Congenital Sternal Cleft along with Persistent Left-Sided Superior Vena Cava: A Rare Presentation

Congenital sternal cleft is a rare abnormality resulting from fusion failure of sternum. It occurs in isolation or along with defects of abdominal wall, diaphragm, pericardium, and heart. Early surgical correction is required to protect the underlying structures for risk of cardiac compression. Here we report a case of 20-day female child presenting with congenital sternal cleft associated with multiple congenital heart disease and left-sided superior vena cava. She was operated by the cardiothoracic surgical team successfully and is doing well on followup. We discuss this rare case, imaging studies, and surgical strategy. 1. Case History A 20-day old female baby, with a birth weight of 2.2？kg, born by caesarean section at term to primi mother was referred to our neonatal intensive care unit with complain of a central depression over the chest wall in inspiration with movements with respiration. There was a prominent midline raphe over the skin starting from anterior neck and visible cardiac pulsations in the suprasternal region (Figure 1). Examination revealed a central bony defect under the depression along the midline and a grade II systolic murmur over the precordium. The baby was hemodynamically stable. Chest radiograph revealed an enlarged thymic shadow. Cranial and abdominal ultrasounds were normal. Transthoracic echocardiography revealed a 3？mm ostium secondum atrial septal defect (ASD) with left-to-right shunt and a 2？mm patent ductus arteriosus (PDA) (Figure 2). The child also had a left-sided superior vena cava (SVC) draining to a dilated coronary sinus with absent right SVC. The patient underwent primary surgical correction at 25 days of life. Figure 1: Preoperative appearance of the baby depression over chest wall. Figure 2: Transthoracic echocardiogram, parasternal short axis view showing patent ductus arteriosus. She was operated in supine position through a midline chest incision and the sternal cleft was found. A thickened fibrous raphe was seen tethering the manubrium to the intact pericardial sac. The raphe was excised thus also releasing the neck of the child. The right lobe of thymus was resected. Absent right SVC was confirmed. A U-shaped cartilaginous bar, 3.7？cm long, was found joining the margins of the sternum from middle to lower part. This bar was excised along its entire length, and the two separated parts of the sternum were pulled across and approximated gradually by four ethicon sutures over the underlying mediastinal structures. This position was maintained for 5 minutes to look for any hemodynamic compromise. As there