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Hydrothorax, ascites, and right diaphragmatic hernia
Insights
Right diaphragmatic hernias in children can cause hydrothorax and ascites due to incarcerated sacs. Lymphatic obstruction is the likely cause, potentially leading to life-threatening lung hypoplasia.
Area of Science:
- Pediatric Surgery
- Neonatal Medicine
- Medical Imaging
Background:
- Right diaphragmatic hernia is a congenital defect where abdominal organs, notably the liver, protrude into the chest cavity.
- Associated hydrothorax and ascites are significant clinical findings in affected neonates.
- Understanding the pathophysiology is crucial for timely diagnosis and management.
Observation:
- Five pediatric cases of right diaphragmatic hernia with liver herniation were analyzed.
- Clinical presentations included hydrothorax (3/5), hydrothorax with ascites (1/5), and ascites (1/5).
- Surgical or autopsy findings revealed incarcerated peritoneal sacs containing fluid in the thoracic cavity in cases of large hydrothorax.
Findings:
- Lymphatic congestion and obstruction are implicated as the primary cause of fluid accumulation (hydrothorax/ascites).
- This fluid collection can progressively enlarge, posing a life-threatening risk.
- Two neonates with severe lung hypoplasia secondary to the condition did not survive; intrauterine interventions were attempted.
- Two survivors underwent successful surgical repair at 1 week and 7 months of age.
- Ascites in two patients was attributed to hepatic venous obstruction, similar to Budd-Chiari syndrome.
Implications:
- Early detection via fetal ultrasonography of intrathoracic masses is vital.
- Prompt surgical intervention can lead to favorable outcomes.
- The pathophysiology highlights the critical role of lymphatic and venous systems in congenital diaphragmatic abnormalities.
- This condition underscores the need for specialized neonatal care and surgical expertise.
Abstract:
Hydrothorax and/or ascites may be the most striking finding in children with right diaphragmatic hernia. The clinical, radiographic, and pathologic findings of five children with right diaphragmatic defects through which the liver had herniated are described. Three presented with a right hydrothorax, one with a right hydrothorax and ascites, and another with ascites. All four children with large right hydrothoraxes were found to have an incarcerated peritoneal sac filled with fluid in the right side of the chest at surgery or autopsy. Lymphatic congestion and obstruction was the probable cause for the fluid collection, which tended to enlarge with time. This condition may be life threatening, and two of the four patients died soon after birth because of hypoplasia of the lungs. Fetal ultrasonography in both had disclosed right intrathoracic cystic masses, and in one, intrauterine aspiration to decompress the lungs had been attempted. The other two patients are alive and well following surgical repair at 1 week and 7 months of age. Ascites was present in two patients and was believed to be due to hepatic venous obstruction, a mechanism similar to that responsible for the Budd-Chiari syndrome.
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