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Giant stomach secondary to juvenile polyposis syndrome
Nathalie Wong-Chong1, Wondwossen Hailu Kidanewold, Richard Kirsch
1Department of Surgery, Mount Sinai Hospital, 600 University Avenue, Suite 1225, Toronto, ON, M5G 1X5, Canada.
Insights
Juvenile polyposis syndrome (JPS) can cause massive gastric polyposis, leading to severe complications. This case highlights the need for timely intervention, including total gastrectomy, for life-threatening JPS manifestations.
Area of Science:
- Gastroenterology
- Genetics
- Pathology
Background:
- Juvenile polyposis syndrome (JPS) is a rare autosomal dominant disorder.
- Typically, polyps affect the colon, but gastric and small intestine involvement can occur.
Observation:
- A 27-year-old woman presented with severe anemia and severe gastric polyposis.
- The condition progressed over 5 years, causing hematemesis, hypoproteinemia, and gastric outlet obstruction.
Findings:
- Histopathology confirmed juvenile polyposis.
- Massive gastric polyposis led to severe gastromegaly (stomach enlargement).
Implications:
- This case demonstrates an unusual presentation of JPS with severe gastric involvement.
- Total gastrectomy was necessary to manage life-threatening complications of massive gastric polyposis.
Introduction:
Juvenile polyposis syndrome (JPS) is a rare, autosomal dominant condition. The polyps predominate in the colon but may be seen less commonly in the stomach or small intestine. We report an unusual case of JPS associated with massive gastric polyposis, resulting in a giant stomach, severe anemia, hematemesis, protein-losing enteropathy, and gastric outlet obstruction. Progressive complications ultimately necessitated a total gastrectomy.
Case:
A 27-year-old woman presented in 2005 with severe anemia. Gastroscopy revealed severe gastric polyposis. Histopathology confirmed juvenile polyposis. Gastrectomy was initially declined. Progressive hematemesis, hypoproteinemia, and gastric outlet obstruction, however, resulted in a total gastrectomy 5 years following initial presentation. Massive gastric polyposis resulted in severe gastromegaly.
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