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Protein-losing enteropathy in an infant with a lymphangiomyoma
A G Thomas1, D Livingstone, V Miller
1Booth Hall Children's Hospital, Manchester, England.
Insights
A rare infant lymphangiomyoma caused intractable diarrhea and protein-losing enteropathy. Pancreatic enzyme therapy initially improved symptoms, but the condition later recurred and proved refractory to treatment.
Area of Science:
- Pediatric Gastroenterology
- Rare Diseases
- Gastrointestinal Motility Disorders
Background:
- Lymphangiomyoma is a rare benign tumor typically affecting adult women.
- Infantile lymphangioma is exceptionally rare, with limited documented cases.
- Protein-losing enteropathy presents with significant gastrointestinal protein loss and malnutrition.
Observation:
- A 3-month-old infant presented with intractable diarrhea and protein-losing enteropathy.
- The condition was found to be secondary to an undiagnosed lymphangiomyoma.
- Enteral nutrition was not tolerated, necessitating parenteral nutrition for two months.
Findings:
- Pancreatic enzyme supplementation led to rapid clinical improvement and normalized serum albumin levels.
- The infant was successfully weaned from parenteral nutrition, with improvement in nutrient deficiencies.
- A subsequent recurrence of symptoms occurred despite normal pancreatic function, indicating treatment refractoriness.
Implications:
- This case highlights the first reported instance of lymphangiomyoma causing protein-losing enteropathy in an infant.
- Pancreatic enzyme supplementation may offer a therapeutic option for similar cases, though not curative.
- Further research is needed to understand the pathogenesis and develop effective treatments for infantile lymphangiomyoma-associated enteropathy.
Abstract:
A 3-month-old girl with intractable diarrhoea had protein-losing enteropathy secondary to a lymphangiomyoma. This is the first reported case of lymphangiomyoma in an infant. As enteral feeds were not tolerated, she was maintained on parenteral nutrition for 2 months. Pancreatic enzyme supplementation produced a rapid clinical improvement and normalization of serum albumin level. Weaning was subsequently tolerated and the vitamin, mineral, and trace element deficiencies improved. Subsequent recurrence was associated with normal pancreatic function and has proved refractory to treatment.