{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T16:18:10Z","timestamp":1793377090672,"version":"4.5.0"},"reference-count":24,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2018,11]]},"abstract":"<jats:sec><jats:title>OBJECTIVE<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>METHODS<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>RESULTS<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>CONCLUSIONS<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec>","DOI":"10.3171\/2018.5.peds17582","type":"journal-article","created":{"date-parts":[[2018,8,10]],"date-time":"2018-08-10T13:55:40Z","timestamp":1533909340000},"page":"584-590","source":"Crossref","is-referenced-by-count":25,"title":["Currarino syndrome: repair of the dysraphic anomalies and resection of the presacral mass in a combined neurosurgical and general surgical approach"],"prefix":"10.3171","volume":"22","author":[{"given":"Michael D.","family":"Cearns","sequence":"first","affiliation":[{"name":"Department of Paediatric Neurosurgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samantha","family":"Hettige","sequence":"additional","affiliation":[{"name":"Department of Paediatric Neurosurgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"De Coppi","sequence":"additional","affiliation":[{"name":"Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital, London, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominic N. P.","family":"Thompson","sequence":"additional","affiliation":[{"name":"Department of Paediatric Neurosurgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1","series-title":"Am J Hum Genet","doi-asserted-by":"publisher","first-page":"312","DOI":"10.1086\/302723","article-title":"Involvement of the HLXB9 homeobox gene in Currarino syndrome","volume":"66","author":"Belloni E","year":"2000","unstructured":"Belloni E, Martucciello G, Verderio D, Ponti E, Seri M, Jasonni V, : Involvement of the HLXB9 homeobox gene in Currarino syndrome. Am J Hum Genet 66:312\u2013319, 200010.1086\/30272310631160"},{"key":"b2","series-title":"Surg Neurol Int","doi-asserted-by":"publisher","first-page":"77","DOI":"10.4103\/sni.sni_439_16","article-title":"Multidisciplinary surgical treatment of presacral meningocele and teratoma in an adult with Currarino triad","volume":"8","author":"Chakhalian D","year":"2017","unstructured":"Chakhalian D, Gunasekaran A, Gandhi G, Bradley L, Mizell J, Kazemi N: Multidisciplinary surgical treatment of presacral meningocele and teratoma in an adult with Currarino triad. Surg Neurol Int 8:77, 201710.4103\/sni.sni_439_16"},{"key":"b3","series-title":"Wiley Interdiscip Rev Dev Biol","doi-asserted-by":"publisher","first-page":"213","DOI":"10.1002\/wdev.71","article-title":"Neural tube defects\u2014disorders of neurulation and related embryonic processes","volume":"2","author":"Copp AJ","year":"2013","unstructured":"Copp AJ, Greene ND: Neural tube defects\u2014disorders of neurulation and related embryonic processes. Wiley Interdiscip Rev Dev Biol 2:213\u2013227, 20132400903410.1002\/wdev.71"},{"key":"b4","series-title":"Hum Mutat","doi-asserted-by":"publisher","first-page":"903","DOI":"10.1002\/humu.20718","article-title":"Spectrum of HLXB9 gene mutations in Currarino syndrome and genotype-phenotype correlation","volume":"29","author":"Cr\u00e9tolle C","year":"2008","unstructured":"Cr\u00e9tolle C, Pelet A, Sanlaville D, Z\u00e9rah M, Amiel J, Jaubert F, : Spectrum of HLXB9 gene mutations in Currarino syndrome and genotype-phenotype correlation. Hum Mutat 29:903\u2013910, 