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Lymphatic Leakage in Pediatric Heart Transplantation: Early Recognition and Timely Management with Interventional
Carolena Trocchia1, Tian Mauer1, Sally Mitchell2
1From the Department of Pediatrics, Johns Hopkins All Children's Hospital, Saint Petersburg, FL.
Insights
Protein-losing enteropathy (PLE) is a severe Fontan complication. Endoscopic sclerotherapy successfully treated hepatoduodenal lymphatic leakage in a pediatric heart transplant patient, stopping fluid loss.
Area of Science:
- Cardiology
- Gastroenterology
- Pediatric Medicine
Background:
- Protein-losing enteropathy (PLE) is a serious complication following the Fontan procedure, leading to systemic issues from intestinal protein loss.
- Hepatoduodenal lymphatic leakage, caused by elevated lymphatic pressure, is a recognized complication contributing to PLE.
Observation:
- A pediatric heart transplant recipient presented with persistent PLE symptoms, necessitating frequent albumin infusions.
- The patient exhibited lymphatic fluid leakage into the duodenum, confirmed via diagnostic lymphangiography and endoscopy.
Findings:
- Endoscopic sclerotherapy utilizing ethanolamine injection was successfully employed to treat the identified site of lymphatic leakage.
- The intervention effectively halted lymphatic fluid effusion, suggesting a potential therapeutic avenue for PLE-related lymphatic complications.
Implications:
- This case underscores the critical need for prompt diagnosis and intervention in managing PLE and its sequelae.
- Radiological and endoscopic techniques offer a viable treatment strategy for hepatoduodenal lymphatic leakage associated with PLE.
Abstract:
Protein-losing enteropathy (PLE) is a severe complication of the Fontan procedure that leads to systemic complications owing to enteric protein loss. Hepatoduodenal lymphatic leakage resulting from increased lymphatic pressure is one such complication. We present the case of a pediatric heart transplant patient who experienced refractory PLE symptoms requiring serial albumin infusions and exhibited lymphatic leakage into the duodenum. Using diagnostic lymphangiography and endoscopy, we identified the affected area and treated it successfully with endoscopic sclerotherapy using ethanolamine injection. This treatment allowed for the cessation of lymphatic fluid and may serve as a potential intervention for PLE-associated hepatoduodenal lymphatic leakage. The present case highlights the importance of early recognition and timely intervention with radiology and endoscopic therapy to manage PLE and its associated complications.
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