Epidemiological and Clinical Aspects of Congenital Hydrocephalus in the Neonatal Department of Gabriel TouréTeaching Hospital Bamako Mali

Objective: The aim of our work was to access the epidemiological and clinical aspects of congenital hydrocephalus in the pediatrics department of Gabriel Touré teaching Hospital Bamako. Methods: We conducted a retro and prospective study that ran from January, 1st 2018 to July, 30, 2019. All newborns of 72 hours of life or less with hydrocephalus confirmed by sonography or CT scan were enrolled in this study. Results: During the study period, 5416 patients were hospitalized in the neonatology department. Among them, 39 patients (0.72%) presented congenital hydrocephalus and congenital infectious causes accounted for 25.6%. The sex ratio (M/F) was 1.78. Mothers were housewives and not educated in 79.5% and 64.1% respectively. The parents resided outside Bamako in 61.5% of cases. Prenatal consultations were conducted in 32 patients (82%). Consanguinity between the 2 parents was present in 38.5%. The antenatal diagnosis was made in 8 patients (20.5%). Vaginal delivery was the main mode of birth (67%). The average birth weight was 2930 g (1000 to 5400 g) and the average head circumference was 37.82 cm (26 - 55 cm). In 87.2% of cases, newborns were eutrophic. The main clinical signs were bulging anterior fontanel (46.2%), sunset gaze (20.5%), prominent forehead (48.7%), reduced face (20.5%), enlarged cranial sutures (43.6%), macrocrania (25.6%). Transfontanellar ultrasound and CT scan were performed in 51.3% and 48.7% of cases respectively. Associated malformations were spina bifida in 30.8% of cases followed by Dandy Walker malformation (5.1%), and omphalocele (2.5%). The neurological signs were diminished neonatal reflexes (51.3%), hypotonia (30.8%), motor deficit (38.4%), intracranial hypertension (25.6%), seizures 10.25% and psychomotor delay (43.6%). Surgery was performed in 8 neonates (20.5%). Ventriculo-peritoneal shunt (VPB) was the main treatment. Postoperative complications were infectious (37.5%) and mechanical complications (12.5%). The postoperative mortality rate was 12.5%.The overall mortality was 13 cases (33.3%). Conclusion: The hospital frequency of congenital hydrocephalus in our environment seems low but does not reflect reality.

β-Catenin Deletion in Regional Neural Progenitors Leads to Congenital Hydrocephalus in Mice

Congenital hydrocephalus is a major neurological disorder with high rates of morbidity and mortality;however,the underlying cellular and molecular mechanisms remain largely unknown.Reproducible animal models mirroring both embryonic and postnatal hydrocephalus are also limited.Here,we describe a new mouse model of congenital hydrocephalus through knockout ofβ-catenin in Nkx2.1-expressing regional neural progenitors.Progressive ventriculomegaly and an enlarged brain were consistently observed in knockout mice from embryonic day 12.5 through to adulthood.Transcriptome profiling revealed severe dysfunctions in progenitor maintenance in the ventricular zone and therefore in cilium biogenesis afterβ-catenin knockout.Histological analyses also revealed an aberrant neuronal layout in both the ventral and dorsal telencephalon in hydrocephalic mice at both embryonic and postnatal stages.Thus,knockout ofβ-catenin in regional neural progenitors leads to congenital hydrocephalus and provides a reproducible animal model for studying pathological changes and developing therapeutic interventions for this devastating disease.

Pressure-adjustable shunt in treatment of hydrocephalus associated with congenital intracranial anomalous venous return

Objective To investigate the value of Codman Hakim pressure adjustable shunt in the treatment of hydrocephalus associated with congenital intracranial anomalous venous return. Methods 2 patients whose diagnosis were confirmed by CT,MRI,MRV and DSA and programmable ventriculoperitoneal shunting was given.