20081844989810.1002\/humu.20718"},{"key":"b5","series-title":"AJR Am J Roentgenol","doi-asserted-by":"publisher","first-page":"395","DOI":"10.2214\/ajr.137.2.395","article-title":"Triad of anorectal, sacral, and presacral anomalies","volume":"137","author":"Currarino G","year":"1981","unstructured":"Currarino G, Coln D, Votteler T: Triad of anorectal, sacral, and presacral anomalies. AJR Am J Roentgenol 137:395\u2013398, 198110.2214\/ajr.137.2.3956789651"},{"key":"b6","series-title":"Pediatr Neurosurg","doi-asserted-by":"publisher","first-page":"223","DOI":"10.1159\/000028655","article-title":"A novel embryogenetic mechanism for Currarino\u2019s triad: inadequate dorsoventral separation of the caudal eminence from hindgut endoderm","volume":"28","author":"Dias MS","year":"1998","unstructured":"Dias MS, Azizkhan RG: A novel embryogenetic mechanism for Currarino\u2019s triad: inadequate dorsoventral separation of the caudal eminence from hindgut endoderm. Pediatr Neurosurg 28:223\u2013229, 1998973225310.1159\/000028655"},{"key":"b7","series-title":"Childs Nerv Syst","doi-asserted-by":"publisher","first-page":"547","DOI":"10.1007\/s00381-013-2274-6","article-title":"Currarino syndrome: report of five consecutive patients","volume":"30","author":"Duru S","year":"2014","unstructured":"Duru S, Karabagli H, Turkoglu E, Er\u015fahin Y: Currarino syndrome: report of five consecutive patients. Childs Nerv Syst 30:547\u2013552, 201410.1007\/s00381-013-2274-624013264"},{"key":"b8","series-title":"J Pediatr Surg","doi-asserted-by":"publisher","first-page":"1238","DOI":"10.1016\/j.jpedsurg.2005.05.004","article-title":"The Currarino triad: the variable expression","volume":"40","author":"Emans PJ","year":"2005","unstructured":"Emans PJ, Kootstra G, Marcelis CL, Beuls EA, van Heurn LW: The Currarino triad: the variable expression. J Pediatr Surg 40:1238\u20131242, 200510.1016\/j.jpedsurg.2005.05.00416080925"},{"key":"b9","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"924","DOI":"10.1227\/01.NEU.0000209945.87233.6A","article-title":"The Currarino triad: neurosurgical considerations","volume":"58","author":"Emans PJ","year":"2006","unstructured":"Emans PJ, van Aalst J, van Heurn EL, Marcelis C, Kootstra G, Beets-Tan RG, : The Currarino triad: neurosurgical considerations. Neurosurgery 58:924\u2013929, 20061663932810.1227\/01.NEU.0000209945.87233.6A"},{"key":"b10","series-title":"Surg Case Rep","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1186\/s40792-018-0419-2","article-title":"Surgical management for a huge presacral teratoma and a meningocele in an adult with Currarino triad: a case report","volume":"4","author":"Emoto S","year":"2018","unstructured":"Emoto S, Kaneko M, Murono K, Sasaki K, Otani K, Nishikawa T, : Surgical management for a huge presacral teratoma and a meningocele in an adult with Currarino triad: a case report. Surg Case Rep 4:9, 201810.1186\/s40792-018-0419-2"},{"key":"b11","series-title":"Childs Nerv Syst","doi-asserted-by":"publisher","first-page":"522","DOI":"10.1007\/PL00007296","article-title":"Diagnostic steps and staged operative approach in Currarino\u2019s triad: a case report and review of the literature","volume":"16","author":"Ilhan H","year":"2000","unstructured":"Ilhan H, Tokar B, Atasoy MA, Kulali A: Diagnostic steps and staged operative approach in Currarino\u2019s triad: a case report and review of the literature. Childs Nerv Syst 16:522\u2013524, 200010.1007\/PL0000729611007505"},{"key":"b12","series-title":"Pediatr Neurosurg","doi-asserted-by":"publisher","first-page":"110","DOI":"10.1159\/000319007","article-title":"Currarino