Neurosurgical management of Currarino syndrome: A case series and review of literature

Surg Neurol Int. 2019 Apr 24;10:70. doi: 10.25259/SNI-26-2019. eCollection 2019.

Abstract

Background: The Currarino syndrome (CS), defined by the triad of anorectal malformations, sacral bone deformities, and presacral masses, is rare. There are few surgical series that discuss conservative management versus the surgical approaches to these lesions. Here, we describe utilizing a combined anterior and posterior approach for resecting these lesions in four patients.

Methods: Four patients with CS were treated with two-stage approaches performed by a multidisciplinary team, including pediatric neurosurgery and general surgery. The first anterior laparoscopic approach mobilized the presacral mass from its ventral attachments. The second posterior procedure detethered the spinal cord, repaired the dural defect, and facilitated removal of the presacral mass.

Results: Gross total resection of all four presacral masses was accomplished without intraoperative complication; all patients clinically improved.

Conclusion: The CS is characterized by a large presacral mass. Here, one must rule out malignancy and also consider diagnosis/resection due to the risks for malignant transformation. The operative approach we described in four patients utilized standard anterior mobilization of the mass, followed by posterior detethering, dural repair, and ultimate resection.

Keywords: Currarino syndrome; presacral mass; tethered cord; ventral meningocele.