Introduction: Hydrocephalus is a common central nervous system disorder in children. In spite of its importance, it has not been subjected to sufficient epidemiological studies, particularly in the developing countries. The aim of this study was to provide information from a representative series of newly diagnosed cases of infantile hydrocephalus on the birth prevalence, associated factors and mortality. Methodology: A prospective study was conducted over a 1-year period from April 1996 to March 1997 in the city of Al-Madinah Al-Munawarah, Saudi Arabia. Except for neural tube defects and brain tumors, all cases of hydrocephalus diagnosed within the first 28 days of life were included. A control group of 104 subjects was studied for comparison. Information about antenatal, natal and early postnatal periods were obtained by interviewing the mothers of the newborns. Results: During the study period, 26 cases of infantile hydrocephalus were detected. The birth prevalence was 1.6 per 1,000 live births. There was no sex preponderance as the male to female ratio was 1.2:1. Multiple pregnancies were detected in 21 (81%) cases. Nineteen (73%) cases were the product of consanguineous parent and 4 patients had a positive family history of hydrocephalus. The number of preterm infants was 16 (62%). The number of low birth weights (less than 2,500 g) was 18 (69%). An Apgar score of less than 8 occurred in 18 (69%) cases. The mode of delivery was vaginal in 15 (58%) women. The 6 months mortality rate was 23% (6 infants). Conclusion: The birth prevalence of infantile hydrocephalus in this study was significantly higher than in the developed countries. A positive family history of hydrocephalus, low birth weight, low Apgar score and abdominal delivery were found to be associated factors. The mortality rate in the first 6 months of life was significantly higher in hydrocephalus infants than in controls.

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