triad: surgical management and follow-up results of four [correction of three] cases","volume":"46","author":"Isik N","year":"2010","unstructured":"Isik N, Elmaci I, Gokben B, Balak N, Tosyali N: Currarino triad: surgical management and follow-up results of four [correction of three] cases. Pediatr Neurosurg 46:110\u2013119, 201010.1159\/00031900720664237"},{"key":"b13","series-title":"J Neurosurg Pediatr","doi-asserted-by":"publisher","first-page":"685","DOI":"10.3171\/2014.3.PEDS13534","article-title":"Currarino syndrome and spinal dysraphism","volume":"13","author":"Kole MJ","year":"2014","unstructured":"Kole MJ, Fridley JS, Jea A, Bollo RJ: Currarino syndrome and spinal dysraphism. J Neurosurg Pediatr 13:685\u2013689, 20142474534210.3171\/2014.3.PEDS13534"},{"issue":"1 Suppl","key":"b14","series-title":"J Neurosurg","first-page":"158","article-title":"Complete familial Currarino triad. Report of three cases in one family","volume":"94","author":"Kurosaki M","year":"2001","unstructured":"Kurosaki M, Kamitani H, Anno Y, Watanabe T, Hori T, Yamasaki T: Complete familial Currarino triad. Report of three cases in one family. J Neurosurg 94 (1 Suppl):158\u2013161, 200111147855"},{"key":"b15","series-title":"J Pediatr Surg","doi-asserted-by":"publisher","first-page":"58","DOI":"10.1016\/S0022-3468(97)90094-4","article-title":"Currarino triad: anorectal malformation, sacral bony abnormality, and presacral mass\u2014a review of 11 cases","volume":"32","author":"Lee SC","year":"1997","unstructured":"Lee SC, Chun YS, Jung SE, Park KW, Kim WK: Currarino triad: anorectal malformation, sacral bony abnormality, and presacral mass\u2014a review of 11 cases. J Pediatr Surg 32:58\u201361, 1997902157010.1016\/S0022-3468(97)90094-4"},{"key":"b16","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"E597","DOI":"10.1227\/01.NEU.0000171836.67266.75","article-title":"Presacral meningocele associated with hereditary sacral agenesis and treated surgically: evaluation in three members of the same family","volume":"57","author":"Marin-Sanabria EA","year":"2005","unstructured":"Marin-Sanabria EA, Nagashi T, Yamamoto K, Nakamura Y, Aihara H, Kohmura E: Presacral meningocele associated with hereditary sacral agenesis and treated surgically: evaluation in three members of the same family. Neurosurgery 57:E597, 200510.1227\/01.NEU.0000171836.67266.7516145509"},{"key":"b17","series-title":"J Pediatr Surg","doi-asserted-by":"publisher","first-page":"1305","DOI":"10.1016\/j.jpedsurg.2004.05.003","article-title":"Currarino syndrome: proposal of a diagnostic and therapeutic protocol","volume":"39","author":"Martucciello G","year":"2004","unstructured":"Martucciello G, Torre M, Belloni E, Lerone M, Pini Prato A, Cama A, : Currarino syndrome: proposal of a diagnostic and therapeutic protocol. J Pediatr Surg 39:1305\u20131311, 200410.1016\/j.jpedsurg.2004.05.00315359381"},{"key":"b18","series-title":"Childs Nerv Syst","doi-asserted-by":"publisher","first-page":"722","DOI":"10.1007\/s00381-003-0814-1","article-title":"Giant anterior sacral meningocele and posterior sagittal approach","volume":"19","author":"Massimi L","year":"2003","unstructured":"Massimi L, Calisti A, Koutzoglou M, Di Rocco C: Giant anterior sacral meningocele and posterior sagittal approach. Childs Nerv Syst 19:722\u2013728, 200310.1007\/s00381-003-0814-114576957"},{"key":"b19","series-title":"Eur J Med Genet","doi-asserted-by":"publisher","first-page":"648","DOI":"10.1016\/j.ejmg.2013.09.011","article-title":"Novel MNX1 mutations and clinical analysis of familial and sporadic