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intestinal lymphangiectasia
Etiology:
1) hypoplastic lymphatics
2) lymphatic obstruction
Pathology:
1) hypoplastic lymphatics cause lymph to leak into the intestine
2) immunodeficiency may result
3) small bowel biopsy
a) shows dilated lacteals & lymphatics in the lamina propria
b) lymphatics may contain lipid-laden macrophages
c) similar biopsy findings seen in:
1] obstruction of mesenteric lymph nodes due to:
a] lymphoma
b] Whipple's disease
c] Crohn's disease
2] obstruction of venous flow into the heart
a] constrictive pericarditis
b] severe right-sided heart failure
Clinical manifestations:
1) edema (often unilateral leg edema)
2) chylous peritoneal or pleural effusions
3) steatorrhea
4) protein-losing enteropathy
Laboratory:
1) complete blood count (CBC): lymphopenia (generally 600/mm3)
2) decreased serum proteins: serum albumin & serum globulins
Special laboratory:
- endoscopy with small bowel biopsy
Radiology: small bowel shows edematous folds
Management:
1) low-fat diet
2) medium-chain triglycerides
3) surgical excision of involved bowel segment is useful if a lesion is isolated
General
intestinal disease
lymphatic disease
References
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed)
Lippincott-Raven, Philadelphia, 1998, pg 298
- Medical Knowledge Self Assessment Program (MKSAP) 11, American
College of Physicians, Philadelphia 1998