Pentalogy of Cantrell - A Case Report from Nigeria

FB was delivered at home to a para three low income mother, at term after an unsupervised pregnancy. At birth she was noticed to have an anterior chest wall defect with a protruding pulsating mass. Further examination revealed a defect from the suprasternal notch to the xiphoid process (bifid sternum) with the heart exposed and pulsating (ectopia cordis). The pericardium was absent and the great vessels were exposed. There was an epigastric omphalocoele and a ventral diaphragmatic defect. The diagnosis of Pentallogy of Cantrell was made. The challenges involved in the evaluation and management of this case in a Nigerian setting are discussed.