Craniorachisis Totalis

Abstract

Introduction: Craniorachischisis totalis is the most severe form of neural tube anomalies. It is a rare malformation of the central nervous system and the most complete form of craniorachisis, which presents together with anencephaly and
total spina bifida, and is fatal.
Objective: To report the rare case of a newborn with malformation due to craniorachischisis totalis.
Case report: A 32-year-old pregnant woman, with health history, pregnancy 7, for 4, 2 spontaneous abortions, came to the New Amsterdam Hospital in Guyana, with imprecise amenorrhea, she refered intermittent intense pain in the lower
abdomen, radiating towards the lumbar region, together with fluid loss. Eutocic delivery was performed. She was assisted by personnel from the Cuban Medical Brigade. A severely depressed newborn was received, weighing 1400 g. the
newborn showed total opening of the spine and back of the skull, with exposure of the nervous tissue - spinal cord and brain-, and double facial massif. He passed away 5 minutes after birth.
Conclusions: Craniorachischisis totalis jointly presents anencephaly and total spina bifida, and it is lethal. Prenatal diagnosis is possible by ultrasound monitoring. Preconception care should be extended to all women of reproductive age, as part of medical care in primary care, in all countries, to prevent children from being born with this type of congenital malformations.