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[Currarino triad: different forms of presentation]
E Calleja Aguayo1, B Estors Sastre, P Bragagnini Rodríguez
1Servicio de Cirugía Pediátrica, Hospital Miguel Servet, Zaragoza. dracalleja@gmail.com
Summary
Currarino syndrome (CS) involves anorectal malformations and sacral agenesis. Management requires a multidisciplinary approach, with constipation being the most common symptom and rectomeningeal fistula posing a high mortality risk.
Area of Science:
- Pediatric Surgery
- Medical Genetics
- Clinical Urology
Background:
- Currarino syndrome (CS) is a rare congenital disorder characterized by a specific combination of anomalies.
- This study details clinical experiences and findings in three pediatric cases of Currarino syndrome.
Observation:
- Case 1: Newborn with myelomeningocele, partial sacral agenesis, horseshoe kidney, and anorectal malformation.
- Case 2: 14-month-old male with constipation, anal stricture, presacral lipomeningocele, teratoma, and partial sacral agenesis.
- Case 3: 8-month-old male with recurrent meningitis, anterior sacral meningocele, rectal fistula, anal stricture, and hemisacrum.
Findings:
- Surgical interventions varied, including pull-through procedures, tumor removal, anal dilation, and colostomy.
- Complications included surgical failure due to vascular anomalies, recto-cutaneous fistula, and hydrocephalus requiring valve implantation.
- Constipation is the most frequent symptom; rectomeningeal fistula presents a significant mortality risk (56%).
Implications:
- Anal stricture is the most common anorectal malformation in CS, with complexity correlating to incontinence risk.
- Successful management and long-term follow-up of Currarino syndrome necessitate a coordinated multidisciplinary team approach.
- Early diagnosis and tailored interventions are crucial for improving outcomes in patients with Currarino syndrome.
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