Congenital left hemidiaphragm agenesis and use of glatiramer acetate

Abstract

Congenital Diaphragmatic Hernia (CDH) is a congenital defect of the diaphragm through which intestine and other viscera herniate in to the chest. In extreme form of diaphragmatic maldevelopment, there might be a complete agenesis of diaphragm. Neonates with CDH present postnatally with respiratory distress and a characteristic absence of breath sounds in the ipsilateral chest. Here we present a 26-year-old gravida 1, para 1 woman with Multiple Sclerosis (MS) on glatiramer acetate who was admitted for elective caesarean section at 38 weeks of gestation. Abdominal ultrasonography had been performed for mother monthly from month 3 and all were reported normal. A baby girl (3300g) was born. Shortly after birth neonate became cyanotic with heavy shallow respiration. Chest x-ray showed massive gas filled intestinal herniation in left thoracic cavity along with right sided mediastinal shift and right sided pneumothorax. Orogastric tube was placed, intubation was performed and she was placed immediately on a ventilator but in the way to operating room she was expired.