eprintid: 10054851 rev_number: 28 eprint_status: archive userid: 608 dir: disk0/10/05/48/51 datestamp: 2018-08-29 11:03:45 lastmod: 2021-12-05 01:02:37 status_changed: 2018-08-29 11:03:45 type: article metadata_visibility: show creators_name: Cearns, MD creators_name: Hettige, S creators_name: De Coppi, P creators_name: Thompson, DNP title: Currarino syndrome: repair of the dysraphic anomalies and resection of the presacral mass in a combined neurosurgical and general surgical approach ispublished: pub subjects: GOSH divisions: UCL divisions: B02 divisions: D13 divisions: G22 divisions: G26 keywords: Currarino syndrome, anorectal malformation, spinal dysraphism, anterior meningocele, teratoma, tethered spinal cord, spine note: This version is the version of record. For information on re-use, please refer to the publisher’s terms and conditions. abstract: OBJECTIVE: It is well established that Currarino syndrome (CS) may be associated with spinal dysraphism. Here, the authors report on 10 CS patients with dysraphic anomalies who had undergone a combined neurosurgical and general surgical approach to repair the dysraphic anomalies and resect the presacral mass in a single operation. They discuss the spectrum of spinal dysraphism that may coexist in CS in the context of its developmental etiology. METHODS: Children with a confirmed CS diagnosis who had undergone the combined operative approach were identified from a departmental database. Presenting features were recorded and preoperative imaging was analyzed to record features of the presacral mass and the dysraphic anomalies. The histopathological nature of the resected presacral mass and the outcomes postoperatively and at the last follow-up were reviewed. RESULTS: Between 2008 and 2015, 10 patients presented with CS, 9 with constipation. Median age at the time of surgery was 1.3 years. Six of the 10 patients had anorectal malformation consisting of anal stenosis, rectal stenosis, or imperforate anus. Spinal anomalies included anterior meningocele (5 cases), low-lying conus (8), terminal syrinx (4), fatty filum (5), caudal lipoma (3), and intraspinal cyst (1). In all cases, the lumbosacral spinal canal was accessed via a midline approach with laminoplasty, allowing spinal cord untethering and repair of the dysraphic anomalies. Following dural closure, the incision was extended inferiorly to incorporate a posterior sagittal approach to resect the presacral mass. The histopathological nature of the mass was mature teratoma (8 cases), complex hamartomatous malformation (1), or neurenteric cyst (1). There were no new instances of neurological deterioration, with most instances of persisting morbidity related to constipation (6 cases) or neurogenic bladder dysfunction (8). There were no infective complications, no instances of cerebrospinal fluid fistula, no recurrences of the presacral mass, and no cases of retethering of the spinal cord. CONCLUSIONS: Although not part of the original triad, spinal dysraphic anomalies are common in CS and in keeping with a disorder of secondary neurulation. Lumbosacral MRI is an essential investigation when CS is suspected. Children are at risk of sphincter impairment due to the anorectal malformation; however, both spinal cord tethering and compression from the presacral mass may further compromise long-term continence. A combined operative approach to repair the dysraphic anomalies and resect the presacral mass is described with good postoperative and long-term outcomes. date: 2018-11 date_type: published official_url: http://dx.doi.org/10.3171/2018.5.PEDS17582 oa_status: green full_text_type: pub language: eng primo: open primo_central: open_green verified: verified_manual elements_id: 1577831 doi: 10.3171/2018.5.PEDS17582 lyricists_name: De Coppi, Paolo lyricists_name: Thompson, Dominic lyricists_id: PDECO50 lyricists_id: DTHOM60 actors_name: Thompson, Dominic actors_id: DTHOM60 actors_role: owner full_text_status: public publication: Journal of Neurosurgery: Pediatrics volume: 22 number: 5 pagerange: 467-599 event_location: United States issn: 1933-0707 citation: Cearns, MD; Hettige, S; De Coppi, P; Thompson, DNP; (2018) Currarino syndrome: repair of the dysraphic anomalies and resection of the presacral mass in a combined neurosurgical and general surgical approach. Journal of Neurosurgery: Pediatrics , 22 (5) pp. 467-599. 10.3171/2018.5.PEDS17582 <https://doi.org/10.3171/2018.5.PEDS17582>. Green open access document_url: https://discovery.ucl.ac.uk/id/eprint/10054851/1/Thompson_Currarino%20syndrome.%20Repair%20of%20the%20dysraphic%20anomalies%20and%20resection%20of%20the%20presacral%20mass%20in%20a%20combined%20neurosurgical%20and%20general%20surgical%20approach_VoR.pdf