Abstract
Background/purpose
The Currarino syndrome (CS) is a peculiar form of caudal regression syndrome (CRS)
characterized by the association of hemisacrum, anorectal malformation (ARM), and
presacral mass. The authors analyzed retrospectively their series, and they propose
a multidisciplinary diagnostic and therapuetic protocol that until now has not been
introduced.
Methods
A series of 6 patients with CS is presented. Five of them were treated initially in
other centers. None of them had an early diagnosis. All presented associated anomalies;
in 50%, Hirschsprung’s disease (HD) and other dysganglionoses were present. One patient
died of a presacral ectopic nephroblastoma.
Results
Depending on the expressivity, 3 types of CS can be identified, complete, mild, and
minimal. Dysganglionoses and HD can be considered part of CS. A multidisciplinary
diagnostic and therapeutic protocol is presented. Main points are sacrum x-Ray, molecular
genetic diagnosis, radiologic evaluation of every member of CS families, magnetic
resonance (MR) evaluation of patient spine and pelvis, suction rectal biopsies, and
search for associated anomalies.
Conclusions
This protocol could give a valid contribution to the treatment of CS, allowing an
early diagnosis and proposing a rational timing of multidisciplinary surgical procedures.
Early diagnosis and treatment are essential to avoid morbidity and mortality from
an undiagnosed presacral mass.
Keywords
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Article info
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Copyright
© 2004 Elsevier Inc. Published by Elsevier Inc. All rights reserved.
