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PubMed This is a summary of 15 peer-reviewed journal articles Updated
Pediatric Surgery

Surgical Strategy & Building Your Care Team

At a Glance

Treatment for Currarino syndrome requires a multidisciplinary team including colorectal surgeons, neurosurgeons, urologists, and geneticists. Surgery aims to remove presacral masses, repair anorectal malformations, and release tethered spinal cords, followed by long-term MRI and bowel monitoring.

Because Currarino syndrome involves multiple organs and the central nervous system, treatment is never the responsibility of a single doctor. Instead, care is built around a multidisciplinary team—a group of specialists who coordinate their expertise to address the “triad” of findings [1][2].

Building Your Specialized Care Team

A highly experienced care team for Currarino syndrome typically includes the following specialists [2][3]:

  • Pediatric or Colorectal Surgeon: Manages the anorectal malformation (ARM) and bowel function [4].
  • Neurosurgeon: Focuses on the presacral mass (especially if it is an anterior sacral meningocele) and ensures the spinal cord is not “tethered” or damaged [2][5].
  • Urologist: Monitors bladder health, as the nerves that control the bladder can be affected by sacral defects or surgery [4][6].
  • Medical Geneticist: Helps the family understand the MNX1 gene mutation and provides screening for relatives [7].

Surgical Strategy and Sequencing

The goal of surgery is to remove any masses, repair structural issues, and prevent serious infections like meningitis [5][8]. There is no single “correct” order for these surgeries; the plan is tailored to the individual’s anatomy [9][10].

Managing the Presacral Mass and Spine

If a presacral mass (like a meningocele or teratoma) is present, a neurosurgeon is essential to ensure it is safely separated from the spinal canal [2][5].

  • Intraoperative Ultrasound: During surgery, doctors often use intraoperative ultrasound to get real-time images. This helps them identify vital nerves and may reduce the need for more invasive exploration of the spinal canal [11][12].
  • Tethered Cord Repair: If the spinal cord is “tethered” (stuck), surgeons will carefully release it to prevent future neurological issues [11][2].

Correcting Anorectal Malformations

The surgical approach for the anorectal malformation (the narrowing or blockage of the anus) often follows standard protocols for these defects. A common method is the posterior sagittal approach, which allows surgeons a clear view of the rectum and the area in front of the sacrum [13][9].

Stomas and Multi-Stage Procedures: In some cases, patients may require a temporary colostomy (a stoma) to divert stool while the area heals before the definitive posterior sagittal repair. This multi-stage approach ensures the delicate surgical site is protected from infection. In other anatomies, a “single-stage” surgery is possible, where both the neurosurgical and colorectal repairs are done during the same operation [13][10].

Long-Term Monitoring

Surgery is often successful in correcting structural issues, but long-term follow-up is vital.

  • Bowel Management: Even after successful surgery, many patients require a long-term plan for managing constipation or incontinence [4][14].
  • Surveillance: Because presacral masses can sometimes recur or undergo changes, regular MRI scans are scheduled for several years following surgery [15][2].
  • Infection Risk: Until any connections (fistulas) between the gut and the spine are fully repaired, there remains a risk of meningitis [8][5].

Once the mass is removed, it must be analyzed. Continue to the next section to learn about Pathology & Risk.

Common questions in this guide

Which specialists should be on a Currarino syndrome care team?
A comprehensive care team typically includes a pediatric or colorectal surgeon, a neurosurgeon, a urologist, and a medical geneticist. This multidisciplinary approach ensures all aspects of the condition, from bowel function to spinal health, are properly managed.
What is the overall surgical strategy for Currarino syndrome?
The primary goals are to remove presacral masses, correct anorectal malformations, and ensure the spinal cord is not tethered. Because every patient's anatomy is different, the exact timing and order of these procedures are highly individualized.
Is surgery for Currarino syndrome done in one step or multiple stages?
Depending on the specific anatomy, some patients undergo a single-stage operation where both neurosurgical and colorectal repairs are completed at once. Others require a multi-stage approach, which might involve a temporary colostomy to allow delicate areas to heal safely.
What follow-up care is needed after Currarino syndrome surgery?
Patients generally need regular MRI scans for several years to ensure presacral masses do not recur. Additionally, ongoing bowel management is often required to help with constipation or incontinence, alongside urological monitoring for bladder function.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.How many patients with Currarino syndrome has this surgical team treated in the last five years?
  2. 2.Do you recommend a 'single-stage' approach where the neurosurgeon and colorectal surgeon work together, or separate surgeries?
  3. 3.How will you use intraoperative ultrasound to protect the nerves during the procedure?
  4. 4.What is the plan for monitoring my/my child's bladder function after surgery?
  5. 5.Does the surgical plan address both the physical mass and any possible connections (fistulas) to the spine?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (15)
  1. 1

    Clinical and radiological evaluation of caudal regression syndrome.

