The Currarino Triad: Neurosurgical Considerations

Emans, Pieter J.; Aalst, Jasper van; Heurn, L.W.E. van; Marcelis, Carlo; Kootstra, G.; Beets-Tan, R. G. H.; Vles, Johan S.H.; Beuls, E. A. M.

doi:10.1227/01.neu.0000209945.87233.6a

Cited by 46 publications

(49 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The mass can be a teratoma, an anterior meningocele, an anterior myelomeningocele or a combination of both. These patients usually have constipation with the cause hypothetically related to either anorectal malfomations or to the size of the presacral mass [6].…”

Section: Discussionmentioning

confidence: 99%

“…The caudal regression syndrome ranges from a congenital absence of sacral, lumbar and lower thoracic vertebrae to absence of the coccyx, with the majority of abnormalities involving only the sacrum [2]. SA is associated with multiple organ system abnormalities including the genitourinary tract, the hindgut and the respiratory system [2][3][4][5][6]. Bony defects of the sacrum can have a rare association with terminal myelomeningocele, a rare form of occult spinal dysraphism that may present as a lumbosacral mass [7].…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

A tail of sacral agenesis: delayed presentation of meningocele in sacral agenesis

2012

View full text Add to dashboard Cite

Introduction Sacral agenesis is a congenital condition associated with multiple orthopedic, spinal, abdominal and thoracic organ deformities. Meningocele is commonly found among patients with sacral agenesis. Description We present the first case in the literature describing a delayed presentation of terminal (posterior) meningocele in an adult patient born with sacral agenesis. Conclusion Surgical repair was performed and is the best treatment option for significantly large lesions, with postoperative CSF leak being the main complication.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

A tail of sacral agenesis: delayed presentation of meningocele in sacral agenesis

2012

View full text Add to dashboard Cite

show abstract

“…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: 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%

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

Isik¹,

Elmacı²,

Gökben³

et al. 2010

Pediatr Neurosurg

View full text Add to dashboard Cite

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.

show abstract

“…2,15 The Currarino triad (anorectal malformation, presacral mass, and sacral bone abnormalities) is also a syndrome that is associated with tethered cord. 7 Interestingly, genetic studies suggest that TCS may be genetically transmissible. 3,16,26 More recently a link between the TBX1 gene, 22q11.2 deletion, and trisomy 21 with TCS has been described.…”

Section: Primary and Secondary Causesmentioning

confidence: 99%

Spinal cord traction, vascular compromise, hypoxia, and metabolic derangements in the pathophysiology of tethered cord syndrome

Filippidis

Kalani²,

Theodore³

et al. 2010

FOC

View full text Add to dashboard Cite

Object The definition of tethered cord syndrome (TCS) relies mainly on radiological criteria and clinical picture. The presence of a thickened filum terminale and a low-lying conus medullaris in symptomatic patients is indicative of TCS. The radiological definition of TCS does not take into account cases that involve a normal-lying conus medullaris exhibiting symptoms of the disease. Methods The authors performed a MEDLINE search using the terms “tethered cord” and “pathophysiology.” The search returned a total of 134 studies. The studies were further filtered to identify mostly basic research studies in animal models or studies related to the biomechanics of the filum terminale and spinal cord. Results Spinal cord traction and the loss of filum terminale elasticity are the triggers that start a cascade of events occurring at the metabolic and vascular levels leading to symptoms of the disease. Traction on the caudal cord results in decreased blood flow causing metabolic derangements that culminate in motor, sensory, and urinary neurological deficits. The untethering operation restores blood flow and reverses the clinical picture in most symptomatic cases. Conclusions Although classically defined as a disease of a low-lying conus medullaris, the pathophysiology of TCS is much more complex and is dependent on a structural abnormality, with concomitant altered metabolic and vascular sequelae. Given the complex mechanisms underlying TCS, it is not surprising that the radiological criteria do not adequately address all presentations of the disease.

show abstract

The Currarino Triad: Neurosurgical Considerations

Cited by 46 publications

References 15 publications

A tail of sacral agenesis: delayed presentation of meningocele in sacral agenesis

A tail of sacral agenesis: delayed presentation of meningocele in sacral agenesis

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

Spinal cord traction, vascular compromise, hypoxia, and metabolic derangements in the pathophysiology of tethered cord syndrome

Contact Info

Product

Resources

About