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Follow-up of defecation, urination, growth and development in children with Currarino syndrome
Xinyue Tan1, Guantong Li1, Yuyan Jin1
1Department of Neonatal Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Insights
Early surgery for Currarino syndrome (CS) improves bowel function. However, children with CS experience growth issues, requiring ongoing nutritional monitoring.
Area of Science:
- Pediatric Surgery
- Genetics
- Developmental Biology
Background:
- Currarino syndrome (CS) is a rare congenital disorder characterized by anorectal malformations (ARMs), presacral masses, and sacral dysplasia.
- While survival rates have improved, long-term functional and growth outcomes in pediatric CS patients require further evaluation.
- Understanding these long-term outcomes is crucial for optimizing patient care and management strategies.
Purpose of the Study:
- To evaluate long-term defecation and urinary function in pediatric patients with Currarino syndrome.
- To assess growth patterns and identify potential growth faltering in this cohort.
- To analyze the impact of surgical timing, specifically anoplasty, on functional outcomes.
Main Methods:
- Retrospective analysis of 15 children with complete CS who underwent anoplasty.
- Functional outcomes assessed using Rintala scores and Dysfunctional Voiding Symptom Scores (DVSS) for patients over 4 years old.
- Growth parameters evaluated using Z-scores for weight, height, and BMI for patients over 2 years old.
- Systematic literature review pooling data from 74 patients to analyze surgical timing effects.
Main Results:
- At a mean follow-up of 67.9 months, generally favorable functional outcomes were observed (mean Rintala score: 16.3; median DVSS: 0).
- Growth parameters showed concerning trends, with mean Z-scores for weight, height, and BMI below population norms.
- Age-stratified analysis revealed progressive growth deterioration in older children.
- Pooled analysis of 89 patients indicated that anoplasty within the first year of age was linked to a lower rate of defecation dysfunction (P=0.03).
Conclusions:
- Performing anoplasty within the first year of life is associated with improved defecation outcomes in Currarino syndrome patients.
- Age-related growth faltering is a significant concern, necessitating sustained nutritional surveillance.
- Early surgical intervention and ongoing monitoring are vital for optimizing functional and growth outcomes in pediatric CS.
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.
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