Introduction
The difficulty in prognosticating the clinical and intellectual outcome of fetuses presenting with a Dandy-Walker malformation (DWM) comes from the great variety of cystic, median, and retrocerebellar malformations that probably have nothing in common and the variability of the definitions given to these lesions. In addition, many of these lesions can mimic each other. A correct diagnosis cannot be made without a good quality MRI including sagittal views of the vermis and T2-weighted images. We limited the diagnosis of DWM to those malformations with all of the following features: 1) a large median posterior fossa cyst widely communicating with the fourth ventricle, 2) a small, rotated, raised cerebellar vermis, 3) an upwardly displaced tentorium, 4) an enlarged posterior fossa, 5) antero-laterally displaced but apparently normal cerebellar hemispheres, 6) a normal brain stem. If any one of the previous criteria were not met, the malformation was considered distinct from DWM.

Dandy-Walker malformation: prenatal diagnosis and prognosis

Object The aim of this study was to assess the efficacy of various treatment options available for children with Dandy–Walker malformation (DWM) and to evaluate the role of endoscopic procedures in the treatment of this disorder. Methods The authors conducted a retrospective review of 72 children who underwent surgical treatment for DWM during a 16-year period. All patients underwent computed tomography scanning, and 26 underwent magnetic resonance (MR) imaging. The initial surgical treatment included ventriculoperitoneal (VP) shunt placement in 21 patients, cystoperitoneal (CP) shunt placement in 24, and combined VP and CP shunt insertion in three. Twenty-one patients underwent endoscopic procedures (endoscopic third ventriculostomy [ETV] alone in 16 patients, ETV with aqueductal stent placement in three, and ETV with fenestration of the occluding membrane in two). Three patients underwent membrane excision via a posterior fossa craniectomy. In the 26 patients who had undergone preoperative MR imaging, a...

Treatment options for Dandy-Walker malformation.

Objects: Forty-two patients with Dandy-Walker syndrome who were treated with different surgical modalities over a period of 8 years, from 1988 to 1996, at the Sanjay Gandhi Post Graduate Institute of Medical Sciences were reviewed in the present study. Methods: All the patients presented with hydrocephalus at the time of diagnosis. Association of other CNS anomalies was detected in 9 (22%) patients. Vermian hypoplasia was present in 36 (88%) cases, while cerebellar hypoplasia was documented in 27 (59%) of these patients. The treatment modality for these cases has continued to change in our institution over the years. Initially the ventriculoperitoneal shunt was the treatment of choice and was performed in 28 (66%) patients. Subsequently shunting of the cyst to the peritoneal cavity was performed in 7 (16%) patients. More recently, 3 of our patients were treated by fenestration of the cyst membrane and 4 others (9%), by ventriculocystoperitoneal shunting procedures. In this study the additional insertion of cystoperitoneal shunts was required in 8 (27%) of the 28 patients who had primary ventriculoperitoneal shunt procedures, because of either nonresolution or reappearance of a posterior fossa cyst. Six out of the 7 cases of primary cystoperitoneal shunts required additional ventriculoperitoneal shunt diversion because of persistent ventricular dilatation. In the group of 4 patients with primary ventriculocystoperitoneal shunts, only 1 patient required shunt revision. The patients on whom cyst membrane fenestration was performed required no additional procedures, except for 1 who already had a ventriculoperitoneal shunt in situ. Conclusions: Therefore, 18 of the total 42 patients could ultimately be made asymptomatic by ventriculocystoperitoneal shunting for one or the other of these reasons.

Dandy-Walker syndrome: different modalities of treatment and outcome in 42 cases

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.

https://journals.sagepub.com/doi/pdf/10.1177/0883073809338410

Dandy-Walker Malformation: Analysis of 19 Cases:

OBJECTIVE AND IMPORTANCE: Dandy-Walker malformation has conventionally been managed with placement of cystoperitoneal or ventriculoperitoneal shunts. However, associated aqueductal obstruction requires simultaneous drainage of both the supratentorial and infratentorial compartments. CLINICAL PRESENTATION: Three children with Dandy-Walker malformation and aqueductal obstruction were managed with endoscopic third ventriculostomy and placement of a stent from the third ventricle to the posterior fossa cyst. INTERVENTION: After an endoscopic third ventriculostomy was performed, the stent was placed from the third ventricle to the posterior fossa cyst through the thinnest part of the posteroinferior wall of the aqueduct. There was no operative morbidity. In one patient, the stent was malpositioned, requiring a repositioning. The endoscopic third ventriculostomy was successful in two patients, whereas it failed in one, requiring a ventriculoperitoneal shunt placement. CONCLUSION: Cystoventricular stent placement with endoscopic third ventriculostomy is a promising alternative in patients with Dandy-Walker malformation with aqueductal obstruction.

Endoscopic Third Ventriculostomy with Cystoventricular Stent Placement in the Management of Dandy-Walker Malformation: Technical Case Report of Three Patients

Upward transtentorial herniation of a Dandy-Walker cyst is a potentially fatal complication, particularly after solitary shunting of the lateral ventricle for associated hydrocephalus. Two patients with upward transtentorial herniation of their Dandy-Walker cysts are presented to illustrate the diagnostic value of magnetic resonance (MR) imaging with the demonstration of unique 'keyhole' and 'snail' signs on the axial and sagittal MR images, respectively. In both patients, double and simultaneous shunting en-Y of a lateral ventricle and the posterior fossa cyst resulted in the resolution of the herniation syndrome.

Dandy-Walker Cyst Upward Herniation: The Role of Magnetic Resonance Imaging and Double Shunts

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Dandy-Walker Cyst Upward Herniation: The Role of Magnetic Resonance Imaging and Double Shunts