An Unusual Case of the Complete Currarino Triad: Case Report, Discussion of the Literature and the Embryogenic Implications

An Unusual Case of the Complete Currarino Triad: Case Report, Discussion of the Literature and... AbstractOBJECTIVE AND IMPORTANCE:We present and illustrate an unusual case of the complete familial Currarino triad (an association between a bony sacral defect, a presacral mass, and an anorectal malformation) in which the teratoma arose from the conus medullaris and contained mature neurons, glia, and branching ependymal canals that were in communication with a terminal syrinx. The embryogenic implications are discussedCLINICAL PRESENTATION:The patient was a term neonate when discovered to have imperforate anus. Further workup revealed lumbosacral dysraphism with a presacral mass, a rectovaginal fistula, and a single pelvic kidney. The family pedigree revealed a familial transmission pattern; the patient had a second cousin with anal atresia and a first cousin with similar sacral anomalies. The motor level was L4 with trace L5, and there was absent sensation in the sacral dermatomesINTERVENTION:A diverting colostomy was performed on Day 14, and the infant returned at 3 months of age to undergo near-total resection through the previous abdominal approach. Only a subtotal resection was possible because the mass arose from the low-lying conus and was firmly adherent to the sacral nerve roots and iliac vessel. Follow-up magnetic resonance imaging performed 18 months after surgery revealed that the residual tumor had not progressed.CONCLUSION:Complete Currarino triad is rare and is familial in half of the cases. The special features of the tumor in our case were the presence of mature neurons with ependymal canals and its origin from the conus. The possible embryogenesis may provide evidence that the caudal notochord is important for organized secondary neurulation. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Neurosurgery Oxford University Press

An Unusual Case of the Complete Currarino Triad: Case Report, Discussion of the Literature and the Embryogenic Implications

An Unusual Case of the Complete Currarino Triad: Case Report, Discussion of the Literature and the Embryogenic Implications

6 5 8 Gegg et al. Sm ith (3), a m e ch a n ism that is atypically An Unusual Case of the Complete Currarino associated with an o m alies of the caudal region (10, 15). Triad: Case Report, Discussion of the W e p resen t and illustrate an unusual familial case of the com plete Currarino Literature and the Embryogenic Implications triad in w hich the presacral teratoma appeared to arise from a low-lying co­ nus m ed u llaris with branching ependy­ mal canals co m m u n ica tin g between the Christopher A. Gegg, M.D., Dennis G. Vollmer, M.D., teratoma and a terminal syrinx. The tera­ Micam W . Tullous, M.D., Kathleen S. Kagan-Hallet, M.D. toma histopathology was also remarkable Division of Neurosurgery (CAG, D G V , M W T ) and Department of Pathology (KSK-H), for containing mature neurons in a glial University of Texas Health Science Center, San Antonio, Texas stroma. A discussion of this rare triad and the plausible embryogenesis follows. OBJECTIVE AND IMPORTANCE: We present and illustrate an unusual case CASE REPORT of the complete familial Currarino triad (an association between a bony The patient w as the product of a 38 sacral defect, a presacral mass, and an anorectal malformation) in which and fiv e-sev en th s-w eek gestation to a the teratoma arose from the conus medullaris and contained mature healthy 21-year-old m other with an un­ neurons, glia, and branching ependymal canals that were in communica­ rem arkab le antenatal history. A physi­ tion with a terminal syrinx. The embryogenic implications are discussed. cal exam in ation revealed that the infant CLINICAL PRESENTATION: The patient was a term neonate when discov­ had a p ro tu b eran t ab d o m en with imper­ ered to have imperforate anus. Further workup revealed lumbosacral forate anus. A neurological assessment dysraphism with a presacral mass, a rectovaginal fistula, and a single revealed significant weakness of both pelvic kidney. The family pedigree revealed a...
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Publisher
Congress of Neurological Surgeons
Copyright
© Published by Oxford University Press.
ISSN
0148-396X
eISSN
1524-4040
D.O.I.
10.1097/00006123-199903000-00127
Publisher site
See Article on Publisher Site

Abstract

AbstractOBJECTIVE AND IMPORTANCE:We present and illustrate an unusual case of the complete familial Currarino triad (an association between a bony sacral defect, a presacral mass, and an anorectal malformation) in which the teratoma arose from the conus medullaris and contained mature neurons, glia, and branching ependymal canals that were in communication with a terminal syrinx. The embryogenic implications are discussedCLINICAL PRESENTATION:The patient was a term neonate when discovered to have imperforate anus. Further workup revealed lumbosacral dysraphism with a presacral mass, a rectovaginal fistula, and a single pelvic kidney. The family pedigree revealed a familial transmission pattern; the patient had a second cousin with anal atresia and a first cousin with similar sacral anomalies. The motor level was L4 with trace L5, and there was absent sensation in the sacral dermatomesINTERVENTION:A diverting colostomy was performed on Day 14, and the infant returned at 3 months of age to undergo near-total resection through the previous abdominal approach. Only a subtotal resection was possible because the mass arose from the low-lying conus and was firmly adherent to the sacral nerve roots and iliac vessel. Follow-up magnetic resonance imaging performed 18 months after surgery revealed that the residual tumor had not progressed.CONCLUSION:Complete Currarino triad is rare and is familial in half of the cases. The special features of the tumor in our case were the presence of mature neurons with ependymal canals and its origin from the conus. The possible embryogenesis may provide evidence that the caudal notochord is important for organized secondary neurulation.

Journal

NeurosurgeryOxford University Press

Published: Mar 1, 1999

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