Congenital paraplegia. A complication of multifetal pregnancy reduction?

Abstract

A 31 year old woman was admitted to a tertiary level perinatal centre in preterm labour at 26 weeks of gestation. This was an in vitro fertilisation pregnancy following an eight-year history of infertility. At implantation there were four viable conceptuses. At ten weeks of gestation there had been an attempt at multifetal pregnancy reduction, with the aim of reducing the quadruplet pregnancy to a twin pregnancy. Both the in vitro fertilisation and selective reduction procedures had been performed overseas, and it was not possible to obtain further details of these.Her first local antenatal visit was at 14 -15 weeks of gestation. Ultrasonography at that time recorded the presence of three viable fetuses and one non-viable fetus who appeared anencephalic. Ultrasound examinations at 22 and 25 weeks of gestation showed that one of the three viable fetuses had oedema and talipes of both feet. It is presumed the fourth fetus with anencephaly had been resorbed.Following the onset of spontaneous preterm labour at 26 weeks the mother was given antenatal steroids. At 28 weeks she had an emergency caesarean section for unsuppressed labour. Three live born infants were delivered, two girls and one boy. Their birthweights were appropriate for gestational age.Triplet one was found to have fixed flexion contractures of her hips, knees and ankles (Fig. 1). Her hips were widely abducted and her ankles were in the equinovarus position. There was no spontaneous movement of her lower limbs and no response to noxious stimuli. Her lower limbs were wasted and arreflexic; however, there was no abnormality of her upper limbs. Her buttocks were wasted and a sacral 'dimple' was evident, the anus was patulous, and there was constant dribbling of urine. A sensory level was approximated at T10. She was not dysmorphic, and her head circumference was on the 90th centile. The other two infants were normal.Her neonatal course was complicated by respiratory distress syndrome requiring exogenous surfactant, and she was intubated for two days. She had apnoea of prematurity and was on continuous positive airways pressure until day 35. A patent ductus arteriosus was closed with indomethacin and she was treated with phototherapy for hyperbilirubinaemia.Ultrasound examination of her head and kidneys was normal. Ultrasound examinations of her spine showed a 2 mm  3 mm  5 mm cystic lesion in the cord at thoracic vertebrae T10-11 level. There was no bony abnormality of the vertebrae and sacrum, thus excluding spina bifida and sacral agenesis. A skeletal survey showed a scoliosis, there being eleven ribs on the right with fusion of ribs five and six. There were no other bony abnormalities. Magnetic resonance imaging of the spine revealed a thin dysplastic cord caudally from T5 -7. Cystic areas were confirmed at T10 -11 and lumbar vertebrae L2-L3. The spinal cord was tethered at L5-S1. Her placental pathology showed a pale underperfused placental plate with focal amnionitis. The placenta was trichorionic and triamniotic. Serology for toxoplasmosis, rubel...