Currarino cases","volume":"56","author":"Merello E","year":"2013","unstructured":"Merello E, De Marco P, Ravegnani M, Riccipetitoni G, Cama A, Capra V: Novel MNX1 mutations and clinical analysis of familial and sporadic Currarino cases. Eur J Med Genet 56:648\u2013654, 20132409582010.1016\/j.ejmg.2013.09.011"},{"key":"b20","series-title":"J Pediatr Surg","doi-asserted-by":"publisher","first-page":"1112","DOI":"10.1053\/jpsu.2000.7838","article-title":"Posterior sagittal approach for Currarino syndrome with anterior sacral meningocele: a case report","volume":"35","author":"Otagiri N","year":"2000","unstructured":"Otagiri N, Matsumoto Y, Yoshida Y: Posterior sagittal approach for Currarino syndrome with anterior sacral meningocele: a case report. J Pediatr Surg 35:1112\u20131114, 20001091730810.1053\/jpsu.2000.7838"},{"key":"b21","series-title":"BMJ Case Rep","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2014-204276","article-title":"Currarino\u2019s syndrome in twins presenting as neonatal intestinal obstruction\u2014identical presentation in non-identical twins","volume":"2014","author":"Patel RV","year":"2014","unstructured":"Patel RV, De Coppi P, Kiely E, Pierro A: Currarino\u2019s syndrome in twins presenting as neonatal intestinal obstruction\u2014identical presentation in non-identical twins. BMJ Case Rep 2014, 20142519918710.1136\/bcr-2014-204276"},{"key":"b22","series-title":"J Pediatr Surg","doi-asserted-by":"publisher","first-page":"1643","DOI":"10.1016\/j.jpedsurg.2014.05.038","article-title":"Urological outcome in patients with Currarino syndrome","volume":"49","author":"Pio L","year":"2014","unstructured":"Pio L, Piatelli G, Rossi A, Scarsi P, Merello E, Capra V, : Urological outcome in patients with Currarino syndrome. J Pediatr Surg 49:1643\u20131646, 20142547581010.1016\/j.jpedsurg.2014.05.038"},{"key":"b23","series-title":"J Pediatr Surg","doi-asserted-by":"publisher","first-page":"1790","DOI":"10.1053\/jpsu.2000.19258","article-title":"Currarino triad\u2014diagnostic dilemma and a combined surgical approach","volume":"35","author":"Samuel M","year":"2000","unstructured":"Samuel M, Hosie G, Holmes K: Currarino triad\u2014diagnostic dilemma and a combined surgical approach. J Pediatr Surg 35:1790\u20131794, 200010.1053\/jpsu.2000.19258"},{"key":"b24","series-title":"Pediatr Surg Int","doi-asserted-by":"publisher","first-page":"677","DOI":"10.1007\/s00383-010-2615-4","article-title":"Long-term functional outcomes in children with Currarino syndrome","volume":"26","author":"Yoshida A","year":"2010","unstructured":"Yoshida A, Maoate K, Blakelock R, Robertson S, Beasley S: Long-term functional outcomes in children with Currarino syndrome. Pediatr Surg Int 26:677\u2013681, 201010.1007\/s00383-010-2615-420473613"}],"container-title":["Journal of Neurosurgery: Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/22\/5\/article-p584.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/22\/5\/article-p584.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/22\/5\/article-p584.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,15]],"date-time":"2023-04-15T13:42:32Z","timestamp":1681566152000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/22\/5\/article-p584.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,11]]},"references-count":24,"journal-issue":{"issue":"5"},"URL":"https:\/\/doi.org\/10.3171\/2018.5.peds17582","relation":{},"ISSN":["1933-0707","1933-0715"],"issn-type":[{"value":"1933-0707","type":"print"},{"value":"1933-0715","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,11]]}}}