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    Case of the Day
    Sacral agenesis

    Modality: MRI Modality: MRI

    View here all images of this case in different sizes !



    Go to the top of the page ID: 20061111154853
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     Sacral agenesis   
     Available images: There are MRI images available for this case. [ MRI ]   
     Author/s:

    Samuel Stafrace (Raigmore Hospital/Inverness/Scotland), H. Shannon (Raigmore Hospital/Inverness/Scotland)  

     
     Email Address:

    Viewable for logged on visitors (Log on)  

     
     Age:

    3 Years  

     
     Gender:

    Female  

     
     History:

    3 year old girl with chronic constipation, repeated urinary infections and delayed milestones.  

     
     Pathomorphology or Pathophysiology of this disease :

    Congenital developmental abnormality of neural tube - related to maternal diabetes in 20%.
    Recent literature may suggest relation to vascular developmental anomaly.
     

     
     Radiological findings:


    MRI 1 <- view MRI 1

    MRI 1: Sagittal T2 weighted MRI of the spine shows a truncated appearence of the lower cord and absence of the lower sacral elements. A grossly distended bladder is also noted.


    MRI 2 <- view MRI 2

    MRI 2: Axial T2 image of the kidneys shows a dilated left renal system.

     

     
     Diagnosis confirmation:

    Expert's opinion  

     
     Which DD would be also possible with the radiological findings:

    Nil  

     
     Course / Prognosis / Frequency / Other :

    1:7500 births
    Results in lower limb abnormalities, spinal cord abnormalities, urinary tract anomalies secondary to neurogenic bladder and chronic constipation.  

     
     Comments of the author about the case:

    Excellent example showing sacral, urinary and cord abnormalities.
     

     
     First description / History:

    N/A  

     
     Literature:

    Barkovich AJ, Raghavan N, Chuang S, Peck WW.
    The wedge-shaped cord terminus: a radiographic sign of caudal regression.
    AJNR Am J Neuroradiol. 1989 Nov-Dec;10(6):1223-31

    Pang D
    Sacral agenesis and caudal spinal cord malformations.
    Neurosurgery. 1993 May;32(5):755-78; discussion 778-9.

    Hentschel J, Stierkorb E, Schneider G, Goedde S, Siemer S, Gortner L.
    Caudal regression sequence: vascular origin?
    J Perinatol. 2006 Jul;26(7):445-7

     

     
     Region-Organ:

    Spinal canal / Myelon  

     
     Most likely etiology:

    congenital  

     
     Available images: There are MRI images available for this case. [ MRI ]   
    More cases from these authors: Search Samuel Stafrace in Medline Samuel Stafrace (3)   Search Samuel Stafrace in Medline H. Shannon (2)     




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