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LETTER TO THE EDITOR
Year : 2013  |  Volume : 8  |  Issue : 3  |  Page : 256-257
 

Sacral agenesis with club shaped conus


1 Department of Neurology, Sree Chitra Tirunal Institute of Medical Sciences and Technology, Thiruvananthapuram, Kerala, India
2 Department of Imaging Sciences and Intervention Radiology, Sree Chitra Tirunal Institute of Medical Sciences and Technology, Thiruvananthapuram, Kerala, India

Date of Web Publication26-Dec-2013

Correspondence Address:
Sujit Abajirao Jagtap
Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram - 695 011, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1817-1745.123705

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How to cite this article:
Jagtap SA, Kambale HJ, Nair M D. Sacral agenesis with club shaped conus. J Pediatr Neurosci 2013;8:256-7

How to cite this URL:
Jagtap SA, Kambale HJ, Nair M D. Sacral agenesis with club shaped conus. J Pediatr Neurosci [serial online] 2013 [cited 2019 Aug 24];8:256-7. Available from: http://www.pediatricneurosciences.com/text.asp?2013/8/3/256/123705


Dear Sir,

A 4-month-old boy presented with club feet and flattened buttock. The antenatal period was normal without a history of exposure to radiation, toxins, drugs or intrauterine infections or gestational diabetes. At birth parents noticed bilateral club feet with flattened buttocks. His motor, language and social development were normal. He had equinovarus deformity of left foot with reduced gluteal mass and abnormal skin folds over the buttocks [Figure 1]a, without any evidence of spina bifida, dimple or dermal sinus or other bony deformity. His other systemic examination as well as 2D echo and ultrasound abdomen was normal. Radiograph pelvis showed total absence of sacral bones with iliac wings fused to lateral borders of lower most vertebras with normal transverse pelvic diameter. Magnetic resonance imaging of spine revealed sacral agenesis (SA) with abruptly ending club shaped conus at the level of T11 [Figure 1]b-d.
Figure 1: (a) Clinical photograph showing flattened buttocks, shallow gluteal fold, (b) T2 weighted sagittal image of lumbosacral spine shows non tapering conus medullaris at upper border of D11 vertebral body (c) wedge shape of conus is due to dorsal half extending further than ventral half and is due to relative preservation of dorsal sacral roots and dorsal ganglia central T2 hyperintensity might be due to prominent terminal ventricle. Stretched out nerve roots are seen distally with narrow distal canal (d) radiograph pelvis anteroposterior view shows total absence of sacral bones with iliac wings fused to lateral borders of lower vertebra with normal transverse pelvic diameter

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SA is a rare congenital disorder which involves absence of only coccyx to sacral, lumbar and even thoracic vertebrae with incidence of 0.01-0.05/1000 live births. [1],[2] The exact etiology is not known but is reported in association with maternal diabetes, genetic factors and vascular hypoperfusion. Nearly 12-22% of SA cases are associated with maternal diabetes mellitus. [3] The clinical presentation varies from flattened buttocks with abnormal skin fold, shortening of the intergluteal cleft, small gluteal masses and club feet to severe form with genitourinary, respiratory and nervous system abnormalities. The position of conus defines two distinct groups in patients with SA. In group 1, conus terminate abruptly at T11 or T12 vertebral body level with club/wedge shape while in group 2 conus ends below L2 with tethered cord. [4] In our case, S1 vertebral body is absent suggesting high bony aplasia.

 
   References Top

1.Alexander E, Nashold BS. Agenesis of the sacrococcygeal region. J Neurosurg 1956; 13:507-13.  Back to cited text no. 1
    
2.Caird MS, Hall JM, Bloom DA, Park JM, Farley FA. Outcome study of children, adolescents, and adults with sacral agenesis. J Pediatr Orthop 2007;27:682-5.  Back to cited text no. 2
[PUBMED]    
3.Stroustrup Smith A, Grable I, Levine D. Case 66: Caudal regression syndrome in the fetus of a diabetic mother. Radiology 2004;230:229-33.  Back to cited text no. 3
[PUBMED]    
4.Renshaw TS. Sacral agenesis. J Bone Joint Surg Am 1978;60:373-83.  Back to cited text no. 4
    


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