PED Case 2: Congenital diaphragmatic hernia
Imaging Sciences URMC 2008
Publication Date: 2009-06-04
Supine AP chest radiograph demonstrates multiple lucencies within the right hemithorax (black arrows) with near-complete opacification of the right lung field. Tip of the Replogle tube lies within the left left upper quadrant. ET tube tip projects beyond the thoracid inlet.
Congenital diaphragmatic hernia (CDH) is caused by failure of closure of the pleuroperitoneal fold by the 9th week of gestational age. Herniated contents may include stomach, small bowel, colon and liver. CDH occurs in one in every 2000 to 4000 live births and accoutns for 8% of all major congenital anomalies. These defects are usually posterior (Bochdalek hernia) with a left-sided predominance (5:1) which may be due to the protective effect of the liver/ earlier closure of the pleuroperitoneal canal on the right.
Initial radiographs of a neonate with delivery room respiratory distress may reveal dense hemithorax due to lack of gas within herniated contents. Early suspicious findings include paucity of intra-abdominal gas, abnormal position of support apparatus (such as NG tube) and an antenatal history of polyhydramnios. Over time, as gas passes through the herniated viscera, lucencies appear within the dense right hemithorax. There is associated lack of aeration of the ipsilateral lung, decreased aeration of the contralateral lung and mediastinal shift.
Age at presentation and mortality depend on the degree of pulmonary hypoplasia.
This patient was placed on extracorporeal membrane oxygenation (ECMO) for persistent desaturation episodes despite supportive therapy. He was weaned off ECMO on day 9 and underwent repair of CDH. Intra-operatively, a rim of rudimentary diaphragm was identified. Herniated contents included the right lobe of the liver, portions of the colon and small bowel loops. CDH repair with a Gore-Tex patch was performed. Subsequent radiographs demonstrated re-expansion of severely hypoplastic right lung (white arrows) with associated hydrothorax (black arrows). However, the patient subsequently succumbed to ECMO-associated coagulopathy.
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