Craniorachischisis Totalis: Gross And Microscopic Findings In A Rare Lethal Neural Tube Defect

ABSTRACT Craniorachischisis totalis is a rare, lethal neural tube defect caused by complete failure of neural tube closure. A 41-year-old multigravida (G3P2L2) with intermittent vaginal spotting underwent ultrasonography, which revealed a 13-week-3-day fetus. A 45 g male fetus was spontaneously expelled vaginally. Gross examination showed absent calvaria and cerebral tissue with a completely open vertebral column and exposed neural tissue, consistent with craniorachischisis totalis. No gross abnormalities were identified in the thoracic or abdominal viscera. This case highlights the characteristic morphology of this fatal anomaly and underscores the importance of early antenatal diagnosis, folic acid supplementation, and genetic counselling.