Case of the Week #605
Femicare, Center of prenatal ultrasonographic diagnostics, Martin, Slovakia
A 35-year-old primigravida presented to our office at 20 weeks of gestation for routine second-trimester screening. The following images and videos show an interesting cranial anomaly.
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Answer
We present a case of Dandy-Walker malformation
Our images revealed following cranial findings:
- Upwardly rotated hypoplastic cerebellar vermis (brainstem-vermis angle 64,45°, craniocaudal vermian diameter 5,3 mm, anteroposterior vermian diameter 8,5 mm)
- High insertion of the tentorium, wide brainstem-tentorium angle (38,78°)
- Cystic structure within posterior fossa with inferolateral location of the choroid plexuses outside of the cystic structure
- No other anomalies of the brain were seen
A female infant was delivered at term and the findings were confirmed. At the time of publication, the patient is four months old and doing well. Her neurologic examination was normal. The spectrum of the severity may vary from severe to mild, which is represented by our case.
The differential diagnosis includes other anomalies of the Dandy-Walker spectrum, such as Blake’s pouch cyst or vermian hypoplasia. The above mentioned findings, the inferolateral displacement of the taenia–tela choroidea complex, and choroid plexus distant from the vermis are in favor of Dandy-Walker malformation [1-4].
References:
[1] Volpe P, De Robertis V, Volpe G, et al. Position of the choroid plexus of the fourth ventricle in first- and second-trimester fetuses: a novel approach to early diagnosis of cystic posterior fossa anomalies. Ultrasound Obstet Gynecol. 2021 Oct;58(4):568-575.
[2] Paladini D, Donarini G, Parodi S, Chaoui R. Differentiating features of posterior fossa at 12-13 weeks' gestation in fetuses with Dandy-Walker malformation and Blake's pouch cyst. Ultrasound Obstet Gynecol. 2019 Jun;53(6):850-852.
[3] Whitehead MT, Vezina G, Schlatterer SD, et al. Taenia-tela choroidea complex and choroid plexus location help distinguish Dandy-Walker malformation and Blake pouch cysts. Pediatr Radiol. 2021 Jul;51(8):1457-1470.
[4] Whitehead MT, Barkovich MJ, Sidpra J, et al. Refining the Neuroimaging Definition of the Dandy-Walker Phenotype. AJNR Am J Neuroradiol. 2022 Oct;43(10):1488-1493.
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