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KMID : 1152420200260020046
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Advances in Pediatric Surgery
2020 Volume.26 No. 2 p.46 ~ p.53
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Clinical Characteristics and Treatment of Currarino Syndrome: A Single Institutional Experience
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Kim Hyun-Jeong
Ho In-Geol
Ihn Kyong
Han Seok-Joo
Oh Jung-Tak
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Abstract
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Purpose: Currarino syndrome is a rare disease, and understanding its clinical characteristics is important because it involves complex anomalies and it requires a multidisciplinary approach for treatment. However, the accumulation of treatment experiences is challenging, and studies on this disease remain insufficient. Our study aimed to review the clinical characteristics and treatment of Currarino syndrome at our institution.
Methods: The medical records of patients diagnosed as Currarino triad or Currarino syndrome at the authors institution from 1997 to 2018 were retrospectively reviewed. Thirteen patients were included in this study.
Results: No significant difference in disease prevalence was observed in terms of sex (male:female, 7:6), and the median age at diagnosis was 7 months (1 day to 35 years). Currarino syndrome was diagnosed during the neonatal period in only one patient, whereas its diagnosis was delayed in the other patients. The most common initial symptom or diagnosis was anorectal malformation (ARM) followed by constipation and sacrococcygeal mass. All patients underwent simple abdominal X-ray and magnetic resonance imaging for the diagnosis of this disease. The most common sacral anomaly was partial sacral agenesis (type III, 62%) followed by hemisacrum (type IV, 38%). The most common presacral mass was mature teratoma. No pathologic malignant features were observed. In ARM, nine patients had anal stenosis, and posterior sagittal anorectoplasty was the most common operative method for ARM. Twelve patients were followed up beyond the age of three, and the median follow-up age was 8.1 years (range: 3.1?30.0 years). Among the 12 patients, 4 patients did not show symptoms of functional abnormality, whereas 8 patients showed constipation, fecal incontinence, urinary dysfunction, or gait disturbance.
Conclusion: The diagnosis of Currarino syndrome could be delayed when sacral bony anomaly and anorectal stenosis are not given attention. A careful follow up is essential because poor long-term functional outcomes are common.
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KEYWORD
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Currarino syndrome, Anorectal stenosis, Sacral agenesis
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