Pentalogy of Cantrell; complete expression in a nine-month-old-boy
A 9-month-old boy was first seen at the age of 5 months being delivered with multiple congenital malformations involving the anterior abdominal and chest walls. The child was delivered at term gestation and at home; the pregnancy was not adversely eventful and she had no antenatal care. However, the child has defect on the anterior abdominal wall with a huge swelling extending from the lower anterior abdomen to the lower anterior chest wall, which was pulsatile and covered by a thin membrane which was initially reddish at birth but later became thickened and darker following daily cleaning and dressing. There was no history of vomiting or constipation, child sucked directly from the breast, though interrupted occasionally to catch his breath, there was no history of difficulty with breathing and no darkening of the lips or mucous membrane. The mother was a 25-year-old stay at home mother with four other children in a non-consanguineous marriage; she neither smoked cigarette nor drank alcohol and had no adverse medical record. No similar problem was found in the family. On examination, there was a big pendulous and pulsatile mass extending from slightly above the symphysis pubis to the epigastrum measuring 12 × 10 cm [] with an epithelised membranous covering; it had visible peristaltic waves, it was pundunculated, with cardiac pulsation felt in the epigastric region []. Bowel and heart sounds were heard over the mass below the level of the diaphragm. There was a lower sternal cleft with absence of the xiphoid process. He was not tachypneic or tachycardic and had first and second heart sounds with a systolic murmur. Bowel sounds were audible and normal. His chest X-ray showed a midline heart with inferior displacement [], abdominal ultrasound showed the swelling to contain loops of bowl and echocardiography revealed a 6 mm secundum atrial septal defect. A diagnosis of pentalogy of Cantrell was made. The child is currently being followed up awaiting staged repairs of the defects.