Abstract
A healthy 42-year-old G10 P7 mother with seven healthy children with the same partner conceived naturally while taking prenatal folic acid supplementation. She was referred to a perinatal assessment team at a tertiary hospital following a 21-week ultrasound showing extensive lumbosacral meningomyelocele and Chiari II malformation. Serial ultrasounds documented progressive polyhydramnios, massive ventriculomegaly, poorly developed mantle, 120° kyphoscoliosis and club feet. The referring family physician and obstetrics, genetics, neonatology, neurosurgery and palliative care were involved throughout the pregnancy. The consultants’ impressions of profound neurological damage led to counselling about options for termination of pregnancy, comfort measures alone, or resuscitative care and re-evaluation after delivery. The parents hoped for a live-born infant and time to hold her while alive. Following extensive discussions with their family physician, they chose a trial of resuscitative care with immediate neonatology assessment. A scheduled Caesarean section under spinal anesthetic, preceded by ultrasound-guided ventriculocentesis to facilitate delivery, was performed at 37 weeks’ gestation. Resuscitation included intubation and administration of intravenous fluid. The neonatologist confirmed a large open lumbosacral meningomyelocele. The baby became increasingly hypoxic despite attempts to optimize ventilation and, in discussion with the parents, the neonatologist discontinued mechanical ventilation while the baby was held in her father’s arms. Death occurred at 2 h of age.
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