carlosbresil[at]hotmail.com

                                

Reviewers

Mateus Nardelli, Fernando Amorim, José Nelson Mendes Vieira,  MD, Ph.D., Viviane Santuari Parisotto Marino, MD, Ph.D.

 

Translated by

Bruno Campos Santos, 6th year medical student at Universidade Federal de Minas Gerais.

E-mail: bruno_campos[at]outlook.com

Test question

(Medical Residence 2015 – EBSERH - ADAPTED) The foetal CNS anomaly in which, at US, the liquid located at an dilated cisterna magna  clearly communicates with the IV ventricle through a malformation in the cerebellar vermis and the cerebellar hemispheres are obviously distinct, with usual ventriculomegaly, is named:

a) Arnold Chiari malformation.

25%

b) Dandy-Walker syndrome.

25%

c) Agenesis of the corpus callosum.

25%

d) Hydrocephaly.

25%

e) Myelomeningocele.

25%
   

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