Diagnostic steps and staged operative approach in Currarino’s triad: a case report and review of the literature

Ilhan, Huseyin; Tokar, Baran; Atasoy, Metin Ant; Kulali, Alparslan

doi:10.1007/pl00007296

Cited by 32 publications

(36 citation statements)

References 13 publications

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“…These sites share a common embryological origin in the tail bud. So far, more than 300 CS cases have been reported in the literature, and additional associated malformations have been described, namely: neural tube defects (tethered cord, intra-and/or extraspinal lipoma, syringomyelia, and hydromyelia), renal or ureteral duplications, hydronephrosis, horseshoe kidney, vesicoureteral reflux, neurogenic bladder, and bicornuate uterus [De Lagausie et al, 1991;Kochling et al, 1996;Lee et al, 1997;Gegg et al, 1999;Belloni et al, 2000;Ilhan et al, 2000;Gereige and Frias, 2002;MarinSanabria et al, 2005]. Presacral mass may result in severe complications such as presacral abscess or meningitis due to communication between the presacral mass and the spinal canal.…”

Section: Introductionmentioning

confidence: 99%

Spectrum ofHLXB9gene mutations in Currarino syndrome and genotype-phenotype correlation

et al. 2008

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Communicated by Sergio OttolenghiCurrarino syndrome (CS) is a rare congenital malformation described in 1981 as the association of three main features: typical sacral malformation (sickle-shaped sacrum or total sacral agenesis below S2), hindgut anomaly, and presacral tumor. In addition to the triad, tethered cord and/or lipoma of the conus are also frequent and must be sought, as they may lead to severe complications if not treated. The HLXB9 gene, located at 7q36, is disease-causing. It encodes the HB9 transcription factor and interacts with DNA through a highly evolutionarily conserved homeodomain early in embryological development.

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Section: Introductionmentioning

confidence: 99%

Spectrum ofHLXB9gene mutations in Currarino syndrome and genotype-phenotype correlation

et al. 2008

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“…Resection of a presacral mass may not relieve patients from constipation because constipation may also be caused by a narrow anal canal or spinal cord tethering [1,2]. It is recognized that spinal cord tethering leads to bladder and bowel dysfunction [2,5,15]. The incidence of the association between the Currarino triad and tethered cord syndrome is low in the series reported by Lee et al [4] (18%).…”

Section: Discussionmentioning

confidence: 84%

“…Approximately 300 similar cases have been reported to date in the literature[1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25]. The majority of these lesions have been reported as sporadic, isolated cases.…”

Section: Discussionmentioning

confidence: 90%

“…This is referred to as ‘split notochord syndrome’ and partial resorption of this connection would result in the formation of an ASM or a retrorectal enteric cyst. The combination of mesodermal tissue with these enteric and neuroectodermal elements leads to the formation of a presacral teratoma [1,2,3,5,6,9,27]. …”

Section: Discussionmentioning

confidence: 99%

“…The Currarino triad is a rare complex of congenital caudal anomalies including three main features: a sacral bony deformity, anorectal malformations (ARMs), and a presacral mass [1,2,3,4,5,6,7]. It is seen more commonly in children; females are typically affected more than males [8].…”

Section: Introductionmentioning

confidence: 99%

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Currarino Triad: Surgical Management and Follow-Up Results of Three Cases

Isik¹,

Elmacı²,

Gökben³

et al. 2010

Pediatr Neurosurg

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The Currarino syndrome is a rare triad that is a combination of a presacral mass, a congenital sacral bony abnormality and an anorectal malformation. We present 4 children with complete Currarino triad who were diagnosed using MRI. Our aim was to report the neurosurgical management of Currarino syndrome in children. All of the patients had chronic constipation and pain in the lumbosacral region. In the plain radiograph, 3 patients had a sacral scimitar-shaped bony abnormality, and 1 patient had total sacral agenesis. There was a narrow anal canal or narrow ventrally displaced anus in all patients. Their anorectal malformations were characterized as anal stenoses (4 patients), associated with Hirschsprung’s disease in 2 cases. In 3 patients, MRI showed tethered cord syndrome in addition to the presacral mass. There was hydrocephalus in 1 patient. Anal stenosis was treated by anal dilatation. In 2 patients, rectal biopsy and temporary colostomy (2 patients) had been performed previously due to Hirschsprung’s disease. We performed a posterior procedure via lumbar and sacral partial laminectomy-laminoplasty and transdural ligation of the neck of the meningocele for anterior sacral meningoceles, or alternatively, tumor excision for other types of presacral lesions. Histopathologically, 3 were cases of anterior sacral meningoceles and 1 was a teratoma. One of them also had a spinal abscess. He required reoperation (twice) and appeared at the time to have improved with medical therapy. All patients improved and stabilized. There were no additional neurological deficits and no recurrence of the presacral mass over the follow-up period (6 years, on average). The family pedigree did not reveal any familial transmission pattern. In cases of Currarino triad, MRI can allow the characterization of the presacral masses. If it is an anterior sacral meningocele or a solid tumor without severe anorectal malformation, it can be managed with posterior lumbar and sacral procedures. Such approaches are performed easily by transdural ligation of the neck of the anterior sacral meningocele or through tumor excision.

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Malignant degeneration of presacral teratoma in the Currarino anomaly

Urioste

Garcia-Andrade

Valle

et al. 2004

American J of Med Genetics Pt A

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The autosomal dominant Currarino anomaly (CA) comprises a presacral mass, partial sacral agenesis, and anorectal defects. Chronic constipation in childhood related to anorectal defects is the most common presenting symptom and hemisacrum the most frequent malformation. The presacral mass may be an anterior meningomyelocele, teratoma, hamartoma, dermoid cyst, neuroenteric cyst, or a combination of these. Sepsis and meningitis are frequent serious problems related to the anterior meningomyelocele, whilst malignant transformation of presacral teratoma is a rare, severe complication in CA. Here, we report on a three-generation family segregating the CA, presenting with anorectal defects, severe constipation, and sacral involvement in affected relatives. Teratoma was the most frequent component of the presacral mass. In this kindred a 22-year-old man died of a neuroendocrine tumor, probably related to malignant change in a presacral teratoma. A novel mutation in HLXB9 consisting of a 24-bp deletion and insertion of 2-bp into exon 1, was identified in all patients and in also three asymptomatic members of this family. Anterior meningomyelocele is the most frequently reported component of the presacral masses in CA; however, presacral teratomas carry an inherent risk for malignancy that must be considered in the counseling, surgical treatment options, and follow-up of CA patients.

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Diagnostic steps and staged operative approach in Currarino’s triad: a case report and review of the literature

Cited by 32 publications

References 13 publications

Spectrum ofHLXB9gene mutations in Currarino syndrome and genotype-phenotype correlation

Spectrum ofHLXB9gene mutations in Currarino syndrome and genotype-phenotype correlation

Currarino Triad: Surgical Management and Follow-Up Results of Three Cases

Malignant degeneration of presacral teratoma in the Currarino anomaly

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