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CLINICAL IMAGE
Year : 2021  |  Volume : 18  |  Issue : 3  |  Page : 217-218

Caudal regression sequence: A costly vascular steal


Department of Radiology, Indraprastha Apollo Hospital, New Delhi, India

Date of Submission04-Jun-2021
Date of Decision31-Jul-2021
Date of Acceptance01-Aug-2021
Date of Web Publication18-Aug-2021

Correspondence Address:
Nitin P Ghonge
Department of Radiology, Indraprastha Apollo Hospital, New Delhi - 110 076, India
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/am.am_49_21

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How to cite this article:
Ghonge NP. Caudal regression sequence: A costly vascular steal. Apollo Med 2021;18:217-8

How to cite this URL:
Ghonge NP. Caudal regression sequence: A costly vascular steal. Apollo Med [serial online] 2021 [cited 2021 Dec 6];18:217-8. Available from: https://www.apollomedicine.org/text.asp?2021/18/3/217/324063



A 4-year-old male child presented with inability to walk with urinary and fecal incontinence. Physical examination showed muscle wasting in the pelvis and lower limbs. Plain radiograph [Figure 1] showed the absence of lumbar vertebrae and sacrum. The iliac bones are hypoplastic and apposed (marked *) with abnormal morphology of hip joints. Lateral radiograph [Figure 2] and Sagittal T2W magnetic resonance imaging (MRI) [[Figure 3], Panel B] showed the absence of lumbar vertebrae and sacrum (arrows) and abrupt termination of the spinal cord with “cigar-shaped appearance” (arrowhead). Coronal T2W MRI [[Figure 3], Panel A] also shows fused midline “pancake” kidneys (marked *). The findings are consistent with caudal regression sequence (CRS).
Figure 1: Plain radiograph showing absence of lumbar vertebrae and sacrum. The iliac bones are hypoplastic and apposed (marked *) with abnormal morphology of hip joints

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Figure 2: Lateral radiograph showing the absence of lumbar vertebrae and sacrum (arrow).

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Figure 3: (a) Coronal T2W MRI [Figure 3, Panel A] showing fused midline “pancake” kidneys (marked *). (b) Sagittal T2W MRI [Figure 3, Panel B] showing absence of lumbar vertebrae and sacrum (arrows) and abrupt termination of the spinal cord with “cigar-shaped appearance” (arrowhead)

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CRS is a rare congenital defect of lower spinal segments with loss of the bladder and bowel control and limb fusion in its most severe form (sirenomelia).[1] Gastrointestinal and genitourinary anomalies are often associated as shown in this case. The pathophysiology is likely to be in-utero “vascular steal” by vitelline artery.[2] The level of steal often determines the clinical spectrum and the consequent prognosis. CRS is often associated with maternal diabetes and is divided into two subtypes, based on the status of the spinal cord.[3] In type 1, the conus medullaris is high with abrupt termination along with associated vertebral aplasia, whereas type 2 CRS shows low lying tethered conus medullaris due to associated spinal abnormality. Detailed imaging evaluation is the key to the diagnosis, prognostication, and to decide the treatment options,[4] as highlighted in these images.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Rare Diseases Database, NORD. Available from: https://rarediseases.org/rare-diseases/caudal-regression-syndrome/. [Last accessed on 2021 Jun 30].  Back to cited text no. 1
    
2.
Hentschel J, Stierkorb E, Schneider G, Goedde S, Siemer S, Gortner L. Caudal regression sequence: Vascular origin? J Perinatol 2006;26:445-7.  Back to cited text no. 2
    
3.
Paolo TD, Andrea R. Pediatric Neuroradiology Textbook. Apollo Medicine; Wolters Kluwer; 2005. p. 1533-608.  Back to cited text no. 3
    
4.
Boruah DK, Dhingani DD, Achar S, Prakash A, Augustine A, Sanyal S, et al. Magnetic resonance imaging analysis of caudal regression syndrome and concomitant anomalies in pediatric patients. J Clin Imaging Sci 2016;6:36.  Back to cited text no. 4
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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