Caudal regression syndrome: a review for the radiologist.

Congress:

ECR 2020

Poster Number:

C-01531

Type:

Educational Exhibit

Keywords:

Performed at one institution, Not applicable, Congenital, Diagnostic procedure, MR, CT, Conventional radiography, Paediatric, Neuroradiology spine, Musculoskeletal system

Authors:

E. M. Flores Armas¹, L. M. Paramo², M. A. Cruz Marmolejo¹, N. Ramirez Pedraza¹, M. A. ALCALAN ANCHEYTA¹; ¹Ciudad de Mexico/MX, ²Mexicali/MX

DOI:

10.26044/ecr2020/C-01531

DOI-Link:

https://dx.doi.org/10.26044/ecr2020/C-01531

Fig. 5: Sag T2WI of type III SA with blunt ending conus above the lower border of L1.

Fig. 7: Sag T2WI in type I SA with tethered cord.

Fig. 10: Tethered cord in patient with Type 1 SA.

Fig. 15: Coronal STIR showing a uterus didelphys a class III Müllerian duct anomaly...

Learning objectives

Review the epidemiology and spectrum of findings in caudal regression syndrome (CRS). CRS spectrum of findings including sacral agenesis, genitourinary and associated abnormalities assessment with conventional radiography, ultrasound, tomography,and magnetic resonance.

Background

Sacral agenesis is defined as partial to complete absence of sacral and coccygeal bones. Caudal regression syndrome (CRS) represents a spectrum of abnormalities of the caudal region in addition to partial to complete coccygeal and lumbosacral agenesis [1,2]. Embryology of the spine: Pathogenesis of CRS involves the complex embryology in the differentiation of of the developing spine, spinal cord, and caudal mesoderm. Notochord development occurs during gastrulation, around the third week of gestation. The mesoderm differentiates into paraxial, intermediate and lateral on the sides of...

Findings and procedure details

We review the wide spectrum of findings in CRS including sacral agenesis and associated urogenital, anorectal and other spinal abnormalities using all imaging modalities (conventional radiography, fluoroscopic procedures, spinal ultrasound, computed tomography and magnetic resonance). Prenatal ultrasound in diabetic mothers must be methodical and keep in mind the possibility of CRS. First trimester findings are non specific such as short CRL and increased nuchal translucency [4,9]. Second and third trimester findings include abrupt termination of the spine, abnormal lower extremity positioning, “shield like” appearing iliac...

Conclusion

Caudal regression syndrome is a complex multi-spectrum entity which includes multiple abnormalities, among these are urogenital, gastrointestinal, neurologic, and spinal abnormalities. All imaging modalities must be used in defining the multiple findings associated to this spectrum of abnormalities in order to aid in appropriate and timely diagnosis and treatment.

Personal information and conflict of interest

Dr. Eduardo Miguel Flores Armas- nothing to disclose Magnetic Resonance Imaging Section, /Departament of Radiology, Hospital Infantil de Mexico Federico Gomez, Ciudad de Mexico, Mexico. Email:[email protected] Dr. Laura Michell Paramo Garcia- nothing to disclose Pediatric Radiology Fellow, Hospital Infantil de Mexico Federico Gomez, Ciudad de Mexico, Mexico. Email:[email protected] Dr. Miguel Angel Cruz Marmolejo- nothing to disclose Resident in Training, Department of Radiology, Hospital de Especialidades "Dr. Bernardo Sepulveda Gutierrez" Centro Medico Nacional Siglo XXI, Ciudad de Mexico, Mexico. Email: [email protected] Dr. Natalia Ramírez Pedraza- nothing...

References

Boulas MM. Recognition of caudal regression syndrome.Adv Neonatal Care. 2009;9(2):61-69, quiz 70-71. Pang D. Sacral agenesis and caudal spinal cord malformations.Neurosurgery. 1993;32(5):755-778, discussion 778-779. Kaplan, Kevin M., et al. Embryology of the Spine and Associated Congenital Abnormalities.The Spine Journal, vol. 5, no. 5, 11 Sept. 2005, pp. 564–576, 10.1016/j.spinee.2004.10.044. Temizkan, Osman, et al. “Prenatal Diagnosed Caudal Regression Syndrome.”Open Journal of Obstetrics and Gynecology, vol. 03, no. 02, 20 Jan. 2013, pp. 227–231, 10.4236/ojog.2013.32042. Boruah DK, Dhingani DD, Achar S, Prakash A, Augustine A, Sanyal S,et...