What is the management plan for Currarino triad?

Management of Currarino Triad

Currarino triad requires a multidisciplinary surgical approach with specific interventions for each component of the triad (anorectal malformation, sacral bony defect, and presacral mass) to optimize morbidity, mortality, and quality of life outcomes. 1

Diagnostic Evaluation

• MRI is essential for characterizing the presacral mass and determining the exact nature and extent of the lesion before surgery 2
• Evaluate for:
  • Tethered cord syndrome (present in many cases) 3
  • Hydrocephalus (may be present in some cases) 3
  • Specific type of presacral mass (meningocele, teratoma, or dermoid/epidermoid cyst) 1
  • Sacral bony defect (typically "scimitar sacrum" or sacral agenesis) 1
  • Anorectal malformation (stenosis, agenesis, or fistula) 1

Surgical Management Algorithm

1. Anorectal Malformation Management

• For anal stenosis: Initial management with anal dilatation 3
• For severe anorectal malformations: Temporary colostomy may be required 3
• For Hirschsprung's disease (when present): Rectal biopsy and appropriate surgical correction 3

2. Presacral Mass Management

• For anterior sacral meningocele:

  • Posterior approach via lumbar and sacral partial laminectomy-laminoplasty
  • Transdural ligation of the neck of the meningocele 3

• For solid tumors (teratoma, dermoid/epidermoid cyst):

  • Complete surgical excision via appropriate approach 3
  • Consider supine lithotomy position for better access to deeply located presacral masses 2

3. Tethered Cord Management (when present)

• Surgical untethering during the same procedure as presacral mass removal 3

4. Hydrocephalus Management (when present)

• Appropriate neurosurgical intervention (shunting or endoscopic third ventriculostomy) 3

Position Considerations for Surgery

• For males: Prone position is standard for posterior sagittal anorectoplasty (PSARP) 2
• For females: Consider supine position as an alternative (anterior sagittal anorectoplasty) 2
• Important caveat: Supine lithotomy position provides better access to explore the presacral space, especially with deeply located cysts 2

Follow-up and Monitoring

• Regular neurological assessment to detect any new deficits 3
• Imaging to ensure no recurrence of presacral mass 3
• Bowel function monitoring and management 3
• Long-term follow-up is essential (average follow-up in successful cases: 6 years) 3

Special Considerations

• Dual pathology: In rare cases, the presacral mass may contain both meningocele and teratoma, requiring careful surgical planning 4
• Infection risk: Spinal abscess can occur as a complication, requiring reoperation and medical therapy 3
• Genetic counseling: Although most cases are sporadic, familial transmission has been reported 3

Outcomes

With appropriate surgical management, patients typically show improvement with:

• No additional neurological deficits
• No recurrence of presacral mass
• Improved bowel function
• Stabilization of symptoms 3

Pitfalls to Avoid

• Overlooking the characteristic notched sacrum (sacral scimitar) on plain X-ray, especially with small sacral defects 2
• Inadequate exposure of deeply located presacral masses due to inappropriate positioning 2
• Incomplete excision of presacral masses with dual pathology 4
• Failure to address all components of the triad 1