6-year-old girl, no clinical history

 

Images

Doctor's Information

Name : ----------------
Family :Kharrazi
Affiliation : Head of Radiology Department of Shohada Hospital
Academic Degree: ----------------
Email : This email address is being protected from spambots. You need JavaScript enabled to view it.
Resident : Hussein Soleiman Tabar

 

Patient's Information

Gender : Female
Age : 6

 

Case Section

Pediatric Radiology

 

Clinical Summary

6-year-old girl, no clinical history

 

 

Imaging Procedures and Findings

CT images clearly depict complete agenesis of sacrum and coccyx.

 

Discussion

Caudal regression syndrome refers to a spectrum of findings comprising absence of the lower portion of the caudal spine, urologic abnormalities such as renal aplasia or dysplasia and neurogenic bladder, malformed external genitalia, anal atresia, and sirenomelia (fusion of the lower extremities). The main findings involving the bony spine and spinal cord are absence of the lower end of the bony spine and of the spinal canal below T9. The most common location for agenesis is at S1 (22% to 58%), followed by Ll to L5 (17% to 40%), T11 or T12 (33%), and T9 (25%). Failure of the coccyx to form, without other parts of the bony spine being involved, is an incidental finding, and patients are asymptomatic. The distal end of the spinal cord may be abnormal, with the conus having a bulbous or wedge-shaped appearance instead of the normal conical shape, and the conus may be abnormal in location (anywhere from the level of the beginning of the absence to as high as T8 or T9). Children with caudal regression syndrome present with a combination of urologic, orthopedic, and neuro- logic symptoms. The syndrome is more common in males than in females (2.5:1). The radiologic diagnosis and evaluation is best performed with plain radiographs of the spine and CT to evaluate the bony absence. T1-weighted MR images will provide detail of the abnormal conus and the absence of the distal spine. Other abnormalities such as spinal canal stenosis, dysraphic defects, and segmentation anomalies can be demonstrated on multiplanar T1- and T2-weighted MR images.

 

Final Diagnosis

Caudal regression syndrome

 

References

Caffey's pediatric diagnostic imaging-l0th ed./[edited by] jerald P. Kuhn, Thomas L.Siovis,jack O. Haller.

 

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