Abstract
Malformations (or cysts) are developmental malformations in which the Cerebellar Vermis may be partially or completely absent, with resulting dilation of the 4th Ventricle forming a Cyst. It is thought that the defect arises from abnormal embryogenesis of the roof of the 4th Ventricle. A milder form is termed the Dandy-Walker Variant. This disorder may account for approximately 5% to 10% of cases of Ventriculomegaly. This study aims to reveal particular cases of Danty Walker Cases when associated with hydrocephalus, in the African context. It is a retrospective and descriptive study carried out at the Neurosurgery department of the Friendship hospital of Kipe in Guinea for 36 months, from June 2012 to August 2015. Of the 120 patients hospitalized for hydrocephalus, 32 cases have exhibited the Dandy Walker cyst. Among the 32 patients, 15 were of male and 17 were of female gender at a sex ratio (H/F) of 0.88; 1. The dominant clinical symptoms that were observed in our subjects were macrocrania (32 cases), bulging fontanelle (25 cases), sunsetting eyes (19 cases). The degree of macrocrania was noted to be minimal in 1 case only, mild in 5 cases, moderate in 8 cases, and excessive in 18 cases. Furthermore, CT scan results showed evidence of triventricular hydrocephalus in 14 cases, and tetraventricular hydrocephalus in 18 cases. The anatomical variations encountered among others were, MDW in 15 cases, DWV in 11 cases et DWC in 6 cases. The calculated Evan’s index was equal to 0.3 in 6 patients and greater than 0.3 in 26 patients. The operative techniques most commonly widely conducted were DVP (16 caes), VCS (13 cases) and DKP (3 cases). The average duration of hospitalization was 20 days with extreme range of 1 to 49 days. The post operative period was uneventful in 16 cases, clinically unchanged in 5 cases and post operative deaths were reported in 11 cases. The principal causes of death originated from the presence co-existing infections, respiratory distress, anemia and malarial sickness.
Highlights
Dandy-Walker malformation (DWM) is a rare congenital disorder which involves the posterior cerebral fossa
The patients selected for the study had a hydrocephalus which was associated with a Dandy walker cyst confirmed by CT and/or transfontanellar ultrasound imaging
We have assembled a total of 597 hospitalized patients in the neurosurgical service during the period of study, with 120 cases of hydrocephalus among which 32 cases were associated with DWM
Summary
Dandy-Walker malformation (DWM) is a rare congenital disorder which involves the posterior cerebral fossa. Its incidence is noted to be betwen 1/25000 and 1/35000 per live births and is often associated with a hydrocephalus. Hydrocephalus associated with DWM in infants is a pathology which can be observed at any age, in which the etiologies are multiple and often modulated by genetic factors, and/or environmental factors. It can ensue as a consequence of congenital malformations, infections, trauma, disequilibrium in cerebrospinal fluid (CSF) pressure, vascular lesions [2]. The imaging techniques available for antenatal diagnosis used are ultrasound, magnetic resonance imagin (MRI), CT imaging, for establishing a precise anatomical mapping, and to locate any of the associated malformations: agenesis of the corpus callosum, occipital meningocele, meningomyelocele, Joubert syndrome, WalkerWalburg syndrome, Arnold Chiari [5]
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