586 - Neurologic morbidity in infants with congenital end-stage kidney disease requiring kidney replacement therapy

Publication Number: 3567.586

Sarah E. Taylor, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States; Betul Derinkuyu, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States; J. Michael Taylor, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States; Stefanie L. Riddle, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States; Alexander Bondoc, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States; Charu Venkatesan, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States; Meredith P. Schuh, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States

Background: Infants with severe congenital anomalies of the kidney and urologic tract (CAKUT) require kidney replacement therapy (KRT) shortly after birth. Survival of this population has increased through advances in fetal and neonatal interventions. Patients have highly complex medical needs, and the acute and long-term neurological morbidities are poorly characterized.

Objective: Characterize acute and long-term neurologic morbidity in infants with end-stage kidney disease requiring KRT.

Design/Methods: This was a single center, IRB-approved retrospective cohort study of 26 patients prenatally diagnosed with CAKUT requiring initiation of KRT within 60 days after birth. Their postnatal course was reviewed for presence of neurologic morbidity, including acute neurologic events and longer-term neurodevelopmental outcomes.

Results: Median gestational age at birth was 36 weeks 3 days. The majority, 23 patients (88.5%), started KRT via hemodialysis in the first two weeks of life; 3 (11.5%) did not undergo hemodialysis and started peritoneal dialysis by 60 days of life. Acute neurologic events that occurred early in the patients’ postnatal course included stroke, seizures, and intracranial infection. Of 26 patients, 8 (30.8%) suffered a stroke in the first 6 months of life; 3 (11.5%) suffered two discrete strokes. Stroke types included both ischemic and hemorrhagic infarcts. Notably, strokes were more likely to be asymptomatic (63.6%) than symptomatic (36.4%). There were 8 patients (30.8%) who had seizures in the first year of life. Seizures occurred in the setting of stroke, systemic infection, hyponatremia, or were cryptogenic. Posterior reversible encephalopathy syndrome (PRES) occurred in 2 patients (7.7%) in early childhood and was accompanied by seizures in both cases. Apart from stroke or PRES, MRI revealed further pathology such as microhemorrhages and diffuse cerebral volume loss. Follow-up of neurodevelopmental outcomes in 14 patients older than 3 years identified delay or deficits in motor (78.5% delayed, 28.6% non-ambulatory), language (21.4% delayed, 14.3% non-verbal), and feeding (93% requiring gastrostomy tube at 3 years old) skills. About half of patients with acute symptomatic seizures developed epilepsy.

Conclusion(s): Neurologic morbidity is common in neonates with CAKUT requiring KRT. Morbidity includes acute events like stroke or seizures, as well as delays or deficits in neurodevelopment. Prenatal counseling should review this high risk for neurologic complications. As survival continues to improve, our focus should shift towards reducing these morbidities.