Pentalogy of Cantrell

Abstract

Pentalogy of Cantrell is a rare syndrome that consists of defects involving the diaphragm, abdominal wall, pericardium, heart and lower sternum. A 2 –day- old male neonate presented to the pediatric surgical unit in the Maternity and Child Teaching hospital\AL-diwaniya with abdominal wall defect,precious baby deliverd by C\S to the mother with history of primary infertility for more than 13 year, The first clinical evaluation revealed epigastric omphalocele ( liver ,spleen and transverse colon were exposed with rupture sac , sternal defect , and ectopia cordis. U\S ,echocardogram and radiological studies were done to exclude others associated anomalies , Staged closure decided because the primary closure will significantly compromise ventilation or abdominal viscera .The first surgery was closure of both abdominal and thoracic defect ,bilateral flank and lateral chest wall release incisions and skin flap done for this closure. The patient had an uncomplicated postoperative course. A chest CT was performed at 2 weeks of life and demonstrated a very hypoplastic appearing anterior chest wall and a stenosis of the left mainstem bronchus as the cause of the patient's left lung hyperinflation. Monthly evaluation were done till he became at age of two years and every thinks were normal a part from abdominal distention (herniated viscera) which affected and limited the physical activity so the second stage was done, reinforced Dacron mesh placed to the anterior abdominal wall to protects the herniated bowel and redirects the pressure in the abdomen and to promotes enlargement of abdominal cavity.