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Transient protein-losing enteropathy secondary to elevated caval pressures and caval obstruction after the Mustard
Insights
Protein-losing enteropathy occurred in a child after a Mustard operation for complete transposition. This complication may be linked to inferior vena cava obstruction and might be more common than previously thought.
Area of Science:
- Pediatric Cardiology
- Gastroenterology
Background:
- The Mustard operation is a palliative surgical procedure for complete transposition of the great arteries.
- Protein-losing enteropathy (PLE) is a condition characterized by excessive loss of serum proteins into the gastrointestinal tract.
Observation:
- A 24-month-old girl developed PLE following a Mustard operation.
- Diagnostic studies, including 51Cr-labeled albumin excretion, confirmed PLE.
- Angiocardiograms revealed apparent postoperative inferior vena cava obstruction with elevated caval pressures.
Findings:
- PLE was documented in a pediatric patient post-Mustard operation.
- Inferior vena cava obstruction was identified as a potential contributing factor.
- No primary renal, hepatic, or cardiac failure was evident.
Implications:
- Inferior vena cava obstruction following the Mustard operation may lead to protein-losing enteropathy.
- This complication could be underdiagnosed in patients who have undergone this surgical procedure.
- Further investigation into the incidence and management of PLE post-Mustard operation is warranted.
Abstract:
Protein-losing enteropathy, documented by 51Cr-labeled albumin excretion studies, was demonstrated in a 24-month-old girl following the Mustard operation for complete transposition. Apparent postoperative inferior vena cava obstruction had been diagnosed by angiocardiograms and both caval pressure were found to be elevated. There was no evidence of primary renal or hepatic disease or cardiac failure. This problem may occur more frequently than has been recognized.