Right-Sided Congenital Diaphragmatic Hernia Caused by Hepatopulmonary Fusion

Abstract

Introduction: Hepatopulmonary fusion is a very rare finding associated with right-sided congenital diaphragmatic hernia. With less than 50 reported cases, management and outcomes of hepatopulmonary fusion are poorly understood. This report highlights that clinical presentation is not a reliable indicator of outcomes in this rare disease. Case Presentation. A term neonate admitted for tachypnea and complete opacification of the right hemithorax was diagnosed with right-sided congenital diaphragmatic hernia. Preoperative respiratory support was minimal, and the only symptom exhibited was tachypnea. During surgical repair, fusion of the lung and liver were noted, consistent with a diagnosis of hepatopulmonary fusion. Postoperatively, the patient's pulmonary hypertension worsened and required extracorporeal membrane oxygenation.

Conclusions: Many patients with hepatopulmonary fusion and only mild symptoms die postoperatively from severe pulmonary hypertension and progressive respiratory failure. Preoperative clinical status is not indicative of postoperative outcomes, and literature suggests that patients who require less support preoperatively have high mortality rates. The availability of ECMO for postoperative complications may be necessary in patients requiring repair of hepatopulmonary fusion.

Figure 1

Radiographs upon admission. (a) Anterior-posterior view depicting complete opacification of right hemithorax with leftward mediastinal shift. (b) Cross-table lateral view depicting suspected hernia of abdominal contents into chest cavity.

Figure 2

Computed tomography scans one day after birth. (a) Coronal view of the liver in the right hemithorax extending to the apex with leftward mediastinal shift. (b) Axial images depicting the apex of the lungs, with the right lung apex completely compressed by the liver. (c) Axial view showing the leftward mediastinal shift and the extent of aeration of the right middle lobe. (d) Sagittal view of right lung at the level of greatest aeration. (e) Axial view depicting the right mainstem bronchus and cardiothymic shift. (f) Axial view suggesting possible aplasia of the right lower lobe.

Figure 3

Follow-up radiograph at 5 months of age. (a) Anterior-posterior view suggesting minimal right lung aeration. (b) Lateral view showing the different locations of the two diaphragms, suggesting profound right pulmonary hypoplasia.