485 - Developmental Assessment Using Ages and Stages Questionnaires in children with Congenital Anomalies of the Kidney and Urinary Tract.

Poster Number: 485
Publication Number: 485.1576

Background: Congenital Anomalies of the Kidney and Urinary Tract (CAKUT) are common. Severe forms can lead to kidney failure that have been historically lethal even before birth. Now care options may include fetal intervention (i.e. vesicoamniotic shunt, amnioinfusions), infant dialysis, and eventual kidney transplant; leading to a new population surviving into childhood and beyond. Little is known about developmental screening of children with CAKUT receiving fetal intervention, though there is high risk of delays in pediatric chronic kidney disease (CKD). Ages and Stages Questionnaires (ASQ) are validated tools for developmental screening in children.

Objective: The aim of this study is to evaluate the ASQ results in children with fetal CAKUT.

Design/Methods: Retrospective, chart review of patients with CAKUT from 2010-2022. Demographic, obstetric, and pediatric data were collected. The ASQ was completed by the primary caregiver at prescribed ages. The ASQ has 5 domains: communication, gross motor, fine motor, problem solving and personal-social. For each domain, based on their score, children are categorized as “normal” (above the mean), “at risk” (1–2 standard deviations below the mean) and “abnormal” (>2 standard deviations below mean).

Results: Of 25 patients, 68% were males and 36% underwent some type of fetal intervention at a median gestational age (GA) of 21 weeks, delivered at a median GA of 37 weeks. Median length of NICU stay was 26 days (IQR 2, 80). 45% had CKD; 7 (28%) received infant dialysis and were transplanted at median age of 24 months. 47 ASQs were completed. Figure 1 reflects mean scores across ages and Figure 2 categorized by age group, with greatest compromise seen in gross motor domain (45% at risk or abnormal); scores improved after 22-24 months across domains. 12 (26%) were globally delayed (affecting >2 domains). Figure 3 compares groups requiring fetal interventions versus not; lower scores across all domains were seen in those requiring fetal interventions.

Conclusion(s): Infants with severe CAKUT require close developmental follow up especially after fetal interventions, as there is significant risk for motor and global delays. Most infants fall below the mean score on any of the five domains between 6-12 months of age, showing trend towards improvement at 22-24 months. This improvement in scores after 22-24 months may be related to intensive therapies received as well as kidney transplant. Further work dedicated to understanding neurodevelopmental outcomes following fetal kidney failure is needed.