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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
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Year : 2020  |  Volume : 25  |  Issue : 4  |  Page : 254-255
 

Isolated asternia: An extremely rare entity


1 Department of Pediatric Surgery, AIIMS, New Delhi, India
2 Department of Radiodiagnosis, AIIMS, New Delhi, India

Date of Submission25-Dec-2019
Date of Decision02-Feb-2020
Date of Acceptance08-Feb-2020
Date of Web Publication24-Jun-2020

Correspondence Address:
Dr. Minu Bajpai
Department of Pediatric Surgery, AIIMS, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.JIAPS_223_19

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How to cite this article:
Anand S, Sharma K, Bajpai M, Goel P, Jana M. Isolated asternia: An extremely rare entity. J Indian Assoc Pediatr Surg 2020;25:254-5

How to cite this URL:
Anand S, Sharma K, Bajpai M, Goel P, Jana M. Isolated asternia: An extremely rare entity. J Indian Assoc Pediatr Surg [serial online] 2020 [cited 2020 Jul 11];25:254-5. Available from: http://www.jiaps.com/text.asp?2020/25/4/254/287656




A 2-day-old male baby presented to the emergency department with chest wall deformity. There was no associated history of cyanosis or respiratory distress. Clinical examination revealed asternia (complete absence of sternum) with parchment-like membrane [Figure 1] covering the sternal defect. Visible cardiac contractions were evident through the sternal defect. His systemic examination was unremarkable without any obvious associated anomalies. Subsequently, a computed tomogram of the chest confirmed asternia with normal intrathoracic structures [Figure 2]a, [Figure 2]b, [Figure 2]-c. Other investigations including echocardiography and ultrasonography of the abdomen ruled out associated cardiac or urinary anomalies.
Figure 1: Clinical photograph of the baby showing complete absence of sternum (asternia) with parchment-like skin covering (black arrow) the underlying defect

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Figure 2: (a-c) Computed tomogram of the chest. Axial bone window (a), sagittal reformatted bone window (b), and volume-rendered (c) images show the absence of sternum in its normal location (marked by * in a and c and arrows in b)

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Asternia usually occurs in association with other congenital anomalies, such as in children with Cantrell's pentalogy and Leiber's syndrome. Isolated asternia is extremely rare.[1] Complete failure of fusion of the sternal plates (concentration of mesenchymal cells) in a developing embryo results in asternia.[1],[2] Timing of surgical reconstruction is debatable. However, it is agreed upon that the best time to operate is infancy as the thoracic cage is pliable and the gap is less wide.[1] These children continue to have a benign course due to a lack of syndromic associations. However, long-term follow-up is necessary for the evaluation of cardiorespiratory function.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Kohli V, Nooreyazdan S, Das BN, Kaul S, Singh J, Parmar V. Surgical reconstruction for absence of sternum and pericardium in a newborn. Indian J Pediatr 2006;73:367-8.  Back to cited text no. 1
    
2.
ur Rahman S, Newman C, Ein SH. Isolated asternia: A case report. Pediatr Surg Int 2002;18:496-7.  Back to cited text no. 2
    


    Figures

  [Figure 1], [Figure 2]



 

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