    Krishnan V, Jaganathan S, Jayappa S, et al.

    Pediatric radiology 2024; (54(9)):1451-1461 doi:10.1007/s00247-024-05945-1.

    PMID: 38750326
  2. 2

    Atypical presentation of currarino syndrome: A case report.

    Hage P, Kseib C, Adem C, et al.

    International journal of surgery case reports 2019; (57()):102-105 doi:10.1016/j.ijscr.2019.02.047.

    PMID: 30933899
  3. 3

    Currarino Syndrome Presenting in Adulthood: A Rare Case.

    Verma A, Attry S, Haldenia K, et al.

    Cureus 2023; (15(3)):e36497 doi:10.7759/cureus.36497.

    PMID: 37090396
  4. 4

    The Currarino triad: What pediatric surgeons need to know.

    AbouZeid AA, Mohammad SA, Abolfotoh M, et al.

    Journal of pediatric surgery 2017; (52(8)):1260-1268 doi:10.1016/j.jpedsurg.2016.12.010.

    PMID: 28065719
  5. 5

    Surgical management of Currarino syndrome in elderly patient with infected pre-sacral mass: Technical nuances and review of literature.

    Saway BF, Rafka HE, Gunasekaran A, et al.

    Clinical neurology and neurosurgery 2022; (222()):107470 doi:10.1016/j.clineuro.2022.107470.

    PMID: 36265244
  6. 6

    Think of the Conus Medullaris at the Time of Diagnosis of Fetal Sacral Agenesis.

    Mottet N, Martinovic J, Baeza C, et al.

    Fetal diagnosis and therapy 2017; (42(2)):137-143 doi:10.1159/000451080.

    PMID: 27794580
  7. 7

    Sacrum agenesis and scimitar sacrum in Currarino syndrome.

    Corrêa DG, Daltro PA

    Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 2020; (36(7)):1337-1338 doi:10.1007/s00381-020-04653-z.

    PMID: 32447453
  8. 8

    Naked sacrococcygeal teratoma associated with dorsal meningocoele and sacrococcygeal inversion: an atypical presentation of Currarino syndrome.

    Datta D, Wilson N, McAuley D

    Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 2025; (41(1)):276 doi:10.1007/s00381-025-06911-4.

    PMID: 40936035
  9. 9

    Selection of operative approach in children with Currarino syndrome.

    Pang W, Chen Y, Wang K, et al.

    Pediatric surgery international 2023; (39(1)):72 doi:10.1007/s00383-022-05345-2.

    PMID: 36607405
  10. 10

    Multidisciplinary surgical treatment of presacral meningocele and teratoma in an adult with Currarino triad.

    Chakhalian D, Gunasekaran A, Gandhi G, et al.

    Surgical neurology international 2017; (8()):77 doi:10.4103/sni.sni_439_16.

    PMID: 28584680
  11. 11

    Presacral mature cystic teratoma associated with Currarino syndrome in an adolescent with androgen insensitivity: illustrative case.

    Koskay G, Opperman P, Mezzacappa FM, et al.

    Journal of neurosurgery. Case lessons 2022; (4(18)).

    PMID: 36317236
  12. 12

    Intraoperative Ultrasound and Contrast-Enhanced Ultrasound in Surgical Treatment of Intramedullary Spinal Tumors.

    Han B, Wu D, Jia W, et al.

    World neurosurgery 2020; (137()):e570-e576 doi:10.1016/j.wneu.2020.02.059.

    PMID: 32081827
  13. 13

    Complete Currarino Triad Presenting With Chronic Constipation.

    Aldabbab HY, Al Ghadeer HA, Alnosair AA, et al.

    Cureus 2022; (14(4)):e23743 doi:10.7759/cureus.23743.

    PMID: 35509736
  14. 14

    Differentiating presacral masses in anorectal malformations and isolated sacrococcygeal teratomas.

    Dewberry L, Peña A, Meyers ML, et al.

    Pediatric surgery international 2019; (35(9)):979-983 doi:10.1007/s00383-019-04510-4.

    PMID: 31256295
  15. 15

    Managing Recurrent Teratoma in Currarino Syndrome.

    Chung YC, Centauri S, Nguyen TC

    Cureus 2023; (15(11)):e48780 doi:10.7759/cureus.48780.

    PMID: 38098935

This page provides educational information about surgical approaches for Currarino syndrome. It does not replace professional medical advice. Always consult your multidisciplinary care team to determine the safest surgical plan for your specific anatomy.

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