Mejia Leon Andres, Pérez Hidalgo Juan, Roa Benavides Jose, Acosta Izquierdo Laura
Final Pr. ID: Poster #: EDU-089
Caudal Regression Syndrome (CRS) is a rare and severe form of abnormal sacral development, with an estimated frequency in the general population ranging from 0.005% to 0.01%. It exhibits a slightly higher incidence among children born to diabetic mothers. The majority of CRS cases are sporadic, and the clinical presentation varies depending on the extent of the abnormalities. CRS has been associated with other anomalies, including Currarino syndrome, VACTERL (vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, limb abnormalities) association, caudal duplication syndrome, and more.
As radiologists, understanding the key features of Caudal Regression Syndrome and its associated syndromes is essential. This knowledge can aid clinicians in defining the extent of the disease and facilitating early and targeted treatment.
The primary goal of this educational exhibit is to provide a comprehensive review of CRS and its associated syndromes. Using clinical cases and various imaging modalities, we aim to enhance your understanding of these conditions.
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Authors: Mejia Leon Andres , Pérez Hidalgo Juan , Roa Benavides Jose , Acosta Izquierdo Laura
Keywords: Caudal regression syndrome, Currarino syndrome, Caudal duplication syndrome
Smyth Anna, Nowik Christina, Pugash Denise, Rosenbaum Daniel
Final Pr. ID: Poster #: CR-003
Caudal duplication syndrome is a rare entity that involves duplication of various structures arising from the embryonic cloaca and notochord.
This presentation outlines the case of a 38-year-old G2P1 woman referred to our institution at 21 weeks gestation for anomalies detected on antenatal ultrasound. Antenatal ultrasound demonstrated sagittal duplication of the bladder, duplex phallus, a bifid scrotum and a horseshoe kidney. A subsequent fetal MRI confirmed those findings and also demonstrated apparent duplication of the colon and dysmorphic lumbosacral spine.
Following delivery, the baby passed urine via both urethras and meconium via a right-sided anus; there was a left-sided anal dimple with an imperforate anus. VCUG showed no communication between the two bladders. A colovesical fistula was demonstrated between the left bladder and colon, which is likely the redundant duplicated colon with the imperforate anus. MRI of the abdomen and pelvis showed a lipomeningocele with attempted sacral duplication, redemonstrated duplication of the pelvic organs, and confirmed absence of a left-sided rectum and sphincteric complex.
Caudal duplication syndrome is a complex malformation, the management of which often requires a multidisciplinary approach involving radiology, general surgery, urology, and neurosurgery. This case illustrates the findings of this rare entity with good correlation between fetal and postnatal imaging. It also highlights respective contributions of the various imaging modalities in guiding management, which usually entails staged surgical correction.
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Authors: Smyth Anna , Nowik Christina , Pugash Denise , Rosenbaum Daniel
Keywords: Caudal duplication