Surgical Treatment of 13 Pediatric Patients with Dandy-Walker Syndrome

Yüceer, Nurullah; Mertol, Tansu; Arda, Nuri

doi:10.1159/000106383

Cited by 14 publications

(7 citation statements)

References 50 publications

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“…In this regard, prominent gyral development occurs after 25 weeks [ 38 ] while the gestational ages of fetuses with DWM at the time of MRI were mostly less than 25 weeks in this study. We also observed associated extracerebral anomalies, including congenital heart diseases, urinary tract malformations, and craniofacial abnormalities, as previously reported [ 39 , 40 ]. Fetuses with chromosomal abnormalities tend to have multiple anomalies; the results from this study indicate that fetuses presented with typical clinical demographics that are observed in children with DWM.…”

Section: Discussionsupporting

confidence: 88%

Regional brain development in fetuses with Dandy-Walker malformation: A volumetric fetal brain magnetic resonance imaging study

et al. 2022

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Dandy-Walker malformation (DWM) is a common prenatally diagnosed cerebellar malformation, characterized by cystic dilatation of the fourth ventricle, upward rotation of the hypoplastic vermis, and posterior fossa enlargement with torcular elevation. DWM is associated with a broad spectrum of neurodevelopmental abnormalities such as cognitive, motor, and behavioral impairments, which cannot be explained solely by cerebellar malformations. Notably, the pathogenesis of these symptoms remains poorly understood. This study investigated whether fetal structural developmental abnormalities in DWM extended beyond the posterior fossa to the cerebrum even in fetuses without apparent cerebral anomalies. Post-acquisition volumetric fetal magnetic resonance imaging (MRI) analysis was performed in 12 fetuses with DWM and 14 control fetuses. Growth trajectories of the volumes of the cortical plate, subcortical parenchyma, cerebellar hemispheres, and vermis between 18 and 33 weeks of gestation were compared. The median (interquartile range) gestational ages at the time of MRI were 22.4 (19.4–24.0) and 23.9 (20.6–29.2) weeks in the DWM and control groups, respectively (p = 0.269). Eight of the 12 fetuses with DWM presented with associated cerebral anomalies, including hydrocephalus (n = 3), cerebral ventriculomegaly (n = 3), and complete (n = 2) and partial (n = 2) agenesis of the corpus callosum (ACC); 7 presented with extracerebral abnormalities. Chromosomal abnormalities were detected by microarray analysis in 4 of 11 fetuses with DWM, using amniocentesis. Volumetric analysis revealed that the cortical plate was significantly larger in fetuses with DWM than in controls (p = 0.040). Even without ACC, the subcortical parenchyma, whole cerebrum, cerebellar hemispheres, and whole brain were significantly larger in fetuses with DWM (n = 8) than in controls (p = 0.004, 0.025, 0.033, and 0.026, respectively). In conclusion, volumetric fetal MRI analysis demonstrated that the development of DWM extends throughout the brain during the fetal period, even without apparent cerebral anomalies.

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Section: Discussionsupporting

confidence: 88%

Regional brain development in fetuses with Dandy-Walker malformation: A volumetric fetal brain magnetic resonance imaging study

et al. 2022

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“…There are various treatment options available for children with Dandy-Walker malformation, such as shunt placement, either ventriculoperitoneal, cystoperitoneal, or combined ventriculoperitoneal, and cystoperitoneal shunt, membrane excision, and endoscopic procedures. 4,5,13,14 Shunt placement in patients with Dandy-Walker malformation resulted in significant lower morbidity and mortality. 13 However, permanent shunting is not without complications.…”

Section: Discussionmentioning

confidence: 99%

“…In older children, symptoms of increased intracranial pressure such as irritability and vomiting and signs of cerebellar dysfunction such as gait disturbance and lack of muscle coordination may occur. 4,5 Other symptoms include cranial nerves dysfunction and abnormal breathing patterns. 6 Dandy-Walker malformation is frequently associated with disorders from other brain structures and with various extracranial abnormalities.…”

Section: Discussionmentioning

confidence: 99%

Dandy-Walker Malformation: Analysis of 19 Cases

2009

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Dandy-Walker malformation is a congenital disorder that involves the cerebellum and fourth ventricle. Regarding treatment, there is still controversy over the optimum surgical management. In the current study, we present 19 consecutive cases of Dandy-Walker malformation diagnosed between January 1992 and January 2008 that were treated in our institute. All patients presented with hydrocephalus at the time of diagnosis and were treated surgically. Combined drainage of the ventricular system and posterior fossa cyst, using a 3-way connector was performed in 5 patients. Posterior fossa cyst drainage alone was performed in 10 patients and the remaining 4 patients were treated by ventricular drainage alone. All patients improved after treatment. Dandy-Walker malformation is a developmental abnormality of the central nervous system associated with various brain and extracranial abnormalities. Surgical treatment remains controversial, whereas prognosis varies greatly according to the severity of syndrome and associated comorbidities.

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“…In older children with Dandy-Walker syndrome, symptoms of increased intracranial pressure (ICP), including irritability and vomiting, and signs of cerebellar dysfunction, including gait disturbance and lack of muscle coordination, may occur [2]. In the present case, the patient had general muscular dystrophy and macrocephalus as well as craniofacial abnormalities such as micrognathia and cleft palate, which can make airway management challenging.…”

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confidence: 87%