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 2a-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
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)
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 The patient's parents understand that the patient's name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.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
- 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. doi: 10.1007/BF02825837. [DOI] [PubMed] [Google Scholar]
- 2.ur Rahman S, Newman C, Ein SH. Isolated asternia: A case report. Pediatr Surg Int. 2002;18:496–7. doi: 10.1007/s00383-002-0828-x. [DOI] [PubMed] [Google Scholar]
