intestinal lymphangiectasia

Etiology

• hypoplastic lymphatics
• lymphatic obstruction

Pathology

• hypoplastic lymphatics cause lymph to leak into the intestine
• immunodeficiency may result
• small bowel biopsy
  • shows dilated lacteals & lymphatics in the lamina propria
  • lymphatics may contain lipid-laden macrophages
  • similar biopsy findings seen in:
    • obstruction of mesenteric lymph nodes due to:
      • lymphoma
      • Whipple's disease
      • Crohn's disease
    • obstruction of venous flow into the heart
      • constrictive pericarditis
      • severe right-sided heart failure

Clinical manifestations

• edema (often unilateral leg edema)
• chylous peritoneal or pleural effusions
• steatorrhea
• protein-losing enteropathy

Laboratory

• complete blood count (CBC): lymphopenia (generally 600/mm3)
• decreased serum proteins: serum albumin & serum globulins

Diagnostic procedures

• endoscopy with small bowel biopsy

Radiology

small bowel shows edematous folds

Management

• low-fat diet
• medium-chain triglycerides
• surgical excision of involved bowel segment is useful if a lesion is isolated

More general terms

• intestinal disease
• lymphatic disease