A newborn infant presents with a sternal cleft. She is stable on room air. Her parents request the defect be closed as soon as possible. What is the best next step in the baby’s management?

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[size=20px]A Renal ultrasound

 

B Cardiac echo

 

C Abdomen CT

 

D Head MRI[/size]​

B

B

correct answer

B Cardiac echo

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Sternal cleft is an exceedingly rare congenital anomaly, accounting for about 0.15% of all congenital chest wall anomalies. There are four types in the Shamberger classification: (i) thoracic ectopia cordis; (ii) cervical ectopia cordis, (iii) thoracoabdominal ectopia cordis, and (iv) sternal cleft or bifid sternum. A simple sternal cleft has normal skin and the heart in the orthotopic position with an intact pericardium.

 

Sternal clefts are most commonly associated with cardiac anomalies, hence the need for cardiac echo prior to repair. Superior sternal clefts are associated with rare anomalies such as cervicofacial hemangiomas, midline raphe from the tip of the cleft to the umbilicus, and PHACES (posterior fossa malformations, facial hemangiomas, arterial anomalies with coarctation of aorta, cardiac defects, eye abnormalities, sternal cleft, and supraumbilical raphe) syndrome. Inferior partial clefts are associated with thoraco-abdominal ectopia cordis as part of the Pentalogy of Cantrell (omphalocele, anterior diaphragmatic hernia, sternal cleft, ectopia cordis, ventricular septal defect/left ventricular diverticulum).

 

Repair of simple sternal cleft is often undertaken before 5 months of age while the chest wall remains compliant and involves reapproximation of the sternal bars.

 

Later repairs may require more complex measures including sterno-clavicular disarticulation, sternal isolation, inferior sternal osteotomy, medialization of the sternocleidomastoid muscles, or use of prosthetic materials. Reconstruction of ectopia cordis may require the use of skin grafts, prosthetic materials and slow reduction of the heart into the thoracic cavity to prevent hemodynamic compromise from compression of the heart or great vessels.