Follow-up of defecation, urination, growth and development in children with Currarino syndrome.

Background: Currarino syndrome (CS) is a rare congenital disorder associated with anorectal malformations (ARMs), presacral masses, and sacral dysplasia. While survival has improved, long-term functional and growth outcomes remain lacking in evaluation. This study aims to evaluate long-term defecation/urinary function and growth patterns in pediatric CS patients and to summarize infant treatment experience. Methods: We conducted a retrospective analysis of 15 children with complete CS who underwent anoplasty between April 2015 and August 2024. Functional outcomes were assessed using Rintala scores and Dysfunctional Voiding Symptom Scores (DVSS) in children older than 4 years. Growth parameters were evaluated through Z-scores for weight, height, and body mass index (BMI) in children above 2 years. Additionally, we performed a systematic literature review, pooling data from 74 patients to analyze the impact of surgical timing on functional outcomes. Results: At a mean follow-up of 67.9±37.6 months, the cohort demonstrated generally favorable functional outcomes (mean Rintala score: 16.3±3.0; median DVSS: 0). However, growth assessment revealed concerning trends, with mean Z-scores for weight, height, and BMI all below population norms (-0.42±1.30, -0.28±1.45, and -0.46±1.50, respectively). Age-stratified analysis indicated progressive deterioration in growth in older children. Additionally, pooled analysis of 89 patients showed that anoplasty within the first year of age was associated with a lower rate of defecation dysfunction (P=0.03). Conclusions: Early anoplasty (within the first year) correlates with better defecation outcomes. Age-related growth faltering highlights the need for sustained nutritional surveillance.