Gastrointestinal Tract Involvement of Gorham's Disease with Expression of D2-40 in Duodenum

We present a case of a 13-year-old boy with Gorham's disease involving the thoracic and lumbar spine, femur, and gastrointestinal (GI) tract, which was complicated by recurrent chylothorax and GI bleeding. The presenting symptoms were intermittent abdominal pain, back pain, and melena. Esophago...

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Published inPediatric gastroenterology, hepatology & nutrition Vol. 17; no. 1; pp. 52 - 56
Main Authors Choi, Bong Seok, Hong, Suk Jin, Chu, Mi Ae, Lee, Seok Jong, Lee, Jong-Min, Bae, Han Ik, Choe, Byung-Ho
Format Journal Article
LanguageEnglish
Published Korea (South) The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition 01.03.2014
대한소아소화기영양학회
Subjects
Case Report
소아과학
Gastrointestinal tract
Melena
Osteolysis
Gorham disease
Child
Occult blood
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Summary:We present a case of a 13-year-old boy with Gorham's disease involving the thoracic and lumbar spine, femur, and gastrointestinal (GI) tract, which was complicated by recurrent chylothorax and GI bleeding. The presenting symptoms were intermittent abdominal pain, back pain, and melena. Esophagogastroduodenoscopy and colonoscopy showed no abnormal lesions, but duodenal biopsy showed marked dilation of the lymphatics in the mucosa and submucosa, which revealed positive staining with a D2-40 immunohistochemical marker. In cases of GI bleeding with osteolysis, the expression of a D2-40 marker in the lymphatic endothelium of the GI tract may help to diagnose GI involvement in Gorham's disease. To the best of our knowledge, this is the first case report to pathologically demonstrate intestinal lymphatic malformation as a cause of GI bleeding in Gorham's disease.
Bibliography:G704-001424.2014.17.1.009
ISSN:2234-8646
2234-8840
DOI:10.5223/pghn.2014.17.1.52